Chapter 1

Anatomy of Ear
brain
The sound for your
-

amplifies
The ear is divided into: of the pinna. It has two deficiencies-the "fissures of San-
torini" in this part of the cartilage and through them the
1. External ear
parotid or superficial mastoid infections can appear in the
2. Middle ear
canal or vice versa. The skin covering the cartilaginous ca-
3. Internal ear or the labyrinth
nal is thick and contains ceruminous and pilosebaceous
glands which secrete wax. Hair is only confined to the
outer canal and therefore furuncles (staphylococcal infec-
THE EXTERNAL EAR tion of hair follicles) are seen only in the outer one-third
of the canal.
The external ear consists of the (i) auricle or pinna, (ii)
external acoustic canal and (iii) tympanic membrane 2. Bony Part
(Figure l.l A).
It forms inner two-thirds (16 mm). Skin lining the bony
canal is thin and continuous over the tympanic mem-
A. AURICLE OR PINNA brane. It is devoid of hair and ceruminous glands. About
6 mm lateral to tympanic membrane, the bony mea-
The entire pinna except its lobule and the outer part of
tus presents a narrowing called isthmus. Foreign bodies,
external acoustic canal are made up of a framework of a
lodged medial to the isthmus, get impacted, and are dif-
single piece of yellow elastic cartilage covered with skin.
ficult to remove. Anteroinferior part of the deep meatus,
The latter is closely adherent to the perichondrium on
beyond the isthmus, presents a recess called anterior re-
its lateral surface while it is slightly loose on the me-
cess, which acts as a cesspool for discharge and debris in
dial (cranial) surface. The various elevations and depres-
cases of external and middle ear infections (Figure 1.2).
sions seen on the lateral surface of pinna are shown in
Anteroinferior part of the bony canal may present a de-
Figure l. lB.
ficiency (foramen of Huschke) in children up to the age of
There is no cartilage between the tragus and crus
four or sometimes even in adults, permitting infections to
of the helix, and this area is called incisura terminalis
and from the parotid.
(Figure 1.lC). An incision made in this area will not cut
through the cartilage and is used for endaural approach
in surgery of the external auditory canal or the mastoid. C. TYMPANI( MEMBRANE OR
Pinna is also the source of several graft materials for the THE DRUMHEAD
surgeon. Cartilage from the tragus, perichondrium from
the tragus or concha and fat from the lobule are fre- It forms the partition between the external acoustic canal
quently used for reconstructive surgery of the middle ear. and the middle ear. It is obliquely set and as a result, its
The conchal cartilage has also been used to correct the posterosuperior part is more lateral than its anteroinferior
depressed nasal bridge while the composite grafts of the part. It is 9-10 mm tall, 8-9 mm wide and 0.1 mm thick.
skin and cartilage from the pinna are sometimes used for Tympanic membrane can be divided into two parts:
repair of defects of nasal ala.
1. Pars Tensa
It forms most of tympanic membrane. Its periphery is
B. EXTERNAL ACOUSTIC (AUDITORY) CANAL thickened to form a fibrocartilaginous ring called an-
It extends from the bottom of the concha to the tympan- nulus tympanicus, which fits in the tympanic sulcus.
ic membrane and measures about 24 mm along its poste- The central part of pars tensa is tented inwards at the
rior wall. It is not a straight tube; its outer part is directed level of the tip of malleus and is called umbo. A bright
upwards, backwards and medially while its inner part is cone of light can be seen radiating from the tip of mal-
directed downwards, forwards and medially. Therefore, to leus to the periphery in the anteroinferior quadrant
see the tympanic membrane, the pinna has to be pulled (Figure 1.3).
upwards, backwards and laterally so as to bring the two
parts in alignment. 2. Pars Flaccida (Shrapnell's Membrane)
The canal is divided into two parts: (i) cartilaginous This is situated above the lateral process of malleus be-
and (ii) bony. tween the notch of Rivinus and the anterior and posterior
malleal folds (earlier called malleolar folds). It is not so
1. Cartilaginous Part taut and may appear slightly pinkish. Various landmarks
It forms outer one-third (8 mm) of the canal. Cartilage is a seen on the lateral surface of tympanic membrane are
continuation of the cartilage which forms the framework shown in Figure 1.4.

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4 SECTION I — Diseases of Ear

Figure 1.1. (A) The ear and its divisions. (B) The elevations and depressions on the lateral surface of pinna. (C) The auricular cartilage.

Figure 1.2. Anterior recess of the meatus. It is important to clean Figure 1.4. Landmarks of a normal tympanic membrane of right
discharge and debris from this area. side.

Layers of Tympanic Membrane

Tympanic membrane consists of three layers:
• Outer epithelial layer, which is continuous with the
skin lining the meatus (Figure 1.3).
• Inner mucosal layer, which is continuous with the mu-
cosa of the middle ear.
• Middle fibrous layer, which encloses the handle of
malleus and has three types of fibres—the radial, circu-
lar and parabolic (Figure 1.5).
Figure 1.3. Coronal section through tympanic membrane and
external ear canal showing structures of pars tensa and pars flaccida of Fibrous layer in the pars flaccida is thin and not organ-
tympanic membrane. Scutum forms a part of lateral attic wall. ized into various fibres as in pars tensa.

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 Chapter 1 — Anatomy of Ear 5

External Auditory Canal

1. Anterior wall and roof: auriculotemporal (V3).
2. Posterior wall and floor: auricular branch of vagus
(CN X).
3. Posterior wall of the auditory canal also receives sen-
sory fibres of CN VII through auricular branch of vagus
(see Hitzelberger’s sign on p. 125).
In herpes zoster oticus, lesions are seen in the distri-
bution of facial nerve, i.e. concha, posterior part of tym-
panic membrane and postauricular region.

Tympanic Membrane
1. Anterior half of lateral surface: auriculotemporal (V3).
2. Posterior half of lateral surface: auricular branch of va-
Figure 1.5. Radial, circular and parabolic fibres of pars tensa of gus (CN X).
tympanic membrane. 3. Medial surface: tympanic branch of CN IX (Jacobson’s
nerve).

RELATIONS OF EXTERNAL ACOUSTIC MEATUS

• Superiorly: Middle cranial fossa

THE MIDDLE EAR *
• Posteriorly: Mastoid air cells and the facial nerve The middle ear together with the eustachian tube, adi-
• Inferiorly: Parotid gland tus, antrum and mastoid air cells is called middle ear cleft
• Anteriorly: Temporomandibular joint (Figure 1.7). It is lined by mucous membrane and filled
with air.
Posterosuperior part of deeper canal near the tympanic The middle ear extends much beyond the limits of
membrane is related to the mastoid antrum. “Sagging” of tympanic membrane which forms its lateral boundary
this area may be noticed in acute mastoiditis. and is sometimes divided into: (i) mesotympanum (ly-
ing opposite the pars tensa), (ii) epitympanum or the
NERVE SUPPLY OF THE EXTERNAL EAR attic (lying above the pars tensa but medial to Shrap-
nell’s membrane and the bony lateral attic wall) and
Pinna (iii) hypotympanum (lying below the level of pars tensa)
1. Greater auricular nerve (C2,3) supplies most of the (Figure 1.8). The portion of middle ear around the tym-
medial surface of pinna and only posterior part of the panic orifice of the eustachian tube is sometimes called
lateral surface (Figure 1.6). protympanum.
2. Lesser occipital (C2) supplies upper part of medial Middle ear can be likened to a six-sided box with a
surface. roof, a floor, medial, lateral, anterior and posterior walls
3. Auriculotemporal (V3) supplies tragus, crus of helix (Figure 1.9).
and the adjacent part of the helix. The roof is formed by a thin plate of bone called teg-
4. Auricular branch of vagus (CN X), also called Arnold’s men tympani. It also extends posteriorly to form the roof
nerve, supplies the concha and corresponding emi- of the aditus and antrum. It separates tympanic cavity
nence on the medial surface. from the middle cranial fossa.
5. Facial nerve, which is distributed with fibres of auricu- The floor is also a thin plate of bone, which separates
lar branch of vagus, supplies the concha and retroau- tympanic cavity from the jugular bulb. Sometimes, it is
ricular groove. congenitally deficient and the jugular bulb may then

Figure 1.6. Nerve supply of pinna. (A) Lateral surface of pinna. (B) Medial or cranial surface of pinna.

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6 SECTION I — Diseases of Ear

Figure 1.7. Middle ear cleft. Figure 1.8. Divisions of middle ear into epi-, meso- and hypotym-
panum.

Figure 1.9. Walls of middle ear and the structures related to them. (1) Canal for tensor tympani, (2) Opening of eustachian tube, (3) Oval
window, (4) Round window, (5) Processus cochleariformis, (6) Horizontal canal, (7) Facial nerve, (8) Pyramid, (9) Aditus, (10) Chorda tympani,
(11) Carotid artery, (12) Jugular bulb.

project into the middle ear; separated from the cavity The medial wall (Figure 1.11) is formed by the
only by the mucosa. labyrinth. It presents a bulge called promontory which
The anterior wall has a thin plate of bone, which sep- is due to the basal coil of cochlea; oval window into
arates the cavity from internal carotid artery. It also has which is fixed the footplate of stapes; round window or
two openings; the lower one for the eustachian tube and the fenestra cochleae which is covered by the second-
the upper one for the canal of tensor tympani muscle. ary tympanic membrane. Above the oval window is
The posterior wall lies close to the mastoid air cells. the canal for facial nerve. Its bony covering may some-
It presents a bony projection called pyramid through the times be congenitally dehiscent and the nerve may lie
summit of which appears the tendon of the stapedius exposed making it very vulnerable to injuries or infec-
muscle to get attachment to the neck of stapes. Aditus, tion. Above the canal for facial nerve is the prominence
an opening through which attic communicates with the of lateral semicircular canal. Just anterior to the oval
antrum, lies above the pyramid. Facial nerve runs in the window, the medial wall presents a hook-like projec-
posterior wall just behind the pyramid. Facial recess or tion called processus cochleariformis. The tendon of ten-
the posterior sinus is a depression in the posterior wall lat- sor tympani takes a turn here to get attachment to the
eral to the pyramid. It is bounded medially by the vertical neck of malleus. The cochleariform process also marks
part of VIIth nerve, laterally by the chorda tympani and the level of the first genu of the facial nerve which is
above, by the fossa incudis (Figure 1.10). Surgically, facial an important landmark for surgery of the facial nerve.
recess is important, as direct access can be made through Medial to the pyramid is a deep recess called sinus tym-
this into the middle ear without disturbing posterior ca- pani, which is bounded by the subiculum below and the
nal wall (intact canal wall technique, see p. 80). ponticulus above (Figure 1.10).

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 Chapter 1 — Anatomy of Ear 7

Figure 1.10. (A) Facial recess lies lateral and sinus tympani medial to the pyramidal eminence and vertical part of the facial nerve. (B) Exposure
of facial recess through posterior tympanotomy as seen at mastoid surgery. SCC, semicircular canal.

The lateral wall is formed largely by the tympanic mem- zontal canal lies on its medial side while the fossa incudis,
brane and to a lesser extent by the bony outer attic wall to which is attached the short process of incus, lies later-
called scutum (Figure 1.3). The tympanic membrane is semi- ally. Facial nerve courses just below the aditus.
transparent and forms a “window” into the middle ear. It
is possible to see some structures of the middle ear through THE MASTOID AND ITS AIR CELL SYSTEM
the normal tympanic membrane, e.g. the long process of (FIGURE 1.13)
incus, incudostapedial joint and the round window.
The mastoid consists of bony cortex with a “honeycomb”
of air cells underneath. Depending on development of air
MASTOID ANTRUM cell, three types of mastoid have been described.
It is a large, air-containing space in the upper part of mas- 1. Well-pneumatized or cellular. Mastoid cells are well-
toid and communicates with the attic through the aditus. developed and intervening septa are thin.
Its roof is formed by tegmen antri, which is a continuation 2. Diploetic. Mastoid consists of marrow spaces and a
of the tegmen tympani and separates it from the middle few air cells.
cranial fossa. The lateral wall of antrum is formed by a 3. Sclerotic or acellular. There are no cells or marrow
plate of bone which is on an average 1.5 cm thick in the spaces.
adult. It is marked externally on the surface of mastoid by
suprameatal (MacEwen’s) triangle (Figure 1.12). With any type of mastoid pneumatization, antrum is
always present. In sclerotic mastoids, antrum is usually
ADITUS AD ANTRUM small and the sigmoid sinus is anteposed.
Depending on the location, mastoid air cells are di-
Aditus is an opening through which the attic communi- vided into:
cates with the antrum. The bony prominence of the hori-
1. Zygomatic cells (in the root of zygoma).
2. Tegmen cells (extending into the tegmen tympani).
3. Perisinus cells (overlying the sinus plate).
4. Retrofacial cells (round the facial nerve).

Figure 1.11. Medial wall of middle ear. (1) Promontory, (2) Proces- Figure 1.12. MacEwen’s (suprameatal) triangle. It is bounded by tem-
sus cochleariformis, (3) CN VII, (4) Oval window, (5) Horizontal ca- poral line (a), posterosuperior segment of bony external auditory canal
nal, (6) Pyramid, (7) Ponticulus, (8) Sinus tympani, (9) Subiculum, (b) and the line drawn as a tangent to the external canal (c). It is an
(10) Round window, (11) Tympanic plexus. important landmark to locate the mastoid antrum in mastoid surgery.

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8 SECTION I — Diseases of Ear

Figure 1.13. Air cells in the temporal bone.

5. Perilabyrinthine cells (located above, below and be- important as it may cause difficulty in locating the an-
hind the labyrinth, some of them pass through the trum and the deeper cells; and thus may lead to incom-
arch of superior semicircular canal. These cells may plete removal of disease at mastoidectomy (Figure 1.14).
communicate with the petrous apex). Mastoid antrum cannot be reached unless the Korner’s
6. Peritubal (around the eustachian tube. Along with hy- septum has been removed.
potympanic cells they also communicate with the pe-
trous apex). Petrous apex and its cell system
7. Tip cells (which are quite large and lie medial and lat- The petrous apex lies anterior and medial to the laby-
eral to the digastric ridge in the tip of mastoid). rinth. It may be pneumatised in 30% of individuals, with
8. Marginal cells (lying behind the sinus plate and may cell tracts running either from the mastoid or hypotym-
extend into the occipital bone). panum (Figure 1.15). They run inferior, superior or an-
9. Squamosal cells (lying in the squamous part of tempo- terior to the bony capsule of the labyrinth and cochlea.
ral bones). Thus various surgical approaches have been used to drain
Abscesses may form in relation to these air cells and the inflammatory or cystic lesions of the petrous apex.
may sometimes be located far from the mastoid region. 1. Inferior route. This is the most common route. Two
approaches are used:
Development of Mastoid a. Infralabyrinthine. Access is through mastoid, and
Mastoid develops from the squamous and petrous bones. cell tracts run below the labyrinth.
The petrosquamosal suture may persist as a bony plate— b. Infracochlear. Access is through the ear canal, and
the Korner’s septum, separating superficial squamosal cells tract runs from the hypotympanum to the bony
from the deep petrosal cells. Korner’s septum is surgically cochlea to petrous apex.

Figure 1.14. Korner’s septum (A) as seen on mastoid exploration, (B) in coronal section of mastoid; in its presence there is difficulty in locating
the antrum which lies deep to it.

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 Chapter 1 — Anatomy of Ear 9

Figure 1.15. A pneumatised petrous apex. Figure 1.16. Ear ossicles and their parts.

2. Superior route. Various approaches are used. They are sounds thus preventing noise trauma to the inner ear.
from the middle cranial fossa; through the arch of superi- Stapedius is a second arch muscle and is supplied by a
or canal; through the attic region or the root of zygoma. branch of CN VII while tensor tympani develops from
3. Anterior route. Anterior cell tract runs from the hypo- the first arch and is supplied by a branch of mandibular
tympanum, anterior to the cochlea towards the petrous nerve (V3).
apex. Various approaches have earned the eponyms of
Lempert, Ramadier or Eagleton approaches.
TYMPANIC PLEXUS
Another approach to petrous apex is the translabyrin-
thine, where the labyrinth is also removed. This results It lies on the promontory and is formed by (i) tympanic
in total sensorineural loss and is used when useful branch of glossopharyngeal and (ii) sympathetic fibres
hearing is already non-existent. from the plexus round the internal carotid artery. Tym-
panic plexus supplies innervation to the medial surface
of the tympanic membrane, tympanic cavity, mastoid air
OSSICLES OF THE MIDDLE EAR cells and the bony eustachian tube. It also carries secreto-
There are three ossicles in the middle ear—the malleus, motor fibres for the parotid gland. Section of tympanic
incus and stapes (Figure 1.16). branch of glossopharyngeal nerve can be carried out in
The malleus has head, neck, handle (manubrium), a the middle ear in cases of Frey’s syndrome.
lateral and an anterior process. Head and neck of malleus Course of secretomotor fibres to the parotid:
lie in the attic. Manubrium is embedded in the fibrous Inferior salivary nucleus → CN IX → Tympanic
layer of the tympanic membrane. The lateral process branch → Tympanic plexus → Lesser petrosal nerve → Otic
forms a knob-like projection on the outer surface of the ganglion → Auriculotemporal nerve → Parotid gland.
tympanic membrane and gives attachment to the ante-
rior and posterior malleal (malleolar) folds. CHORDA TYMPANI NERVE
The incus has a body and a short process, both of
which lie in the attic, and a long process which hangs It is a branch of the facial nerve which enters the middle
vertically and attaches to the head of stapes. ear through posterior canaliculus, and runs on the medial
The stapes has a head, neck, anterior and posterior surface of the tympanic membrane between the handle
crura, and a footplate. The footplate is held in the oval of malleus and long process of incus, above the attach-
window by annular ligament. ment of tendon of tensor tympani. It carries taste from
The ossicles conduct sound energy from the tympanic anterior two-thirds of tongue and supplies secretomotor
membrane to the oval window and then to the inner ear fibres to the submaxillary and sublingual salivary glands
fluid. (Figure 14.12, p.107).

INTRATYMPANIC MUSCLES LINING OF THE MIDDLE EAR CLEFT

There are two muscles—tensor tympani and the stape- Mucous membrane of the nasopharynx is continuous
dius; the former attaches to the neck of malleus and with that of the middle ear, aditus, antrum and the mas-
tenses the tympanic membrane while the latter attaches toid air cells. It wraps the middle ear structures—the os-
to the neck of stapes and helps to dampen very loud sicles, muscles, ligaments and nerves—like peritoneum

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10 SECTION I — Diseases of Ear

TABLE 1.1 LYMPHATIC DRAINAGE OF EAR

Area Nodes
Concha, tragus, fossa triangularis and external cartilaginous canal Preauricular and parotid nodes
Lobule and antitragus Infra-auricular nodes
Helix and antihelix Postauricular nodes, deep jugular and spinal accessory nodes
Middle ear and eustachian tube Retropharyngeal nodes → upper jugular chain
Inner ear No lymphatics

wraps various viscera in the abdomen—raising several

THE INTERNAL EAR &
folds and dividing the middle ear into various compart-
ments. Middle ear contains nothing but the air; all the The internal ear or the labyrinth is an important organ
structures lie outside the mucous membrane. of hearing and balance. It consists of a bony and a mem-
Histologically, the eustachian tube is lined by ciliated branous labyrinth. The membranous labyrinth is filled
epithelium, which is pseudostratified columnar in the with a clear fluid called endolymph while the space be-
cartilaginous part, columnar in the bony part with sev- tween membranous and bony labyrinths is filled with
eral mucous glands in the submucosa. Tympanic cavity is perilymph.
lined by ciliated columnar epithelium in its anterior and
inferior part which changes to cuboidal type in the poste-
rior part. Epitympanum and mastoid air cells are lined by BONY LABYRINTH (FIGURE 1.17A)
flat, nonciliated epithelium. It consists of three parts: the vestibule, the semicircular
canals and the cochlea.
BLOOD SUPPLY OF MIDDLE EAR
1. VESTIBULE. It is the central chamber of the laby-
Middle ear is supplied by six arteries, out of which two rinth. In its lateral wall lies the oval window. The in-
are the main, i.e. side of its medial wall presents two recesses, a spherical
recess, which lodges the saccule, and an elliptical recess,
1. Anterior tympanic branch of maxillary artery which
which lodges the utricle. Below the elliptical recess is
supplies tympanic membrane.
the opening of aqueduct of vestibule through which
2. Stylomastoid branch of posterior auricular artery
passes the endolymphatic duct. In the posterosuperior
which supplies middle ear and mastoid air cells.
part of vestibule are the five openings of semicircular
Four minor vessels are: canals (Figure 1.17C).
1. Petrosal branch of middle meningeal artery (runs along
2. SEMICIRCULAR CANALS. They are three in number, the
greater petrosal nerve).
lateral, posterior and superior, and lie in planes at right
2. Superior tympanic branch of middle meningeal artery
angles to one another. Each canal has an ampullated
traversing along the canal for tensor tympani muscle.
end which opens independently into the vestibule and
3. Branch of artery of pterygoid canal (runs along eus-
a nonampullated end. The nonampullated ends of poste-
tachian tube).
rior and superior canals unite to form a common channel
4. Tympanic branch of internal carotid.
called crus commune. Thus, the three canals open into the
Veins drain into pterygoid venous plexus and superior vestibule by five openings.
petrosal sinus.
3. COCHLEA. The bony cochlea is a coiled tube making
2.5 to 2.75 turns round a central pyramid of bone called
LYMPHATIC DRAINAGE OF EAR
modiolus. The base of modiolus is directed towards inter-
Lymphatic drainage of the ear is shown in Table 1.1. The nal acoustic meatus and transmits vessels and nerves to
inner ear doesn’t have any lymphatics. the cochlea. Around the modiolus and winding spirally

Figure 1.17. (A) Left bony labyrinth. (B) Left membranous labyrinth. (C) A cut section of the bony labyrinth.

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 Chapter 1 — Anatomy of Ear 11

It appears triangular on cross-section and its three walls

are formed by:
(a) the basilar membrane, which supports the organ of
Corti;
(b) the Reissner’s membrane, which separates it from the
scala vestibule; and
(C) the stria vascularis, which contains vascular epithe-
lium and is concerned with secretion of endolymph.
Cochlear duct is connected to the saccule by ductus
reuniens (Figure 1.17B). The length of basilar membrane
increases as we proceed from the basal coil to the apical
coil. It is for this reason that higher frequencies of sound
are heard at the basal coil while lower ones are heard at
the apical coil.
Figure 1.18. A section through the cochlea to show scala media
2. UTRICLE AND SACCULE. The utricle lies in the posterior
(cochlear duct), scala vestibuli and scala tympani.
part of bony vestibule. It receives the five openings of the
three semicircular ducts. It is also connected to the sac-
like the thread of a screw, is a thin plate of bone called cule through utriculosaccular duct. The sensory epithe-
osseous spiral lamina. It divides the bony cochlea incom- lium of the utricle is called macula and is concerned with
pletely and gives attachment to the basilar membrane. linear acceleration and deceleration. The saccule also lies
The bony bulge in the medial wall of middle ear, the in the bony vestibule, anterior to the utricle and opposite
promontory, is due to the basal coil of the cochlea. The the stapes footplate. Its sensory epithelium is also called
bony cochlea contains three compartments: macula. Its exact function is not known. It probably also
(a) Scala vestibuli, responds to linear acceleration and deceleration. In Mé-
(b) Scala tympani, nière’s disease, the distended saccule lies against the sta-
(c) Scala media or the membranous cochlea (Figure 1.18). pes footplate and can be surgically decompressed by per-
forating the footplate.
The scala vestibuli and scala tympani are filled with
perilymph and communicate with each other at the apex 3. SEMICIRCULAR DUCTS. They are three in number and
of cochlea through an opening called helicotrema. Scala correspond exactly to the three bony canals. They open
vestibuli is closed by the footplate of stapes which sepa- in the utricle. The ampullated end of each duct contains
rates it from the air-filled middle ear. The scala tympani is a thickened ridge of neuroepithelium called crista ampul-
closed by secondary tympanic membrane; it is also con- laris.
nected with the subarachnoid space through the aqueduct
of cochlea (Figure 1.19). 4. ENDOLYMPHATIC DUCT AND SAC. Endolymphatic duct
is formed by the union of two ducts, one each from the
saccule and the utricle. It passes through the vestibular
MEMBRANOUS LABYRINTH (FIGURE 1.17B)
aqueduct. Its terminal part is dilated to form endolym-
It consists of the cochlear duct, the utricle and saccule, phatic sac, which lies between the two layers of dura on
the three semicircular ducts, and the endolymphatic duct the posterior surface of the petrous bone.
and sac. Endolymphatic sac is surgically important. It is ex-
posed for drainage or shunt operation in Ménière’s
1. COCHLEAR DUCT (FIGURE 1.18). Also called membra- disease.
nous cochlea or the scala media. It is a blind coiled tube.
INNER EAR FLUIDS AND THEIR CIRCULATION
There are two main fluids in the inner ear: perilymph and
endolymph.

1. PERILYMPH. It resembles extracellular fluid and is

rich in Na ions. It fills the space between the bony and
the membranous labyrinth. It communicates with CSF
through the aqueduct of cochlea which opens into the
scala tympani near the round window. In fact this duct is
not a direct communication but contains connective tis-
sue resembling arachnoid through which perilymph per-
colates. There are two views regarding the formation of
perilymph: (i) It is a filtrate of blood serum and is formed
Figure 1.19. A diagrammatic representation of the perilymphatic by capillaries of the spiral ligament and (ii) it is a direct
system. CSF passes into the scala tympani through the aqueduct of continuation of CSF and reaches the labyrinth via aque-
the cochlea. duct of cochlea.

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12 SECTION I — Diseases of Ear

TABLE 1.2 COMPOSITION OF INNER EAR FLUIDS

Endolymph Perilymph CSF
Na+ (mEq/L) 5 140 152
K+ (mEq/L) 144 10 4
Protein (mg/dL) 126 200–400 20–50
Glucose (mg/dL) 10–40 85 70

Values are average and may differ slightly according to the site of collection of endolymph (cochlea, utricle, sac) and perilymph (scala tympani or scala
vestibuli).

2. ENDOLYMPH. It fills the entire membranous labyrinth 2. Blood supply to cochlea and vestibular labyrinth is
and resembles intracellular fluid, being rich in K ions. It segmental, therefore, independent ischaemic damage
is secreted by the secretory cells of the stria vascularis of can occur to these organs causing either cochlear or
the cochlea and by the dark cells (present in the utricle vestibular symptoms.
and also near the ampullated ends of semicircular ducts).
There are two views regarding its flow: (i) longitudinal, i.e.
endolymph from the cochlea reaches saccule, utricle and DEVELOPMENT OF EAR
endolymphatic duct and gets absorbed through endolym-
phatic sac, which lies in the subdural space and (ii) ra- AURICLE. First branchial cleft is the precursor of external
dial, i.e. endolymph is secreted by stria vascularis and also auditory canal. Around the 6th week of embryonic life,
gets absorbed by the stria vascularis. This view presumes a series of six tubercles appear around the first branchi-
that endolymphatic sac is a vestigial structure in man and al cleft. They progressively coalesce to form the auricle
plays no part in endolymph absorption. Composition of (Figure 1.22). Tragus develops from the tubercle of the
endolymph, perilymph and CSF is given in Table 1.2. first arch while the rest of the pinna develops from the
remaining five tubercles of the second arch. Faulty fusion
between the first and the second arch tubercles causes
BLOOD SUPPLY OF LABYRINTH
preauricular sinus or cyst, which is commonly seen be-
The entire labyrinth receives its arterial supply through tween the tragus and crus of helix. By the 20th week,
labyrinthine artery, which is a branch of anterior-inferior pinna achieves adult shape. Initially, the pinna is located
cerebellar artery but sometimes from the basilar. In the low on the side of the neck and then moves on to a more
internal auditory canal it divides in the manner shown in lateral and cranial position.
Figures 1.20 and 1.21.
Venous drainage is through three veins, namely inter- EXTERNAL AUDITORY MEATUS. It develops from the first
nal auditory vein, vein of cochlear aqueduct and vein of branchial cleft. By about the 16th embryonic week, cells
vestibular aqueduct, which ultimately drain into inferior proliferate from the bottom of ectodermal cleft and form
petrosal sinus and lateral venous sinus. a meatal plug. Recanalization of this plug forms the epi-
It is to be noted that: thelial lining of the bony meatus. Recanalization begins
1. Blood supply to the inner ear is independent of blood from the deeper part near the tympanic membrane and
supply to middle ear and bony otic capsule, and there progresses outwards, and that explains why deeper mea-
is no cross circulation between the two. tus is sometimes developed while there is atresia of canal
in the outer part. External ear canal is fully formed by the
28th week of gestation.

TYMPANIC MEMBRANE. It develops from all the three ger-

minal layers. Outer epithelial layer is formed by the ec-
toderm, inner mucosal layer by the endoderm and the
middle fibrous layer by the mesoderm.

MIDDLE EAR CLEFT. The eustachian tube, tympanic

cavity, attic, antrum and mastoid air cells develop from
the endoderm of tubotympanic recess which arises from
the first and partly from the second pharyngeal pouches
(Figure 1.23).
Malleus and incus are derived from mesoderm of the
first arch while the stapes develop from the second arch
except its footplate and annular ligament which are de-
rived from the otic capsule.

Figure 1.20. Divisions of the labyrinthine artery which supply blood MEMBRANOUS INNER EAR. Development of the inner ear
to various parts of the labyrinth. starts in the 3rd week of fetal life and is complete by the

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 Chapter 1 — Anatomy of Ear 13

Figure 1.21. Blood supply of the labyrinth.

Figure 1.22. Development of pinna. Six hillocks of His around the Figure 1.23. Development of the external auditory canal and mid-
first branchial cleft and the corresponding parts of pinna which de- dle ear.
velop from them.

16th week. Ectoderm in the region of hindbrain thickens development of the inner ear. It is therefore not unusual
to form an auditory placode, which is invaginated to form to see malformed and nonfunctional inner ear in the pres-
auditory vesicle or the otocyst. The latter then differenti- ence of normal external and middle ears, and vice versa.
ates into the endolymphatic duct and sac; the utricle, the The cochlea is developed sufficiently by 20 weeks of
semicircular ducts; and saccule and the cochlea. Devel- gestation (Table 1.3) and the fetus can hear in the womb
opment of phylogenetically older part of labyrinth—pars of the mother. This probably explains how Abhimanyu,
superior (semicircular canals and utricle) takes place earlier while still unborn, could have heard the conversation be-
than pars inferior (saccule and cochlea). tween his mother and father (Arjuna) in the legend given
The embryologic source and the time of development in the Great Indian epic of Mahabharata written thou-
of external and middle ears are quite independent of the sands of years ago.

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14 SECTION I — Diseases of Ear

TABLE 1.3 TIMING OF DEVELOPMENT OF THE EAR IN THE WEEK OF GESTATIONa

Development Pinna Meatus Middle ear Vestibular labyrinth Cochlea
Begins 6th 8th 3rd 3rd 3rd
Completes 20th 28th 30th 20th 20th
aSource: Gulya AJ. Developmental Anatomy of the Ear. In: Glasscock and Shambaugh, editors. Surgery of the Ear. Philadelphia: W.B. Saunders Company,
1990.

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 Chapter 2
Peripheral Receptors and Physiology
of Auditory and Vestibular Systems

AUDITORY SYSTEM pathways is complex. From cochlear nuclei, the main

nuclei in the ascending auditory pathways, sequentially,
ORGAN OF CORTI (FIGURE 2.1) from below upwards are:
Organ of Corti is the sense organ of hearing and is situ- 1. Superior olivary complex
ated on the basilar membrane. Important components of 2. Nucleus of lateral lemniscus
the organ of Corti are: 3. Inferior colliculus
4. Medial geniculate body
1. TUNNEL OF CORTI. It is formed by the inner and outer 5. Auditory cortex
rods. It contains a fluid called cortilymph. The exact func-
The auditory fibres travel via the ipsilateral and con-
tion of the rods and cortilymph is not known.
tralateral routes and have multiple decussation points.
Thus each ear is represented in both cerebral hemispheres.
2. HAIR CELLS. They are important receptor cells of
The area of cortex, concerned with hearing is situated in
hearing and transduce sound energy into electrical en-
the superior temporal gyrus (Brodmann’s area 41). For au-
ergy. Inner hair cells form a single row while outer hair
ditory pathways, remember the mnemonic E.COLI-MA:
cells are arranged in three or four rows. Inner hair cells
Eighth nerve, Cochlear nuclei, Olivary complex, Lateral
are richly supplied by afferent cochlear fibres and are
lemniscus, Inferior colliculus, Medial geniculate body
probably more important in the transmission of audito-
and Auditory cortex.
ry impulses. Outer hair cells mainly receive efferent in-
nervation from the olivary complex and are concerned
with modulating the function of inner hair cells. Dif-
ferences between inner and outer hair cells are given in PHYSIOLOGY OF HEARING
Table 2.1.
Any vibrating object causes waves of compression and
rarefaction and is capable of producing sound. In the air,
3. SUPPORTING CELL. Deiters’ cells are situated between
at 20°C and at sea level, sound travels at a speed of 344 m
the outer hair cells and provide support to the latter. Cells
(1120 ft) per second. It travels faster in liquids and solids
of Hensen lie outside the Deiters’ cells.
than in the air. Also, when sound energy has to pass from
air to liquid medium, most of it is reflected because of the
4. TECTORIAL MEMBRANE. It consists of gelatinous ma-
impedance offered by the liquid.
trix with delicate fibres. It overlies the organ of Corti. The
shearing force between the hair cells and tectorial mem-
brane produces the stimulus to hair cells. MECHANISM OF HEARING
A sound signal in the environment is collected by the
NERVE SUPPLY OF HAIR CELLS pinna, passes through external auditory canal and strikes
the tympanic membrane. Vibrations of the tympanic
Ninety-five per cent of afferent fibres of spiral ganglion
membrane are transmitted to stapes footplate through
supply the inner hair cells while only five per cent supply
a chain of ossicles coupled to the tympanic membrane.
the outer hair cells. Efferent fibres to the hair cells come
Movements of stapes footplate cause pressure changes
from the olivocochlear bundle. Their cell bodies are situ-
in the labyrinthine fluids, which move the basilar mem-
ated in superior olivary complex. Each cochlea sends in-
brane. This stimulates the hair cells of the organ of Corti.
nervation to both sides of the brain.
It is these hair cells which act as transducers and convert
the mechanical energy into electrical impulses, which
AUDITORY NEURAL PATHWAYS AND THEIR travel along the auditory nerve. Thus, the mechanism of
NUCLEI (FIGURE 2.2) hearing can be broadly divided into:
Hair cells are innervated by dendrites of bipolar cells of 1. Mechanical conduction of sound (conductive appara-
spiral ganglion which is situated in Rosenthal’s canal (ca- tus).
nal running along the osseous spiral lamina). Axons of 2. Transduction of mechanical energy to electrical im-
these bipolar cells form the cochlear division of CN VIII pulses (sensory system of cochlea).
and end in the cochlear nuclei, the dorsal and ventral, 3. Conduction of electrical impulses to the brain (neural
on each side of the medulla. Further course of auditory pathways).

15

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16 SECTION I — Diseases of Ear

Figure 2.1. Structure of organ of Corti.

TABLE 2.1 DIFFERENCES BETWEEN INNER AND OUTER HAIR CELLS

Inner hair cells Outer hair cells
Total no. 3500 12,000
Rows One row Three or four rows
Shape Flask shaped Cylindrical
Nerve supply Primarily afferent fibres and very few efferent Mainly efferent fibres and very few afferent
Development Develop earlier Develop late
Function Transmit auditory stimuli Modulate function of inner hair cells
Vulnerability More resistant Easily damaged by ototoxic drugs and high intensity noise

1. Conduction of Sound
A person under water cannot hear any sound made in
the air because 99.9% of the sound energy is reflected
away from the surface of water because of the imped-
ance offered by it. A similar situation exists in the ear
when air-conducted sound has to travel to cochlear
fluids. Nature has compensated for this loss of sound
energy by interposing the middle ear which converts
sound of greater amplitude but lesser force, to that of
lesser amplitude but greater force. This function of the
middle ear is called impedance matching mechanism or the
transformer action.
It is accomplished by:
(a) Lever action of the ossicles. Handle of malleus is 1.3
times longer than long process of the incus, provid-
ing a mechanical advantage of 1.3.
(b) Hydraulic action of tympanic membrane. The area of
tympanic membrane is much larger than the area of
stapes footplate, the average ratio between the two
being 21:1. As the effective vibratory area of tym-
panic membrane is only two-thirds, the effective ar-
Figure 2.2. Auditory pathways from the right cochlea. Note bilateral eal ratio is reduced to 14:1, and this is the mechani-
route through brainstem and bilateral cortical representation. cal advantage provided by the tympanic membrane
(Figure 2.3).
The product of areal ratio and lever action of os-
sicles is 18:1.

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 Chapter 2 — Peripheral Receptors and Physiology of Auditory and Vestibular Systems 17

Figure 2.4. Frequency localization in the cochlea. Higher frequencies

Figure 2.3. Transformer action of the middle ear. Hydraulic effect of
are localized in the basal turn and then progressively decrease towards
tympanic membrane and lever action of ossicles combine to compen-
the apex.
sate the sound energy lost during its transmission from air to liquid
medium.
2. Transduction of Mechanical Energy to
According to some workers (Wever and Lawrence) Electrical Impulses
out of a total of 90 mm2 area of human tympanic Movements of the stapes footplate, transmitted to the
membrane, only 55 mm2 is functional and given the cochlear fluids, move the basilar membrane and set up
area of stapes footplate (3.2 mm2), the areal ratio is shearing force between the tectorial membrane and the
17:1 and total transformer ratio (17× 1.3) is 22.1. hair cells. The distortion of hair cells gives rise to cochlear
(c) Curved membrane effect. Movements of tympanic microphonics, which trigger the nerve impulse.
membrane are more at the periphery than at the cen- A sound wave, depending on its frequency, reaches
tre where malleus handle is attached. This too pro- maximum amplitude on a particular place on the basilar
vides some leverage. membrane and stimulates that segment (travelling wave
theory of von Bekesy). Higher frequencies are represented
PHASE DIFFERENTIAL BETWEEN OVAL AND ROUND WIN- in the basal turn of the cochlea and the progressively low-
DOWS. Sound waves striking the tympanic membrane do er ones towards the apex (Figure 2.4).
not reach the oval and round windows simultaneously.
There is a preferential pathway to the oval window be- 3. Neural Pathways
cause of the ossicular chain. Thus, when oval window is
Hair cells get innervation from the bipolar cells of
receiving wave of compression, the round window is at
spiral ganglion. Central axons of these cells collect to
the phase of rarefaction. If the sound waves were to strike
form the cochlear nerve which goes to the ventral and
both the windows simultaneously, they would cancel
dorsal cochlear nuclei. From there, both crossed and
each other’s effect with no movement of the perilymph
uncrossed fibres travel to the superior olivary nucleus,
and no hearing. This acoustic separation of windows is
lateral lemniscus, inferior colliculus, medial geniculate
achieved by the presence of intact tympanic membrane
body and finally reach the auditory cortex of the tem-
and a cushion of air in the middle ear around the round
poral lobe (Brodmann’s area 41 situated at the superior
window. Phase differential between the windows contrib-
aspect of the temporal lobe along the floor of the lateral
utes 4 dB when tympanic membrane is intact.
cerebral fissure).
NATURAL RESONANCE OF EXTERNAL AND MIDDLE EAR.
Inherent anatomic and physiologic properties of the ex- ELECTRICAL POTENTIALS OF COCHLEA
ternal and middle ear allow certain frequencies of sound AND CN VIII
to pass more easily to the inner ear due to their natu-
ral resonances. Natural resonance of external ear canal is Four types of potentials have been recorded; three from
3000 Hz and that of middle ear 800 Hz. Frequencies most the cochlea and one from CN VIII fibres. They are:
efficiently transmitted by ossicular chain are between
500 and 2000 Hz while that by tympanic membrane is 1. Endocochlear potential
800–1600 Hz. Thus greatest sensitivity of the sound trans- 2. Cochlear microphonic from cochlea
mission is between 500 and 3000 Hz and these are the 3. Summating potential
frequencies most important to man in day-to-day conver- 4. Compound action potential from nerve fibres
sation (Table 2.2).
1. ENDOCOCHLEAR POTENTIAL. It is a direct current (DC)
TABLE 2.2 NATURAL RESONANCE AND potential recorded from scala media. It is +80 mV and
EFFICIENCY OF AUDITORY APPARATUS is generated from the stria vascularis by Na+/K+-ATPase
pump and provides source of energy for cochlear trans-
External auditory canal 3000 Hz
duction (Figure 2.5). It is present at rest and does not
Tympanic membrane 800–1600 Hz
require sound stimulus. This potential provides a sort of
Middle ear 800 Hz
Ossicular chain 500–2000 Hz “battery” to drive the current through hair cells when
they move in response to a sound stimulus.

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18 SECTION I — Diseases of Ear

Figure 2.5. Davis’ battery model of cochlear transduction. Scala

media has a DC potential of 180 mV. Stimulation of hair cells pro-
duces intracellular potential of 240 mV. This provides flow of current
of 120 mV through the top of hair cells.

Figure 2.6. Structure of ampullary end of semicircular duct. Over the

2. COCHLEAR MICROPHONIC (CM). When basilar mem- crista lie sensory hair cells interspersed with supporting cells. Hair from
brane moves in response to sound stimulus, electrical re- sensory cells project into the gelatinous substance of cupula.
sistance at the tips of hair cells changes allowing flow of
K+ through hair cells and produces voltage fluctuations
from the surface of crista to the ceiling of the ampulla
called cochlear microphonic. It is an alternating current
and forms a water tight partition, only to be displaced to
(AC) potential.
one or the other side like a swing door, with movements
of endolymph. The gelatinous mass of cupula consists of
3. SUMMATING POTENTIAL (SP). It is a DC potential and
polysaccharide and contains canals into which project
follows “envelope” of stimulating sound. It is produced
the cilia of sensory cells.
by hair cells. It may be negative or positive. SP has been
Hair cells are of two types (Figure 2.7). Type I cells are
used in diagnosis of Ménière’s disease. It is superimposed
flask-shaped with a single large cup-like nerve terminal
on VIII nerve action potential.
surrounding the base. Type II cells are cylindrical with
Both CM and SP are receptor potentials as seen in oth-
multiple nerve terminals at the base. From the upper sur-
er sensory end-organs. They differ from action potentials
face of each cell, project a single hair, the kinocilium and
in that: (i) they are graded rather than all or none phe-
a number of other cilia, the stereocilia. The kinocilium is
nomenon, (ii) have no latency, (iii) are not propagated
thicker and is located on the edge of the cell. Sensory cells
and (iv) have no postresponse refractory period.
are surrounded by supporting cells which show microvilli
on their upper ends.
4. COMPOUND ACTION POTENTIAL. It is an all or none
response of auditory nerve fibres.
(B) STRUCTURE OF A MACULA. A macula consists mainly
of two parts: (i) a sensory neuroepithelium, made up of
type I and type II cells, similar to those in the crista; (ii)
VESTIBULAR SYSTEM an otolithic membrane, which is made up of a gelatinous
mass and on the top, the crystals of calcium carbonate
PERIPHERAL RECEPTORS called otoliths or otoconia (Figure 2.8). The cilia of hair cells
They are of two types:

1. Cristae
They are located in the ampullated ends of the three
semicircular ducts. These receptors respond to angular ac-
celeration.

2. Maculae
They are located in otolith organs (i.e. utricle and sac-
cule). Macula of the utricle lies in its floor in a horizontal
plane. Macula of the saccule lies in its medial wall in a
vertical plane. They sense position of head in response to
gravity and linear acceleration.

(A) STRUCTURE OF A CRISTA (FIGURE 2.6). It is a crest-

like mound of connective tissues on which lie the sensory
epithelial cells. The cilia of the sensory hair cells project Figure 2.7. Sensory hair cells of the vestibular organs. Type I (left)
into the cupula, which is a gelatinous mass extending and Type II (right).

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 Chapter 2 — Peripheral Receptors and Physiology of Auditory and Vestibular Systems 19

Figure 2.8. Structure of macula, the sensory end organ of the utricle and the saccule.

project into the gelatinous layer. The linear, gravitational 5. Vestibular nuclei of the opposite side.
and head tilt movements cause displacement of otolithic 6. Cerebral cortex (temporal lobe). This is responsible for
membrane and thus stimulate the hair cells which lie in subjective awareness of motion.
different planes.

VESTIBULAR NERVE PHYSIOLOGY OF VESTIBULAR SYSTEM

Vestibular or Scarpa’s ganglion is situated in the lateral Vestibular system is conveniently divided into:
part of the internal acoustic meatus. It contains bipolar
1. Peripheral, which is made up of membranous labyrinth
cells. The distal processes of bipolar cells innervate the
(semicircular ducts, utricle and saccule) and vestibular
sensory epithelium of the labyrinth while its central pro-
nerve.
cesses aggregate to form the vestibular nerve.
2. Central, which is made up of nuclei and fibre tracts in
the central nervous system to integrate vestibular im-
CENTRAL VESTIBULAR CONNECTIONS pulses with other systems to maintain body balance.
The fibres of vestibular nerve end in vestibular nuclei and
some go to the cerebellum directly. SEMICIRCULAR CANALS
Vestibular nuclei are four in number, the superior,
They respond to angular acceleration and deceleration.
medial, lateral and descending. Afferents to these nuclei
The three canals lie at right angles to each other but the
come from:
one which lies at right angles to the axis of rotation is
1. Peripheral vestibular receptors (semicircular canals, stimulated the most. Thus horizontal canal will respond
utricle and saccule) maximum to rotation on the vertical axis and so on. Due
2. Cerebellum to this arrangement of the three canals in three different
3. Reticular formation planes, any change in position of head can be detected.
4. Spinal cord Stimulation of semicircular canals produces nystagmus
5. Contralateral vestibular nuclei and the direction of nystagmus is determined by the
plane of the canal being stimulated. Thus, nystagmus is
Thus, information received from the labyrinthine re-
horizontal from horizontal canal, rotatory from the supe-
ceptors is integrated with information from other soma-
rior canal and vertical from the posterior canal.
tosensory systems.
The stimulus to semicircular canal is flow of endo-
Efferents from vestibular nuclei go to:
lymph which displaces the cupula. The flow may be to-
1. Nuclei of CN III, IV, VI via medial longitudinal bundle. wards the cupula (ampullopetal) or away from it (am-
It is the pathway for vestibulo-ocular reflexes and this pullofugal), better called utriculopetal and utriculofugal.
explains the genesis of nystagmus. Ampullopetal flow is more effective than ampullofugal
2. Motor part of spinal cord (vestibulospinal fibres). This for the horizontal canal. The quick component of nys-
coordinates the movements of head, neck and body in tagmus is always opposite to the direction of flow of en-
the maintenance of balance. dolymph. Thus, if a person is rotated to the right for
3. Cerebellum (vestibulocerebellar fibres). It helps to co- sometime and then abruptly stopped, the endolymph
ordinate input information to maintain the body bal- continues to move to the right due to inertia (i.e. am-
ance. pullopetal for left canal), the nystagmus will be hori-
4. Autonomic nervous system. This explains nausea, zontal and directed to the left (Figure 2.9). Remember
vomiting, palpitation, sweating and pallor seen in ves- nystagmus is in the direction opposite to the direction
tibular disorders (e.g. Ménière’s disease). of flow of endolymph.

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20 SECTION I — Diseases of Ear

and pull system. In static neutral position, each side con-

tributes equal sensory information, i.e. push and pull sys-
tem of one side is equal to that of the other side. If one
side pulls more than the other, balance of the body is
disturbed. During movement, i.e. turning or tilt, there is
a temporary change in the push and pull system, which
is corrected by appropriate reflexes and motor outputs to
the eyes (vestibulo-ocular reflex), neck (vestibulocervical
Figure 2.9. Rotation test. At the end of rotation to the right, semicir- reflex), and trunk and limbs (vestibulospinal reflex) to
cular canals stop but endolymph continues to move to the right, i.e.
maintain new position of head and body, but if any com-
towards the left ampulla but away from the right, causing nystagmus
ponent of push and pull system of one side is disturbed
to the left.
for a longer time due to disease, vertigo and ataxia will
develop.
UTRICLE AND SACCULE
VERTIGO AND DIZZINESS
Utricle is stimulated by linear acceleration and decelera-
tion or gravitational pull during the head tilts. The sen- Disorientation in space causes vertigo or dizziness and
sory hair cells of the macula lie in different planes and are can arise from disorders of any of the three systems: ves-
stimulated by displacement of otolithic membrane dur- tibular, visual or somatosensory. Normally, the impulses
ing the head tilts. reaching the brain from the three systems are equal and
The function of saccule is similar to that of utricle as opposite. If any component on one side is inhibited or
the structure of maculae in the two organs is similar but stimulated, the information reaching the cortex is mis-
experimentally, the saccule is also seen to respond to matched, resulting in disorientation and vertigo. The
sound vibrations. vestibular inhibition on one side (e.g. acute vestibular
The vestibular system thus registers changes in the failure, labyrinthectomy, Ménière’s disease, VIIIth nerve
head position, linear or angular acceleration and decel- section) causes vertigo. Similarly, stimulation of labyrinth
eration, and gravitational effects. This information is sent by thermal or rotational stimulus causes vertigo. Dizzi-
to the central nervous system where information from ness can similarly result from the ocular causes, e.g. high
other systems—visual, auditory, somatosensory (muscles, errors of refraction or acute extraocular muscle paralysis
joints, tendons, skin)—is also received. All this informa- with diplopia.
tion is integrated and used in the regulation of equilib- Vertigo and its causes are discussed in detail in
rium and body posture. Chapter 7.
Cerebellum, which is also connected to vestibular end
organs, further coordinates muscle movements in their
MOTION SICKNESS
rate, range, force and duration and thus helps in the
maintenance of balance. It is characterized by nausea, vomiting, pallor and sweat-
ing during sea, air, bus or car travel in certain susceptible
individuals. It can be induced by both real and apparent
MAINTENANCE OF BODY EQUILIBRIUM
motion and is thought to arise from the mismatch of in-
A useful clinical approach to understand the physiology formation reaching the vestibular nuclei and cerebellum
of equilibrium is to imagine that the balance system (ves- from the visual, labyrinthine and somatosensory systems.
tibular, visual and somatosensory) is a two-sided push It can be controlled by the usual labyrinthine sedatives.

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 Chapter 3
Audiology and Acoustics

This section aims to introduce certain terms which are power, i.e. watts/cm2 or as pressure, i.e. dynes/cm2. In au-
frequently used in audiology and acoustics. diology, sound is measured as sound pressure level (SPL).
It is compared with the reference sound which has an
SOUND. It is a form of energy produced by a vibrating SPL of 0.0002 dynes/cm2 or 20 µPa (micropascals), which
object. A sound wave consists of compression and rarefac- roughly corresponds to the threshold of hearing in nor-
tion of molecules of the medium (air, liquid or solid) in mal subjects at 1000 Hz. Decibel notation was introduced
which it travels. Velocity of sound is different in differ- in audiology to avoid dealing with large figures of sound
ent media. In the air, at 20 °C, at sea level, sound travels pressure level (0.0002 dynes/cm2 at normal threshold of
344 m (1120 ft) per second, and is faster in liquid and still hearing to 200 dynes/cm2 which causes pain in the ear.
faster in a solid medium. The latter is 1,000,000 times the former).
Formula for decibel is
FREQUENCY. It is the number of cycles per second. The
unit of frequency is Hertz (Hz) named after the German Power of S1
Sound in dB = 10 log
scientist Heinrich Rudolf Hertz. A sound of 1000 Hz Power of S0
means 1000 cycles per second.
S1= sound being described
S0= reference sound
PURE TONE. A single frequency sound is called a pure
tone, e.g. a sound of 250, 500 or 1000 Hz. In pure tone
audiometry, we measure the threshold of hearing in deci- or 10 log
(SPL of S1 )2
bels for various pure tones from 125 to 8000 Hz. (SPL of S0 )2
(because power of sound is proportional to square of SPL)
COMPLEX SOUND. Sound with more than one frequency
is called a complex sound. Human voice is a complex SPL of S1
sound. or 20 log
SPL of S0

PITCH. It is a subjective sensation produced by frequency If a sound has an SPL of 1000, i.e. (103) times the refer-
of sound. Higher the frequency, greater is the pitch. ence sound, it is expressed as 20 × 3 = 60 dB. Similarly, a
sound of 1,000,000, i.e. (106) times the reference sound
OVERTONES. A complex sound has a fundamental fre- SPL is expressed simply as 120 dB and so on.
quency, i.e. the lowest frequency at which a source vi-
brates. All frequencies above that tone are called the over- NOISE. It is defined as an aperiodic complex sound. There
tones. The latter determine the quality or the timbre of are three types of noise:
sound. a. White noise. It contains all frequencies in audible
spectrum and is comparable to the white light which
INTENSITY. It is the strength of sound which determines contains all the colours of the visible spectrum. It is a
its loudness. It is usually measured in decibels. At a dis- broad-band noise and is used for masking.
tance of 1 m, intensity of b. Narrow band noise. It is white noise with certain fre-
Whisper = 30 dB quencies, above and below the given noise, filtered
Normal conversation = 60 dB out. Thus, it has a frequency range smaller than the
Shout = 90 dB broad-band white noise. It is used to mask the test fre-
Discomfort of the ear = 120 dB quency in pure tone audiometry.
Pain in the ear = 130 dB c. Speech noise. It is a noise having frequencies in the
speech range (300–3000 Hz). All other frequencies are
filtered out.
LOUDNESS. It is the subjective sensation produced by in-
tensity. More the intensity of sound, greater the loudness. MASKING. It is a phenomenon to produce inaudibility of
one sound by the presentation of another. In clinical au-
DECIBEL (dB). It is 1/10th of a bel and is named after diometry, one ear is kept busy by a sound while the other
Alexander Graham Bell, the inventor of telephone. It is is being tested. Masking of nontest ear is essential in all
not an absolute figure but represents a logarithmic ratio bone conduction tests, but for air conduction tests, it is
between two sounds, namely the sound being described required only when difference of hearing between two
and the reference sound. Sound can be measured as ears exceeds 40 dB.

21

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22 SECTION I — Diseases of Ear

SOUND PRESSURE LEVEL. The SPL of a sound in decibels above his threshold. For a normal person, this would be
is 20 times the logarithm to the base 10, of the pressure of a sound of 0 + 40, i.e. 40 dB HL, but for one with a hear-
a sound to the reference pressure. The reference pressure ing loss of say 30 dB, it would be 30 + 40, i.e. 70 dB HL.
is taken as 0.0002 dynes/cm2 or 20 µPa (micropascals) for In other words, sensation level refers to the sound which will
a frequency of 1000 Hz and represents the threshold of produce the same sensation, as in normally hearing person.
hearing in normally hearing young adults. In speech audiometry, discrimination scores are tested at
30–40 dB SL. Stapedial reflex is elicited with a sound of
FREQUENCY RANGE IN NORMAL HEARING. A normal per- 70–100 dB SL.
son can hear frequencies of 20–20,000 Hz but in routine
audiometric testing only 125–8000 Hz are evaluated. MOST COMFORTABLE LEVEL (MCL). It is the intensity
level of sound that is most comfortable for the person.
SPEECH FREQUENCIES. Frequencies of 500, 1000 and
2000 Hz are called speech frequencies as most of human LOUDNESS DISCOMFORT LEVEL. It is the level of sound
voice falls within this range. PTA (pure tone average) is which produces discomfort in the ear. Usually, it is 90–
the average threshold of hearing in these three speech 105 dB SL. It is important to find the loudness discomfort
frequencies. It roughly corresponds to the speech recep- level of a person when prescribing a hearing aid.
tion threshold.
DYNAMIC RANGE. It is the difference between the most
AUDIOMETRIC ZERO. Threshold of hearing, i.e. the faint- comfortable level and the loudness discomfort level. The
est intensity which a normal healthy person can hear will dynamic range is reduced in patients with positive re-
vary from person to person. The International Standards cruitment phenomenon, as is the case in cochlear type
Organization (ISO) adopted a standard for this, which is of hearing loss.
represented as the zero level on the audiometer. According
to ISO, audiometric zero is the mean value of minimal audible SOUND LEVEL METER. It is an instrument to measure
intensity in a group of normally hearing healthy young adults. level of noise and other sounds. Sound level meters have
different weighting networks (e.g. A, B or C) for different
HEARING LEVEL (HL). It is the sound pressure level pro- sensitivities at different frequencies. When describing a
duced by an audiometer at a specific frequency. It is meas- sound measured by a sound level meter, the weighting
ured in decibels with reference to audiometric zero. If an network must be indicated.
audiometer delivers a sound at 70 dB, it is represented as Noise levels are often expressed as dB(A) which refers
70 dB HL. to sound pressure level measured with ‘A’ network where
the low and extremely high frequencies are given much
SENSATION LEVEL (SL). It refers to the level of sound less weightage compared to those in the middle range
above the threshold of hearing for an individual. If some- which are more important and are responsible for noise-
one is tested at 40dB SL, it means he was tested at 40 dB induced hearing loss.

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 Chapter 4
Assessment of Hearing

Hearing loss can be of three types: 1. Finger Friction Test

It is a rough but quick method of screening and consists
1. CONDUCTIVE HEARING LOSS. It is caused by any disease of rubbing or snapping the thumb and a finger close to
process interfering with the conduction of sound from patient’s ear.
the external ear to the stapediovestibular joint. Thus the
cause may lie in the external ear (obstructions), tympanic 2. Watch Test
membrane (perforation), middle ear (fluid), ossicles (fixa- A clicking watch is brought close to the ear and the dis-
tion or disruption) or the eustachian tube (obstruction). tance at which it is heard is measured. It had been popular
as a screening test before the audiometric era but is prac-
2. SENSORINEURAL (SN) HEARING LOSS. It results from tically obsolete now. Clicking watches are also obsolete.
lesions of the cochlea (sensory type) or VIIIth nerve and
its central connections (neural type). The term retrococh- 3. Speech (Voice) Tests
lear is used when hearing loss is due to lesions of VIIIth Normally, a person hears conversational voice at 12 m
nerve, and central deafness, when it is due to lesions of (40 ft) and whisper (with residual air after normal expira-
central auditory connections. tion) at 6 m (20 ft) but for purposes of test, 6 m is taken as
normal for both conversation and whisper.
3. MIXED HEARING LOSS. In this type, elements of both The test is conducted in reasonably quiet surround-
conductive and sensorineural deafness are present in the ings. The patient stands with his test ear towards the
same ear. There is air-bone gap indicating conductive el- examiner at a distance of 6 m. His eyes are shielded to
ement, and impairment of bone conduction indicating prevent lip reading and the nontest ear is blocked by
sensorineural loss. Mixed hearing loss is seen in some intermittent pressure on the tragus by an assistant. The
cases of otosclerosis and chronic suppurative otitis media. examiner uses spondee words (e.g. black-night, football,
While assessing the auditory function it is important daydream) or numbers with letters (X3B, 2AZ, M6D) and
to find out: gradually walks towards the patient.
(a) Type of hearing loss (conductive, sensorineural or The distance at which conversational voice and the
mixed). whispered voice are heard is measured. The disadvantage
(b) Degree of hearing loss (mild, moderate, moderately se- of speech tests is lack of standardization in intensity and
vere, severe, profound or total). pitch of voice used for testing and the ambient noise of
(c) Site of lesion. If conductive, the lesion may be at ex- the testing place.
ternal ear, tympanic membrane, middle ear, ossicles
or eustachian tube. Clinical examination and tympa- 4. Tuning Fork Tests #
nometry can be helpful to find the site of such lesions. These tests are performed with tuning forks of differ-
If sensorineural, find out whether the lesion is coch- ent frequencies such as 128, 256, 512, 1024, 2048 and
lear, retrocochlear or central. Special tests of hearing 4096 Hz, but for routine clinical practice, tuning fork of
will be required to differentiate these types. 512 Hz is ideal. Forks of lower frequencies produce sense
(d) Cause of hearing loss. The cause may be congenital, trau- of bone vibration while those of higher frequencies have
matic, infective, neoplastic, degenerative, metabolic, a shorter decay time and are thus not routinely preferred.
ototoxic, vascular or autoimmune process. Detailed A tuning fork is activated by striking it gently against
history and laboratory investigations are required. the examiner’s elbow, heel of hand or the rubber heel of
the shoe.
To test air conduction (AC) (Figure 4.1 ), a vibrating
ASSESSMENT OF HEARING fork is placed vertically in line with the meatus, about
2 cm away from the opening of external auditory canal.
Hearing of an individual can be tested by clinical and au- The sound waves are transmitted through the tympanic
diometric tests. membrane, middle ear and ossicles to the inner ear. Thus,
by the air conduction test, the function of both the con-
ducting mechanism and the cochlea are tested. Normally,
A. CLINICAL TESTS OF HEARING hearing through air conduction is louder and heard twice
1. Finger friction test as long as through the bone conduction route.
2. Watch test To test bone conduction (BC), the footplate of vibrating
3. Speech tests tuning fork is placed firmly on the mastoid bone. Cochlea
4. Tuning fork tests is stimulated directly by vibrations conducted through

23

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24 SECTION I — Diseases of Ear

transcranial transmission of sound. In such cases, correct

diagnosis can be made by masking the nontest ear with
Barany’s noise box while testing for bone conduction.
Weber test will further help as it gets lateralized to the
better ear.

(B) WEBER TEST. In this test, a vibrating tuning fork is

placed in the middle of the forehead or the vertex and
the patient is asked in which ear the sound is heard. Nor-
mally, it is heard equally in both ears. It is lateralized to
the worse ear in conductive deafness and to the better ear
in sensorineural deafness. In weber test, sound travels di-
rectly to the cochlea via bone. Lateralization of sound in
weber test with a tuning fork of 512 Hz implies a conduc-
tive loss of 15–25 dB in ipsilateral ear or a sensorineural
loss in the contralateral ear.

(C) ABSOLUTE BONE CONDUCTION (ABC) TEST. Bone

Figure 4.1. Tuning fork tests. (A) Testing for air conduction. (B) Testing conduction is a measure of cochlear function. In ABC test,
for bone conduction. (C) Weber test. patient’s bone conduction is compared with that of the
Scan to play Hearing Tests and Tuning Fork Test. examiner (presuming that the examiner has normal hear-
ing). External auditory meatus of both the patient and ex-
the skull bones. Thus, BC is a measure of the cochlear aminer should be occluded (by pressing the tragus inwards)
function only. to prevent ambient noise entering through AC route. In
The clinically useful tuning fork tests include: conductive deafness, the patient and the examiner hear
the fork for the same duration of time. In sensorineural
(A) RINNE TEST. In this test air conduction of the ear is deafness, the patient hears the fork for a shorter duration.
compared with its bone conduction. A vibrating tuning
fork is placed on the patient’s mastoid and when he stops (D) SCHWABACH’S TEST. Here again BC of patient is com-
hearing, it is brought beside the meatus. If he still hears, pared with that of the normal hearing person (examiner)
AC is more than BC. Alternatively, the patient is asked but meatus is not occluded. It has the same significance
to compare the loudness of sound heard through air and as absolute bone conduction test. Schwabach is reduced
bone conduction. Rinne test is called positive when AC in sensorineural deafness and lengthened in conductive
is longer or louder than BC. It is seen in normal persons deafness.
or those having sensorineural deafness. A negative Rinne Table 4.1 summarizes the interpretation of tuning fork
(BC > AC) is seen in conductive deafness. A negative tests.
Rinne indicates a minimum air-bone gap of 15–20 dB.
A prediction of air-bone gap can be made if tuning (E) BING TEST. It is a test of bone conduction and exam-
forks of 256, 512 and 1024 Hz are used. ines the effect of occlusion of ear canal on the hearing. A
vibrating tuning fork is placed on the mastoid while the
• A Rinne test equal or negative for 256 Hz but positive
examiner alternately closes and opens the ear canal by
for 512 Hz indicates air-bone gap of 20–30 dB.
pressing on the tragus inwards. A normal person or one
• A Rinne test negative for 256 and 512 Hz but positive
with sensorineural hearing loss hears louder when ear ca-
for 1024 Hz indicates air-bone gap of 30–45 dB.
nal is occluded and softer when the canal is open (Bing
• A Rinne negative for all the three tuning forks of 256,
positive). A patient with conductive hearing loss will ap-
512 and 1024 Hz indicates air-bone gap of 45–60 dB.
preciate no change (Bing negative).
Remember that a negative Rinne for 256, 512 and
1024 Hz indicates a minimum AB gap of 15, 30, 45 dB, (F) GELLE’S TEST. It is also a test of bone conduction and
respectively. examines the effect of increased air pressure in ear canal on
FALSE NEGATIVE RINNE. It is seen in severe unilateral the hearing. Normally, when air pressure is increased in
sensorineural hearing loss. Patient does not perceive any the ear canal by Siegel’s speculum, it pushes the tympanic
sound of tuning fork by air conduction but responds membrane and ossicles inwards, raises the intralabyrin-
to bone conduction testing. This response to bone con- thine pressure and causes immobility of basilar mem-
duction is, in reality, from the opposite ear because of brane and decreased hearing, but no change in hearing

TABLE 4.1 TUNING FORK TESTS AND THEIR INTERPRETATION

Test Normal Conductive deafness SN deafness
Rinne AC > BC (Rinne positive) BC > AC (Rinne negative) AC > BC
Weber Not lateralized Lateralized to poorer ear Lateralized to better ear
ABC Same as examiner’s Same as examiner’s Reduced
Schwabach Equal Lengthened Shortened

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 Chapter 4 — Assessment of Hearing 25

(e) Helps to predict speech reception threshold.

2. Speech Audiometry
In this test, the patient’s ability to hear and understand
speech is measured. Two parameters are studied: (i) speech
reception threshold and (ii) discrimination score.

(A) SPEECH RECEPTION THRESHOLD (SRT). It is the mini-

mum intensity at which 50% of the words are repeated
correctly by the patient. A set of spondee words (two sylla-
ble words with equal stress on each syllable, e.g. baseball,
sunlight, daydream, etc.) is delivered to each ear through
the headphone of an audiometer. The word lists are deliv-
ered in the form of recorded tapes or monitored voice and
their intensity varied in 5 dB steps till half of them are cor-
rectly heard. Normally, SRT is within 10 dB of the average
Figure 4.2. Two-room audiometry setup. Audiometrician watches of pure tone threshold of three speech frequencies (500,
responses of the patient sitting across a glass partition. 1000 and 2000 Hz). An SRT better than pure tone average
by more than 10 dB suggests a functional hearing loss.
is observed when ossicular chain is fixed or disconnected.
Gelle’s test is performed by placing a vibrating fork on the (B) SPEECH DISCRIMINATION SCORE. Also called speech
mastoid while changes in air pressure in the ear canal are recognition or word recognition score. It is a measure of pa-
brought about by Siegel’s speculum. Gelle’s test is posi- tient’s ability to understand speech. Here, a list of pho-
tive in normal persons and in those with sensorineural netically balanced (PB) words (single syllable words, e.g.
hearing loss. It is negative when ossicular chain is fixed pin, sin, day, bus, etc.) is delivered to the patient’s each
or disconnected. It was a popular test to find out stapes ear separately at 30–40 dB above his SRT and the percent-
fixation in otosclerosis but has now been superceded by age of words correctly heard by the patient is recorded. In
tympanometry. normal persons and those with conductive hearing loss
a high score of 90–100% can be obtained (Figure 4.3A, B
and Table 4.2).
B. AUDIOMETRIC TESTS
1. Pure Tone Audiometry PERFORMANCE INTENSITY FUNCTION FOR PB WORDS
PB MAX. Instead of using a single suprathreshold in-
An audiometer is an electronic device which produces
tensity of 30–40 dB above SRT as described above, it is
pure tones, the intensity of which can be increased or de-
creased in 5 dB steps (Figure 4.2). Usually air conduction
thresholds are measured for tones of 125, 250, 500, 1000,
2000, 4000 and 8000 Hz and bone conduction thresholds
for 250, 500, 1000, 2000 and 4000 Hz. The amount of
intensity that has to be raised above the normal level is a
measure of the degree of hearing impairment at that fre-
quency. It is charted in the form of a graph called audio-
gram. The threshold of bone conduction is a measure of
cochlear function. The difference in the thresholds of air
and bone conduction (A–B gap) is a measure of the degree
of conductive deafness. It may be noted that audiometer
is so calibrated that the hearing of a normal person, both
for air and bone conduction, is at 0 dB and there is no
A–B gap, while tuning fork tests normally show AC > BC.
When difference between the two ears is 40 dB or
above in air conduction thresholds, the better ear is
masked to avoid getting a shadow curve from the nontest
better ear. Similarly, masking is essential in all bone con-
duction studies. Masking is done by employing narrow-
band noise to the nontest ear.

USES OF PURE TONE AUDIOGRAM

(a) It is a measure of threshold of hearing by air and bone
conduction and thus the degree and type of hearing
loss.
(b) A record can be kept for future reference.
(c) Audiogram is essential for prescription of hearing aid.
(d) Helps to find degree of handicap for medicolegal
purposes. Figure 4.3. Speech audiogram.

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26 SECTION I — Diseases of Ear

TABLE 4.2 ABILITY TO UNDERSTAND SPEECH Various types of tracings obtained are:
AND ITS RELATION TO SPEECH DISCRIMINATION
(SD) SCORE A LIST OF 50 PB WORDS IS
Type I Continuous and pulsed tracings overlap. Seen
PRESENTED AND THE NUMBER CORRECTLY
HEARD IS MULTIPLIED BY 2 in normal hearing or conductive hearing loss.
Type II Continuous and pulsed tracings overlap up
SD score Ability to understand speech to 1000 Hz and then continuous tracing falls.
90–100% Normal Seen in cochlear loss.
76–88% Slight difficulty Type III Continuous tracing falls below pulsed tracing
60–74% Moderate difficulty at 100–500 Hz even up to 40–50 dB. Seen in
40–58% Poor retrocochlear/neural lesion.
Very poor
<40% Type IV Continuous tracing falls below pulsed lesion
at frequencies up to 1000 Hz by more than
25 dB. Seen in retrocochlear/neural lesion.
better to chart PB scores against several levels of speech
Type V Continuous tracing is above pulsed one. Seen
intensity and find the maximum score (PB max) a person
in nonorganic hearing loss.
can attain. Also note the intensity of sound at which PB
max is attained. It is a useful test clinically to set the vol- Bekesy audiometry is seldom performed these days.
ume of hearing aid (Figure 4.3C). Maximum volume of
hearing aid should not be set above PB max.
ROLL OVER PHENOMENON. It is seen in retrocochlear
hearing loss. With increase in speech intensity above a 4. Impedance Audiometry (Figure 4.4)
particular level, the PB word score falls rather than main- It is an objective test, widely used in clinical practice and
tain a plateau as in cochlear type of sensorineural hearing is particularly useful in children. It consists of:
loss (Figure 4.3D).
(a) Tympanometry
Thus speech audiometry is useful in several ways:
(b) Acoustic reflex measurements
(i) To find speech reception threshold which correlates
well with average of three speech frequencies of pure (A) TYMPANOMETRY. It is based on a simple principle,
tone audiogram. i.e. when a sound strikes tympanic membrane, some of
(ii) To differentiate organic from nonorganic (function- the sound energy is absorbed while the rest is reflected. A
al) hearing loss. stiffer tympanic membrane would reflect more of sound
(iii) To find the intensity at which discrimination score is energy than a compliant one. By changing the pressures
best. This is helpful for fitting a hearing aid and set- in a sealed external auditory canal and then measuring
ting its volume for maximum discrimination. the reflected sound energy, it is possible to find the com-
(iv) To differentiate a cochlear from a retrocochlear sen- pliance or stiffness of the tympano-ossicular system and
sorineural hearing loss. thus find the healthy or diseased status of the middle
ear.
3. Bekesy Audiometry Essentially, the equipment consists of a probe which
It is a self-recording audiometry where various pure tone snugly fits into the external auditory canal and has three
frequencies automatically move from low to high while channels: (i) to deliver a tone of 220 Hz, (ii) to pick up the
the patient controls the intensity through a button. Two reflected sound through a microphone and (iii) to bring
tracings, one with continuous and the other with pulsed about changes in air pressure in the ear canal from posi-
tone, are obtained. The tracings help to differentiate a tive to normal and then negative (Figure 4.5). By chart-
cochlear from a retrocochlear and an organic from a func- ing the compliance of tympano-ossicular system against
tional hearing loss. various pressure changes, different types of graphs called

Figure 4.4. (A) Impedance audiometry in progress. (B) Impedance audiometer.

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 Chapter 4 — Assessment of Hearing 27

TESTING FUNCTION OF EUSTACHIAN TUBE. Tympanome-

try has also been used to find function of eustachian tube
in cases of intact or perforated tympanic membrane. A
negative or a positive pressure (−200 or +200 mm H2O) is
created in the middle ear and the person is asked to swal-
low five times in 20 s. The ability to equilibrate the pres-
sure indicates normal tubal function. The test can also be
used to find the patency of the grommet placed in the
tympanic membrane in cases of serous otitis media.

(B) ACOUSTIC REFLEX. It is based on the fact that a loud

sound, 70–100 dB above the threshold of hearing of a
particular ear, causes bilateral contraction of the stapedial
muscles which can be detected by tympanometry. Tone
can be delivered to one ear and the reflex picked from the
same or the contralateral ear. The reflex arc involved is:
Ipsilateral: CN VIII → ventral cochlear nucleus → CN
Figure 4.5. Principle of impedance audiometry. (A) Oscillator to pro- VII nucleus ipsilateral stapedius muscle.
duce a tone of 220 Hz. (B) Air pump to increase or decrease air pres- Contralateral: CN VIII → ventral cochlear nucleus →
sure in the air canal. (C) Microphone to pick up and measure sound
contralateral medial superior olivary nucleus → contralat-
pressure level reflected from the tympanic membrane.
eral CN VII nucleus → contralateral stapedius muscle
(Figure 4.7).
tympanograms are obtained which are diagnostic of cer- This test is useful in several ways:
tain middle ear pathologies.
(i) To test the hearing in infants and young children. It
Types of tympanograms (Figure 4.6)
is an objective method.
(ii) To find malingerers. A person who feigns total deaf-
Type A Normal tympanogram. ness and does not give any response on pure tone
Type As Compliance is lower at or near ambient air audiometry but shows a positive stapedial reflex is a
pressure. Seen in fixation of ossicles, e.g. oto- malingerer.
sclerosis or malleus fixation. (iii) To detect cochlear pathology. Presence of stapedial
Type AD High compliance at or near ambient pressure. reflex at lower intensities, e.g. 40–60 dB than the
Seen in ossicular discontinuity or thin and lax usual 70 dB indicates recruitment and thus a coch-
tympanic membrane. lear type of hearing loss.
Type B A flat or dome-shaped graph. No change in (iv) To detect VIIIth nerve lesion. If a sustained tone of
compliance with pressure changes. Seen in 500 or 1000 Hz, delivered 10 dB above acoustic reflex
middle ear fluid or thick tympanic membrane. threshold, for a period of 10 s, brings the reflex ampli-
Type C Maximum compliance occurs with negative tude to 50%, it shows abnormal adaptation and is in-
pressure in excess of 100 mm H2O. Seen in dicative of VIIIth nerve lesion (stapedial reflex decay).
retracted tympanic membrane and may show (v) Lesions of facial nerve. Absence of stapedial reflex
some fluid in middle ear. when hearing is normal indicates lesion of the facial
nerve, proximal to the nerve to stapedius. The reflex
can also be used to find prognosis of facial paralysis
as the appearance of reflex, after it was absent, indi-
cates return of function and a favourable prognosis.
(vi) Lesion of brainstem. If ipsilateral reflex is present but
the contralateral reflex is absent, lesion is in the area
of crossed pathways in the brainstem.

Figure 4.6. Types of tympanograms. Figure 4.7. Acoustic reflex.

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28 SECTION I — Diseases of Ear

PHYSICAL VOLUME OF EAR CANAL. Acoustic immittance SISI score is seldom more than 15%; it is 70–100% in
can also measure the physical volume of air between the cochlear deafness and 0–20% in nerve deafness.
probe tip and tympanic membrane. Normally it is up to
1.0 mL in children and 2 mL in adults. Any increase in 3. Threshold Tone Decay Test
volume, >2 mL in children and >2.5 mL in adults, in- It is a measure of nerve fatigue and is used to detect ret-
dicates perforation of the tympanic membrane (because rocochlear lesions. Normally, a person can hear a tone
middle ear volume is added up to the volume of external continuously for 60 s. In nerve fatigue, he stops hearing
ear canal). This has also been used to find patency of the earlier. The threshold tone decay test is simple and is per-
ventilation tube. formed in the following manner:
A tone of 4000 Hz is presented at 5 dB above the pa-
tient’s threshold of hearing, continuously for a period of
C. SPECIAL TESTS OF HEARING
60 s. If patient stops hearing earlier, intensity is increased
1. Recruitment by another 5 dB. The procedure is continued till patient
It is a phenomenon of abnormal growth of loudness. The can hear the tone continuously for 60 s, or no level exists
ear which does not hear low intensity sound begins to above the threshold where tone is audible for full 60 s.
hear greater intensity sounds as loud or even louder than The result is expressed as number of dB of decay. A decay
normal hearing ear. Thus, a loud sound which is tolerable more than 25 dB is diagnostic of a retrocochlear lesion.
in normal ear may grow to abnormal levels of loudness 4. Evoked Response Audiometry
in the recruiting ear and thus becomes intolerable. The
patients with recruitment are poor candidates for hear- It is an objective test which measures electrical activity in
ing aids. Recruitment is typically seen in lesions of the the auditory pathways in response to auditory stimuli. It
cochlea (e.g. Ménière’s disease and presbycusis) and thus requires special equipment with an averaging computer.
helps to differentiate a cochlear from a retrocochlear sen- There are several components of evoked electric response
sorineural hearing loss. but only two have gained clinical acceptance. They are:
Alternate binaural loudness balance test is used to detect (a) Electrocochleography (EcoG). It measures electrical
recruitment in unilateral cases. A tone, say of 1000 Hz, is potentials arising in the cochlea and CN VIII in response
played alternately to the normal and the affected ear and to auditory stimuli within first 5 ms. The response is
the intensity in the affected ear is adjusted to match the in the form of three phenomena: cochlear microphon-
loudness in normal ear. The test is started at 20 dB above ics, summating potentials and the action potential of
the threshold of deaf ear and then repeated at every 20 dB VIIIth nerve. The recording electrode is usually a thin
rise until the loudness is matched or the limits of audi- needle passed through the tympanic membrane onto
ometer reached. In conductive and neural deafness, the the promontory. In adults, it can be done under local
initial difference is maintained throughout while in coch- anaesthesia but in children or anxious persons seda-
lear lesions, partial, complete or over-recruitment may be tion or general anaesthesia is required. Sedation does
seen (Figure 4.8). not interfere in these responses. EcoG is useful (i) to
find threshold of hearing in young infants and children
2. Short Increment Sensitivity Index
(SISI Test)
Patients with cochlear lesions distinguish smaller chang-
es in intensity of pure tone better than normal persons
and those with conductive or retrocochlear pathology.
SISI test is thus used to differentiate a cochlear from a
retrocochlear lesion.
In this test, a continuous tone is presented 20 dB above
the threshold and sustained for about 2 min. Every 5 s, the
tone is increased by 1 dB and 20 such blips are presented.
Patient indicates the blips heard. In conductive deafness,

Figure 4.9. Electrocochleography. (A) Normal ear. (B) Ear with Mé-
nière’s disease. Voltage of summating potential (SP) is compared with
that of action potential (AP). Normally SP is 30% of AP. This ratio is
Figure 4.8. Alternate binaural loudness balance test. enhanced in Ménière’s disease.

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 Chapter 4 — Assessment of Hearing 29

5. Auditory Steady State Response (ASSR)

Though ABR, conducted with tone bursts of various fre-
quencies, can produce frequency-specific thresholds of
hearing in infants to fit hearing aid at a young age, it has
limitations. It cannot test hearing losses above 80 dB.
It cannot detect hearing sensitivity in severe to pro-
foundly deaf infants. ASSR is useful in such situations.
It is, like ABR, an electrophysiological test which uses
steady state pure tone signals instead of transient signals
of tone bursts or clicks used in ABR. The steady state
signals are also modulated rapidly in amplitude and fre-
quency and thus gives a frequency-specific audiogram.
Figure 4.10. Brainstem auditory evoked potentials. Hearing losses exceeding 80 dB can be detected. It can
help in selection of children for cochlear implantation
at an early age.
within 5–10 dB and (ii) to differentiate lesions of coch-
lea from those of the VIIIth nerve (Figure 4.9).
(b) Auditory brainstem response (ABR). Also called BAER
6. Otoacoustic Emissions (OAEs)
or BAEP (brainstem auditory evoked response or poten- They are low-intensity sounds produced by outer hair
tial) or BERA (brainstem evoked response audiometry) cells of a normal cochlea and can be elicited by a very
is to elicit brainstem responses to auditory stimulation sensitive microphone placed in the external ear canal and
by clicks or tone bursts. It is a noninvasive technique to analyzed by a computer. Sound produced by outer hair
find the integrity of central auditory pathways through cells travels in a reverse direction: outer hair cells → basi-
the VIIIth nerve, pons and midbrain. In this method, lar membrane → perilymph → oval window → ossicles →
electrical potentials are generated in response to several tympanic membrane → ear canal. OAEs are present when
click stimuli or tone bursts and picked up from the ver- outer hair cells are healthy and are absent when they are
tex by surface electrodes. It measures hearing sensitiv- damaged and thus help to test the function of cochlea.
ity in the range of 1000–4000 Hz. In a normal person, They do not disappear in VIIIth nerve pathology as coch-
seven waves are produced in the first 10 ms. The first, lear hair cells are normal.
third and fifth waves are most stable and are used in
measurements. The waves are studied for absolute la- TYPES OF OAES. Broadly OAEs are of two types: spon-
tency, interwave latency (usually between wave I and V) taneous and evoked. The latter are elicited by a sound
and the amplitude (Figure 4.10). stimulus.
The exact anatomic site of neural generators for vari- (a) Spontaneous OAEs. They are present in healthy nor-
ous waves is disputed but the latest studies indicate the mal hearing persons where hearing loss does not
following sites: exceed 30 dB. They may be absent in 50% of normal
persons.
(b) Evoked OAEs. They are further divided into two types
Wave I Distal part of CN VIII depending on the sound stimulus used to elicit them.
Wave II Proximal part of CN VIII near (i) Transient evoked OAEs (TEOAEs). Evoked by clicks.
the brainstem A series of click stimuli are presented at 80–85 dB
Wave III Cochlear nucleus SPL (sound pressure level) and response recorded.
Wave IV Superior olivary complex (ii) Distortion product OAEs (DPOAEs). Two tones are
Wave V Lateral lemniscus simultaneously presented to the cochlea to pro-
Waves VI and VII Inferior colliculus duce distortion products. They have been used to
test hearing in the range of 1000–8000 Hz.
As an aide memorie remember the mnemonic EE COLI
USES
(eight, eight, cochlear nucleus, olivary complex, lateral
(a) OAEs are used as a screening test of hearing in neo-
lemniscus, inferior colliculus) compare E COLI-MA in
nates and to test hearing in uncooperative or mentally
pathways of hearing.
challenged individuals after sedation. Sedation does
ABR is used:
not interfere with OAEs.
(i) As a screening procedure for infants. (b) They help to distinguish cochlear from retrocochlear
(ii) To determine the threshold of hearing in infants; hearing loss. OAEs are absent in cochlear lesions, e.g.
also in children and adults who do not cooperate ototoxic sensorineural hearing loss. They detect oto-
and in malingerers. toxic effects earlier than pure tone audiometry.
(iii) To diagnose retrocochlear pathology particularly (c) OAEs are also useful to diagnose retrocochlear pathol-
acoustic neuroma. ogy, especially auditory neuropathy. Auditory neurop-
(iv) To diagnose brainstem pathology, e.g. multiple scle- athy is a neurologic disorder of CN VIII. Audiometric
rosis or pontine tumours. tests, e.g. SNHL for pure tones, impaired speech dis-
(v) To monitor CN VIII intraoperatively in surgery of crimination score, absent or abnormal auditory brain-
acoustic neuromas to preserve the function of coch- stem response, show a retrocochlear type of lesion but
lear nerve. OAEs are normal.

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30 SECTION I — Diseases of Ear

OAEs are absent in 50% of normal individuals, lesions asked to identify both. Staggered spondaic word test is
of cochlea, middle ear disorders (as sound travelling in the one more often used. Pairs of spondaic words along
reverse direction cannot be picked up) and when hearing with digits or nonsense words are simultaneously pre-
loss exceeds 30 dB. sented to the ears. Patients with temporal lobe lesions
will have difficulty identifying these words when pre-
7. Central Auditory Tests sented to the ear opposite to that of the side of lesion.
Patients with central auditory disorders have difficulty (c) Binaural tests. They are used to identify integration of
in hearing in noisy surroundings or when the speech is information from both ears. Such tests are normal in
distorted and not clearly spoken. Three different types of cortical lesions but affected in lesions of brainstem
speech discrimination tests are used. and thus help to localize the site of lesion. Most com-
mon test used is binaural masking level difference test.
(a) Monotic test. It is presented with speech message which
is distorted. Patients with lesions of brain and cortex Central auditory tests are not used routinely.
have difficulty to understand the message.
(b) Dichotic test. Two different speech messages are pre- 8. Hearing Assessment in Infants and
sented simultaneously, one to each ear and patient is Children (see p. 132)

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 Chapter 5
Hearing Loss

CLASSIFICATION 4. Ossicular interruption with perforation: 38 dB

5. Malleus fixation: 10–25 dB
6. Closure of oval window: 60 dB

Note here that ossicular interruption with intact drum

causes more loss than ossicular interruption with perfo-
rated drum.

MANAGEMENT
Most cases of conductive hearing loss can be managed by
medical or surgical means. Treatment of these conditions
is discussed in respective sections. Briefly, it consists of:
Scan to play Types of Hearing Loss. 1. Removal of canal obstructions, e.g. impacted wax,
foreign body, osteoma or exostosis, keratotic mass, be-
nign or malignant tumours, or meatal atresia.
CONDUCTIVE HEARING LOSS
2. Removal of fluid. Myringotomy with or without
AND ITS MANAGEMENT grommet insertion.
Any disease process which interferes with the conduction 3. Removal of mass from middle ear. Tympanotomy
of sound to reach cochlea causes conductive hearing loss. and removal of small middle ear tumours or cholestea-
The lesion may lie in the external ear and tympanic mem- toma behind intact tympanic membrane.
brane, middle ear or ossicles up to stapediovestibular joint. 4. Stapedectomy, as in otosclerotic fixation of stapes
The characteristics of conductive hearing loss are: footplate.
5. Tympanoplasty. Repair of perforation, ossicular chain
1. Negative Rinne test, i.e. BC > AC. or both.
2. Weber lateralized to poorer ear. 6. Hearing aid. In cases, where surgery is not possible,
3. Normal absolute bone conduction. refused or has failed.
4. Low frequencies affected more.
5. Audiometry shows bone conduction better than air
conduction with air-bone gap. Greater the air-bone
Tympanoplasty #
It is an operation to (i) eradicate disease in the middle
gap, more is the conductive loss (Figure 5.1). ear and (ii) to reconstruct hearing mechanism. It may be
6. Loss is not more than 60 dB. combined with mastoidectomy if disease process so de-
7. Speech discrimination is good. mands. Type of middle ear reconstruction depends on the
damage present in the ear. The procedure may be limited
AETIOLOGY only to repair of tympanic membrane (myringoplasty), or
to reconstruction of ossicular chain (ossiculoplasty),
The cause may be congenital (Table 5.1) or acquired or both (tympanoplasty). Reconstructive surgery of the ear
(Table 5.2). has been greatly facilitated by development of operating
microscope, microsurgical instruments and biocompat-
AVERAGE HEARING LOSS SEEN IN ible implant materials.
DIFFERENT LESIONS OF CONDUCTIVE From the physiology of hearing mechanism, the follow-
APPARATUS ing principles can be deduced to restore hearing surgically:
(a) An intact tympanic membrane, to provide large hydrau-
1. Complete obstruction of ear canal: 30 dB lic ratio between the tympanic membrane and stapes
2. Perforation of tympanic membrane 10–40 dB footplate.
(It varies and is directly proportional to (b) Ossicular chain, to conduct sound from tympanic
the size of perforation): membrane to the oval window.
3. Ossicular interruption with intact 54 dB (c) Two functioning windows, one on the scala vestibuli (to
drum: receive sound vibrations) and the other on the scala

31

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32 SECTION I — Diseases of Ear

(e) Functioning eustachian tube, to provide aeration to the

middle ear.
(f) A functioning sensorineural apparatus, i.e. the cochlea
and VIIIth nerve.

TYPES OF TYMPANOPLASTY. Wullstein classified tympa-

noplasty into five types (Figure 5.2).

Type I Defect is perforation of tympanic mem-

brane which is repaired with a graft. It is
also called myringoplasty.
Type II Defect is perforation of tympanic mem-
brane with erosion of malleus. Graft is
placed on the incus or remnant of malleus.
Type III Malleus and incus are absent. Graft is
placed directly on the stapes head. It is
also called myringostapediopexy or columella
Figure 5.1. (A) Audiogram of right ear showing conductive hearing
tympanoplasty.
loss with A–B gap. (B) Symbols used in audiogram charting. Type IV Only the footplate of stapes is present. It
is exposed to the external ear, and graft is
placed between the oval and round win-
TABLE 5.1 CONGENITAL CAUSES OF CONDUCTIVE dows. A narrow middle ear (cavum mi-
HEARING LOSS nor) is thus created to have an air pocket
around the round window. A mucosa-lined
• Meatal atresia
• Fixation of stapes footplate
space extends from the eustachian tube to
• Fixation of malleus head the round window. Sound waves in this
• Ossicular discontinuity case act directly on the footplate while the
• Congenital cholesteatoma round window has been shielded.
Type V Stapes footplate is fixed but round window
is functioning. In such cases, another win-
dow is created on horizontal semicircular
canal and covered with a graft. Also called
TABLE 5.2 ACQUIRED CAUSES OF CONDUCTIVE
fenestration operation.
HEARING LOSS
External ear Any obstruction in the ear canal, e.g. wax,
foreign body, furuncle, acute inflammatory
swelling, benign or malignant tumour or
Several modifications have appeared in the above clas-
atresia of canal. sification and they mainly pertain to the types of ossicu-
Middle ear (a) Perforation of tympanic membrane, lar reconstruction.
traumatic or infective
(b) Fluid in the middle ear, e.g. acute MYRINGOPLASTY. It is repair of tympanic membrane.
otitis media, serous otitis media or Graft materials of choice are temporalis fascia or the
haemotympanum perichondrium taken from the patient. Sometimes, ho-
(c) Mass in middle ear, e.g. benign or mografts such as dura, vein, fascia or cadaver tympan-
malignant tumour ic membrane are also used. Repair can be done by two
(d) Disruption of ossicles, e.g. trauma to
techniques—the underlay or the overlay. In the underlay
ossicular chain, chronic suppurative otitis
media, cholesteatoma
technique, margins of perforation are freshened and the
(e) Fixation of ossicles, e.g. otosclerosis, graft placed medial to perforation or tympanic annulus,
tympanosclerosis, adhesive otitis media if large, and is supported by gelfoam in the middle ear
(f) Eustachian tube blockage, e.g. retracted (Figure 5.3A). In the overlay technique, the graft is placed
tympanic membrane, serous otitis media lateral to fibrous layer of the tympanic membrane after
carefully removing all squamous epithelium from the lat-
eral surface of tympanic membrane remnant (Figure 5.3B
tympani (to act as a relief window). If it is only one and Chapter 83).
window, as in stapes fixation or closure of round win-
dow, there will be no movement of cochlear fluids re- OSSICULAR RECONSTRUCTION. Ossicles are essential for
sulting in conductive hearing loss. transmission of sound from tympanic membrane to laby-
(d) Acoustic separation of two windows, so that sound does rinth. Several types of prosthesis are available to replace
not reach both the windows simultaneously. It can be ossicles depending on the ossicular defects (Table 5.3).
achieved by providing an intact tympanic membrane, Autograft ossicles can be sculptured to bridge the gap.
preferential pathway to one window (usually the oval) Homograft preserved ossicles with or without tympanic
by providing ossicular chain and by the presence of membrane have been used but are difficult to procure and
air in the middle ear. have danger of transmission of disease (Figure 5.4).

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 Chapter 5 — Hearing Loss 33

Figure 5.2. Types of tympanoplasty. The graft is progressively in contact with malleus (type I), incus (type II), stapes (type III), stapes footplate
(type IV), or fenestra in horizontal semicircular canal (type V). In classical type IV, the graft was attached to promontory, this provides sound pro-
tection for round window while footplate was directly exposed.

Figure 5.3. Myringoplasty. (A) Underlay technique—fascia graft is

under the anterior annulus. It is supported by gelfoam in the mid-
dle ear to prevent medial displacement. (B) Overlay technique—fascia
graft lies lateral to anterior annulus onto the anterior bony canal wall.
It is placed medial to malleus handle to prevent lateralization.

TABLE 5.3 MATERIALS USED FOR OSSICULAR

RECONSTRUCTION
Type of graft Material Remarks
Figure 5.4. Ossicular reconstruction. Sculptured autograft or homo-
Autograft • Incus, head of • Risk of harbouring graft ossicles have been used. (A) Malleus—Stapes assembly. Modified
malleus disease incus graft connecting malleus handle with stapes head. (B) Malleus—
• Cortical bone from • Low cost Footplate assembly. Modified malleus connecting malleus handle with
mastoid • Easily available stapes footplate. (C) Modified incus connecting tympanic membrane
Allograft • Plastipore • Readymade (TM) to stapes head. Malleus is missing. (D) Modified incus connect-
(polyethylene • Easy to store and ing TM to stapes footplate.
sponge) use
• Hydroxyapatite • Costly
(HA) implants • Likely to be At the time of ossicular reconstruction in chronic otitis
• Titanium implants extruded media, one should ensure:
• Glass isomer
• Middle ear is healthy and free of mucosal disease and
• Teflon prosthesis
• HA cholesteatoma.
(50%) + Titanium • Eustachian tube function is good. Atelectatic middle
(50%) ear shows poor eustachian tube function.
• HA + Silicon
In cases of canal wall-up mastoidectomy done for cho-
(flex-HA)
• HA + Polyethylene lesteatoma or active mucosal disease, the procedure is de-
(HAPEX) layed for about 6 months to ensure ear is free of disease.
Homograft • Preserved ossicles • Difficult to procure Primary ossicular reconstruction can be performed in:
only • Risk of disease
• Traumatic ossicular disruption
• Ossicles with transmission
• Fixation of ossicles
tympanic
membrane • Canal wall down procedures when there is no mucosal
disease or cholesteatoma

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34 SECTION I — Diseases of Ear

Figure 5.6. Audiogram of right ear showing sensorineural loss with

no A–B gap.

Figure 5.5. Hydroxyapatite TORP and PORP. (A) Centred and (B) off-
set types. (C) Titanium TORP. (D) Titanium PORP.
AETIOLOGY
Congenital
It is present at birth and is the result of anomalies of the
inner ear or damage to the hearing apparatus by prenatal
or perinatal factors (see p. 129).
Types of prosthesis (Figure 5.5)
1. Incus prosthesis. Used when incus is missing but han- Acquired
dle of malleus and stapes with superstructure are pre- It appears later in life. The cause may be genetic or non-
sent and functional. genetic. The genetic hearing loss may manifest late (de-
2. Incus–stapes prosthesis. Used when incus and stapes layed onset) and may affect only the hearing, or be a part
superstructure are missing. Malleus and stapes foot- of a larger syndrome affecting other systems of the body
plate are functional. as well (syndromal). Common causes of acquired SNHL
3. Partial ossicular replacement prosthesis (PORP). include:
Used when malleus and incus are absent. Stapes is pre-
1. Infections of labyrinth—viral, bacterial or spiro-
sent and mobile. PORP is placed between tympanic
chaetal
membrane and stapes head.
2. Trauma to labyrinth or VIIIth nerve, e.g. fractures of
4. Total ossicular replacement prosthesis (TORP).
temporal bone or concussion of the labyrinth or the
Used when malleus, incus and stapes superstructure
ear surgery
are absent. Only the stapes footplate is present and is
3. Noise-induced hearing loss
mobile.
4. Ototoxic drugs
5. Presbycusis
6. Ménière’s disease
SENSORINEURAL HEARING LOSS 7. Acoustic neuroma
AND ITS MANAGEMENT 8. Sudden hearing loss
9. Familial progressive SNHL
Sensorineural hearing loss (SNHL) results from lesions of
10. Systemic disorders, e.g. diabetes, hypothyroidism,
the cochlea, VIIIth nerve or central auditory pathways. It
kidney disease, autoimmune disorders, multiple scle-
may be present at birth (congenital) or start later in life
rosis, blood dyscrasias.
(acquired).
The characteristics of sensorineural hearing loss are:
DIAGNOSIS
1. A positive Rinne test, i.e. AC > BC.
2. Weber lateralized to better ear.
3. Bone conduction reduced on Schwabach and absolute 1. HISTORY. It is important to know whether disease is
bone conduction tests. congenital or acquired, stationary or progressive, associ-
4. More often involving high frequencies. ated with other syndromes or not, involvement of other
5. No gap between air and bone conduction curve on au- members of the family and possible aetiologic factors.
diometry (Figure 5.6).
6. Loss may exceed 60 dB. 2. SEVERITY OF DEAFNESS (MILD, MODERATE, MODER-
7. Speech discrimination is poor. ATELY SEVERE, SEVERE, PROFOUND OR TOTAL). This can
8. There is difficulty in hearing in the presence of noise. be found out on audiometry.

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 Chapter 5 — Hearing Loss 35

3. TYPE OF AUDIOGRAM. Whether loss is high frequency, of 2 or the late form, manifesting at the age of 8–20 years.
low frequency, mid-frequency or flat type. Syphilitic involvement of the inner ear can cause:
(a) Sudden sensorineural hearing loss, which may be uni-
4. SITE OF LESION. i.e. cochlear, retrocochlear or central.
lateral or bilateral. The latter is usually symmetrical in
high frequencies or is a flat type.
5. LABORATORY TESTS. They depend on the aetiology
(b) Ménière’s syndrome with episodic vertigo, fluctuat-
suspected, e.g. X-rays or CT scan of temporal bone for
ing hearing loss, tinnitus and aural fullness—a picture
evidence of bone destruction (congenital cholesteatoma,
simulating Ménière’s disease.
glomus tumour, middle ear malignancy or acoustic neu-
(c) Hennebert’s sign. A positive fistula sign in the absence
roma), blood counts (leukaemia), blood sugar (diabetes),
of a fistula. This is due to fibrous adhesions between
serology for syphilis, thyroid functions (hypothyroid-
the stapes footplate and the membranous labyrinth.
ism), kidney function tests, etc.
(d) Tullio phenomenon in which loud sounds produce
vertigo.
MANAGEMENT
Diagnosis of otosyphilis can be made by other clinical
Early detection of SNHL is important as measures can be evidence of late acquired or congenital syphilis (interstitial
taken to stop its progress, reverse it or to start an early re- keratitis, Hutchinson’s teeth, saddle nose, nasal septal per-
habilitation programme, so essential for communication. foration and frontal bossing) and the laboratory tests. Fluo-
Syphilis of the inner ear is treatable with high doses of rescent treponema-absorption test (FTA-ABS) and venereal
penicillin and steroids with improvement in hearing. Hear- disease research laboratory (VDRL) or rapid plasma reagin
ing loss of hypothyroidism can be reversed with replacement (RPR) tests from CSF are useful to establish the diagnosis.
therapy. Serous labyrinthitis can be reversed by attention to Treatment of otosyphilis includes i.v. penicillin and
middle ear infection. Early management of Ménière’s disease steroids.
can prevent further episodes of vertigo and hearing loss.
SNHL due to perilymph fistula can be corrected surgically
by sealing the fistula in the oval or round window with fat. B. FAMILIAL PROGRESSIVE SENSORINEURAL
Ototoxic drugs should be used with care and discontin- HEARING LOSS
ued if causing hearing loss. In many such cases, it may be
possible to regain hearing, total or partial, if the drug is It is a genetic disorder in which there is progressive degen-
stopped. Noise-induced hearing loss can be prevented from eration of the cochlea starting in late childhood or early
further deterioration if the person is removed from the adult life. Hearing loss is bilateral with flat or basin-shaped
noisy surroundings. audiogram but an excellent speech discrimination.
Rehabilitation of hearing impaired with hearing aids
and other devices is discussed in Chapter 20.
C. OTOTOXICITY
Various drugs and chemicals can damage the inner ear
and cause sensorineural hearing loss, tinnitus and some-
SPECIFIC FORMS OF HEARING LOSS times vertigo (Table 5.4).
A. INFLAMMATIONS OF LABYRINTH
1. AMINOGLYCOSIDE ANTIBIOTICS. Streptomycin, gen-
It may be viral, bacterial or syphilitic. tamicin and tobramycin are primarily vestibulotoxic.
They selectively destroy type I hair cells of the crista am-
1. VIRAL LABYRINTHITIS. Viruses usually reach the in- pullaris but, administered in large doses, can also damage
ner ear by blood stream affecting stria vascularis and then the cochlea.
the endolymph and organ of Corti. Measles, mumps and Neomycin, kanamycin, amikacin, sisomycin and di-
cytomegaloviruses are well-documented to cause laby- hydrostreptomycin are cochleotoxic. They cause selective
rinthitis. Several other viruses, e.g. rubella, herpes zoster, destruction of outer hair cells, starting at the basal coil
herpes simplex, influenza and Epstein–Barr are clinically and progressing onto the apex of cochlea.
known to cause deafness but direct proof of their inva- Patients particularly at risk are those:
sion of labyrinth is lacking.
(a) having impaired renal function,
(b) elderly people above the age of 65,
2. BACTERIAL. Bacterial infections reach labyrinth
(c) concomitantly receiving other ototoxic drugs,
through the middle ear (tympanogenic) or through CSF
(d) who have already received aminoglycoside antibiotics,
(meningogenic). Labyrinthitis as a complication of mid-
(e) who are receiving high doses of ototoxic drugs with
dle ear infection is discussed on page 45. Sensorineural
high serum level of drug, and
hearing loss following meningitis is a well-known clini-
(f) who have genetic susceptibility to aminoglycosides.
cal entity. Bacteria can invade the labyrinth along nerves,
Here the antibiotic binds to the ribosome and inter-
vessels, cochlear aqueduct or the endolymphatic sac.
feres with protein synthesis, thus causing death of the
Membranous labyrinth is totally destroyed.
cochlear cells.
3. SYPHILITIC. Sensorineural hearing loss is caused both Symptoms of ototoxicity, hearing loss, tinnitus and/or
by congenital and acquired syphilis. Congenital syphi- giddiness may manifest during treatment or after comple-
lis is of two types: the early form, manifesting at the age tion of the treatment (delayed toxicity).

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36 SECTION I — Diseases of Ear

TABLE 5.4 OTOTOXIC DRUGS 7. DEFEROXAMINE (DESFERRIOXAMINE). It is an iron-

chelating substance used in the treatment of thalassaemic
A. Aminoglycoside antibiotics E. Analgesics patients who receive repeated blood transfusions and in
• Streptomycin • Salicylates
turn have high iron load. Like cisplatin and aminogly-
• Dihydrostreptomycin • Indomethacin
cosides, deferoxamine also causes high-frequency senso-
• Gentamicin • Phenylbutazone
• Tobramycin • Ibuprofen rineural hearing loss. Onset of hearing loss is sudden or
• Neomycin F. Chemicals delayed. It is permanent but in some cases it can be re-
• Kanamycin • Alcohol versible when the drug is discontinued. It causes toxicity
• Amikacin • Tobacco to nerves; children are affected more.
• Netilmycin • Marijuana
• Sisomycin • Carbon monoxide 8. MISCELLANEOUS. Isolated cases of deafness have been
B. Diuretics poisoning reported with erythromycin, ampicillin and chloram-
• Furosemide G. Miscellaneous phenicol, indomethacin, phenylbutazone, ibuprofen,
• Ethacrynic acid • Erythromycin
tetanus antitoxin, propranolol and propylthiouracil.
• Bumetanide • Ampicillin
Alcohol, tobacco and marijuana also cause damage to
C. Antimalarials • Propranolol
• Quinine • Propylthiouracil the inner ear.
• Chloroquine • Deferoxamine
• Hydroxychloroquine 9. TOPICAL EAR DROPS. Topical use of drugs in the mid-
D. Cytotoxic drugs dle ear can also cause damage to the cochlea by absorp-
• Nitrogen mustard tion through oval and round windows. Deafness has
(Mechlorethamine) occurred with the use of chlorhexidine which was used
• Cisplatin in the preparation of ear canal before surgery or use of
• Carboplatin ear drops containing aminoglycoside antibiotics, e.g. ne-
omycin, framycetin and gentamicin. Ototoxic potential
is also present in ear drops containing polymyxin B, pro-
2. DIURETICS. Furosemide, bumetanide and ethacrynic pylene glycol and antifungal agents. Use only approved
acid are called loop diuretics as they block transport of so- ototopical drops for middle ear infection.
dium and chloride ions in the ascending loop of Henle.
They are known to cause oedema and cystic changes in D. NOISE TRAUMA
the stria vascularis of the cochlear duct. In most cases,
Hearing loss associated with exposure to noise has been
the effect is reversible but permanent damage may occur.
well-known in boiler makers, iron- and coppersmiths,
Hearing loss may be bilateral and symmetrical or some-
and artillery men. Lately, noise trauma has assumed
time sudden in onset.
greater significance because of its being an occupational
hazard; the compensations asked for and the responsibili-
3. SALICYLATES. Symptoms of salicylate ototoxicity are
ties thrust upon the employer and the employee to con-
tinnitus and bilateral sensorineural hearing loss particu-
serve hearing. Hearing loss caused by excessive noise can
larly affecting higher frequencies. Site of lesion testing
be divided into two groups:
indicates cochlear involvement, but light and electron
microscopy have failed to show any morphologic chang-
1. ACOUSTIC TRAUMA. Permanent damage to hear-
es in the hair cells. Possibly they interfere at enzymatic
ing can be caused by a single brief exposure to very in-
level. Hearing loss due to salicylates is reversible after the
tense sound without this being preceded by a temporary
drug is discontinued. SNHL has also been noted with oth-
threshold shift. Also called impulse noise, such noise can
er NSAIDs, e.g. naproxen, piroxicam and ketorolac but is
arise from an explosion, gun fire or a powerful cracker
reversible.
and may reach or cross 140 dB. Noise level of a gun or
rifle may reach 140–170 dB SPL (sound pressure level).
4. QUININE. Ototoxic symptoms due to quinine are tin-
Such brief and loud noises mechanically damage organ
nitus and sensorineural hearing loss, both of which are
of Corti, tear Reissner’s membrane, rupture hair cells and
reversible. Higher doses may cause permanent loss. The
allowing mixing of perilymph and endolymph. A severe
symptoms generally appear with prolonged medication
blast, in addition, may concomitantly damage the tym-
but may occur with smaller doses in those who are sus-
panic membrane and disrupt ossicles further adding con-
ceptible. Congenital deafness and hypoplasia of cochlea
ductive loss. Impulse noise may be as brief as 0.2 ms. No
have been reported in children whose mothers received
impulse noise more than 140 dB (A) is permitted.
this drug during the first trimester of pregnancy. Ototoxic
effects of quinine are due to vasoconstriction in the small
2. NOISE-INDUCED HEARING LOSS (NIHL). Hearing loss,
vessels of the cochlea and stria vascularis.
in this case, follows chronic exposure to less intense sounds
than seen in acoustic trauma and is mainly a hazard of
5. CHLOROQUINE AND HYDROXYCHLOROQUINE. Effect
noisy occupations.
is similar to that of quinine and cause reversible SNHL.
Sometimes permanent deafness can result. (a) Temporary threshold shift (TTS). The hearing is impaired
immediately after exposure to noise but recovers after
6. CYTOTOXIC DRUGS. Nitrogen mustard, cisplatin and an interval of a few minutes to a few hours even up
carboplatin can cause cochlear damage. They affect the to 2 weeks. Amount of TTS depends on the noise—its
outer hair cells of the cochlea. intensity, frequency and duration.

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TABLE 5.5 PERMISSIBLE EXPOSURE IN CASES OF TABLE 5.6 PERMISSIBLE LIMITS OF NOISE AS
CONTINUOUS NOISE OR A NUMBER OF SHORT- PER THE NOISE POLLUTION (REGULATION
TERM EXPOSURES [GOVERNMENT OF INDIA, AND CONTROL) RULES 2000, MINISTRY OF
MINISTRY OF LABOUR, MODEL RULES UNDER ENVIRONMENT AND FOREST, GOVT. OF INDIA
FACTORIES ACT 1948 (CORRECTED UP TO 31.3.87)] Day (6 am to 10 pm) Night (10 pm to 6 am)
Noise levela (dBA) Permitted daily exposure (h) Zone/Area Limits in dB(A) Leqa Limits in dB(A) Leq
90 8.0 Industrial 75 70
92 6.0 Commercial 65 55
95 4.0 Residential 55 45
97 3.0 Silence 50 40
100 2.0
aLeq = Energy mean of noise level over a specified period.
102 11/2
105 1.0
110 1/2
115 1/4
a5 dB rule of time intensity states that “any rise of 5 dB noise level will
reduce the permitted noise exposure time to half.”

(b) Permanent threshold shift (PTS). The hearing impair-

ment is permanent and does not recover at all.
The damage caused by noise trauma depends on sev-
eral factors:
(i) Frequency of noise. A frequency of 2000–3000 Hz
causes more damage than lower or higher frequen-
cies.
(ii) Intensity and duration of noise. As the intensity in-
creases, permissible time for exposure is reduced.
Table 5.5 gives the permissible limits of time for
various intensity levels for the safety of ear.
(iii) Continuous vs interrupted noise. Continuous noise is
Figure 5.7. Early case of noise-induced hearing loss. Note dip at
more harmful.
4000 Hz.
(iv) Susceptibility of the individual. Degree of TTS and
PTS varies in different individuals.
(v) Pre-existing ear disease. TABLE 5.7 HEARING ATTENUATION PROVIDED
BY DIFFERENT DEVICES
A noise of 90 dB (A) SPL, 8 h a day for 5 days per week
is the maximum safe limit as recommended by Ministry Cotton wool 5 dB
of Labour, Govt. of India, Model Rules under Factories Ear plug 15–30 dB (mostly in range of 3–5 kHz)
Act (Table 5.5). No exposure in excess of 115 dB (A) is to Ear muffs 30–40 dB (500–1 kHz)
Ear plugs + muffs More than 40 dB
be permitted. No impulse noise of intensity greater than
140 dB (A) is permitted. Note: Hearing protectors provide more attenuation in higher frequencies,
The Noise Pollution (Regulation and Control) Rules 25–40 dB for 1000–8000 Hz while only 10–30 dB attenuation for lower
2000, Ministry of Environment and Forest, Govt. of India frequencies less than 500 Hz.
has defined permissible limits of noise for various zones
or areas (Table 5.6). According to which silence zone is 500, 1000 and 2000 Hz (the speech frequencies) are also
100 m around the premises of hospitals, nursing homes, affected.
educational institutions and courts. Also manufacture, NIHL causes damage to hair cells, starting in the basal
sale and use of fire crackers generating sound level above turn of cochlea. Outer hair cells are affected before the
125 dB (AI) or 145 dB (C) pk from 4 m distance from the inner hair cells.
point of bursting are not permitted (Environment Pro- Noise-induced hearing loss is preventable. Persons
tection Rules 2006) [dB (AI) = A-weighted impulse sound who have to work at places where noise is above 85 dB
pressure level in decibels; dB (C) pk = C-weighted peak (A) should have pre-employment and then annual audio-
sound pressure in decibels]. grams for early detection. Ear protectors (ear plugs or ear
The audiogram in NIHL shows a typical notch, at muffs) should be used where noise levels exceed 85 dB
4 kHz, both for air and bone conduction (Figure 5.7). It (A). They provide protection up to 35 dB (see Table 5.7). If
is usually symmetrical on both sides. At this stage, pa- hearing impairment has already occurred, rehabilitation
tient complains of high-pitched tinnitus and difficulty in is similar to that employed for other sensorineural hear-
hearing in noisy surroundings but no difficulty in day-to- ing losses.
day hearing. As the duration of noise exposure increases,
the notch deepens and also widens to involve lower and 3. NONAUDITORY EFFECTS OF NOISE. Apart from hear-
higher frequencies. Hearing impairment becomes clini- ing loss, noise can affect other systems of the body. It
cally apparent to the patient when the frequencies of interferes with rest and sleep causing chronic fatigue and

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38 SECTION I — Diseases of Ear

stress. Through activation of the autonomic nervous sys- F. SUDDEN HEARING LOSS
tem and pituitary–adrenal axis, it causes annoyance and
irritability. Hypertension and peptic ulcer have also been Sudden SNHL is defined as 30 dB or more of SNHL over
attributed to it. It also adversely affects task performance at least three contiguous frequencies occurring within a
where communication through speech is required. Laryn- period of 3 days or less. Mostly it is unilateral. It may be
geal problems have been noticed in workers who have to accompanied by tinnitus or temporary spell of vertigo.
speak loudly in persistently noisy surroundings.
Aetiology
Most often the cause of sudden deafness remains obscure,
E. AUTOIMMUNE (IMMUNE-MEDIATED) in which case it is called the idiopathic variety. In such
INNER EAR DISEASE cases, three aetiological factors are considered—viral, vas-
Immune-mediated inner ear disease (Syn. autoimmune cular or the rupture of cochlear membranes. Spontaneous
SNHL) causes progressive bilateral sensorineural hearing perilymph fistulae may form in the oval or round window.
loss. It occurs between 40 and 50 years with equal inci- Other aetiological factors which cause sudden deafness
dence in both sexes. Nearly 50% of patients also experi- and must be excluded are listed below. Remember the
ence vestibular symptoms like disequilibrium, motion mnemonic “In The Very Ear Too No Major Pathology.”
intolerance, positional or episodic vertigo. About 15% of 1. Infections. Mumps, herpes zoster, meningitis, enceph-
patients have evidence of other autoimmune disorder such alitis, syphilis, otitis media.
as ulcerative colitis, systemic lupus, rheumatoid arthritis or 2. Trauma. Head injury, ear operations, noise trauma,
multiple sclerosis. Moscicki et al. defined the condition as: barotrauma, spontaneous rupture of cochlear mem-
‘Bilateral SNHL ≥ 30 dB at any frequency and evidence branes.
of progression in at least one ear on two serial audiograms 3. Vascular. Haemorrhage (leukaemia), embolism or
that are done at equal to or less than 3 months apart. thrombosis of labyrinthine or cochlear artery or their
Progression is defined as threshold shift of ≥ 15 dB at one vasospasm. They may be associated with diabetes, hy-
frequency or 10 dB at two or more consecutive frequen- pertension, polycythaemia, macroglobinaemia or sick-
cies or significant change in speech discrimination’. le cell trait.
Investigations 4. Ear (otologic). Ménière’s disease, Cogan’s syndrome,
large vestibular aqueduct.
1. Audiogram. To establish above criteria, repeated au- 5. Toxic. Ototoxic drugs, insecticides.
diograms can be taken at one month intervals. Audio- 6. Neoplastic. Acoustic neuroma. Metastases in cerebel-
gram may show loss at high and low frequencies. lopontine angle, carcinomatous neuropathy.
2. Speech audiogram. Speech discrimination is affected 7. Miscellaneous. Multiple sclerosis, hypothyroidism,
though threshold of pure tones remains the same. sarcoidosis.
3. Evoked response audiometry. To exclude acoustic 8. Psychogenic.
neuroma or multiple sclerosis.
4. Contrast-enhanced MRI. Management
5. Blood tests to exclude systemic autoimmune disor-
As far as possible, the aetiology of sudden hearing loss
ders. Total and differential counts, ESR, rheumatoid
should be discovered by detailed history, physical exami-
factor, antinuclear antibodies, C3 and C4 compli-
nation and laboratory investigations. The investigations
ment levels, Raji cell assay for circulating immune
may include audiometry, vestibular tests, imaging studies
complexes.
of temporal bones, sedimentation rate, tests for syphilis,
6. Western blot essay for anti-Hsp 70 (anti-heat shock
diabetes, hypothyroidism, blood disorders and lipid pro-
protein 70) antibodies. Antigen used in this test is
files. Some cases may require exploratory tympanotomy
crude protein extract from bovine renal cells. It is not
where perilymph fistula is strongly suspected. Where the
a specific test for diagnosis but correlates to both active
cause still remains obscure, treatment is empirical and
disease and steroid responsiveness.
consists of:
Treatment 1. Bed rest.
Prednisolone 1 mg/kg/day up to a total of 60 mg/day (for 2. Steroid therapy. Prednisolone 40–60 mg in a single
adults) for 4 weeks. Sometimes response is late. If no re- morning dose for 1 week and then tailed off in a period
sponse is seen in 4 weeks, steroid is tapered off in 12 days. of 3 weeks. Steroids are anti-inflammatory and relieve
Responders continue till a plateau is reached and then oedema. They have been found useful in idiopathic
continue on maintenance dose of 10–20 mg every other sudden hearing loss of moderate degree.
day for about 6 months. Side effects and risks of long- 3. Inhalation of carbogen (5% CO2 + 95% O2). It increas-
term steroid therapy should be kept in mind. es cochlear blood flow and improves oxygenation.
Those who cannot take steroids can be given metho- 4. Vasodilator drugs.
trexate 15 mg/week for 6–8 weeks and if the patient re- 5. Low molecular weight dextran. It decreases blood
sponds, continue it for 6 months. If no response is ob- viscosity. It is contraindicated in cardiac failure and
tained for 6–8 weeks trial, drug is discontinued. bleeding disorders.
Alternative to methotrexate is cyclophosphamide but 6. Hyperbaric oxygen therapy. Available only in select-
it is more toxic. ed centres, hyperbaric oxygen raises concentration of
Other treatments include intratympanic steroid injec- oxygen in labyrinthine fluids and improves cochlear
tion, systemic IgG injection and plasmapheresis. function (see p. 405).

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 Chapter 5 — Hearing Loss 39

TABLE 5.8 PROGNOSTIC FACTORS IN SUDDEN SNHL 3. STRIAL OR METABOLIC. This is characterized by atro-
phy of stria vascularis in all turns of cochlea. In this, the
Good prognosis Bad prognosis
physical and chemical processes of energy production are
Mild loss Severe loss affected. It runs in families. Audiogram is flat but speech
Low and medium frequency High frequency loss discrimination is good.
loss
Recovery starting in 2 weeks Recovery does not start in 4. COCHLEAR CONDUCTIVE. This is due to stiffening of
2 weeks
the basilar membrane thus affecting its movements. Au-
No history of vertigo History of vertigo
Younger patients Older patients
diogram is sloping type.
Early treatment Late treatment Patients of presbycusis have great difficulty in hearing
in the presence of background noise though they may
hear well in quiet surroundings. They may complain of
speech being heard but not understood. Recruitment phe-
nomenon is positive and all the sounds suddenly become
7. Low-salt diet and a diuretic. It is empirical and has
intolerable when volume is raised. Tinnitus is another
same benefit as in cases of Ménière’s disease.
bothersome problem and in some it is the only complaint.
8. Intratympanic steroids therapy. It raises the lo-
Patients of presbycusis can be helped by a hearing aid.
cal concentration of steroids in cochlear fluids, thus
They should also have lessons in speech reading through
avoiding side effects of systemic therapy.
visual cues. Curtailment of smoking and stimulants like
Treatment tea and coffee may help to decrease tinnitus.
Many treatment protocols have been suggested for idi-
opathic sensorineural sudden hearing loss but none has NONORGANIC HEARING LOSS (NOHL)
shown significant benefit over the benefit of spontane-
ous recovery which occurs in 50–60% cases within first In this type of hearing loss, there is no organic lesion.
2 weeks. None of the drugs, dextran 40, vasodilators, car- It is either due to malingering or is psychogenic. In the
bogen inhalation (5% CO2 with 95% O2), diatrizoate me- former, usually there is a motive to claim some compen-
glumine, have shown significant benefit. sation for being exposed to industrial noises, head injury
Generally prescribed medicines include: or ototoxic medication. Patient may present with any of
the three clinical situations:
1. Steroids.
(i) Total hearing loss in both ears, (ii) total loss in only
2. Inhalation of carbogen.
one ear or (iii) exaggerated loss in one or both ears. The
3. Low-salt diet and a diuretic.
responsibility of the physician is to find out: Is the patient
4. Hyperbaric oxygen.
malingering? If so, what is his actual threshold of hear-
Prognosis ing? This is accomplished by:
Fortunately, about half the patients of idiopathic sensori- 1. HIGH INDEX OF SUSPICION. Suspicion further rises
neural hearing loss recover spontaneously within 15 days. when the patient makes exaggerated efforts to hear, fre-
Chances of recovery are poor after 1 month. Severe hear- quently making requests to repeat the question or placing
ing loss and that associated with vertigo have poor prog- a cupped hand to the ear.
nosis. Younger patients below 40 and those with moder-
ate losses have better prognosis (see Table 5.8). 2. INCONSISTENT RESULTS ON REPEAT PURE TONE AND
SPEECH AUDIOMETRY TESTS. Normally, the results of re-
G. PRESBYCUSIS peat tests are within ±5 dB. A variation greater than 15 dB
is diagnostic of NOHL.
Sensorineural hearing loss associated with physiological
aging process in the ear is called presbycusis. It usually 3. ABSENCE OF SHADOW CURVE. Normally, a shadow
manifests at the age of 65 years but may do so early if curve can be obtained while testing bone conduction, if
there is hereditary predisposition, chronic noise exposure the healthy ear is not masked. This is due to transcranial
or generalized vascular disease. transmission of sound to the healthy ear. Absence of this
Four pathological types of presbycusis have been iden- curve in a patient complaining of unilateral deafness is
tified. diagnostic of NOHL.

1. SENSORY. This is characterized by degeneration of the 4. INCONSISTENCY IN PTA AND SRT. Normally, pure
organ of Corti, starting at the basal coil and progressing tone average (PTA) of three speech frequencies (500, 1000
gradually to the apex. Higher frequencies are affected but and 2000 Hz) is within 10 dB of speech reception thresh-
speech discrimination remains good. old (SRT). An SRT better than PTA by more than 10 dB
points to NOHL.
2. NEURAL. This is characterized by degeneration of the
cells of spiral ganglion, starting at the basal coil and pro- 5. STENGER TEST. It can be done with a pair of identical
gressing to the apex. Neurons of higher auditory path- tuning forks or a double-channel audiometer. Principle in-
ways may also be affected. This manifests with high tone volved is that, if a tone of two intensities, one greater than
loss but speech discrimination is poor and out of propor- the other, is delivered to two ears simultaneously, only the
tion to the pure tone loss. ear which receives tone of greater intensity will hear it. To

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40 SECTION I — Diseases of Ear

do this test, take two tuning forks of equal frequency, strike

and keep them say 25 cm from each ear. Patient will claim
to hear it in the normal ear. Now bring the tuning fork
on the side of feigned deafness to within 8 cm, keeping
the tuning fork on the normal side at the same distance.
The patient will deny hearing anything even though tun-
ing fork on normal side is where it could be heard earlier. A
person with true deafness should continue to hear on the
normal side. Patient should be blindfolded during this test.
This same test can be performed with a two-channel
audiometer using pure tone or speech signals.

6. ACOUSTIC REFLEX THRESHOLD. Normally, stapedial

reflex is elicited at 70–100 dB SL. If patient claims total
deafness but the reflex can be elicited, it indicates NOHL.

7. ELECTRIC RESPONSE AUDIOMETRY (ERA). It is very

useful in NOHL and can establish hearing acuity of the
person to within 5–10 dB of actual thresholds.

SOCIAL AND LEGAL ASPECTS

OF HEARING LOSS
HEARING LOSS AND DEAFNESS
Hearing loss is impairment of hearing and its severity may
vary from mild to severe or profound, while the term
deafness is used, when there is little or no hearing at all. Figure 5.8. Classification of hearing loss. Ninety-five per cent of pop-
In some countries, this rigid differentiation is not made. ulation has thresholds between 210 and 110 dB HL.
They use the term deafness to denote any degree of hear-
ing loss irrespective of its severity. In 1980, WHO recom- of the thresholds of hearing for frequencies of 500, 1000
mended that the term “deaf” should be applied only to and 2000 Hz with reference to ISO: R. 389–1970 (Interna-
those individuals whose hearing impairment is so severe tional Calibration of Audiometers).
that they are unable to benefit from any type of amplifi-
cation. A similar definition is used in India while extend- Degree of hearing loss (Figure 5.8)
ing benefits to the hearing handicapped.
1. Mild 26–40 dB
2. Moderate 41–55 dB
DEFINITION OF DEAF
3. Moderately severe 56–70 dB
(Ministry of Social Welfare, Government of India— 4. Severe 71–91 dB
Scheme of Assistance to Hearing Handicap). 5. Profound More than 91 dB
“The deaf are those in whom the sense of hearing is 6. Total
nonfunctional for ordinary purposes of life.” They do not
hear/understand sounds at all even with amplified speech. From this it is implied that there is no apparent impair-
The cases included in the category will be those having ment of hearing from 0 to 25 dB.
hearing loss more than 90 dB in the better ear (profound The disability to understand speech with different de-
impairment) or total loss of hearing in both ears. grees of hearing loss is given in Table 5.9.
The partially hearing are defined as those falling un-
der any one of the following categories:
IMPAIRMENT, DISABILITY AND HANDICAP
Category Hearing acuity When a disease process strikes an organ or a system it
causes an impairment either in structure or function, but
Mild impairment More than 30 but not more than
this impairment may or may not become clinically mani-
45 dB in better ear
fested. When impairment affects the ability to perform
Serious impairment More than 45 but not more than
certain functions in the range considered normal for that
60 dB in better ear
individual it is called disability. The disability further re-
Severe impairment More than 60 but not more than
stricts the duties and roles expected from an individual by
90 dB in better ear
society and is called a handicap.
To exemplify, injury (disease) to the ear may result in
DEGREE OF HEARING LOSS hearing impairment which, depending on its severity,
will affect the individual’s ability to hear and perform
(WHO CLASSIFICATION)
certain activities (disability) and will be termed handicap
WHO (1980) recommended the following classification by the society:
on the basis of pure tone audiogram taking the average Disease → Impairment → Disability → Handicap.

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 Chapter 5 — Hearing Loss 41

TABLE 5.9 HEARING LOSS AND DIFFICULTY IN HEARING SPEECH

Hearing threshold in better ear Degree of impairment
(average of 500, 1000, 2000 Hz) (WHO classification) Ability to understand speech
0–25 Not significant No significant difficulty with faint speech
26–40 Mild Difficulty with faint speech
41–55 Moderate Frequent difficulty with normal speech
56–70 Moderately severe Frequent difficulty even with loud speech
71–90 Severe Can understand only shouted or amplified speech
91 above Profound Usually cannot understand even amplified speech

DEGREE OF HANDICAP In the above calculation only three speech frequencies

(500, 1000 and 2000 Hz) are taken into account but it is
Sometimes it is desired to express the impairment and felt that frequency of 3000 Hz is important for hearing
handicap in terms of percentage for the purposes of in the presence of noise and should also be taken into
compensation. Different countries and professional bod- account. American Academy of Ophthalmology and Oto-
ies have adopted their own system to calculate this per- laryngology recommends and takes into account the av-
centage. erage of four frequencies 500, 1000, 2000 and 3000 Hz
One of the methods to find hearing handicap is given when calculating the handicap.
below: Government of India reserved certain percentage of
(i) Take an audiogram and calculate the average of vacancies in Group C and D in favour of the physically
thresholds of hearing for frequencies of 500, 1000 handicapped and has extended certain other benefits.
and 2000 Hz say = A. It has also recommended the classification based on
(ii) Deduct from it 25 dB (as there is no impairment percentage of impairment and the test required to be
up to 25 dB), i.e. A − 25. performed (see Table 5.10). (Brochure on Reservations
(iii) Multiply it by 1.5, i.e. (A − 25) × 1.5. and Concessions for Physically Handicapped in Central
Govt. Services published by Ministry of Personnel, Pub-
This is the percentage of hearing impairment for that lic Grievances and Pensions, Dept. of Personnel and
ear. Similarly calculate the percentage of hearing impair- Training.)
ment for the other ear.
Total percentage handicap of an individual
UNILATERAL HEARING LOSS
(better ear% × 5) + worse ear%
= Unilateral loss of hearing, even though total, does not
6
produce a serious handicap or affect speech but it im-
Example: pairs localization of the sound source, difficulty in dis-
crimination of speech in the presence of background
500 Hz 1000 Hz 2000 Hz Average
noise and some difficulty at a meeting or in classroom
Right ear 60 75 90 75 dB when the speaker is on the side of affected ear. It should
Left ear 30 45 60 45 dB also alert the individual that he does not have a “spare
Impairment Right ear: 75 − 25 = 50; 50 × 1.5 = 75% or reserve ear” and has to take all precautions for the
Impairment Left ear: 45 − 25 = 20; 20 × 1.5 = 30% safety of the only hearing ear; also the surgeon should
be careful when he is called upon to operate on this only
(30 × 5) + 75 225 hearing ear. Bone-anchored hearing aids are the treat-
Total handicap = =
6 6 ment of choice for management of single-sided deafness
= 37.5% (see p. 137).
= 38% (rounded off)

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42 SECTION I — Diseases of Ear

TABLE 5.10 RECOMMENDED CATEGORIZATION AND PERCENTAGE OF HEARING IMPAIRMENT (DEPT. OF

PERSONNEL, GOVT. OF INDIA). RECOMMENDATIONS ABOUT THE CATEGORIES AND THE TESTS REQUIRED
I. Recommended classification
Percentage of
S. no. Category Type of impairment dB level and/or Speech discrimination impairment
1. I. Mild hearing impairment 26–40 dB in better ear 80–100% in better ear Less than
40%
2. II. Moderate hearing impairment 41–55 dB in better ear 50–80% in better ear 40–50%
3. III. Severe hearing impairment 56–70 dB hearing 40–50% 50–75%
impairment in better ear
4. IV. (a) Total deafness No hearing No discrimination 100%
(b) Near total deafness 91 dB and above in better -do- 100%
ear
(c) Profound hearing 71 to 90 dB Less than 40% in better 75–100%
impairment ear
(Pure tone average of hearing in 500, 1000 and 2000 Hz by air conduction should be taken as basis for consideration as per the test
recommendations.)
Further it should be noted that:
(a) When there is only an island of hearing present in one or two frequencies in better ear, it should be considered as total loss of
hearing.
(b) Wherever there is no response (NR) at any of the 3 frequencies (500, 100, 2000 Hz), it should be considered as equivalent to 130 dB
loss for the purposes of classification of disability and in arriving at the average. This is based on the fact that maximum intensity
limits in most of the audiometers is 110 dB and some audiometers have additional facilities for 20 dB for testing.
II. Recommendations about the categories of disability (Hearing impairment-Physical aspect only-Test recommended).
(a) Pure tone audiometry (ISO R 389–1970 at present, is being used as Audiometric Standard in most of the audiometers. Hence the
audiometers used in testing should be accordingly calibrated). Three frequency average at 500, 1000 and 2000 Hz by Air Conduction
(AC), will be used for categorization.
(b) Wherever possible the pure tone audiometric results should be supplemented by the speech discrimination score—tested at sensation
level (SL), i.e. the speech discriminations test is conducted at 30–40 dBa the patient’s hearing threshold. The stimuli used be either
phonetically balance words (PB) of the particular language or its equivalent material. At present only a few Indian languages have
standard speech material for testing. Hence wherever the standardized test material is not available, either standardized Indian English
Test could be made use of with English knowing population or equivalent material to PB be used.
(c) Wherever children are tested and pure tone audiometry is not possible, free field testing should be employed.
Suggestions of the facilities to be offered to the disabled for rehabilitation.
Category I No special benefits.
Category II Considered for Hearing Aids at free or concessional costs only.
Category III Hearing aids, free of cost or at concessional rates. Job reservation—benefit of special Employment Exchange. Scholarships
at School. Single language formula.
Category IV Hearing Aids—facilities of reservation-special employment exchange. Special facilities in schools like scholarships.
Hearing aids—exemption from 3 language formula (to study in recommended single language).
It is felt that for consideration of admission under special category for courses conducted by institutions like Indian Institute of Technology
(IIT), Industrial Training Institute (ITI) and others, categories I and II only should be considered for reservation of seats, provided they
fulfill the other educational stipulations for the course.
We have considered the different types of hearing affection, i.e. conductive versus sensorineural, and agree that the disability will
be judged by the conditions prevalent in the patient at the time of referral and examination. In case of failure of surgery or other
therapeutic interventions, the patient will be considered and categorized on the basis of the recommended tests.
aLeft blank is the original recommendations; has been added by the author.

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 Chapter 6
Assessment of Vestibular Functions

Assessment of vestibular functions can be divided into B. FISTULA TEST

two groups:
The basis of this test is to induce nystagmus by pro-
1. Clinical tests ducing pressure changes in the external canal which
2. Laboratory tests are then transmitted to the labyrinth. Stimulation of
labyrinth results in nystagmus and vertigo. The test is
performed by applying intermittent pressure on the tra-
I. CLINICAL TESTS OF VESTIBULAR gus or by using Siegel’s speculum. Normally, the test is
FUNCTION negative because the pressure changes in the external
auditory canal cannot be transmitted to the labyrinth.
A. SPONTANEOUS NYSTAGMUS It is positive when there is erosion of horizontal semi-
circular canal as in cholesteatoma or a surgically created
Nystagmus is an important sign in the evaluation of
window in the horizontal canal (fenestration opera-
vestibular system. It is defined as involuntary, rhythmi-
tion), abnormal opening in the oval window (poststa-
cal, oscillatory movement of eyes. It may be horizontal,
pedectomy fistula) or the round window (rupture of
vertical or rotatory. Vestibular nystagmus has a slow and
round window membrane). A positive fistula also im-
a fast component, and by convention, the direction of
plies that the labyrinth is still functioning; it is absent
nystagmus is indicated by the direction of the fast com-
when labyrinth is dead. A false negative fistula test is also
ponent. Intensity of nystagmus is indicated by its degree
seen when cholesteatoma covers the site of fistula and
(Table 6.1).
does not allow pressure changes to be transmitted to the
To elicit nystagmus, patient is seated in front of the ex-
labyrinth.
aminer or lies supine on the bed. The examiner keeps his
A false positive fistula test (i.e. positive fistula test with-
finger about 30 cm from the patient’s eye in the central
out the presence of a fistula) is seen in congenital syphilis
position and moves it to the right or left, up or down, but
and in about 25% cases of Ménière’s disease (Hennebert’s
not moving at any time more than 30° from the central
sign). In congenital syphilis, stapes footplate is hyper-
position to avoid gaze nystagmus. Presence of spontane-
mobile while in Ménière’s disease it is due to the fibrous
ous nystagmus always indicates an organic lesion.
bands connecting utricular macula to the stapes footplate.
Vestibular nystagmus is called peripheral, when it is due
In both these conditions, movements of stapes result in
to lesion of labyrinth or VIIIth nerve and central, when
stimulation of the utricular macula.
lesion is in the central neural pathways (vestibular nuclei,
brainstem, cerebellum).
Irritative lesions of the labyrinth (serous labyrinthi- C. ROMBERG TEST
tis) cause nystagmus to the side of lesion. Paretic lesions
(purulent labyrinthitis, trauma to labyrinth, section of The patient is asked to stand with feet together and arms
VIIIth nerve) cause nystagmus to the healthy side. Nystag- by the side with eyes first open and then closed. With
mus of peripheral origin can be suppressed by optic fixa- the eyes open, patient can still compensate the imbal-
tion by looking at a fixed point, and enhanced in darkness ance but with eyes closed, vestibular system is at more
or by the use of Frenzel glasses (+20 dioptre glasses) both disadvantage. In peripheral vestibular lesions, the pa-
of which abolish optic fixation. tient sways to the side of lesion. In central vestibular
Nystagmus of central origin cannot be suppressed disorder, patient shows instability. If patient can per-
by optic fixation. Purely torsional nystagmus indicates le- form this test without sway, “sharpened Romberg test”
sion of the brainstem/vestibular nuclei and is seen in is performed. In this the patient stands with one heel in
syringomyelia. Vertical downbeat nystagmus indicates lesion front of toes and arms folded across the chest. Inability
at craniocervical region such as Arnold–Chiari malforma- to perform the sharpened Romberg test indicates vestib-
tion or degenerative lesion of the cerebellum. Vertical up- ular impairment.
beat nystagmus is seen in lesions at the junction of pons
and medulla or pons and midbrain. Pendular nystagmus is
D. GAIT
either congenital or acquired. The latter is seen in multi-
ple sclerosis. Pendular nystagmus may also be disconju- The patient is asked to walk along a straight line to a fixed
gate, i.e. vertical in one eye and horizontal in the other. point, first with eyes open and then closed. In case of un-
Table 6.2 shows differences in the nystagmus of peripheral compensated lesion of peripheral vestibular system, with
and central lesions. eyes closed, the patient deviates to the affected side.

43

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44 SECTION I — Diseases of Ear

TABLE 6.1 DEGREE OF NYSTAGMUSa

1st degree It is weak nystagmus and is present when
patient looks in the direction of fast
component.
2nd degree It is stronger than the 1st degree nystagmus
and is present when patient looks straight
ahead.
3rd degree It is stronger than the 2nd degree nystagmus
and is present even when patient looks in the
direction of the slow component.
aThese degrees are according to Alexander’s law and may not hold true in
case of nystagmus of central origin.

TABLE 6.2 POSITIONAL NYSTAGMUS IN

PERIPHERAL AND CENTRAL LESIONS
OF VESTIBULAR SYSTEM. POSITIONAL
NYSTAGMUS IS ELICITED BY HALLPIKE
MANOEUVRE (VIDE INFRA)
Peripheral Central
Latency 2–20 s No latency
Duration Less than 1 min More than 1 min
Direction of Direction fixed, Direction changing
nystagmus towards the
undermost ear
Fatiguability Fatiguable Nonfatiguable
Accompanying Severe vertigo None or slight
symptoms
Figure 6.1. Dix-Hallpike manoeuvre.

E. PAST-POINTING AND FALLING In central lesions (tumours of IVth ventricle, cerebel-

lum, temporal lobe, multiple sclerosis, vertebrobasilar
The past-pointing, falling and the slow component of insufficiency or raised intracranial tension) nystagmus is
nystagmus are all in the same direction. If there is acute produced immediately, as soon as the head is in critical
vestibular failure, say on the right side, nystagmus is to position without any latency and lasts as long as head is
the left but the past-pointing and falling will be towards in that critical position. Direction of nystagmus also var-
the right, i.e. towards side of the slow component. ies in different test positions (direction changing) and is
nonfatiguable on repetition of test (Table 6.2).
F. DIX-HALLPIKE MANOEUVRE (POSITIONAL TEST)
G. TEST OF CEREBELLAR DYSFUNCTION
This test is particularly useful when patient complains of
vertigo in certain head positions. It also helps to differen- All cases of giddiness should be tested for cerebellar disor-
tiate a peripheral from a central lesion. ders. Disease of the cerebellar hemisphere causes:

Method 1. Asynergia (abnormal finger-nose test)

2. Dysmetria (inability to control range of motion)
Patient sits on a couch. Examiner holds the patient’s head,
3. Adiadochokinesia (inability to perform rapid alternat-
turns it 45° to the right and then places the patient in a
ing movements)
supine position so that his head hangs 30° below the hori-
4. Rebound phenomenon (inability to control move-
zontal (Figure 6.1). Patient’s eyes are observed for nystag-
ment of extremity when opposing forceful restraint is
mus. The test is repeated with head turned to left and then
suddenly released)
again in straight head-hanging position. Four parameters
of nystagmus are observed: latency, duration, direction and Midline disease of cerebellum causes:
fatiguability (see Table 6.2). In benign paroxysmal posi-
1. Wide base gait
tional vertigo, nystagmus appears after a latent period of
2. Falling in any direction
2–20 s, lasts for less than a minute and is always in one
3. Inability to make sudden turns while walking
direction, i.e. towards the ear that is undermost. On rep-
4. Truncal ataxia
etition of the test, nystagmus may still be elicited but lasts
for a shorter period. On subsequent repetitions it disap- Nystagmus observed in midline or hemispheral disorders
pears altogether, i.e. nystagmus is fatiguable. Patient also of cerebellum includes gaze evoked nystagmus, rebound
complains of vertigo when the head is in critical position. nystagmus and abnormal optokinetic nystagmus.

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 Chapter 6 — Assessment of Vestibular Functions 45

and at 44 °C (i.e. 7° below and above normal body

II. LABORATORY TESTS OF VESTIBULAR
temperature) and eyes observed for appearance of nys-
FUNCTION tagmus till its end point. Time taken from the start of
A. CALORIC TEST irrigation to the end point of nystagmus is recorded and
charted on a calorigram (Figure 6.3). If no nystagmus is
The basis of this test is to induce nystagmus by thermal elicited from any ear, test is repeated with water at 20 °C
stimulation of the vestibular system. Advantage of the for 4 min before labelling the labyrinth dead. A gap of
test is that each labyrinth can be tested separately. Patient 5 min should be allowed between two ears. Cold water in-
is also asked whether vertigo induced by the caloric test is duces nystagmus to opposite side and warm water to the
qualitatively similar to the type experienced by him dur- same side (remember mnemonic COWS: cold–opposite,
ing the episode of vertigo. If yes, it proves labyrinthine warm–same). Depending on response to the caloric test,
origin of vertigo. we can find canal paresis or dead labyrinth, directional
preponderance, i.e. nystagmus is more in one particular
1. MODIFIED KOBRAK TEST. It is a quick office proce- direction than in the other, or both canal paresis and di-
dure. Patient is seated with head tilted 60° backwards to rectional preponderance.
place horizontal canal in vertical position. Ear is irrigated (a) CANAL PARESIS. It indicates that response (meas-
with ice water for 60 s, first with 5 mL and if there is no ured as duration of nystagmus) elicited from a particular
response, 10, 20 and 40 mL. Normally, nystagmus beating canal (labyrinth), right or left, after stimulation with cold
towards the opposite ear will be seen with 5 mL of ice wa- and warm water is less than that from the opposite side. It
ter. If response is seen with increased quantities of water can also be expressed as percentage of the total response
between 5 and 40 mL, labyrinth is considered hypoactive. from both ears.
No response to 40mL of water indicates dead labyrinth. L 30 + L 44 × 100
Response from the left ear =
L 30 + L 44 + R 30 + R 44
2. FITZGERALD–HALLPIKE TEST (BITHERMAL CALORIC
TEST). In this test, patient lies supine with head tilted 30°
forward so that horizontal canal is vertical (Figure 6.2).
R 30 + R 44 × 100
Ears are irrigated for 40 s alternately with water at 30 °C Response from the right ear =
L 30 + L 44 + R 30 + R 44

Where l30 is the response from left side with water at 30 °C

and l44 is response from left ear after stimulation with
warm water at 44 °C. Less or no response from a particular
side is indicative of depressed function of the ipsilateral
labyrinth, vestibular nerve or vestibular nuclei and is seen
in Ménière’s disease, acoustic neuroma, postlabyrinthec-
tomy or vestibular nerve section.
(b) DIRECTIONAL PREPONDERANCE. It takes into con-
sideration the duration of nystagmus to the right or left
irrespective of whether it is elicited from the right or
left labyrinth. We know that right beating nystagmus

Figure 6.2. Fitzgerald–Hallpike test. (A) Patient is in supine position

and head raised by 30° to make horizontal canal vertical. (B) Position
of canal and the direction of flow of endolymph. Figure 6.3. Calorigram.

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46 SECTION I — Diseases of Ear

is caused by L30 and R44 and left beating nystagmus is to perform the caloric test. Disadvantage of the test is that
caused by R30 and L44. Therefore, both the labyrinths are simultaneously stimulated during
the rotation process and cannot be tested individually.
L 30 + R 44 The test has now been made more sophisticated by the
Right beating nystagmus = × 100
L 30 + L 44 + R 30 + R 44 use of torsion swings, electronystagmography and com-
puter analysis of the results.
R 30 + L 44
Left beating nystagmus = × 100
L 30 + L 44 + R 30 + R 44 E. GALVANIC TEST
It is the only vestibular test which helps in differentiating
If the nystagmus is 25–30% or more on one side than
an end organ lesion from that of vestibular nerve. Patient
the other, it is called directional preponderance to that
stands with his feet together, eyes closed and arms out-
side.
stretched and then a current of 1 mA is passed to one
It is believed that directional preponderance occurs to-
ear. Normally, person sways towards the side of anodal
wards the side of a central lesion, away from the side in a
current. Body sway can be studied by a special platform.
peripheral lesion; however, it does not help to localize the
lesion in central vestibular pathways.
Canal paresis and directional preponderance can also F. POSTUROGRAPHY
be seen together. It is a method to evaluate vestibular function by measur-
Canal paresis on one side with directional preponder- ing postural stability and is based on the fact that mainte-
ance to the opposite side is seen in unilateral Ménière’s nance of posture depends on three sensory inputs—visual,
disease while canal paresis with directional preponder- vestibular and somatosensory. It uses either a fixed or a
ance to ipsilateral side is seen in acoustic neuroma. moving platform. Visual cues can also be varied. The clini-
cal application of posturography is still under investigation.
3. COLD-AIR CALORIC TEST. This test is done when there
is perforation of tympanic membrane because irrigation
with water in such a case with perforation is contraindi- G. VESTIBULAR EVOKED MYOGENIC
cated. The test employs Dundas Grant tube, which is a POTENTIALS (VEMP)
coiled copper tube wrapped in cloth. The air in the tube This is a test to study function of otolith organs—the sac-
is cooled by pouring ethyl chloride and then blown into cule and utricle. Normally their function is linear accel-
the ear. It is only a rough qualitative test. eration. They can also be stimulated by loud sound of air
or bone conduction. Even tapping the head can stimulate
them. Myogenic potentials can be picked up from either
B. ELECTRONYSTAGMOGRAPHY
the sternocleidomastoid (cervical) muscle or ocular mus-
It is a method of detecting and recording of nystagmus, cle (inferior oblique or superior rectus) and have respec-
which is spontaneous or induced by caloric, positional, tively been called cVEMP and oVEMP.
rotational or optokinetic stimulus. The test depends on Since saccule is supplied by the inferior division of
the presence of corneoretinal potentials which are record- nerve and utricle by the superior division, study of VEMP
ed by placing electrodes at suitable places round the eyes. in neuroma can help us to find its origin from the supe-
The test is also useful to detect nystagmus, which is not rior or inferior division.
seen with the naked eye. It also permits to keep a perma- Reflex arc is:
nent record of nystagmus.
From saccule—inferior vestibular—vestibular nuclei—
ipsilateral vestibular spinal tract—spinal accessory
C. OPTOKINETIC TEST nerve (CN XI)—sternocleidomastoid
From utricle—superior vestibular nerve—vestibular
Patient is asked to follow a series of vertical stripes on a
nuclei—medial longitudinal fasciculus—oculomotor
drum moving first from right to left and then from left
(CNIII) nerve—inferior oblique muscle
to right. Normally it produces nystagmus with slow
component in the direction of moving stripes and fast Air-conducted sounds primarily activate the saccule,
component in the opposite direction. Optokinetic abnor- while bone-conducted sounds activate both the saccule
malities are seen in brainstem and cerebral hemisphere and the utricle.
lesions. Thus this test is useful to diagnose a central lesion. VEMP study is being used clinically and the equipment
is also available but needs further research. VEMP is be-
ing used to find the origin of an acoustic neurons (from
D. ROTATION TEST
superior or inferior vestibular nerve). Ménière’s disease,
Patient is seated in Barany’s revolving chair with his head superior canal dehiscence, vestibular neuritis and locali-
tilted 30° forward and then rotated 10 turns in 20 s. The sations of lesions of posterior cranial fossa, i.e. from the
chair is stopped abruptly and nystagmus observed. Nor- upper or lower brainstem. Vestibulo-ocular reflex is medi-
mally there is nystagmus for 25–40 s. The test is useful as ated through upper brainstem, while vestibulospinal arc
it can be performed in cases of congenital abnormalities is through the lower brainstem
where ear canal has failed to develop and it is not possible VEMP studies are still in investigative state.

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 Chapter 7
Disorders of Vestibular System

Disorders of vestibular system cause vertigo and are di- period of rest. Thus, typical history and Hallpike manoeu-
vided into: vre establishes the diagnosis.
1. Peripheral, which involve vestibular end organs The condition can be treated by performing Epley’s ma-
and their 1st order neurons (i.e. the vestibular nerve). The noeuvre. The principle of this manoeuvre is to reposition
cause lies in the internal ear or the VIIIth nerve. They are the otoconial debris from the posterior semicircular canal
responsible for 85% of all cases of vertigo. back into the utricle. The doctor stands behind the patient
2. Central, which involve central nervous system after and the assistant on the side. The patient is made to sit on
the entrance of vestibular nerve in the brainstem and in- the table so that when he is made to lie down, his head
volve vestibulo-ocular, vestibulospinal and other central is beyond the edge of the table as is done in Dix-Hallpike
nervous system pathways. manoeuvre. His face is turned 45° to the affected side.
Table 7.1 lists the common causes of vertigo of periph- The manoeuvre consists of five positions (Figure 7.1):
eral and central origin.
• Position 1. With the head turned 45°, the patient is
made to lie down in head-hanging position (Dix-Hall-
pike manoeuvre). It will cause vertigo and nystagmus.
I. PERIPHERAL VESTIBULAR DISORDERS Wait till vertigo and nystagmus subside.
• Position 2. Head is now turned so that affected ear is
1. MÉNIÈRE’S DISEASE (ENDOLYMPHATIC HYDROPS). It
facing up at a 90° rotation.
is characterized by vertigo, fluctuating hearing loss, tin-
• Position 3. The whole body and head are now rotated
nitus and sense of pressure in the involved ear. Vertigo is
away from the affected ear to a lateral recumbent posi-
of sudden onset, lasts for a few minutes to 24 h or so. (The
tion in a 90°-rotation face-down position.
disease has been discussed on p. 111).
• Position 4. Patient is now brought to a sitting position
with head still turned to the unaffected side by 45°.
2. BENIGN PAROXYSMAL POSITIONAL VERTIGO (BPPV).
• Position 5. The head is now turned forward and chin
It is characterized by vertigo when the head is placed in a
brought down 20°.
certain critical position. There is no hearing loss or other
neurologic symptoms. Positional testing establishes the There should be a pause at each position till there is
diagnosis and helps to differentiate it from positional no nystagmus or there is slowing of nystagmus, before
vertigo of central origin (Table 7.1). Disease is caused by changing to the next position. After manoeuvre is com-
a disorder of posterior semicircular canal though many plete, patient should maintain an upright posture for
patients have history of head trauma and ear infection. 48 h. Eighty per cent of the patients will be cured by a
It has been demonstrated that otoconial debris, con-
& single manoeuvre. If the patient remains symptomatic,
sisting of crystals of calcium carbonate, is released from the manoeuvre can be repeated. A bone vibrator placed
the degenerating macula of the utricle and floats freely in on the mastoid bone helps to loosen the debris.
the endolymph. When it settles on the cupula of poste-
rior semicircular canal in a critical head position, it causes 3. VESTIBULAR NEURONITIS. It is characterized by severe
displacement of the cupula and vertigo. The vertigo is fa- vertigo of sudden onset with no cochlear symptoms.
tiguable on assuming the same position repeatedly due to Attacks may last from a few days to 2 or 3 weeks. It is
dispersal of the otoconia but can be induced again after a thought to occur due to a virus that attacks vestibular

TABLE 7.1 VESTIBULAR DISORDERS

Peripheral (Lesions of end organs vestibular nerve) Central (Lesions of brainstem and central connections)
• Ménière’s disease • Vertebrobasilar insufficiency
• Benign paroxysmal positional vertigo • Posterior inferior cerebellar artery syndrome
• Vestibular neuronitis • Basilar migraine
• Labyrinthitis • Cerebellar disease
• Vestibulotoxic drugs • Multiple sclerosis
• Head trauma • Tumours of brainstem and fourth ventricle
• Perilymph fistula • Epilepsy
• Syphilis • Cervical vertigo
• Acoustic neuroma

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48 SECTION I — Diseases of Ear

Figure 7.1. Epley’s manoeuvre for BPPV of posterior canal showing position of patient and corresponding position of otolith debris in the pos-
terior canal. (A) Patient sitting facing forward. (B) Patient lying down in Dix-Hallpike position with head hanging and turned 45˚ to right (the
affected ear). (C) Head turned to left Dix-Hallpike position with affected ear up. (D) Head and body both turned as a unit to unaffected side so
that face is turned to the ground. (E) Patient is made to sit with head bent forward by 20˚.

ganglion. Management of acute attack is similar to that those of the maculae. Certain other drugs which cause
in Ménière’s disease. The disease is usually self-limiting. dizziness or unsteadiness are antihypertensives, labyrin-
thine sedatives, oestrogen preparations, diuretics, antimi-
4. LABYRINTHITIS. It has been discussed in detail on p. 88. crobials (nalidixic acid, metronidazole) and antimalarials.
• Circumscribed labyrinthitis is seen in cases of unsafe However, their mode of action may be different.
type of chronic suppurative otitis media (CSOM) and
fistula test is positive. 6. HEAD TRAUMA. Head injury may cause concussion of
• Serous labyrinthitis is caused by trauma or infection labyrinth, completely disrupt the bony labyrinth or VIIIth
(viral or bacterial) adjacent to inner ear but without nerve, or cause a perilymph fistula. Severe acoustic trau-
actual invasion. There is severe vertigo and sensorineu- ma, such as that caused by an explosion, can also disturb
ral hearing loss. A partial or full recovery of inner ear the vestibular end organ (otoliths) and result in vertigo.
functions is possible if treated early.
• Purulent labyrinthitis is a complication of CSOM. There 7. PERILYMPH FISTULA. In this condition, perilymph
is actual bacterial invasion of inner ear with total loss leaks into the middle ear through the oval or round win-
of cochlear and vestibular functions. Vertigo in this dow. It can follow as a complication of stapedectomy, or
condition is due to acute vestibular failure. There is ear surgery when stapes is accidentally dislocated. It can
severe nausea and vomiting. Nystagmus is seen to also result from sudden pressure changes in the middle
the opposite side due to destruction of the affected ear (e.g. barotrauma, diving, forceful Valsalva) or raised
labyrinth. intracranial pressure (weightlifting or vigorous cough-
ing). A perilymph fistula causes intermittent vertigo and
5. VESTIBULOTOXIC DRUGS. Several drugs cause ototoxic- fluctuating sensorineural hearing loss, sometimes with
ity by damaging the hair cells of the inner ear. Some pri- tinnitus and sense of fullness in the ear (compare Mé-
marily affect the cochlear while others affect the vestibular nière’s disease).
labyrinth. Aminoglycoside antibiotics particularly strep-
tomycin, gentamicin and kanamycin have been shown to 8. SYPHILIS. Syphilis of inner ear, both acquired and
affect hair cells of the crista ampullaris and to some extent congenital, causes dizziness in addition to sensorineural

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 Chapter 7 — Disorders of Vestibular System 49

hearing loss. Late congenital syphilis usually manifesting 2. POSTEROINFERIOR CEREBELLAR ARTERY SYNDROME
between 8 and 20 years, mimics Ménière’s disease with (WALLENBERG SYNDROME). Thrombosis of the posterior
episodes of acute vertigo, sensorineural hearing loss and inferior cerebellar artery cuts off blood supply to lateral
tinnitus. Hennebert’s sign, i.e. a positive fistula test in the medullary area. There is violent vertigo along with diplo-
presence of an intact tympanic membrane, is present in pia, dysphagia, hoarseness, Horner syndrome, sensory
congenital syphilis. Neurosyphilis (tertiary acquired) can loss on ipsilateral side of face and contralateral side of
cause central type of vestibular dysfunction. the body, and ataxia. There may be horizontal or rotatory
nystagmus to the side of the lesion (Figure 7.2).
9. ACOUSTIC NEUROMA. It has been classified in periph-
eral vestibular disorders as it arises from CN VIII within 3. BASILAR MIGRAINE. Migraine is a vascular syndrome
internal acoustic meatus. It causes only unsteadiness or producing recurrent headaches with symptom-free inter-
vague sensation of motion. Severe episodic vertigo, as vals. Headache is usually unilateral and of the throbbing
seen in the end organ disease, is usually missing (for de- type. Basilar artery migraine produces occipital headache,
tails refer Chapter 18). visual disturbances, diplopia and severe vertigo which
Other tumours of temporal bone (e.g. glomus tumour, is abrupt and may last for 5–60 min. Basilar migraine is
carcinoma of external or middle ear and secondaries), de- common in adolescent girls with strong menstrual rela-
stroy the labyrinth directly and cause vertigo. tionship and positive family history.

4. CEREBELLAR DISEASE. Cerebellum may be affected

II. CENTRAL VESTIBULAR DISORDERS by haemorrhage (hypertension), infarction (occlusion of
arterial supply), infection (otogenic cerebellar abscess)
1. VERTEBROBASILAR INSUFFICIENCY. It is a common or tumours (glioma, teratoma or haemangioma). Acute
cause of central vertigo in patients over the age of 50 years. cerebellar disease may cause severe vertigo, vomiting and
There is transient decrease in cerebral blood flow. Com- ataxia simulating an acute peripheral labyrinthine dis-
mon cause is atherosclerosis. Ischaemia in these patients order. Tumours are slow growing and produce classical
may also be precipitated by hypotension or neck move- features of cerebellar disease, i.e. incoordination, past-
ments when cervical osteophytes press on the vertebral pointing, adiadochokinesia, rebound phenomenon and
arteries during rotation and extension of head. wide-based gait.
Vertigo is abrupt in onset, lasts several minutes and is
associated with nausea and vomiting. Other neurological 5. MULTIPLE SCLEROSIS. It is a demyelinating disease
symptoms like visual disturbances, drop attacks, diplopia, affecting young adults. Vertigo and dizziness are com-
hemianopia, dysphagia and hemiparesis resulting from mon complaints. There are other multiple neurological
ischaemia to other areas of brain may also accompany signs and symptoms, e.g. blurring or loss of vision, di-
vertigo. plopia, dysarthria, paraesthesia and ataxia. Spontaneous
Some patients only complain of intermittent attacks nystagmus may be seen. Acquired pendular nystagmus,
of dizziness or vertigo on lateral rotation and extension dissociated nystagmus and vertical upbeat nystagmus are
of head. important features in diagnosis.

Figure 7.2. Lateral medullary syndrome.

• Inferior cerebellar peduncle Vertigo, nausea, vomiting and nystagmus
• Spinocerebellar tracts Ataxia
• Nucleus ambiguus (CN X, IX) Hoarseness and dysphagia
• Descending sympathetic tract Horner’s syndrome
• Uncrossed fibres of spinothalamic tract Loss of pain and temperature on ipsilateral face
• Descending nucleus and tract of CN V Pain and numbness over ipsilateral face
• Contralateral spinothalamic tract (crossed fibres) Contralateral loss of pain and temperature of arm, trunk and leg

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50 SECTION I — Diseases of Ear

6. TUMOURS OF BRAINSTEM AND FLOOR OF IVTH VEN- cervical lordosis. Exact mechanism of cervical vertigo is
TRICLE. Gliomas and astrocytomas may arise from pons not known. It may be due to disturbed vertebrobasilar cir-
and midbrain; medulloblastoma, ependymomas, epider- culation, involvement of sympathetic vertebral plexus or
moid cysts or teratomas may arise from floor of IVth ven- alteration of tonic neck reflexes.
tricle. These tumours cause other neurological signs and
symptoms in addition to vertigo and dizziness. Positional
vertigo and nystagmus may also be the presenting fea- OTHER CAUSES OF VERTIGO
tures. CT scan and magnetic resonance imaging are useful
in their diagnosis. 1. OCULAR VERTIGO. Normally, balance is maintained by
integrated information received from the eyes, labyrinths
7. EPILEPSY. Vertigo may occur as an aura in tempo- and somatosensory system. A mismatch of information
ral lobe epilepsy. The history of seizure and/or uncon- from any of these organs causes vertigo and in this case
sciousness following the aura may help in the diagnosis. from the eyes. Ocular vertigo may occur in case of acute
Sometimes, vertigo is the only symptom of epilepsy and extraocular muscle paresis or high errors of refraction.
that may pose a difficult diagnostic problem. Electro-
encephalography may show abnormalities during the 2. PSYCHOGENIC VERTIGO. This diagnosis is suspected
attack. in patients suffering from emotional tension and anxi-
ety. Often other symptoms of neurosis, e.g. palpitation,
8. CERVICAL VERTIGO. Vertigo may follow injuries of breathlessness, fatigue, insomnia, profuse sweating and
neck 7–10 days after the accident. It is usually provoked tremors are also present. Symptom of vertigo is often
with movements of neck to the side of injury. Examina- vague in the form of floating or swimming sensation or
tion shows tenderness of neck, spasms of cervical muscles light headedness. There is no nystagmus or hearing loss.
and limitation of neck movements. X-rays show loss of Caloric test shows an exaggerated response.

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 Chapter 8
Diseases of External Ear

I. DISEASES OF THE PINNA 5. CUP EAR OR LOP EAR. It is hypoplasia of upper third
of the auricle. Upper portion of helix or pinna is cupped.
The pinna may be afflicted by congenital, traumatic, in- Cockle-shell ear or snail-shell ear are greater deformities
flammatory or neoplastic disorders. of cup ear.

A. CONGENITAL DISORDERS 6. CRYPTOTIA (SYN. POCKET EAR). Upper third of the

auricle is embedded under the scalp skin. It can be cor-
The developmental abnormalities of the pinna may be rected by mobilizing the pinna to normal position and
just minor variations from the normal or major abnormali- covering the raw area by a skin graft.
ties.
7. COLOBOMA. There is a transverse cleft in the pinna in
1. ANOTIA. It is complete absence of pinna and lob- the middle.
ule, and usually forms part of the first arch syndrome
(Figure 8.1). 8. MINOR DEFORMITIES. Absence of tragus, Darwin’s tu-
bercle, additional folds (Stahl’s ear), and Satyr ear.
2. MICROTIA (FIGURE 8.2). It is a major developmental
anomaly. Degree of microtia may vary. It is frequently as- • Darwin’s tubercle is a pointed tubercle on the upper
sociated with anomalies of external auditory canal, mid- part of helix and represents apex of pinna of lower ani-
dle and internal ear. The condition may be unilateral or mals.
bilateral. Hearing loss is frequent. Peanut ear is a form of • In Stahl’s ear, helix which should normally be folded
microtia. is flat and the upper crus of antihelix is duplicated and
reaches rim of helix. It can be corrected by a mould in
3. MACROTIA. It is excessively large pinna. the first 6 weeks of life.

4. BAT EAR (SYN. PROMINENT EAR OR PROTRUDING 9. DEFORMITIES OF EAR LOBULE. They are absence of lob-
EAR). This is an abnormally protruding ear. The concha ule, large lobule, bifid lobule or a pixed (attached) lobule.
is large with poorly developed antihelix and scapha. The
deformity can be corrected surgically any time after the 10. PREAURICULAR TAGS OR APPENDAGES. They are
age of 6 years, if cosmetic appearance so demands. skin-covered tags that appear on a line drawn from the
tragus to the angle of mouth. They may contain small
pieces of cartilage (Figure 8.3).

11. PREAURICULAR PIT OR SINUS. Preauricular pit is a de-

pression in front of the crus of helix or above the tragus.

Figure 8.1. Anotia. Note total absence of pinna and external audi-
tory canal on the left side. Figure 8.2. Microtia right ear (peanut ear).

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52 SECTION I — Diseases of Ear

Figure 8.3. Preauricular appendages. Figure 8.5. Cauliflower ear (pugilistic or boxer’s ear).

Figure 8.4. Infected preauricular sinus with pus exuding from the Figure 8.6. Laceration left pinna.
opening.

absorbable sutures. Special care is taken to prevent strip-

Preauricular sinus is an epithelial track and is due to in- ping of perichondrium from cartilage for fear of avascular
complete fusion of tubercles. It may get repeatedly in- necrosis. Skin is closed with fine nonabsorbable sutures.
fected causing purulent discharge. Abscess may also form. Broad-spectrum antibiotics are given for 1 week.
Treatment is surgical excision of the track if the sinus gets
repeatedly infected (Figure 8.4). 3. AVULSION OF PINNA. When pinna is still attached to
the head by a small pedicle of skin, primary reattachment
B. TRAUMA TO THE AURICLE should be considered and it is usually successful. Com-
pletely avulsed pinna can be reimplanted in selected cases
1. HAEMATOMA OF THE AURICLE. It is collection of by the microvascular techniques; in others, the skin of
blood between the auricular cartilage and its perichon- the avulsed segment of pinna is removed and the carti-
drium. Often it is the result of blunt trauma seen in lage implanted under the postauricular skin for later re-
boxers, wrestlers and rugby players. Extravasated blood construction.
may clot and then organize, resulting in a typical de-
formity called Cauliflower ear (pugilistic or boxer’s ear)
4. FROSTBITE. Injury due to frostbite varies between er-
(Figure 8.5). If haematoma gets infected, severe peri-
ythema and oedema, bullae formation, necrosis of skin
chondritis may set in.
and subcutaneous tissue, and complete necrosis with loss
Treatment is aspiration of the haematoma under strict
of the affected part.
aseptic precautions and a pressure dressing, carefully
Treatment of a frostbitten ear consists of:
packing all concavities of the auricle to prevent reaccu-
mulation. Aspiration may need to be repeated. When as- (a) rewarming with moist cotton pledgets at a tempera-
piration fails, incision and drainage should be done and ture of 38–42 °C,
pressure applied by dental rolls tied with through and (b) application of 0.5% silver nitrate soaks for superficial
through sutures. All cases should receive prophylactic an- infection,
tibiotics. (c) analgesics for pain; rapid rewarming of frostbitten ear
causes considerable pain,
2. LACERATIONS (FIGURE 8.6). They are repaired as (d) protection of bullae from rupture,
early as possible. The perichondrium is stitched with (e) systemic antibiotics for deep infection, and

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 Chapter 8 — Diseases of External Ear 53

tibiotics and local application of 4% aluminium acetate

compresses. When abscess has formed, it must be drained
promptly and culture and sensitivity of the pus obtained.
Incision is made in the natural fold and devitalized car-
tilage removed. Some prefer to place a catheter in the
abscess and administer a continuous drip of antibiotics,
selected by culture and sensitivity for 7–10 days.

2. RELAPSING POLYCHONDRITIS. It is a rare autoimmune

disorder involving cartilage of the ear. Other cartilages,
septal, laryngeal, tracheal, costal may also be involved.
The entire auricle except its lobule becomes inflamed and
tender. External ear canal becomes stenotic. Treatment
consists of high doses of systemic steroids.

Figure 8.7. Keloid following piercing of pinna for ornaments. 3. CHONDRODERMATITIS NODULARIS CHRONICA HELICIS.
Small painful nodules appear near the free border of he-
(f) surgical debridement should wait several months as lix in men about the age of 50 years. Nodules are tender
the true demarcation between the dead and living tis- and the patient is unable to sleep on the affected side.
sues appears quite late. Treatment is excision of the nodule with its skin and
cartilage.
5. KELOID OF AURICLE. It may follow trauma or pierc-
ing of the ear for ornaments. Usual sites are the lobule or D. TUMOURS
helix (Figure 8.7). Surgical excision of the keloid usually
results in recurrence. Recurrence of keloid can be avoided See p. 117
by pre- and postoperative radiation with a total dose of
600–800 rad delivered in four divided doses. Some prefer
local injection of steroid after excision.
II. DISEASES OF EXTERNAL AUDITORY
CANAL
C. INFLAMMATORY DISORDERS
The diseases of external auditory canal are grouped as:
1. PERICHONDRITIS (FIGURE 8.8). It results from infec-
tion secondary to lacerations, haematoma or surgical in- • Congenital disorders
cisions. It can also result from extension of infection from • Trauma
diffuse otitis externa or a furuncle of the meatus. Pseu- • Inflammation
domonas and mixed flora are the common pathogens. • Tumours
Initial symptoms are red, hot and painful pinna which • Miscellaneous conditions
feels stiff. Later abscess may form between the cartilage
and perichondrium with necrosis of cartilage as the carti-
A. CONGENITAL DISORDERS
lage survives only on the blood supply from its perichon-
drium. Treatment in early stages consists of systemic an- 1. ATRESIA OF EXTERNAL CANAL. Congenital atresia of
the meatus may occur alone or in association with micro-
tia. When it occurs alone, it is due to failure of canaliza-
tion of the ectodermal core that fills the dorsal part of
the first branchial cleft. The outer meatus, in these cases,
is obliterated with fibrous tissue or bone while the deep
meatus and the tympanic membrane are normal. Atresia
with microtia is more common. It may be associated with
abnormalities of the middle ear, internal ear and other
structures.

2. COLLAURAL FISTULA. This is an abnormality of the first

branchial cleft. The fistula has two openings: one situated
in the neck just below and behind the angle of mandible
and the other in the external canal or the middle ear. The
track of the fistula traverses through the parotid in close
relation to the facial nerve.

B. TRAUMA TO EAR CANAL

Minor lacerations of canal skin result from Q-tip injury
Figure 8.8. Perichondritis pinna. (scratching the ear with hair pins, needles or matchstick)

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hair follicle
54 SECTION I — Diseases of Ear

or unskilled instrumentation by the physician. They usu- Trauma can result from scratching the ear canal with
ally heal without sequelae. hair pins or matchsticks, unskilled instrumentation to
Major lacerations result from gunshot wounds, auto- remove foreign bodies or vigorous cleaning of ear canal
mobile accidents or fights. The condyle of mandible may after a swim when meatal skin is already macerated. Break
force through the anterior canal wall. These cases require in continuity of meatal lining sets the ground for organ-
careful treatment. Aim is to attain a skin-lined meatus of isms to invade.
adequate diameter. Stenosis of the ear canal is a common Common organisms responsible for otitis externa are
complication. Staphylococcus aureus, Pseudomonas pyocyaneus, Bacillus
proteus and Escherichia coli but more often the infection
is mixed.
C. INFLAMMATIONS OF EAR CANAL
- Some cases of otitis externa are secondary to infection
Otitis externa may be divided, on aetiological basis, into: of the middle ear, or allergic sensitization to the topical
ear drops used for chronic suppurative otitis media.
1. Infective Group Clinical features. Diffuse otitis externa may be acute or
chronic with varying degrees of severity.
Acute phase is characterized by hot burning sensa-
tion in the ear, followed by pain which is aggravated by
movements of jaw. Ear starts oozing thin serous discharge
which later becomes thick and purulent. Meatal lining
becomes inflamed and swollen. Collection of debris and
discharge accompanied with meatal swelling gives rise to
conductive hearing loss. In severe cases, regional lymph
2. Reactive Group nodes become enlarged and tender with cellulitis of the
surrounding tissues.
• Eczematous otitis externa
Chronic phase is characterized by irritation and strong
• Seborrhoeic otitis externa
desire to itch. This is responsible for acute exacerbations
• Neurodermatitis
and reinfection. Discharge is scanty and may dry up to
form crusts. Meatal skin which is thick and swollen may
(A) FURUNCLE (LOCALIZED ACUTE OTITIS EXTERNA). A
also show scaling and fissuring. Rarely, the skin becomes
furuncle is a staphylococcal infection of the hair follicle.
hypertrophic leading to meatal stenosis (chronic stenotic
As the hair are confined only to the cartilaginous part of
otitis externa).
the meatus, furuncle is seen only in this part of meatus.
Treatment. Acute phase is treated as follows:
Usually single, the furuncles may be multiple.
Patient usually presents with severe pain and tender- (i) Ear toilet. It is the most important single factor in the
ness which are out of proportion to the size of the furun- treatment of diffuse otitis externa. All exudate and
cle. Movements of the pinna are painful. Jaw movements, debris should be meticulously and gently removed.
as in chewing, also cause pain in the ear. A furuncle of Special attention should be paid to anteroinferior
posterior meatal wall causes oedema over the mastoid meatal recess, which forms a blind pocket where dis-
& with obliteration of the retroauricular groove. Periauric- charge is accumulated. Ear toilet can be done by dry
ular lymph nodes (anterior, posterior and inferior) may mopping, suction clearance or irrigating the canal
also be enlarged and tender. with warm, sterile normal saline.
Treatment in early cases, 6 without abscess formation,
=>
(ii) Medicated wicks. After thorough toilet, a gauze wick
consists of -
systemic antibiotics,
- -
analgesics and local heat. soaked in antibiotic steroid preparation is inserted in
An ear pack of 10% ichthammol glycerine provides splin- the ear canal and patient advised to keep it moist by
tage and reduces pain. Hygroscopic action of glycerine re- instilling the same drops twice or thrice a day. Wick
duces oedema, while ichthammol is mildly antiseptic. If is changed daily for 2–3 days when it can be substi-
abscess has formed, incision and drainage should be done. tuted by ear drops. Local steroid drops help to relieve
In case of recurrent furunculosis, diabetes should be ex- oedema, erythema and prevent itching. Aluminium
cluded, and attention paid to the patient’s nasal vesti- acetate (8%) or silver nitrate (3%) are mild astrin-
bules which may harbour staphylococci and the infection gents and can be used in the form of a wick to form
transferred by patient’s fingers. Staphylococcal infections a protective coagulum to dry-up an oozing meatus.
of the skin as a possible source should also be excluded (iii) Antibiotics. Broad-spectrum systemic antibiotics are
and suitably treated. used when there is cellulitis and acute tender lym-
-

phadenitis.
(B) DIFFUSE OTITIS EXTERNA. It is diffuse inflammation (iv) Analgesics. For relief of pain.
of meatal skin which may spread to involve the pinna
Chronic phase. Treatment aims at (i) reduction of meatal
and epidermal layer of tympanic membrane.
swelling so that ear toilet can be effectively done and (ii)
Aetiology. Disease is commonly seen in hot and humid
alleviation of itching so that scratching is stopped and
climate and in swimmers. Excessive sweating changes
further recurrences controlled.
the pH of meatal skin from that of acid to alkaline which
A gauze wick soaked in 10% ichthammol glycerine and
favours growth of pathogens. Two factors commonly re-
inserted into the canal helps to reduce swelling. This is
sponsible for this condition are:
followed by ear toilet with particular attention to antero-
(i) trauma to the meatal skin and inferior meatal recess. Itching can be controlled by topi-
(ii) invasion by pathogenic organisms. cal application of antibiotic steroid cream.

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When the meatal skin is thickened to the point of ob- fossa, posteriorly to the mastoid and medially into the
struction and resists all forms of medical treatment, i.e. middle ear and petrous bone.
chronic stenotic otitis externa, it is surgically excised, Diagnosis. Severe otalgia in an elderly diabetic patient
bony meatus is widened with a drill and lined by split- with granulation tissue in the external ear canal at its car-
skin graft. tilaginous–bony junction should alert the physician of
necrotizing otitis externa. CT scan may show bony de-
(C) OTOMYCOSIS. Otomycosis is a fungal infection of the struction but is often not helpful. Gallium-67 is more use-
ear canal that often occurs due to Aspergillus niger, A. fu- ful in diagnosis and follow-up of the patient. It is taken
migatus or Candida albicans. It is seen in hot and humid up by monocytes and reticuloendothelial cells, and is in-
climate of tropical and subtropical countries. Secondary dicative of soft tissue infection. It can be repeated every
fungal growth is also seen in patients using topical anti- 3 weeks to monitor the disease and response to treatment.
biotics for treatment of otitis externa or middle ear sup- Technetium 99 bone scan reveals bone infection but test
puration. remains positive for a year or so and cannot be used to
The clinical features of otomycosis include intense itch- monitor the disease.
ing, discomfort or pain in the ear, watery discharge with Treatment. It consists of:
a musty odour and ear blockage. The fungal mass may
(i) Control of diabetes.
appear white, brown or black and has been likened to a
(ii) Toilet of ear canal. Remove discharge, debris and
wet piece of filter paper.
granulations or any dead tissue or bone.
Examined with an otoscope, A. niger appears as black-
(iii) Antibiotic treatment against causative organism,
headed filamentous growth, A. fumigatus as pale blue or
which in most ears is P. aeruginosa, but sometimes oth-
green and Candida as white or creamy deposit. Meatal
er organisms which can be found by culture and sensi-
skin appears sodden, red and oedematous.
tivity. Antibiotic treatment is continued for 6–8 weeks,
Treatment consists of thorough ear toilet to remove all
sometimes more. Antibiotics found effective are:
discharge and epithelial debris which are conducive to
• Gentamicin combined with ticarcillin. They are
the growth of fungus. It can be done by syringing, suc-
given intravenously. Gentamicin is both ototoxic
tion or mopping. Specific antifungal agents can be ap-
and nephrotoxic, and ticarcillin may produce pen-
plied. Nystatin (100,000 units/mL of propylene glycol)
icillin-like reactions.
is effective against Candida. Other broad-spectrum anti-
• Third-generation cephalosporins, e.g. ceftriaxone
fungal agents include clotrimazole and povidone iodine.
1–2 g/day i.v. or ceftazidime 1–2 g/day i.v. are usu-
Two per cent salicylic acid in alcohol is also effective. It
ally combined with an aminoglycoside.
is a keratolytic agent which removes superficial layers of
• Quinolones (ciprofloxacin, ofloxacin and levo-
epidermis, and along with that, the fungal mycelia grow-
floxacin) are also effective and can be given orally.
ing into them. Antifungal treatment should be continued
They can be combined with rifampin. Ciprofloxa-
for a week even after apparent cure to avoid recurrences.
cin 750 mg OD orally can be used. Oral therapy
Ear must be kept dry. Bacterial infections are often associ-
with quinolones obviates the need for admission
ated with otomycosis and treatment with an antibiotic/
for i.v. injections.
steroid preparation helps to reduce inflammation and
oedema and thus permitting better penetration of anti- If patient is not responsive, culture and sensitivity of
fungal agents. ear discharge should guide the surgeon.
Prolonged antibiotic treatment has replaced radical
(D) OTITIS EXTERNA HAEMORRHAGICA. It is character- surgery and resections done earlier for this condition.
ized by formation of haemorrhagic bullae on the tym-
panic membrane and deep meatus. It is probably viral (G) ECZEMATOUS OTITIS EXTERNA. It is the result of hy-
in origin and may be seen in influenza epidemics. The persensitivity to infective organisms or topical ear drops
condition causes severe pain in the ear and blood-stained such as chloromycetin or neomycin, etc. It is marked by
discharge when the bullae rupture. Treatment with anal- intense irritation, vesicle formation, oozing and crusting
gesics is directed to give relief from pain. Antibiotics are in the canal. Treatment is withdrawal of topical antibiotic
given for secondary infection of the ear canal, or middle causing sensitivity and application of steroid cream.
ear if the bulla has ruptured into the middle ear.
(H) SEBORRHOEIC OTITIS EXTERNA. It is associated with
(E) HERPES ZOSTER OTICUS. It is characterized by forma- seborrhoeic dermatitis of the scalp. Itching is the main
tion of vesicles on the tympanic membrane, meatal skin, complaint. Greasy yellow scales are seen in the external
concha and postauricular groove. The VIIth and VIIIth canal, over the lobule and postauricular sulcus. Treatment
cranial nerves may be involved. consists of ear toilet, application of a cream containing
salicylic acid and sulfur, and attention to the scalp for
(F) MALIGNANT (NECROTIZING) OTITIS EXTERNA. It is an seborrhoea.
inflammatory condition caused by pseudomonas infec-
tion usually in the elderly diabetics, or in those on im- (I) NEURODERMATITIS. It is caused by compulsive
munosuppressive drugs. Its early manifestations resemble scratching due to psychological factors. Patient’s main
diffuse otitis externa but there is excruciating pain and complaint is intense itching. Otitis externa of bacterial
appearance of granulations in the ear canal. Facial paral- type may follow infection of raw area left by scratching.
ysis is common. Infection may spread to the skull base Treatment is sympathetic psychotherapy and that meant
and jugular foramen causing multiple cranial nerve pal- for any secondary infection. Ear pack and bandage to the
sies. Anteriorly, infection spreads to temporomandibular ear are helpful to prevent compulsive scratching.

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56 SECTION I — Diseases of Ear

(J) PRIMARY CHOLESTEATOMA OF EXTERNAL AUDITORY from impaction of wax against the tympanic membrane.
CANAL. In contrast to middle ear cholesteatoma, squa- Reflex cough due to stimulation of auricular branch of va-
mous epithelium of the external canal invades its bone. gus may sometimes occur. The onset of these symptoms
Usually there is some abnormality of bone of external ca- may be sudden when water enters the ear canal during
nal which is conducive for epithelium to invade it. It may bathing or swimming and the wax swells up. Long stand-
be post-traumatic or postsurgical. Clinical features include ing impacted wax may ulcerate the meatal skin and result
purulent otorrhoea and pain; tympanic membrane being in granuloma formation (wax granuloma).
normal. Granulations associated with sequestrated bone Treatment of wax consists in its removal by syringing
need histological examination to differentiate it from carci- or instrumental manipulation. Hard impacted mass may
noma, necrotizing otitis externa and a benign sequestrum. sometimes require prior softening with wax solvents.
Treatment consists of removal of necrotic bone and Technique of syringing the ear. Patient is seated with ear
cholesteatoma, and lining the defect with fascia. to be syringed towards the examiner. A towel is placed
round his neck. A kidney tray is placed over the shoulder
and held snugly by the patient. Patient’s head is slightly
D. TUMOURS
tilted over the tray to collect the return fluid.
See p. 118. Pinna is pulled upwards and backwards and a stream of
water from the ear syringe is directed along the postero-
superior wall of the meatus. Pressure of water, built up
E. MISCELLANEOUS CONDITIONS
deeper to the wax, expels the wax out (Figure 8.10 ).
1. IMPACTED WAX OR CERUMEN. Wax is composed of If wax is tightly impacted, it is necessary to create a space
secretion of sebaceous glands, ceruminous glands, hair, between it and the meatal wall for the jet of water to pass,
desquamated epithelial debris, keratin and dirt. otherwise syringing will be ineffective or may even push
Sebaceous and ceruminous (modified sweat glands) the wax deeper. Ear canal should be inspected from time
glands open into the space of the hair follicle (Fig- to time to see if all wax has been removed. Unnecessary
ure 8.9). Sebaceous glands provide fluid rich in fatty acids syringing should be avoided.
while secretion of ceruminous gland is rich in lipids and At the end of the procedure, ear canal and tympanic
pigment granules. Secretion of both these glands mixes membrane must be inspected and dried with a pledget
with the desquamated epithelial cells and keratin shed of cotton. Any ulceration seen in meatal wall as a result
from the tympanic membrane and deep bony meatus to of impacted wax is protected by application of suitable
form wax. antibiotic ointment. Normally, boiled tap water cooled
Wax has a protective function as it lubricates the ear to body temperature is used. If it is too cold or too hot
canal and entraps any foreign material that happens to it would stimulate the labyrinth, as in caloric testing,
enter the canal. It has acidic pH and is bacteriostatic and and cause vertigo. Too much force used in syringing may
fungistatic. rupture the tympanic membrane especially when it has
Normally, only a small amount of wax is secreted, already been weakened by previous disease. Patient com-
which dries up and is later expelled from the meatus by plains of intense pain and may become giddy and even
movements of the jaw. As some people sweat more than faint. It is necessary before syringing to ask the patient
others, the activity of ceruminous glands also varies; ex- for any past history of ear discharge or an existing per-
cessive wax may be secreted and deposited as a plug in foration. A quiescent otitis media may be reactivated by
the meatus. Certain other factors like narrow and tortu- syringing.
ous ear canal, stiff hair or obstructive lesion of the canal, Instrumental manipulation. It should always be done
e.g. exostosis, may favour retention of wax. It may dry up by skilled hands and under direct vision. Cerumen hook,
and form a hard impacted mass. scoop or Jobson-Horne probe are often used. First, a space
Patient usually presents with impairment of hearing is created between the wax and meatal wall, the instru-
or sense of blocked ear. Tinnitus and giddiness may result ment is passed beyond the wax, and whole plug then

Figure 8.9. Structure of skin of cartilaginous meatus.

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 Chapter 8 — Diseases of External Ear 57

Figure 8.10. (A) Irrigation of the ear canal. (B) Illustration to show how a jet of water expels wax or a foreign body.
Scan to play Ear Irrigation.

dragged out in a single piece. If it breaks, syringing may

be used to remove the fragments.
Occasionally, if the wax is too hard and impacted, to
be removed by syringing or instruments, it should be sof-
tened by drops of 5% sodium bicarbonate in equal parts
of glycerine and water instilled two or three times a day
for a few days. Hydrogen peroxide, liquid paraffin or olive
oil may also achieve the same result. Commercial drops
containing ceruminolytic agents like paradichloroben-
zene 2% can also be used and above methods tried again. Figure 8.11. Other methods of wax or foreign body removal:
(A) Suction. (B) Forceps removal.
2. FOREIGN BODIES OF EAR. (a) Nonliving. Children may
Unskilled attempts at removal of foreign bodies may
insert a variety of foreign bodies in the ear; the common
lacerate the meatal lining, damage the tympanic mem-
ones often seen are: a piece of paper or sponge, grain
brane or the ear ossicles.
seeds (rice, wheat, maize), slate pencil, piece of chalk or
(b) Living. Flying or crawling insects like mosquitoes,
metallic ball bearings. An adult may present with a bro-
beetles, cockroach or an ant may enter the ear canal
ken end of matchstick used for scratching the ear or an
and cause intense irritation and pain (Figure 8.12). No
overlooked cotton swab. Vegetable foreign bodies tend to
attempt should be made to catch them alive. First, the
swell up with time and get tightly impacted in the ear
insect should be killed by instilling oil (a household rem-
canal or may even suppurate.
edy), spirit or chloroform water. Once killed, the insect
Methods of removing a foreign body include:
can be removed by any of the methods described above.
(i) Forceps removal
(ii) Syringing
(iii) Suction
(iv) Microscopic removal with special instruments
(v) Postaural approach
Soft and irregular foreign bodies like a piece of paper,
swab or a piece of sponge can be removed with fine croco-
dile forceps (Figure 8.11).
Most of the seed grains and smooth objects can be
removed with syringing. Smooth and hard objects like
steel ball bearing should not be grasped with forceps as
they tend to move inwards and may injure the tympanic
membrane. In all impacted foreign bodies or in those where
earlier attempts at extraction have been made, it is preferable
to use general anaesthetic and an operating microscope. Oc-
casionally, postaural approach is used to remove foreign
bodies impacted in deep meatus, medial to the isthmus or
those which have been pushed into the middle ear. Figure 8.12. Endoscopic view of an insect in the ear canal (arrow).

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58 SECTION I — Diseases of Ear

Maggots in the ear. Flies may be attracted to the foul- (b) Trauma, e.g. lacerations, fracture of tympanic plate,
smelling ear discharge and lay eggs which hatch out into surgery on ear canal or mastoid.
larvae called maggots. They are commonly seen in the (c) Burns—thermal or chemical.
month of August, September and October. There is severe
Treatment is meatoplasty. Using a postaural incision,
pain with swelling round the ear and blood-stained wa-
scar tissue and thickened meatal skin are excised, bony
tery discharge. Maggots may be seen filling the ear canal.
meatus is enlarged and the raw meatal bone is covered
Treatment consists of instilling chloroform water to
with pedicled flaps from meatus or split-skin grafts.
kill the maggots, which can later be removed by forceps.
Usually, such patients have discharging ears with perfora-
tion of the tympanic membrane and syringing may not
be advisable. III. DISEASES OF TYMPANIC MEMBRANE
Diseases of tympanic membrane may be primary or sec-
3. KERATOSIS OBTURANS. Collection of a pearly white
ondary to conditions affecting external ear, middle ear or
mass of desquamated epithelial cells in the deep meatus
eustachian tube.
is called keratosis obturans. This, by its pressure effect,
Normal tympanic membrane. It is shiny and pearly
causes absorption of bone leading to widening of the
grey in colour with a concavity on its lateral surface,
meatus so much so that facial nerve may be exposed and
more marked at the tip of malleus, the umbo. A bright
paralyzed.
cone of light can be seen in the anteroinferior quadrant
(a) Aetiology. It is commonly seen between 5 and
(Figure 8.13). Attic area lies above the lateral process of
20 years and may affect one or both ears. It may some-
malleus and is slightly pinkish. Transparency varies. Some
times be associated with bronchiectasis and chronic si-
middle ear structures can be seen through a transparent
nusitis. Normally, epithelium from surface of tympanic
membrane. A normal tympanic membrane is mobile
membrane migrates onto the posterior meatal wall. Fail-
when tested with pneumatic otoscope or Siegle’s specu-
ure of this migration or obstruction to migration caused
lum (Figure 8.14).
by wax may lead to accumulation of the epithelial plug
in the deep meatus.
1. RETRACTED TYMPANIC MEMBRANE. It appears dull and
(b) Clinical features. Presenting symptoms may be pain
lustreless. Cone of light is absent or interrupted. Handle of
in the ear, hearing loss, tinnitus and sometimes ear dis-
malleus appears foreshortened. Lateral process of malleus
charge.
becomes more prominent. Anterior and posterior malleal
On examination, ear canal may be full of pearly white
folds become sickle shaped (Figure 8.15). A retracted tym-
mass of keratin material disposed in several layers. Re-
panic membrane is the result of negative intratympanic
moval of this mass may show widening of bony meatus
pressure when the eustachian tube is blocked.
with ulceration and even granuloma formation.
(c) Treatment. Keratotic mass is removed either by sy-
2. MYRINGITIS BULLOSA. It is a painful condition charac-
ringing or instrumentation, similar to the techniques em-
terized by formation of haemorrhagic blebs on the tym-
ployed for impacted wax. Secondary otitis externa may be
panic membrane and deep meatus. It is probably caused
present and should be treated. Patient should be periodi-
by a virus or Mycoplasma pneumoniae.
cally checked and any reaccumulations removed. Recur-
rence can be checked to some extent by the use of kerato-
lytic agent such as 2% salicylic acid in alcohol.

4. ACQUIRED ATRESIA AND STENOSIS OF MEATUS. It can

result from:
(a) Infections, e.g. chronic otitis externa—an important
cause (Figure 8.13).

Figure 8.14. Normal tympanic membrane of the right side. Note a

Figure 8.13. Meatal stenosis following chronic otitis externa. bright cone of light at 5’o-clock position.

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Treatment. In a majority of cases, edges of perforation

get inverted towards the middle ear. In such cases, the ear
should be examined under operating microscope and the
edges of perforation repositioned and splinted (see p. 464).
Injuries of tympanic membrane may be associated
with facial paralysis or subluxation of stapes (vertigo and
nystagmus) and sensorineural hearing loss. In such cases,
urgent exploration may be required.

6. ATROPHIC TYMPANIC MEMBRANE. A normal tympanic

membrane consists of outer epithelial, middle fibrous and
inner mucosal layer. In serous otitis media, the middle fi-
brous layer gets absorbed leaving a thin drumhead which
Figure 8.15. A retracted tympanic membrane.
easily gets collapsed with eustachian tube insufficiency.
A perforation of tympanic membrane also heals only by
epithelial and mucosal layers without the intervening
3. HERPES ZOSTER OTICUS. It is a viral infection involv- fibrous layer.
ing geniculate ganglion of facial nerve. It is character-
ized by appearance of vesicles on the tympanic mem- 7. RETRACTION POCKETS AND ATELECTASIS. When the
brane, deep meatus, concha and retroauricular sulcus. tympanic membrane is thin and atrophic, a segment of
It may involve VIIth (more often) and the VIIIth cranial it or the entire membrane may collapse inwards due to
nerves. eustachian tube insufficiency. It may form a retraction
pocket or get plastered onto promontory and also wrap
4. MYRINGITIS GRANULOSA. Nonspecific granulations round the ossicles. A deep retraction pocket may accumu-
form on the outer surface of tympanic membrane. It may late keratin debris and form a cholesteatoma.
be associated with impacted wax, long-standing foreign
body or external ear infection. 8. TYMPANOSCLEROSIS. It is hyalinization and later calci-
fication in the fibrous layer of tympanic membrane. It ap-
5. TRAUMATIC RUPTURE. Tympanic membrane may be pears as chalky white plaque. Mostly, it remains asympto-
ruptured by: matic. It is frequently seen in cases of serous otitis media
(a) Trauma due to a hair pin, matchstick or unskilled at- as a complication of ventilation tube. Tympanosclerosis
tempts to remove a foreign body. mostly affects tympanic membrane but may be seen in-
(b) Sudden change in air pressure, e.g. a slap or a kiss on volving ligaments, joints of ossicles, muscle tendons and
the ear or a sudden blast. Forceful Valsalva may rup- submucosal layer of middle ear cleft, and interferes in the
ture a thin atrophic membrane. conduction of sound.
(c) Pressure by a fluid column, e.g. diving, water sports or
forceful syringing. 9. PERFORATIONS. They may be central, attic or marginal
(d) Fracture of temporal bone. and are associated with chronic otitis media (see p. 88).

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 Chapter 9
Eustachian Tube and Its Disorders

ANATOMY LINING OF THE EUSTACHIAN TUBE

Histologically, the mucosa shows pseudostratified ciliated
Eustachian tube, also called auditory or pharyngotympanic
columnar epithelium interspersed with mucous secreting
tube, connects nasopharynx with the tympanic cavity. In
goblet cells. Submucosa, particularly in the cartilaginous
an adult, it is about 36 mm long and runs downwards,
part of the tube, is rich in seromucinous glands. The cilia
forwards and medially from its tympanic end, forming
beat in the direction of nasopharynx and thus help to
an angle of 45° with the horizontal. It is divided into
drain secretions and fluid from the middle ear into the
two parts: bony, which is posterolateral, forms one-third
nasopharynx.
(12 mm) of the total length and fibrocartilaginous, which
is anteromedial, forms two-thirds (24 mm). The two
parts meet at isthmus which is the narrowest part of the NERVE SUPPLY
tube (Figure 9.1). The fibrocartilaginous part of the tube
Tympanic branch of cranial nerve (CN) IX supplies sen-
is made of a single piece of cartilage folded upon itself
sory as well as parasympathetic secretomotor fibres to the
in such a way that it forms the whole of medial lamina,
tubal mucosa. Tensor veli palatini muscle is supplied by
roof and a part of the lateral lamina; the rest of its lateral
mandibular branch of trigeminal (V3) nerve. Levator veli
lamina is made of fibrous membrane.
palatini and salpingopharyngeus muscles receive motor
The tympanic end of the tube is bony, measures
nerve supply through pharyngeal plexus (cranial part of
5 × 2 mm and is situated in the anterior wall of middle
CN XI through vagus).
ear, a little above the level of floor. The pharyngeal end of
the tube is slit-like, vertically. The cartilage at this end
raises an elevation called torus tubarius, which is situated DIFFERENCES BETWEEN THE INFANT AND
in the lateral wall of the nasopharynx, 1–1.25 cm behind ADULT EUSTACHIAN TUBE
the posterior end of inferior turbinate.
The eustachian tube of infants is wider, shorter and more
horizontal; thus infections from the nasopharynx can
easily reach the middle ear. Even the milk may regurgitate
STRUCTURE into the middle ear if the infants are not fed in head-up
position (see Table 9.1).
MUSCLES RELATED TO EUSTACHIAN TUBE
(FIGURE 9.2)
Three muscles are related to the tube: tensor veli palatini, FUNCTIONS
levator veli palatini and salpingopharyngeus. The medial
Physiologically, eustachian tube performs three main
fibres of the tensor veli palatini are attached to the lateral
functions:
lamina of the tube and when they contract help to open
the tubal lumen. These fibres have also been called dilator 1. Ventilation and thus regulation of middle ear pressure.
tubae muscle. The exact role of the levator veli palatini 2. Protection against (i) nasopharyngeal sound pressure
and the salpingopharyngeus muscles to open the tube is and (ii) reflux of nasopharyngeal secretions.
uncertain. It is believed that the levator veli palatini mus- 3. Clearance of middle ear secretions.
cle, which runs inferior and parallel to the cartilaginous
part of the tube forms a bulk under the medial lamina 1. VENTILATION AND REGULATION OF MIDDLE EAR
and during contraction pushes it upward and medially PRESSURE. For normal hearing, it is essential that pres-
thus assisting in opening the tube. sure on two sides of the tympanic membrane should be
The elastin hinge. The cartilage, at the junction of me- equal. Negative or positive pressure in the middle ear af-
dial, and lateral lamina at the roof, is rich in elastin fibres fects hearing. Thus, eustachian tube should open peri-
which form a hinge. By its recoil it helps to keep the odically to equilibrate the air pressure in the middle ear
tube closed when no longer acted upon by dilator tubae with the ambient pressure. Normally, the eustachian tube
muscle. remains closed and opens intermittently during swallow-
Ostmann’s pad of fat. It is a mass of fatty tissues relat- ing, yawning and sneezing. Posture also affects the func-
ed laterally to the membranous part of the cartilaginous tion; tubal opening is less efficient in recumbent position
tube. It also helps to keep the tube closed and thus pro- and during sleep due to venous engorgement. Tubal func-
tect it from the reflux of nasopharyngeal secretions. tion is also poor in infants and young children and thus

61

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62 SECTION I — Diseases of Ear

responsible for more ear problems in that age group. It normal hearing. Normally, the eustachian tube remains
usually normalizes by the age of 7–10 years. closed and protects the middle ear against these sounds.
A normal eustachian tube also protects the middle ear
2. PROTECTIVE FUNCTIONS. Abnormally, high sound from reflux of nasopharyngeal secretions into the middle
pressures from the nasopharynx can be transmitted to ear. This reflux occurs more readily if the tube is wide in
the middle ear if the tube is open thus interfering with diameter (patulous tube), short in length (as in babies) or
the tympanic membrane is perforated (cause for persis-
tence of middle ear infections in cases of tympanic mem-
brane perforations).
High pressures in the nasopharynx can also force na-
sopharyngeal secretions into the middle ear, e.g. forceful
nose blowing, closed-nose swallowing as in the presence
of adenoids or bilateral nasal obstruction.

3. CLEARANCE OF MIDDLE EAR SECRETIONS. Mucous

membrane of the eustachian tube and anterior part of
the middle ear is lined by ciliated columnar cells. The
cilia beat in the direction of nasopharynx. This helps
to clear the secretions and debris in the middle ear to-
wards the nasopharynx. The clearance function is fur-
ther augmented by active opening and closing of the
tube.

EUSTACHIAN TUBE FUNCTION TESTS

1. VALSALVA TEST. The principle of this test, as also of
Figure 9.1. Horizontal section through the eustachian tube showing politzerization, is to build positive pressure in the naso-
bony and cartilaginous parts, isthmus, tympanic and pharyngeal ends. pharynx so that air enters the eustachian tube. To do this

Figure 9.2. Vertical section through eustachian tube. Note: Cartilage of the tube forms medial wall, roof and part of lateral wall. Elastin is situated
in the roof at the junction of medial and lateral laminae and helps the medial laminae to regain its original position of closure. (A) Eustachian tube
is closed in resting position. (B) Tube is open when tensor veli palatini (dilator tubae) muscle contracts.

TABLE 9.1 DIFFERENCES BETWEEN INFANT AND ADULT EUSTACHIAN TUBE

Infant Adult
Length 13–18 mm at birth (about half as long as in adult) 36 mm (31–38 mm)
Direction More horizontal. At birth, it forms an angle of 10° Forms an angle of 45° with the horizontal
with the horizontal. At age 7 and later it is 45°
Angulation at isthmus No angulation Angulation present
Bony versus cartilaginous part Bony part is slightly longer than one-third of the Bony part one-third; cartilaginous part two-thirds
total length of the tube and is relatively wider
Tubal cartilage Flaccid. Retrograde reflux of nasopharyngeal Comparatively rigid. Remains closed and protects
secretions can occur the middle ear from the reflux
Density of elastin at the hinge Less dense; tube does not efficiently close by recoil Density of elastin more and helps to keep the tube
closed by recoil of cartilage
Ostmann’s pad of fat Less in volume Large and helps to keep the tube closed

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 Chapter 9 — Eustachian Tube and Its Disorders 63

test, patient pinches his nose between the thumb and in- (b) Bleeding from the nose.
dex finger, takes a deep breath, closes his mouth and tries (c) Transmission of nasal and nasopharyngeal infection
to blow air into the ears. If air enters the middle ear, the into the middle ear causing otitis media.
tympanic membrane will move outwards, which can be (d) Rupture of atrophic area of tympanic membrane if
verified by otoscope or the microscope. In the presence too much pressure is used.
of a tympanic membrane perforation, a hissing sound
is produced or if discharge is also present in the middle 4. TOYNBEE’S TEST. While the above three tests use a
ear, cracking sound will be heard. Failure of this test does positive pressure, Toynbee’s manoeuvre causes negative
not prove blockage of the tube because only about 65% pressure. It is a more physiological test. It is performed
of persons can successfully perform this test. This test by asking the patient to swallow while nose has been
should be avoided (i) in the presence of atrophic scar of pinched. This draws air from the middle ear into the na-
tympanic membrane which can rupture and (ii) in the sopharynx and causes inward movement of tympanic
presence of infection of nose and nasopharynx where in- membrane, which is verified by the examiner otoscopi-
fected secretions are likely to be pushed into the middle cally or with a microscope.
ear causing otitis media.
5. TYMPANOMETRY (ALSO CALLED INFLATION–DEFLATION
2. POLITZER TEST. This test is done in children who are TEST). In this test, positive and negative pressures are cre-
unable to perform Valsalva test. In this test, olive-shaped ated in the external ear canal and the patient swallows re-
tip of the Politzer’s bag is introduced into the patient’s peatedly. The ability of the tube to equilibrate positive and
nostril on the side of which the tubal function is desired negative pressures to the ambient pressure indicates nor-
to be tested. Other nostril is closed, and the bag com- mal tubal function. The test can be done both in patients
pressed while at the same time the patient swallows (he with perforated or intact tympanic membranes (see p. 26).
can be given sips of water) or says “ik, ik, ik.” By means of
an auscultation tube, connecting the patient’s ear under 6. RADIOLOGICAL TEST. A radio-opaque dye, e.g. hy-
test to that of the examiner, a hissing sound is heard if paque or lipoidal instilled into the middle ear through a
tube is patent. Compressed air can also be used instead pre-existing perforation and X-rays taken should deline-
of Politzer’s bag. The test is also used therapeutically to ate the tube and any obstruction. The time taken by the
ventilate the middle ear. dye to reach the nasopharynx also indicates its clearance
function. This test is no longer popular now.
3. CATHETERIZATION. In this test, nose is first anaesthe-
tized by topical spray of lignocaine and then a eustachian 7. SACCHARINE OR METHYLENE BLUE TEST. Saccharine
tube catheter, the tip of which is bent, is passed along the solution is placed into the middle ear through a pre-ex-
floor of nose till it reaches the nasopharynx. Here it is ro- isting perforation. The time taken by it to reach the phar-
tated 90° medially and gradually pulled back till it engag- ynx and impart a sweet taste is also a measure of clearance
es on the posterior border of nasal septum (Figure 9.3A). function.
It is then rotated 180° laterally so that the tip lies against Similarly, methylene blue dye can be instilled into the
the tubal opening (Figure 9.3B). A Politzer’s bag is now middle ear and the time taken by it to stain the pharyn-
connected to the catheter and air insufflated. Entry of air geal secretions can be noted.
into the middle ear is verified by an auscultation tube. Indirect evidence of drainage/clearance function is es-
The procedure of catheterization should be gentle as it is tablished when ear drops instilled into the ear with tym-
known to cause complications such as: panic membrane perforation cause bad taste in throat.

(a) Injury to eustachian tube opening which causes scar- 8. SONOTUBOMETRY. A tone is presented to the nose and
ring later. its recording taken from the external canal. The tone is

Figure 9.3. Catheterization of eustachian tube (see text).

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64 SECTION I — Diseases of Ear

heard louder when the tube is patent (compare patulous eu- TABLE 9.3 CAUSES OF EUSTACHIAN TUBE
stachian tube). It also tells the duration for which the tube OBSTRUCTION
remains open. It is a noninvasive technique and provides
information on active tubal opening. Accessory sounds • Upper respiratory infection (viral or bacterial)
• Allergy
produced in the nasopharynx, during swallowing, may in-
• Sinusitis
terfere with the test results. The test is under development. • Nasal polyps
• Deviated nasal septum
• Hypertrophic adenoids
DISORDERS OF EUSTACHIAN TUBE • Nasopharyngeal tumour/mass
• Cleft palate
• Submucous cleft palate
1. TUBAL BLOCKAGE. Normally, eustachian tube is • Down syndrome
closed. It opens intermittently during swallowing, yawn- • Functional
ing and sneezing through the active contraction of tensor
veli palatini muscle. Air, composed of oxygen, carbon di-
oxide, nitrogen and water vapour, normally fills the mid- retracted tympanic membrane, congestion along the
dle ear and mastoid. When tube is blocked, first oxygen is handle of malleus and the pars tensa, transudate behind
absorbed, but later other gases, CO2 and nitrogen also dif- the tympanic membrane, imparting it an amber colour
fuse out into the blood. This results in negative pressure and sometimes a fluid level with conductive hearing loss.
in the middle ear and retraction of tympanic membrane. In severe cases, as in barotrauma, tympanic membrane
If negative pressure is still further increased, it causes is markedly retracted with haemorrhages in subepithelial
“locking” of the tube with collection of transudate and layer, haemotympanum or sometimes a perforation.
later exudate and even haemorrhage. Effects of acute and
long-term tubal blockage are shown in Table 9.2. 2. ADENOIDS AND EUSTACHIAN TUBE FUNCTION. Ad-
Eustachian tube obstruction can be mechanical, func- enoids cause tubal dysfunction by:
tional or both. Mechanical obstruction can result from
(i) intrinsic causes such as inflammation or allergy or (ii) (a) Mechanical obstruction of the tubal opening.
extrinsic causes such as tumour in the nasopharynx or (b) Acting as reservoir for pathogenic organisms.
adenoids. Functional obstruction is caused by collapse of (c) In cases of allergy, mast cells of the adenoid tissue re-
the tube due to increased cartilage compliance, which re- lease inflammatory mediators which cause tubal block-
sists opening of the tube or failure of active tubal-opening age.
mechanism due to poor function of tensor veli palatini. Thus, adenoids can cause otitis media with effusion
The common clinical conditions which can cause tubal or recurrent acute otitis media. Adenoidectomy can help
obstruction are listed in Table 9.3. both these conditions.
Symptoms of tubal occlusion include otalgia, which
may be mild to severe, hearing loss, popping sensation, 3. CLEFT PALATE AND TUBAL FUNCTION. Tubal function
tinnitus and disturbances of equilibrium or even vertigo. is disturbed in cleft palate patients due to:
Signs of tubal occlusion will vary and depend upon
the acuteness of the condition and severity. They include (a) Abnormalities of torus tubarius, which shows high
elastin density making tube difficult to open.
(b) Tensor veli palatini muscle does not insert into the
TABLE 9.2 EFFECTS OF ACUTE AND PROLONGED torus tubarius in 40% cases of cleft palate and where
TUBAL BLOCKAGE it does insert, its function is poor.
Acute Otitis media with effusion is common in these pa-
Acute tubal blockage tients. Even after repair of the cleft palate deformity,
↓ many of them require insertion of grommets to ventilate
Absorption of ME gases the middle ear.
↓
Negative pressure in ME
4. DOWN SYNDROME AND TUBAL FUNCTION. Function of
↓
Retraction of TM
eustachian tube is defective possibly due to poor tone of
↓ tensor veli palatini muscle and abnormal shape of naso-
Transudate in ME/haemorrhage (acute OME) pharynx. Children with this syndrome are prone to fre-
Prolonged quent otitis media or otitis media with effusion.
Prolonged tubal blockage/dysfunction
↓ 5. BAROTRAUMA. See p. 71.
OME (thin watery or mucoid discharge)
↓
Atelectatic ear/perforation
↓
RETRACTION POCKETS AND EUSTACHIAN
Retraction pocket/cholesteatoma TUBE
↓
Erosion of incudostapedial joint
In ventilation of the middle ear cleft, air passes from eus-
tachian tube to mesotympanum, from there to attic, adi-
ME, middle ear; TM, tympanic membrane; OME, otitis media with effusion. tus, antrum and mastoid air cell system. Mesotympanum

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 Chapter 9 — Eustachian Tube and Its Disorders 65

communicates with the attic via anterior and posterior administration of potassium iodide is helpful but some
isthmi, situated in membranous diaphragm between the long-standing cases may require cauterization of the
mesotympanum and the attic. Anterior isthmus is situ- tubes or insertion of a grommet.
ated between tendon of tensor tympani and the stapes.
Posterior isthmus is situated between tendon of stapedius
muscle and pyramid, and the short process of incus. In EXAMINATION OF EUSTACHIAN TUBE
some cases, middle ear can also communicate directly
Pharyngeal end of the eustachian tube can be examined
with the mastoid air cells through the retrofacial cells.
by posterior rhinoscopy, rigid nasal endoscope or flexible
Any obstruction in the pathways of ventilation can
nasopharyngoscope. The extrinsic causes which obstruct
cause retraction pockets or atelectasis of tympanic mem-
this end can be excluded (Figure 9.4).
brane, e.g.
Tympanic end of the tube can be examined by micro-
1. Obstruction of eustachian tube → Total atelectasis of scope or endoscope, if there is a pre-existing perforation.
tympanic membrane. Eustachian tube endoscopy or middle ear endoscopy can
2. Obstruction in middle ear → Retraction pocket in poste- be done with very fine flexible endoscopes. Simple exami-
rior part of middle ear while anterior part is ventilated. nation of tympanic membrane with otoscope or micro-
3. Obstruction of isthmi → Attic retraction pocket. scope may reveal retraction pockets or fluid in the middle
4. Obstruction at aditus → Cholesterol granuloma and ear. Similarly, movements of tympanic membrane with
collection of mucoid discharge in mastoid air cells, respiration point to patulous eustachian tube.
while middle ear and attic appear normal. Further assessment of function of the tube can be made
by Valsalva, politzerization, Toynbee and other tests al-
Depending on the location of pathologic process, oth-
ready described.
er changes such as thin atrophic tympanic membrane,
Aetiologic causes of eustachian tube dysfunction can be
partial or total (due to absorption of middle fibrous layer),
assessed by thorough nasal examination including endos-
cholesteatoma, ossicular necrosis and tympanosclerotic
copy, tests of allergy, CT scan of temporal bones and of
changes may also be found.
paranasal sinuses. MRI may be required to exclude multi-
Principles of management of retraction pockets and
ple sclerosis in patulous eustachian tube.
atelectasis of middle ear would entail correction/repair of
the irreversible pathologic processes and establishment of
the ventilation.

PATULOUS EUSTACHIAN TUBE

In this condition, the eustachian tube is abnormally pat-
ent. Most of the time it is idiopathic but rapid weight loss,
pregnancy especially third trimester, or multiple sclerosis
can also cause it.
Patient’s chief complaints are hearing his own voice
(autophony), even his own breath sounds, which is very
disturbing. Due to abnormal potency, pressure changes
in the nasopharynx are easily transmitted to the middle
ear so much so that the movements of tympanic can be
seen with inspiration and expiration; these movements
are further exaggerated if patient breathes after closing Figure 9.4. Endoscopic view of nasopharynx showing torus tubarius
the opposite nostril. in the right lateral wall of nasopharynx. Note also the fossa of Rosen-
Acute condition of patulous tube is self-limiting and müller which lies behind it. Fossa of Rosenmüller is the commonest site
does not require treatment. In others, weight gain, oral for the origin of carcinoma nasopharynx.

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 Chapter 10
Disorders of Middle Ear

ACUTE SUPPURATIVE OTITIS MEDIA Haemophilus influenzae (20%) and Moraxella catarrhalis
(12%). Other organisms include Streptococcus pyogenes,
It is an acute inflammation of middle ear by pyogenic Staphylococcus aureus and sometimes Pseudomonas aerugi-
organisms. Here, middle ear implies middle ear cleft, i.e. nosa. In about 18–20%, no growth is seen. Many strains of
eustachian tube, middle ear, attic, aditus, antrum and H. influenzae and M. catarrhalis are β-lactamase producing.
mastoid air cells.

AETIOLOGY PATHOLOGY AND CLINICAL FEATURES

The disease runs through the following stages:
It is more common especially in infants and children of
lower socioeconomic group. Typically, the disease follows
1. STAGE OF TUBAL OCCLUSION. Oedema and hyperae-
viral infection of upper respiratory tract but soon the pyo-
mia of nasopharyngeal end of eustachian tube blocks the
genic organisms invade the middle ear.
tube leading to absorption of air and negative intratym-
panic pressure. There is retraction of tympanic membrane
ROUTES OF INFECTION with some degree of effusion in the middle ear but fluid
may not be clinically appreciable.
Symptoms. Deafness and earache are the two symptoms
1. VIA EUSTACHIAN TUBE. It is the most common route.
but they are not marked. There is generally no fever.
Infection travels via the lumen of the tube or along sub-
Signs. Tympanic membrane is retracted with handle
epithelial peritubal lymphatics. Eustachian tube in infants
of malleus assuming a more horizontal position, promi-
and young children is shorter, wider and more horizontal
nence of lateral process of malleus and loss of light reflex.
and thus may account for higher incidence of infections in
Tuning fork tests show conductive deafness.
this age group. Breast or bottle feeding in a young infant
in horizontal position may force fluids through the tube
2. STAGE OF PRESUPPURATION. If tubal occlusion is pro-
into the middle ear and hence the need to keep the infant
longed, pyogenic organisms invade tympanic cavity caus-
propped up with head a little higher. Swimming and diving
ing hyperaemia of its lining. Inflammatory exudate ap-
can also force water through the tube into the middle ear.
pears in the middle ear. Tympanic membrane becomes
congested.
2. VIA EXTERNAL EAR. Traumatic perforations of tym-
Symptoms. There is marked earache which may disturb
panic membrane due to any cause open a route to middle
sleep and is of throbbing nature. Deafness and tinnitus
ear infection.
are also present, but complained only by adults. Usually,
child runs high degree of fever and is restless.
3. BLOOD-BORNE. This is an uncommon route.
Signs. To begin with, there is congestion of pars tensa.
Leash of blood vessels appear along the handle of malleus
PREDISPOSING FACTORS and at the periphery of tympanic membrane imparting it -

a cart-wheel appearance. Later, whole of tympanic mem-

Anything that interferes with normal functioning of eus-
brane including pars flaccida becomes uniformly red.
tachian tube predisposes to middle ear infection. It could
Tuning fork tests will again show conductive type of
be:
hearing loss.
1. Recurrent attacks of common cold, upper respiratory
tract infections and exanthematous fevers like mea- 3. STAGE OF SUPPURATION. This is marked by formation
sles, diphtheria or whooping cough. of pus in the middle ear and to some extent in mastoid
2. Infections of tonsils and adenoids. air cells. Tympanic membrane starts bulging to the point
3. Chronic rhinitis and sinusitis. of rupture.
4. Nasal allergy. Symptoms. Earache becomes excruciating. Deafness in-
5. Tumours of nasopharynx, packing of nose or naso- creases, child may run fever of 102–103 °F. This may be
pharynx for epistaxis. accompanied by vomiting and even convulsions.
6. Cleft palate. Signs. Tympanic membrane appears red and bulging
with loss of landmarks. Handle of malleus may be en-
BACTERIOLOGY. Most common organisms in infants gulfed by the swollen and protruding tympanic mem-
and young children are Streptococcus pneumoniae (30%), brane and may not be discernible. A yellow spot may be
L

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 68 SECTION I — Diseases of Ear

seen on the tympanic membrane where rupture is im- 2. DECONGESTANT NASAL DROPS. Ephedrine nose drops
minent. In preantibiotic era, one could see a nipple-like (1% in adults and 0.5% in children) or oxymetazoline
protrusion of tympanic membrane with a yellow spot on (Nasivion) or xylometazoline (Otrivin) should be used to
its summit. Tenderness may be elicited over the mastoid relieve eustachian tube oedema and promote ventilation
antrum. of middle ear.
X-rays of mastoid will show clouding of air cells be-
cause of exudate. 3. ORAL NASAL DECONGESTANTS. Pseudoephedrine (Su-
dafed) 30 mg twice daily or a combination of decongest-
4. STAGE OF RESOLUTION. The tympanic membrane rup- ant and antihistaminic (Triominic) may achieve the same
tures with release of pus and subsidence of symptoms. In- result without resort to nasal drops which are difficult to
flammatory process begins to resolve. If proper treatment administer in children.
is started early or if the infection was mild, resolution
may start even without rupture of tympanic membrane. 4. ANALGESICS AND ANTIPYRETICS. Paracetamol helps to
Symptoms. With evacuation of pus, earache is relieved, relieve pain and bring down temperature.
fever comes down and child feels better.
Signs. External auditory canal may contain blood- 5. EAR TOILET. If there is discharge in the ear, it is dry-
tinged discharge which later becomes mucopurulent. mopped with sterile cotton buds and a wick moistened
& Usually, a small perforation is seen in anteroinferior with antibiotic may be inserted.
quadrant of pars tensa. Hyperaemia of tympanic mem-
brane begins to subside with return to normal colour and 6. DRY LOCAL HEAT. Helps to relieve pain.
landmarks.
7. MYRINGOTOMY. It is incising the drum to evacuate
5. STAGE OF COMPLICATION. If virulence of organism is pus and is indicated when (i) drum is bulging and there is
high or resistance of patient poor, resolution may not acute pain, (ii) there is an incomplete resolution despite
&

take place and disease spreads beyond the confines of antibiotics when drum remains full with persistent con-
&

middle ear. It may lead to acute mastoiditis, subperiosteal ductive hearing loss and (iii) there is persistent effusion
abscess, facial paralysis, labyrinthitis, petrositis, extra- beyond 12 weeks.
dural abscess, meningitis, brain abscess or lateral sinus All cases of acute suppurative otitis media should be
thrombophlebitis. carefully followed till tympanic membrane returns to its
normal appearance and conductive hearing loss disap-
TREATMENT pears (Figure 10.1).

1. ANTIBACTERIAL THERAPY (TABLE 10.1). It is indi-

cated in all cases with fever and severe earache. As the ACUTE NECROTIZING OTITIS MEDIA
most common organisms are S. pneumoniae and H. influ-
enzae, the drugs which are effective in acute otitis media It is a variety of acute suppurative otitis media, often seen
are ampicillin (50 mg/kg/day in four divided doses) and &
in children suffering from measles, scarlet fever or influ-
amoxicillin (40 mg/kg/day in three divided doses). Those enza. Causative organism is β-haemolytic streptococcus.
allergic to these penicillins can be given cefaclor, co-tri- There is rapid destruction of whole of tympanic mem-
moxazole or erythromycin. In cases where β-lactamase- brane with its annulus, mucosa of promontory, ossicular
producing H. influenzae or M. catarrhalis are isolated, chain and even mastoid air cells. There is profuse otor-
antibiotics like amoxicillin clavulanate, augmentin, ce- rhoea. In these cases, healing is followed by fibrosis or
furoxime axetil or cefixime may be used. Antibacterial ingrowth of squamous epithelium from the meatus (sec-
therapy must be continued for a minimum of 10 days, ondary acquired cholesteatoma).
till tympanic membrane regains normal appearance and Treatment is early institution of antibacterial therapy.
hearing returns to normal. Early discontinuance of ther- It is continued for at least 7–10 days, even if response is
apy with relief of earache and fever, or therapy given in seen early. Cortical mastoidectomy may be indicated if
inadequate doses may lead to secretory otitis media and medical treatment fails to control or the condition gets
residual hearing loss. complicated by acute mastoiditis.

TABLE 10.1 ANTIBACTERIAL AGENTS AND THEIR DOSAGE IN ACUTE OTITIS MEDIA
Drug Trade names Total daily dose* Divided dose
Amoxicillin Novamox, Biomox 40 mg/kg 3
Ampicillin Biocillin 50–100 mg/kg 4
Co-amoxiclav Augmentin, Enhancin 40 mg/kg 2–3
Erythromycin Emycin, Althrocin 30–50 mg/kg 4
Cefaclor (II generation) Keflor, Distaclor 20 mg/kg 2–3
Cefixime (III generation) Taxim-0, Biotax-0 8 mg/kg 1 or 2
Cefpodoxime proxetil Cepodem, Cefoprox 10 mg/kg (max. 400 mg/day) 2
Ceftibuten (III generation) Procadax 9 mg/kg 1
Co-trimoxazole (Trimethoprim + Sulfamethoxazole) Ciplin, Septran 8 mg (TMP) + 40 mg (SMZ)/kg 2

*Follow the dosage and instructions of the manufacturer.

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 and sorons Semetic all
↑ no of mucus

Chapter 10 — Disorders of Middle Ear 69

(c) Chronic tonsillitis. Enlarged tonsils mechanically

obstruct the movements of soft palate and interfere
-

with the physiological opening of eustachian tube.

(d)
g Benign and malignant tumours of nasopharynx. This
cause should always be excluded in unilateral serous
otitis media in an adult.
(e) Palatal defects, e.g. cleft palate, palatal paralysis.
-

*
2. ALLERGY. Seasonal or perennial allergy to inhalants or
foodstuff is common in children. This not only obstructs
eustachian tube by oedema but may also lead to increased
-
secretory activity as middle ear mucosa acts as a shock
organ in such cases.
Y
3. UNRESOLVED OTITIS MEDIA. Inadequate antibiotic
therapy in acute suppurative otitis media may inactivate

unresolved infection but fail to resolve it completely. Low-grade in-

fection lingers on. This acts as stimulus for mucosa to se-
crete more fluid. The number of goblet cells and mucous
OM
glands also increase. Recent increase in the incidence of
this disease seems to be due to this factor. -

4. VIRAL INFECTIONS. Various adeno- and rhinoviruses

of upper respiratory tract may invade middle ear mucosa
and stimulate it to increased secretory activity.

All opaque
~
I

CLINICAL FEATURES
yello
1. SYMPTOMS. The disease affects children of 5–8 years of
age. The symptoms include:
Figure 10.1. Treatment of acute otitis media.
(a) Hearing loss. This is the presenting and sometimes
the only symptom. It is insidious in onset and rarely
OTITIS MEDIA WITH EFFUSION exceeds 40 dB. Deafness may pass unnoticed by the
-
parents and may be accidentally discovered during
SYN. SEROUS OTITIS MEDIA, SECRETORY
-
audiometric screening tests.
OTITIS MEDIA, MUCOID OTITIS MEDIA, (b) Delayed and defective speech. Because of hearing loss,
“GLUE EAR”
-
* development of speech is delayed or defective.
-
F (c) Mild earaches. There may be history of upper respira-
This is an insidious condition characterized by accumula- -
tory tract infections with mild earaches.
tion of nonpurulent effusion in the middle ear cleft. Of-
ten the effusion is thick and viscid but sometimes it may 2. OTOSCOPIC FINDINGS. Tympanic membrane is often
be thin and serous. The fluid is nearly sterile. The condi- dull and opaque with loss of light reflex. It may appear
-
--
tion is commonly seen in school-going children. yellow, grey or bluish in colour.

Ju
Thin leash of blood vessels may be seen along the han-
PATHOGENESIS dle of malleus or at the periphery of tympanic membrane
and differs from marked congestion of acute suppurative
Two main mechanisms are thought to be responsible.
otitis media. congestion
Tympanic membrane may show varying degree of re-
1. MALFUNCTIONING OF EUSTACHIAN TUBE. Eustachian
tube fails to aerate the middle ear and is also unable to traction. Sometimes, it may appear full or slightly bulging
drain the fluid. in its posterior part due to effusion.

2. INCREASED SECRETORY ACTIVITY OF MIDDLE EAR

Fluid level and air bubbles may be seen when fluid is
thin and tympanic membrane transparent (Figure 10.2).
#
MUCOSA. Biopsies of middle ear mucosa in these cases Mobility of the tympanic membrane is restricted.
have confirmed increase in number of mucus or serous-
secreting cells.
HEARING TESTS
AETIOLOGY 1. Tuning fork tests show conductive hearing loss.
2. Audiometry. There is conductive hearing loss of 20–
40 dB. Sometimes, there is associated sensorineural
1. MALFUNCTIONING OF EUSTACHIAN TUBE. The causes are:
hearing loss due to fluid pressing on the round win-
(a) Adenoid hyperplasia. dow membrane. This disappears with evacuation of
(b) Chronic rhinitis and sinusitis. fluid.

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70 SECTION I — Diseases of Ear

Figure 10.3. To aspirate thick mucus, two incisions may be required

in the tympanic membrane.
Scan to play Myringotomy.

Figure 10.2. Otitis media with effusion. Note appearance of bubbles

in the middle ear on Valsalva manoeuvre.

3. Impedance audiometry. It is an objective test useful

in infants and children. Presence of fluid is indicated
by reduced compliance and flat curve with a shift to
negative side.
4. X-ray mastoids. There is clouding of air cells due to
fluid.

TREATMENT
The aim of treatment is removal of fluid and prevention
of its recurrence.

1. Medical Figure 10.4. Grommet in the tympanic membrane (A and B).

(A) DECONGESTANTS. Topical decongestants in the form
of nasal drops, sprays or systemic decongestants help to anterosuperior quadrant to aspirate thick, glue-like secre-
relieve oedema of eustachian tube. tions (Figure 10.3 ) on “beer-can” principle.

(B) ANTIALLERGIC MEASURES. Antihistaminics or some- (B) GROMMET INSERTION. If myringotomy and aspiration
times steroids may be used in cases of allergy. If possible, combined with medical measures have not helped and
allergen should be found and desensitization done. fluid recurs, a grommet is inserted to provide continued
aeration of middle ear (Figure 10.4). It is left in place for
(C) ANTIBIOTICS. They are useful in cases of upper res- weeks or months or till it is spontaneously extruded.
piratory tract infections or unresolved acute suppurative
otitis media. (C) TYMPANOTOMY OR CORTICAL MASTOIDECTOMY. It is
sometimes required for removal of loculated thick fluid
(D) MIDDLE EAR AERATION. Patient should repeatedly or other associated pathology such as cholesterol granu-
perform Valsalva manoeuvre. Sometimes, politzerization loma.
or eustachian tube catheterization has to be done. This
helps to ventilate middle ear and promote drainage of (D) SURGICAL TREATMENT OF CAUSATIVE FACTOR. Ade-
fluid. Children can be given chewing gum to encourage noidectomy, tonsillectomy and/or wash-out of maxillary
repeated swallowing which opens the tube. antra may be required. This is usually done at the time of
myringotomy.
2. Surgical
When fluid is thick and medical treatment alone does not BIOFILM
help, fluid must be surgically removed.
It is a protective mechanism of bacteria which ensures
(A) MYRINGOTOMY AND ASPIRATION OF FLUID. An inci- their survival and propagation. Bacteria first adhere to an
sion is made in tympanic membrane and fluid aspirat- organic or inorganic material, and then secrete a protec-
ed with suction. Thick mucus may require installation tive layer of complex polysaccharides. This layer permits
of saline or a mucolytic agent like chymotrypsin solu- diffusion of nutrients into the bacterial cells and exit to
tion to liquefy mucus before it can be aspirated. Some- bacterial excretory products but prevents the action of
times, two incisions are made in the tympanic mem- white blood cells, antibodies and antibiotics on the bac-
brane, one in the anteroinferior and the other in the terial cell. Small proportions of bacterial colonies can also

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 Chapter 10 — Disorders of Middle Ear 71

detach and set up new colonies. Biofilms are responsi- 6 bouts in 1 year, insertion of a tympanostomy tube is
ble for bacterial resistance and persistance of infection. recommended.
In ENT, they are implicated in chronic otitis media with 4. Adenoidectomy with or without tonsillectomy.
effusion, chronic rhinosinusitis, and tonsil and adenoid 5. Management of inhalant or food allergy.
infections. They also form on tympanostomy tubes, st-
ents and catheters kept for a long time. Biofilm formation
can be prevented by antibiotic-coated tubes and stents
and an early removal of tubes and stents, if no longer AERO-OTITIS MEDIA (OTITIC
required. BAROTRAUMA)
It is a nonsuppurative condition resulting from failure of
SEQUELAE OF CHRONIC SECRETORY OTITIS eustachian tube to maintain middle ear pressure at ambi-
MEDIA ent atmospheric level. The usual cause is rapid descent
during air flight, underwater diving or compression in
1. ATROPHIC TYMPANIC MEMBRANE AND ATELECTASIS
pressure chamber.
OF THE MIDDLE EAR. In prolonged effusions, there is dis-
solution of fibrous layer of tympanic membrane. It be-
comes thin and atrophic and retracts into the middle ear. MECHANISM
Eustachian tube allows easy and passive egress of air from
2. OSSICULAR NECROSIS. Most commonly, long process
middle ear to the pharynx if middle ear pressure is high.
of incus gets necrosed. Sometimes, stapes superstructure
In the reverse situation, where nasopharyngeal air pres-
also gets necrosed. This increases the conductive hearing
sure is high, air cannot enter the middle ear unless tube is
loss to more than 50 dB.
actively opened by the contraction of muscles as in swal-
lowing, yawning or Valsalva manoeuvre. When atmos-
3. TYMPANOSCLEROSIS. Hyalinized collagen with chalky
pheric pressure is higher than that of middle ear by criti-
deposits may be seen in tympanic membrane, around the
cal level of 90 mm Hg, eustachian tube gets “locked,” i.e.
ossicles or their joints, leading to their fixation.
soft tissues of pharyngeal end of the tube are forced into
its lumen. In the presence of eustachian tube oedema,
4. RETRACTION POCKETS AND CHOLESTEATOMA. Thin
even smaller pressure differentials cause “locking” of the
atrophic part of pars tensa may get invaginated to form
tube. Sudden negative pressure in the middle ear causes
retraction pockets or cholesteatoma. Similar pockets may
retraction of tympanic membrane, hyperaemia and en-
be seen in the attic region.
gorgement of vessels, transudation and haemorrhages.
Sometimes, though rarely, there is rupture of labyrin-
5. CHOLESTEROL GRANULOMA. This is due to stasis of se-
thine membranes with vertigo and sensorineural hearing
cretions in middle ear and mastoid.
loss.

CLINICAL FEATURES
RECURRENT ACUTE OTITIS MEDIA
Severe earache, hearing loss and tinnitus are common
Infants and children between the age of 6 months and complaints. Vertigo is uncommon. Tympanic membrane
6 years may get recurrent episodes of acute otitis media. appears retracted and congested. It may get ruptured.
Such episodes may occur four to five times in a year. Usu- Middle ear may show air bubbles or haemorrhagic ef-
ally, they occur after acute upper respiratory infection, fusion. Hearing loss is usually conductive but sensorineu-
the child being free of symptoms between the episodes. ral type of loss may also be seen.
Recurrent middle infections may sometimes be superim-
posed upon an existing middle ear effusion. Sometimes,
TREATMENT
the underlying cause is recurrent sinusitis, velopharyn-
geal insufficiency, hypertrophy of adenoids, infected ton- The aim is to restore middle ear aeration. This is done by
sils, allergy and immune deficiency. Feeding the babies in catheterization or politzerization. In mild cases, decon-
supine position without propping up the head may also gestant nasal drops or oral nasal decongestant with anti-
cause the milk to enter the middle ear directly that can histaminics are helpful. In the presence of fluid or failure
lead to middle ear infection. of the above methods, myringotomy may be performed
Management of such children involves: to “unlock” the tube and aspirate the fluid.
1. Finding the cause and eliminating it, if possible.
2. Antimicrobial prophylaxis. Amoxicillin (20 mg/kg for PREVENTION
3–6 months) or sulfisoxazole have been used but they
Aero-otitis can be prevented by the following measures:
prevent only 1–2 bouts of otitis media in a year and
have the disadvantage of creating antimicrobial resist- 1. Avoid air travel in the presence of upper respiratory
ance or hypersensitivity reaction and thus not pre- infection or allergy.
ferred by many in favour of early insertion of tympa- 2. Swallow repeatedly during descent. Sucking sweets or
nostomy tubes. chewing gum is useful.
3. Myringotomy and insertion of tympanostomy tube. If the 3. Do not permit sleep during descent as number of swal-
child has 4 bouts of acute otitis media in 6 months or lows normally decrease during sleep.

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72 SECTION I — Diseases of Ear

4. Autoinflation of the tube by Valsalva should be per- 6. In recurrent barotrauma, attention should be paid to
formed intermittently during descent. nasal polyps, septal deviation, nasal allergy and chron-
5. Use vasoconstrictor nasal spray and a tablet of anti- ic sinus infections.
histaminic and systemic decongestant, half an hour
before descent in persons with previous history of this
episode.

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 in the Middle Ear .
fre of 1st
Chapter 11 a
- Matrix o

Cho/esteatoma and Chronic

·

o Keratin
Otitis Media ↓ in
o

debris
skin place --
-

verong

Keratoma
Epidermoni ,

retraction pockets (Figure 11.2) (Wittmaack's theory). #

CHOLESTEATOMA
The outer surface of tympanic membrane is lined by
Normally, middle ear cleft is lined by different types of stratified squamous epithelium which after invagina-
epithelium in different regions: ciliated columnar in the tion forms the matrix of cholesteatoma and lays down
anterior and inferior part, cuboidal in the middle part keratin in the pocket.
and pavement-like in the attic. The middle ear is no- 3. Basal cell hyperplasia (Ruedi's theory). The basal cells of
where lined by keratinizing squamous epithelium. It is germinal layer of skin proliferate under the influence
the presence of latter type of epithelium in the middle of infection and lay down keratinizing squamous epi-
ear or mastoid that constitutes a cholesteatoma. In other thelium.
words, cholesteatoma is a "skin in the wrong place. " The 4. Epithelial invasion (Habermann's theory). The epithe-
term cholesteatoma is a misnomer because it neither con- lium from the meatus or outer drum surface grows
-

tains cholesterol crystals nor it is a tumour to merit the into the middle ear through a pre-existing perforation
suffix
- "oma." However, the term has been retained be- especially of the marginal type where part of annulus
cause of its wider usage. tympanicus has already been destroyed.
Essentially, cholesteatoma consists of two parts: (i) the 5. Metaplasia (Sade's theory). Middle ear mucosa, like res-
matrix, which is made up of keratinizing squamous epi- piratory mucosa elsewhere, undergoes metaplasia due
-

thelium resting on a thin stroma of fibrous tissues and (ii) to repeated infections and transforms into squamous
a central white mass, consisting of keratin debris produced epithelium.
by the matrix (Figure 11.1 [!]). For this reason, it has also
been named epidermosis or keratoma.
-

ORIGIN OF CHOLESTEATOMA
Genesis of cholesteatoma is a matter of debate. Any theo-
ry of its genesis must explain how squamous epithelium
appeared in the middle ear cleft. The various views ex-
pressed are:
1. Presence of congenital cell rests. ·
2. Invagination of tympanic membrane from the attic Retraction pocket
or posterosuperior part of pars tensa in the form of
-

Fibrous_
stroma

m Basal cell hyperplasia

mass

Epithelial invasion through posterosuperior perforation

Figure 11.1. Schematic structure of a cholesteatoma. Figure 11.2. Genesis of a cholesteatoma.
[!] Scan to play Origin of Cholesteatoma.
73
 I
-
/

-
74 SECTION I — Diseases of Ear

CLASSIFICATION OF CHOLESTEATOMA -(b) Basal cell hyperplasia. There is proliferation of the ba-
(FIGURE 11.3) sal layer of pars flaccida induced by subclinical child-
hood infections. Expanding cholesteatoma then breaks
The cholesteatoma is classified into:
- through pars flaccida forming an attic perforation.
1. Congenital (c) Squamous metaplasia. Normal pavement epithelium
2. Acquired, primary of attic undergoes metaplasia, keratinizing squa-
3. Acquired, secondary mous epithelium due to subclinical infections. Such
* a change has also been demonstrated in cases of otitis
=
media with effusion.
1. CONGENITAL CHOLESTEATOMA. It arises from the em-
bryonic epidermal cell rests in the middle ear cleft or
temporal bone. Congenital cholesteatoma occurs at three
-
3. SECONDARY ACQUIRED CHOLESTEATOMA. In these
important sites: middle ear, petrous apex and the cerebel- cases, there is already a pre-existing perforation in pars
lopontine angle, and produces symptomatology depend- tensa. This is often associated with posterosuperior mar-
ing on its location. ginal perforation or sometimes large central perforation.
A middle ear congenital cholesteatoma presents as a Theories on its genesis include:
white mass behind an intact tympanic membrane and (a) Migration of squamous epithelium. Keratinizing squa-
causes conductive
-
hearing loss. It may sometimes be mous epithelium of external auditory canal or outer
discovered on routine examination of children or at the surface of tympanic membrane migrates through the
time of myringotomy. perforation into the middle ear. Perforations, involv-
It may also spontaneously rupture through the tym- ing tympanic annulus as in acute necrotizing otitis
panic membrane and present with a discharging ear in- media, are more likely to allow in-growth of squa-
distinguishable from a case of chronic suppurative otitis mous epithelium.
media. (b) Metaplasia. Middle ear mucosa undergoes metaplasia
due to repeated infections of middle ear through the
2. PRIMARY ACQUIRED CHOLESTEATOMA (FIGURE 11.2). It pre-existing perforation.
is called primary as there is no history of previous otitis
media or a pre-existing perforation. Theories on its gen-
esis are:
EXPANSION OF CHOLESTEATOMA
AND DESTRUCTION OF BONE #
(a) Invagination of pars flaccida. Persistent negative pres-
-

- sure in the attic causes a retraction pocket which ac- Once cholesteatoma enters the middle ear cleft, it invades
cumulates keratin debris. When infected, the keratin the surrounding structures, first by following the path of
mass expands towards the middle ear. Thus, attic per- least resistance, and then by enzymatic bone destruction.
foration is in fact the proximal end of an expanding An attic cholesteatoma may extend backwards into the
invaginated sac. aditus, antrum and mastoid; downwards into the meso-
tympanum; medially, it may surround the incus and/or
head of malleus. -
Cholesteatoma has the property to destroy bone. It
may cause destruction of ear ossicles, erosion of bony lab-
> Karate
- 2 -
yrinth, canal of facial nerve, sinus plate or tegmen tympa-
debris ni and thus cause several complications. Bone destruction
by cholesteatoma has been attributed to various enzymes
superplasia such as collagenase, acid phosphatase and proteolytic en-
zymes, liberated by osteoclasts and mononuclear inflam-
matory cells, seen in association with cholesteatoma. The
earlier theory that cholesteatoma causes destruction of
bone by pressure necrosis is not accepted these days.

-
Y CHRONIC SUPPURATIVE OTITIS MEDIA E
Chronic suppurative otitis media (CSOM) is a long-stand-
V ing infection of a part or whole of the middle ear cleft
characterized by ear discharge and a permanent perfora-
-
tion. A perforation becomes permanent when its edges
-

are covered by squamous epithelium and it does not heal

* # spontaneously. A permanent perforation can be likened
F
to an epithelium-lined fistulous track (Figure 11.4).

EPIDEMIOLOGY
Incidence of CSOM is higher in developing countries be-
Figure 11.3. Genesis of primary and secondary cholesteatomas. cause of poor socioeconomic standards, poor nutrition

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 urafe
dangerous
M .

AA ,
Chapter 11 — Cholesteatoma and Chronic Otitis Media 75
fore
Partit
granulation

Deion
·
safer

-aufal
-

Figure 11.4. Retracted tympanic membrane with attic retraction pocket (arrow) due to negative pressure in the middle ear.

and lack of health education. It affects both sexes and all

2. Ascending infections via the eustachian tube. Infec-
age groups. In India, the overall prevalence rate is 46 andtion from tonsils, adenoids and infected sinuses may
-
-

16 persons per thousand in rural and urban population, be responsible for persistent or recurring otorrhoea. As-
respectively. It is also the single most important cause ofcending infection to middle ear occurs more easily in
hearing impairment in rural population. the presence of infection.
& tubotympanca3. Persistent mucoid otorrhoea
z
*
is sometimes the result of
cantal allergy to ingestants such as milk, eggs, fish, etc.
-

TYPES OF CSOM -
-

Clinically, it is divided into two types: Pathology

The tubotympanic disease remains localized to the mu- -

1. TUBOTYMPANIC. Also called the safe or benign type; it cosa and, that too, mostly to anteroinferior part of the
-

involves anteroinferior part of middle ear cleft, i.e. eus- middle ear cleft. Like any other chronic infection, the
tachian tube and mesotympanum and is associated with processes of healing and destruction go hand in hand
a central perforation. There is no risk of serious complica- and either of them may take advantage over the other,
tions. depending on the virulence of organism and resistance
of the patient. Thus, acute exacerbations are not uncom-
2. ATTICOANTRAL. Also called unsafe or dangerous type; mon. The pathological changes seen in this type of CSOM
it involves posterosuperior part of the cleft (i.e. attic, are:
antrum and mastoid) and is associated with an attic or
a marginal perforation. The disease is often associated 1. PERFORATION OF PARS TENSA. It is a central perfora-
with a bone-eroding process such as cholesteatoma, tion and its size and position varies (Figure 11.5).
granulations or osteitis. Risk of complications is high in
this variety. 2. MIDDLE EAR MUCOSA. It may be normal when dis-
Table 11.1 shows differences between the two types of ease is quiescent or inactive. It is oedematous and velvety
CSOM. when disease is active.
-
=

pale
3. POLYP. A polyp is a smooth mass of oedematous and
A. TUBOTYMPANIC TYPE inflamed mucosa which has protruded through a perfora-
Aetiology tion and presents in the external canal. It is usually pale
in contrast to pink, fleshy polyp seen in atticoantral dis-
The disease starts in childhood and is therefore common ease (Figure 11.6).
-
in that age group.
1. It is the sequela of acute otitis media usually follow- 4. OSSICULAR CHAIN. It is usually intact and mobile but
-

ing exanthematous fever and leaving behind a large may show some degree of necrosis, particularly of the
-
long process of incus.
Bcentral perforation.
-

TABLE 11.1 DIFFERENCES BETWEEN TUBOTYMPANIC AND ATTICOANTRAL TYPE OF CSOM

Tubotympanic or safe type Atticoantral or unsafe type
Discharge Profuse, mucoid, odourless Scanty, purulent, foul smelling
Perforation Central Attic or marginal
Granulations Uncommon Common
Polyp Pale Red and fleshy
Cholesteatoma Absent Present
Complications Rare Common
Audiogram Mild to moderate conductive deafness Conductive or mixed deafness

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76 SECTION I — Diseases of Ear

Y V

Figure 11.5. Perforation of tympanic membrane. Note: Attic and posterosuperior marginal perforation are seen in dangerous type of CSOM and
are often associated with a cholesteatoma. Stratified squamous epithelium from the external auditory canal can grow into the middle ear in any
type of marginal perforation by immigration and form a cholesteatoma. Therefore, all marginal perforations are considered dangerous. Central
perforations are considered safe as cholesteatomas are usually not associated with them.

Figure 11.6. (A) Polyp in the ear canal. (B) Schematic illustration of a polyp arising from the promontory passing through the perforation and
presenting in the ear canal.

Y
5. TYMPANOSCLEROSIS. It is hyalinization and subsequent tensa with inflammation of mucosa and mucopurulent
calcification of subepithelial connective tissue. It is seen discharge. It is called “inactive” when there is a perma-
in remnants of tympanic membrane or under the muco- nent perforation of pars tensa but middle ear mucosa is
sa of middle ear. It is seen as white chalky deposit on the
-
not inflamed and there is no discharge. Permanent perfo-
promontory, ossicles, joints, tendons and oval and round ration implies that squamous epithelium on the external
windows. Tympanosclerotic masses may interfere with the surface of pars tensa and mucosa lining its inner surface
mobility of these structures and cause conductive deafness. have fused across its edge. Healed chronic otitis media is the
condition when tympanic membrane has healed (usually
6. FIBROSIS AND ADHESIONS. They are the result of heal- O by two layers), is atrophic and easily retracted if there is
O
ing process and may further impair mobility of ossicular negative pressure in the middle ear. Healed otitis media
chain or block the eustachian tube. may also have patches of tympanosclerosis in tympanic
membrane, or in middle ear involving promontory, os-
Bacteriology sicles, tendons of stapedius and tensor tympani. Fibrotic
Pus culture in both types of- aerobic and
-
anaerobic CSOM tissue may appear in middle ear. It is always associated
may show multiple organisms. Common aerobic organ- with some degree of conductive hearing loss.
⑧
isms are6 Pseudomonas aeruginosa, Proteus, Escherichia coli ⑧ Atticoantral disease has been called squamosal disease
D
and Staphylococcus aureus, while anaerobes include Bacte- of middle ear. It may be “inactive” when there are retrac-
roides fragilis and anaerobic Streptococci. tion pockets in pars tensa (usually the posterosuperior
region) or pars flaccida. There is no discharge but there
Alternative Classification of Chronic is a possibility of squamous debris in retraction pockets
Otitis Media to become infected and start discharging. Some retrac-
Tubotympanic disease of middle ear is a mucosal disease tion pockets are shallow and self-cleansing. “Active” squa-
with no evidence of invasion of squamous epithelium. mosal disease of middle ear implies presence of cholestea-
It is called “active” when there is a perforation of pars toma of posterosuperior region of pars tensa or in the pars
=> -

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 Chapter 11 — Cholesteatoma and Chronic Otitis Media 77

Figure 11.7. Classification of chronic otitis media.

O
-

Figure 11.8. Types of perforations seen in the tympanic membrane in CSOM.

flaccida. It erodes bone, forms granulation tissue and has 4. MIDDLE EAR MUCOSA. It is seen when the perfora-
purulent offensive discharge (Figure 11.7). tion is large. Normally, it is pale pink and moist; when
inflamed it looks red, oedematous and swollen. Occasion-
Clinical Features * ally, a polyp may be seen.
1. EAR DISCHARGE. It is nonoffensive, mucoid or mucop-
-

urulent, constant or intermittent. The discharge appears

-

mostly at time of upper respiratory tract infection or on

accidental entry of water into the ear.

2. HEARING LOSS. It is conductive type; severity varies

but rarely exceeds 50 dB. Sometimes, the patient reports
of a paradoxical effect, i.e. hears better in the presence of
discharge than when the ear is dry. This is due to “round
window shielding effect” produced by discharge which
#
helps to maintain phase differential. In the dry ear with
perforation, sound waves strike both the oval and round
windows simultaneously, thus cancelling each other’s ef-
fect (see physiology of hearing).
In long standing cases, cochlea may suffer damage due
to absorption of toxins from the oval and round windows
and hearing loss becomes mixed type.

3. PERFORATION. Always central, it may lie anterior,

=
posterior or inferior to the handle of malleus. It may be
small, medium or large or extending up to the annulus,
i.e. subtotal (Figures 11.8 and 11.9). Figure 11.9. A large central perforation.

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 · aural forlet
.
78 SECTION I — Diseases of Ear · Ear drop-polymym
Assessment done three or four times a day. Acid pH helps to eliminate
1. EXAMINATION UNDER MICROSCOPE (FIGURE 11.10). It pseudomonas infection, and irrigations with 1.5% acetic
-
is essential in every case and provides useful informa- acid are useful.
tion regarding presence of granulations, in-growth of Care should be taken as ear drops are likely to cause
squamous epithelium from the edges of perforation, sta- maceration of canal skin, local allergy, growth of fungus
tus of ossicular chain, tympanosclerosis and adhesions. or resistance of organisms. Some ear drops are potentially
An ear which appears dry may show hidden discharge ototoxic.
under the microscope. Rarely, cholesteatoma may coex-
ist with a central perforation and can be seen under a 3. SYSTEMIC ANTIBIOTICS. They are useful in acute ex-
-

microscope. acerbation of chronically infected ear, otherwise role

of systemic antibiotics in the treatment of CSOM is
limited.
V
4. PRECAUTIONS. Patients are instructed to keep water
out of the ear during bathing, swimming and hair wash.
Rubber inserts can be used. Hard nose blowing can also
push the infection from nasopharynx to middle ear and
should be avoided.
-
5. TREATMENT OF CONTRIBUTORY CAUSES. Attention
should be paid to treat concomitantly infected tonsils,
adenoids, maxillary antra and nasal allergy.
- -
=

⑧ 6. SURGICAL TREATMENT. Aural polyp or granulations, *

if present, should be removed before local treatment
with antibiotics. It will facilitate ear toilet and permit ear
drops to be used effectively. An aural polyp should never be
-

avulsed as it may be arising from the stapes, facial nerve or

Figure 11.10. Examination of the ear under a microscope.
horizontal canal and thus lead to facial paralysis or laby-
rinthitis.

2. AUDIOGRAM. It gives an assessment of degree of hear- 7. RECONSTRUCTIVE SURGERY. Once ear is dry, myrin- Ou
-
ing loss and its type. Usually, the loss is conductive but a O
goplasty with or without ossicular reconstruction can be
sensorineural element may be present. done to restore hearing. Closure of perforation will also
check repeated infection from the external canal.
3. CULTURE AND SENSITIVITY OF EAR DISCHARGE. It
-
helps to select proper antibiotic ear drops. B. ATTICOANTRAL TYPE
O
-

4. MASTOID X-RAYS/CT SCAN TEMPORAL BONE. Mastoid It involves posterosuperior part of middle ear cleft (attic,
is usually sclerotic
-
but may be pneumatized with cloud- antrum, posterior tympanum and mastoid) and is associ-
- -

ing of air cells. There is no evidence of bone destruction. ated with cholesteatoma, which, because of its bone erod-
Presence of bone destruction is a feature of atticoantral ing properties, causes risk of serious complications. For
disease. this reason, the disease is also called unsafe or dangerous
type.
Treatment Aetiology
The aim is to control infection and eliminate ear dis- Aetiology of atticoantral disease is same as of cholestea-
charge and at a later stage to correct the hearing loss by toma and has been discussed earlier. It is seen in sclerotic
surgical means. mastoid, and whether the latter is the cause or effect of
# disease is not yet clear.
1. AURAL TOILET. Remove all discharge and debris from
the ear. It can be done by dry mopping with absorbent Pathology
cotton buds, suction clearance under microscope or irri- Atticoantral diseases are associated with the following
gation (not forceful syringing) with sterile normal saline. pathological processes:
Ear must be dried after irrigation.
1. CHOLESTEATOMA *
2. EAR DROPS. Antibiotic ear drops containing neomy-
V
cin, polymyxin, chloromycetin or gentamicin are used. 2. OSTEITIS AND GRANULATION TISSUE. Osteitis involves
They are combined with steroids which have local anti- outer attic wall and posterosuperior margin of the tym-
&

inflammatory effect. To use ear drops, patient lies down panic ring. A mass of granulation tissue surrounds the
with the diseased ear up, antibiotic drops are instilled and area of osteitis and may even fill the attic, antrum, poste-
then intermittent pressure applied on the tragus for an- rior tympanum and mastoid. A fleshy red polypus may be
tibiotic solution to reach the middle ear. This should be seen filling the meatus.

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 Chapter 11 — Cholesteatoma and Chronic Otitis Media 79

V 3. OSSICULAR NECROSIS. It is common in atticoantral dis-

ease. Destruction may be limited to the long process of
incus or may also involve stapes superstructure, handle
of malleus or the entire ossicular chain. Therefore, hear-
ing loss is always greater than in disease of tubotympanic
type. Occasionally, the cholesteatoma bridges the gap
caused by the destroyed ossicles and hearing loss is not
apparent (cholesteatoma hearer).

Y4. CHOLESTEROL GRANULOMA. It is a mass of granulation

tissue with foreign body giant cells surrounding the cho-
lesterol crystals. It is a reaction to long-standing retention
of secretions or haemorrhage, and may or may not coex-
ist with cholesteatoma. When present in the mesotympa-
num, behind an intact drum, the latter appears blue.

Bacteriology
Same as in tubotympanic type.

Symptoms
1. EAR DISCHARGE. Usually-- scanty, but always foul-
smelling due to bone destruction. Discharge may be so
Z
scanty that the patient may not even be aware of it. Total
cessation of discharge from an ear which has been active
till recently should be viewed seriously, as perforation in
these cases might be sealed by crusted discharge, inflam-
matory mucosa or a polyp, obstructing the free flow of
discharge. Pus, in these cases, may find its way internally
and cause complications.

2. HEARING LOSS. Hearing is normal when ossicular

chain
-
is intact or when cholesteatoma, having destroyed
the ossicles, bridges the gap caused by destroyed ossicles
(cholesteatoma hearer). Hearing loss is mostly conductive
but sensorineural element may be added. =

3. BLEEDING. It may occur from granulations or the pol-

yp when cleaning the ear. -

Signs Figure 11.11. (A) Attic perforation. (B) Case with double perforation
O
1. PERFORATION. It is either attic or posterosuperior mar-
ginal type (Figure 11.11). A small attic perforation may
(1) in the pars tensa posterior to the handle of malleus and (2) in the
attic area with destruction of the lateral attic wall (arrows).
be missed due to presence of a small amount of crusted
discharge. Sometimes, the area of perforation is masked
can be lifted from the promontory with suction tip. It
by a small granuloma.
also balloons up when N2O is used during anaesthe-
sia. Tympanic membrane is thin because its collagen-
~2. RETRACTION POCKET. An invagination of tympanic
ous middle layer has been absorbed due to prolonged
membrane is seen in the attic or posterosuperior area of
retraction. In these cases long process of incus and
pars tensa. Degree of retraction and invagination varies.
stapes superstructure are absorbed. Placement of a
In early stages, pocket is shallow and self-cleansing but
ventilation tube helps to restore the position of tym-
later when pocket is deep, it accumulates keratin mass
panic membrane.
and gets infected.
(d) Stage IV. Also called adhesive otitis media. Tympanic
Stages of retraction pockets. There are four stages of tym-
membrane is very thin and wraps the promontory and
panic membrane retraction.
ossicles. There is no middle ear space, mucosal lining
(a) Stage I. Tympanic membrane is retracted but does not of the middle ear is absent and tympanic membrane
contact the incus. It is a mild form of retraction. gets adherent to the promontory. Retraction pockets
(b) Stage II. Tympanic membrane is retracted deep and are formed which may collect keratin plugs and form
contacts the incus; middle ear mucosa is not affected. cholesteatoma. Erosion of the long process of incus
(c) Stage III. Also called middle ear atelectasis. Tympanic and stapes superstructure is common in such cases.
membrane comes to lie on the promontory and ossi-
cles. Middle ear space is totally or partially obliterated O3. CHOLESTEATOMA. Pearly-white flakes of cholesteato-
but middle ear mucosa is intact. Tympanic membrane ma can be sucked from the retraction pockets. Suction

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80 SECTION I — Diseases of Ear

clearance and examination under operating microscope 7. IRRITABILITY AND NECK RIGIDITY. (meningitis).
forms an important part of the clinical examination and
assessment of any type of CSOM. 8. DIPLOPIA. (Gradenigo syndrome) petrositis.

Assessment - 9. ATAXIA. (LABYRINTHITIS OR CEREBELLAR ABSCESS).

1. EXAMINATION UNDER MICROSCOPE. All patients of
chronic middle early disease should be examined un- 10. ABSCESS ROUND THE EAR. (mastoiditis).
der microscope (Figure 11.9). It may reveal presence It is not uncommon for a patient of CSOM, residing in
of cholesteatoma, its site and extent, evidence of bone a far-flung village, where medical facilities are poor, to go
destruction, granuloma, condition of ossicles and pockets to a doctor for the first time, presenting with complica-
of discharge. tions. It then demands urgent attention and emergency
- medical or surgical treatment.
2. TUNING FORK TESTS AND AUDIOGRAM. They are essen-
tial for preoperative assessment and to confirm the degree Treatment
and type of hearing loss. 1. SURGICAL. It is the mainstay of treatment. Primary aim
- in surgical treatment is to remove the disease and render
3. X-RAY MASTOIDS/CT SCAN TEMPORAL BONE. They the ear safe, and second in priority is to preserve or recon-
indicate extent of bone destruction and degree of mastoid struct hearing but never at the cost of the primary aim.
pneumatization. They are useful to indicate a low-lying Two types of surgical procedures are done to deal with
dura or an anteposed sigmoid sinus when operation is cholesteatoma:
being contemplated on a sclerotic mastoid. Cholestea-
(a) Canal wall down procedures. They leave the mas- *
toma causes destruction in the area of attic and antrum
toid cavity open into the external auditory canal-
(key area), better seen in lateral view. CT scan of temporal
so that the diseased area is fully exteriorized. The
bone gives more information and is preferred to X-ray
commonly performed operations for atticoantral
mastoids. - disease are atticotomy, modified radical mastoid-
ectomy and rarely, the radical mastoidectomy (see
4. CULTURE AND SENSITIVITY OF EAR DISCHARGE. It helps
operative surgery).
to select proper antibiotic for local or systemic use.
(b) Canal wall up procedures. Here disease is removed by
Features Indicating Complications in CSOM combined approach through the meatus and mastoid
O
1. PAIN. Pain is uncommon in uncomplicated CSOM. Its
presence is considered serious as it may indicate extra-
but retaining the posterior bony meatal wall intact,
thereby avoiding an open mastoid cavity. It gives dry
ear and permits easy reconstruction of hearing mecha-
dural, perisinus or brain abscess. Sometimes, it is due to
nism. However, there is danger of leaving some cho-
otitis externa associated with a discharging ear.
lesteatoma behind. Incidence of residual or recurrent

O
2. VERTIGO. It indicates erosion of lateral semicircular
canal which may progress to labyrinthitis or meningitis.
cholesteatoma in these cases is very high and there-
fore long-term follow-up is essential. Some surgeon’s
even advise routine re-exploration in all cases after
Fistula test should be performed in all cases.
6 months or so. Canal wall up procedures are advised

-
3. PERSISTENT HEADACHE. It is suggestive of an intracra-
nial complication.
only in selected cases. In combined approach or intact
canal wall mastoidectomy, disease is removed both
permeatally, and through cortical mastoidectomy and

E
4. FACIAL WEAKNESS. indicates erosion of facial canal.
posterior tympanotomy approach, in which a win-
dow is created between the mastoid and middle ear,
through the facial recess, to reach sinus tympani
5. A LISTLESS CHILD REFUSING TO TAKE FEEDS. and easily
(see p. 7).
going to sleep (extradural abscess).
See Table 11.2 for the comparison of canal wall up and
6. FEVER, NAUSEA AND VOMITING. (intracranial infection). canal wall down procedures.

TABLE 11.2 COMPARISON OF CANAL WALLV

UP AND CANAL WALL DOWN PROCEDURES
Canal wall up procedure Canal
=
wall down procedure ~
~
Meatus Normal appearance Widely open meatus communicating with mastoid
Dependence Does not require routine cleaning Dependence on doctor for cleaning mastoid cavity once
-
-
or twice a year
-
Recurrence or residual disease High rate of recurrent or residual Low rate of recurrence or residual disease and thus a
cholesteatoma N safe procedure
Y
Second look surgery O
Requires second look surgery after 6 months
or so to rule out cholesteatoma
Not required

Patients limitations No limitation. Patient allowed swimming Swimming can lead to infection of mastoid cavity and it
&
is thus curtailed
Auditory rehabilitation Easy to wear a hearing aid if needed Problems in fitting a hearing aid due to large meatus
and mastoid cavity which sometimes gets infected

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 Chapter 11 — Cholesteatoma and Chronic Otitis Media 81

2. RECONSTRUCTIVE SURGERY. Hearing can be restored get filled with pale granulations. Caries of bone and os-
by myringoplasty or tympanoplasty. It can be done at the sicles may occur leading to complications. Mastoiditis,
time of primary surgery or as a second stage procedure. facial paralysis, postauricular fistula, osteomyelitis with
formation of bony sequestra and profound hearing loss
3. CONSERVATIVE TREATMENT. It has a limited role in the are often seen in these cases.
management of cholesteatoma but can be tried in selected
cases, when cholesteatoma is small and easily accessible
to suction clearance under operating microscope. Repeat-
ed suction clearance and periodic checkups are essential. CLINICAL FEATURES
It can also be tried out in elderly patients above 65 and 1. PAINLESS EAR DISCHARGE. Earache is characteristi-
those who are unfit for general anaesthesia or those refus- cally absent in cases of tubercular otitis media. Discharge
ing surgery. Polyps and granulations can also be surgically is often foul-smelling because of the underlying bone
removed by cup forceps or cauterized by chemical agents destruction.
like silver nitrate or trichloroacetic acid. Other measures
like aural toilet and dry ear precautions are also essential. 2. PERFORATION. Multiple perforations, two or three in
number, are seen in pars tensa and form a classical sign
Figure 11.12 summarizes the management of CSOM.
of disease. These may coalesce into a single large perfora-
tion then it becomes indistinguishable from nonspecific
TUBERCULAR OTITIS MEDIA CSOM.

AETIOLOGY 3. HEARING LOSS. There is severe hearing loss, out of

proportion to symptoms. Mostly conductive, it may
In most of the cases, infection is secondary to pulmonary
have sensorineural component due to involvement of
tuberculosis; infection reaches the middle ear through
labyrinth.
eustachian tube. Sometimes, it is blood-borne from
tubercular focus in the lungs, tonsils, cervical or mesen-
teric lymph nodes. Disease is mostly seen in children and 4. FACIAL PARALYSIS. It is a common complication and
young adults. may come unexpectedly. This may be the presenting fea-
ture in a child.
PATHOLOGY
The process is slow and insidious. Tubercles appear in the
DIAGNOSIS
submucosal layers of middle ear cleft and caseate. There is
painless necrosis of tympanic membrane. In the presence of secondary pyogenic infection, tu-
Multiple perforations may form which coalesce to bercular otitis media may be indistinguishable from
form a single large perforation. Middle ear and mastoid chronic suppurative otitis media. Culture of ear discharge

V
Y

Figure 11.12. Management of chronic suppurative otitis media (CSOM).

*CWU, canal wall up; CWD, canal wall down.

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82 SECTION I — Diseases of Ear

for tubercle bacilli, histopathological examination of

SYPHILITIC OTITIS MEDIA
granulations and X-ray chest, and other evidence of tu-
berculosis in the body help to confirm the diagnosis. It is a rare condition. Spirochaetes reach middle ear
Presently DNA probe and PCR (polymerase chain reac- through eustachian tube when syphilitic lesions are pre-
tion) from the ear discharge can give early diagnosis in sent in the nose or nasopharynx. Infection may also be
3–7 days. blood-borne. Sensory end organs of the inner ear and
their nerves are soon invaded by spirochaetes leading to
profound sensorineural hearing loss, tinnitus and vertigo.
TREATMENT Bone necrosis and sequestrum formation are common
and they lead to foetid ear discharge. Secondary pyogenic
1. SYSTEMIC ANTITUBERCULAR THERAPY. As being car-
infection may occur, giving a clinical picture very much
ried for primary disease.
like chronic suppurative otitis media.
Definite diagnosis of syphilitic otitis media can only be
2. LOCAL TREATMENT. In the form of aural toilet and made by specific treponemal antigen tests such as trepone-
control of secondary pyogenic infection. mal pallidum immobilization (TPI) test and fluorescent
treponemal antibody absorption test (FTA-ABS). VDRL and
3. MASTOID SURGERY. It is indicated for complications. RPR (reactive plasma reagin) tests are nonspecific but useful
Healing is delayed in tuberculous cases. Wound break- to monitor disease, however false positive tests may occur.
down and fistula formation are common. Reconstructive Treatment consists of antisyphilitic therapy with at-
surgery of middle ear is delayed till antitubercular therapy tention to aural toilet and control of secondary infection.
has been completed. Surgery may be required for removal of sequestra.

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 mastoiditi Clarification
Stasitalis
8

·
I Chapter 12
·
intratemporal I in
fail thi
-
-

tha
Complications of Suppurative
·

O
Otitis Media temporal
bore

o intracranial

Though there is a general decline in the incidence of In acute and chronic middle ear infection, disease pro-
complications, they are still frequently seen in India. cess is limited only to the mucoperiosteal lining of the
The causes are poor socioeconomic conditions, lack of cleft but if it spreads into the bony walls of the cleft or
education and awareness about healthcare (middle ear beyond it, various complications can arise.
discharge is still being considered merely a nuisance
rather than a potentially dangerous condition), and lack
of availability of trained specialists in the far-flung rural PATHWAYS OF SPREAD OF INFECTION
areas where transportation facilities are still inadequate.

1. DIRECT BONE EROSION. In acute infections, it is the

FACTORS INFLUENCING DEVELOPMENT process of hyperaemic decalcification. In chronic infec-
OF COMPLICATIONS tion, it may be osteitis, erosion by cholesteatoma or gran-
ulation tissue.

1. AGE. Most of the complications occur in the first decade 2. VENOUS THROMBOPHLEBITIS. Veins of Haversian ca-
of life or in the elderly when the patient’s resistance is low. nals are connected with dural veins which in turn con-
nect with dural venous sinuses and superficial veins of
2. POOR SOCIOECONOMIC GROUP. Several factors such as brain. Thus, infection from the mastoid bone can cause
overcrowding, poor health education and personal hygiene, thrombophlebitis of venous sinuses and even cortical
and limited access to healthcare play an important part. vein thrombosis. This mode of spread is common in
acute infections.
3. VIRULENCE OF ORGANISMS. Many organisms are de-
veloping resistance to antibiotics and acute infections are 3. PREFORMED PATHWAYS
either not controlled or progress to subacute or chronic (a) Congenital dehiscences, e.g. in bony facial canal,
otitis media. Insufficient dose, less effective drug or insuf- floor of middle ear over the jugular bulb.
ficient period of administration of antibiotic can cause (b) Patent sutures, e.g. petrosquamous suture.
complications. Streptococcus pneumoniae type III (earlier (c) Previous skull fractures. The fracture sites heal only by
called pneumococcus type III) is very virulent due to pro- fibrous scar which permits infection.
duction of autolysin and pneumolysin. Haemophilus influ- (d) Surgical defects, e.g. stapedectomy, fenestration and
enzae is developing resistance to β-lactam antibiotics and mastoidectomy with exposure of dura.
chloramphenicol. Other resistant strains are Pseudomonas (e) Oval and round windows.
aeruginosa and methicillin resistant Staphylococcus aureus. (f) Infection from labyrinth can travel along internal
acoustic meatus, aqueducts of the vestibule and that
4. IMMUNE-COMPROMISED HOST. Patients suffering from of the cochlea to the meninges.
AIDS, uncontrolled diabetes, transplant patients receiving
immunosuppressive drugs and cancer patients receiving
chemotherapy are more prone to develop complications. CLASSIFICATION
5. PREFORMED PATHWAYS. Infection can easily travel beyond Complications of otitis media are classified into two main
the middle ear cleft if preformed pathways exist, e.g. dehis- groups (Figure 12.1 ):
cence of bony facial canal, previous ear surgery, fracture of
temporal bone, stapedectomy, perilymph fistula or congeni-
A. INTRATEMPORAL (WITHIN THE
tally enlarged aqueduct of vestibule (as in Mondini abnor-
mality of inner ear) or dehiscence in the floor of middle ear.
CONFINES OF TEMPORAL BONE)
1. Mastoiditis O
6. CHOLESTEATOMA. Osteitis or granulation tissue in 2. Petrositis
chronic otitis media destroys the bone and helps infec- 3. Facial paralysis
tion to penetrate deeper. 4. Labyrinthitis

83

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84 SECTION I — Diseases of Ear

Figure 12.1. Complications of otitis media.

Scan to play Cholesteatoma and Its Complications.

B. INTRACRANIAL Aetiology
1. Extradural abscess Acute mastoiditis usually accompanies or follows acute
2. Subdural abscess suppurative otitis media, the determining factors be-
3. Meningitis ing high virulence of organisms or lowered resistance
4. Brain abscess of the patient due to measles, exanthematous fevers,
5. Lateral sinus thrombophlebitis poor nutrition or associated systemic disease such as
6. Otitic hydrocephalus. diabetes.
Acute mastoiditis is often seen in mastoids with well-
developed air cell system. Children are affected more.
Beta-haemolytic streptococcus is the most common caus-
SEQUELAE OF OTITIS MEDIA ative organism though other organisms responsible for
They are the direct result of middle ear infection and acute otitis media may also be seen. Very often, anaerobic
should be differentiated from complications. They include: organisms are also associated with mastoiditis and need
antibacterial therapy against them.
1. Perforation of tympanic membrane
2. Ossicular erosion Pathology
3. Atelectasis and adhesive otitis media Two main pathological processes are responsible:
4. Tympanosclerosis
5. Cholesteatoma formation 1. Production of pus under tension.
6. Conductive hearing loss due to ossicular erosion or 2. Hyperaemic decalcification and osteoclastic resorption
fixation of bony walls.
7. Sensorineural hearing loss Extension of inflammatory process to mucoperiosteal
8. Speech impairment lining of air cell system increases the amount of pus pro-
9. Learning disabilities duced due to large surface area involved. Drainage of
The last two are secondary to loss of hearing in the this pus, through a small perforation of tympanic mem-
developmental phase of the infant or child. brane and/or eustachian tube, cannot keep pace with the
amount being produced. Swollen mucosa of the antrum
and attic also impede the drainage system resulting in ac-
cumulation of pus under tension.
I. INTRATEMPORAL COMPLICATIONS Hyperaemia and engorgement of mucosa causes disso-
OF OTITIS MEDIA lution of calcium from the bony walls of the mastoid air
cells (hyperaemic decalcification).
A. (i) ACUTE MASTOIDITIS
Both these processes combine to cause destruction and
Inflammation of mucosal lining of antrum and mastoid coalescence of mastoid air cells, converting them into a
air cell system is an invariable accompaniment of acute single irregular cavity filled with pus (empyema of mas-
otitis media and forms a part of it. The term “mastoiditis” toid).
is used when infection spreads from the mucosa, lining Pus may break through mastoid cortex leading to sub-
the mastoid air cells, to involve bony walls of the mastoid periosteal abscess which may even burst on surface lead-
air cell system. ing to a discharging fistula.

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 Chapter 12 — Complications of Suppurative Otitis Media 85

Clinical Features pus bursts through bony cortex, a subperiosteal fluc-

SYMPTOMS. They are similar to that of acute suppurative tuant abscess is formed (Figures 12.2 and 12.3) which
otitis media. In a case of acute middle ear infection, it may further burst on skin or form a fistula.
is the change in the character of these symptoms which 6. Hearing loss. Conductive type of hearing loss is always
is significant and a pointer to the development of acute present.
mastoiditis. 7. General findings. Patient appears ill and toxic with low-
grade fever. In children, fever is high with a rise in
1. Pain behind the ear. Pain is seen in acute otitis media pulse rate.
but it subsides with establishment of perforation or
treatment with antibiotics. It is the persistence of pain, Investigations
increase in its intensity or recurrence of pain, once it 1. BLOOD COUNTS show polymorphonuclear leucocytosis.
had subsided. These are significant pointers of pain.
2. Fever. It is the persistence or recurrence of fever in a case 2. ERYTHROCYTE SEDIMENTATION RATE is usually raised.
of acute otitis media, in spite of adequate antibiotic
treatment that points to the development of mastoiditis. 3. X-RAY MASTOID. CT scan temporal bone. There is cloud-
3. Ear discharge. In mastoiditis, discharge becomes profuse ing of air cells due to collection of exudate in them. Bony
and increases in purulence. In some cases, discharge partitions between air cells become indistinct, but the sinus
may cease due to obstruction to its drainage but other plate is seen as a distinct outline. In later stages, a cavity
symptoms would worsen. Any persistence of discharge may be seen in the mastoid.
beyond 3 weeks, in a case of acute otitis media, points
to mastoiditis. 4. EAR SWAB. for culture and sensitivity.

SIGNS Differential Diagnosis

1. Mastoid tenderness. This is an important sign. Tender- 1. SUPPURATION OF MASTOID LYMPH NODES. Scalp in-
ness is elicited by pressure over the middle of mastoid fection may cause mastoid lymph node enlargement and
process, at its tip, posterior border or the root of zy-
goma. Tenderness elicited over the suprameatal triangle
may not be diagnostic of acute mastoiditis as it is seen
even in cases of the acute otitis media due to inflam-
mation of mastoid antrum (antritis). Tenderness should
always be compared with that of the healthy side.
2. Ear discharge. Mucopurulent or purulent discharge, of-
ten pulsatile (light-house effect), may be seen coming
through a central perforation of pars tensa.
3. Sagging of posterosuperior meatal wall. It is due to perios-
titis of bony party wall between the antrum and deeper
posterosuperior part of bony canal.
4. Perforation of tympanic membrane. Usually, a small perfo-
ration is seen in pars tensa with congestion of the rest
of tympanic membrane. Perforation may sometimes
appear as a nipple-like protrusion. Sometimes, tympan-
ic membrane is intact but dull and opaque especially in
those who have received inadequate antibiotics.
5. Swelling over the mastoid. Initially, there is oedema of per-
iosteum, imparting a smooth “ironed out” feel over the
mastoid. Later retroauricular sulcus becomes obliterated Figure 12.2. Acute mastoiditis. Note: Pinna is pushed downward
and pinna is pushed forwards and downwards. When and forward.

Figure 12.3. (A) Burst mastoid abscess exuding pus. (B) Mastoid fistula.

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86 SECTION I — Diseases of Ear

then suppuration leading to abscess formation, but in 4. CORTICAL MASTOIDECTOMY. It is indicated when there is:
such cases there is no history of preceding otitis media,
(a) Subperiosteal abscess.
ear discharge or deafness. Abscess is usually superficial.
(b) Sagging of posterosuperior meatal wall.
(c) Positive reservoir sign, i.e. meatus immediately fills
2. FURUNCULOSIS OF MEATUS. It is differentiated from
with pus after it has been mopped out.
acute mastoiditis by:
(d) No change in condition of patient or it worsens in
(a) Absence of preceding acute otitis media. spite of adequate medical treatment for 48 h.
(b) Painful movements of pinna; pressure over the tragus (e) Mastoiditis, leading to complications, e.g. facial pa-
or below the cartilaginous part of meatus causes ex- ralysis, labyrinthitis, intracranial complications, etc.
cruciating pain.
Aim of cortical mastoidectomy is to exenterate all the
(c) Swelling of meatus is confined to the cartilaginous
mastoid air cells and remove any pockets of pus. Ade-
part only.
quate antibiotic treatment must be continued at least for
(d) Discharge is never mucoid or mucopurulent. Mucoid
5 days following mastoidectomy.
element in discharge can only come from the middle
ear and not from the external ear which is devoid of
mucus-secreting glands. Complications of Acute Mastoiditis
(e) Enlargement of pre- or postauricular lymph nodes. 1. Subperiosteal abscess
(f) Conductive hearing loss is usually mild and is due to 2. Labyrinthitis
the occlusion of meatus. 3. Facial paralysis
(g) An absolutely normal looking tympanic membrane 4. Petrositis
excludes possibility of acute mastoiditis. 5. Extradural abscess
(h) X-ray mastoid with clear air-cell system excludes 6. Subdural abscess
acute mastoiditis. Sometimes, difficulty arises when 7. Meningitis
air-cell system appears hazy due to superimposed soft 8. Brain abscess
tissue swelling in cases of furunculosis. 9. Lateral sinus thrombophlebitis
10. Otitic hydrocephalous.
3. INFECTED SEBACEOUS CYST

Treatment Abscesses in Relation to Mastoid Infection

1. HOSPITALIZATION OF THE PATIENT. Patient is hospital- 1. POSTAURICULAR ABSCESS (FIGURE 12.3). This is the
ized if not already done. commonest abscess that forms over the mastoid. Pinna is
displaced forwards, outwards and downwards. In infants
2. ANTIBIOTICS. In the absence of culture and sensitivity, and children, abscess forms over the MacEwen’s triangle;
start with amoxicillin or ampicillin. Specific antimicro- pus in these cases travels along the vascular channels of
bial is started on the receipt of sensitivity report. Since lamina cribrosa.
anaerobic organisms are often present, chloramphenicol
or metronidazole is added. 2. ZYGOMATIC ABSCESS. It occurs due to infection of
zygomatic air cells situated at the posterior root of zy-
3. MYRINGOTOMY. When pus is under tension it is relieved goma. Swelling appears in front of and above the pinna
by wide myringotomy (see operative surgery). Early cases (Figure 12.4 A and B). There is associated oedema of the
of acute mastoiditis respond to conservative treatment upper eyelid. In these cases, pus collects either superficial
with antibiotics alone or combined with myringotomy. or deep to the temporalis muscle.

Figure 12.4. (A) Abscesses in relation to mastoid: (1) postauricular, (2) zygomatic and (3) Bezold abscess. (B) Citelli, postauricular and Bezold
abscesses seen from behind.

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 Chapter 12 — Complications of Suppurative Otitis Media 87

6. PARAPHARYNGEAL OR RETROPHARYNGEAL ABSCESS.

This results from infection of the peritubal cells due to
acute coalescent mastoiditis.

A. (ii) MASKED (LATENT) MASTOIDITIS

It is a condition of slow destruction of mastoid air cells
but without the acute signs and symptoms often seen in
acute mastoiditis. There is no pain, no discharge, no fever
and no mastoid swelling but mastoidectomy may show
extensive destruction of the air cells with granulation tis-
sue and dark gelatinous material filling the mastoid. It is
not surprising to find erosion of the tegmen tympani and
sinus plate with an extradural or perisinus abscess.

Figure 12.5. Bezold abscess. Pus bursting through the medial side of
Aetiology
the tip of mastoid and collecting under the sternomastoid or digastric The condition often results from inadequate antibiotic
triangle. therapy in terms of dose, frequency and duration of ad-
ministration. Most often it results from use of oral peni-
cillin given in cases of acute otitis media when acute
3. BEZOLD ABSCESS. It can occur following acute coales-
symptoms subside but smouldering infection continues
cent mastoiditis when pus breaks through the thin me-
in the mastoid.
dial side of the tip of the mastoid and presents as a swell-
ing in the upper part of neck. The abscess may (i) lie deep Clinical Features
to sternocleidomastoid, pushing the muscle outwards,
Patient is often a child, not entirely feeling well, with
(ii) follow the posterior belly of digastric and present as a
mild pain behind the ear but with persistent hearing loss.
swelling between the tip of mastoid and angle of jaw, (iii)
Tympanic membrane appears thick with loss of trans-
be present in upper part of posterior triangle, (iv) reach
lucency. Slight tenderness may be elicited over the mas-
the parapharyngeal space or (v) track down along the ca-
toid. Audiometry shows conductive hearing loss of vari-
rotid vessels (Figure 12.5).
able degree. X-ray of mastoid will reveal clouding of air
Clinical features. Onset is sudden. There is pain, fever,
cells with loss of cell outline.
a tender swelling in the neck and torticollis. Patient gives
history of purulent otorrhoea. Treatment
A Bezold abscess should be differentiated from:
Cortical mastoidectomy with full doses of antibiotics is
(a) acute upper jugular lymphadenitis. the treatment of choice. This may cause tympanic mem-
(b) abscess or a mass in the lower part of the parotid gland. brane to return to normal with improvement in hearing.
(c) an infected branchial cyst.
(d) parapharyngeal abscess. B. PETROSITIS
(e) jugular vein thrombosis.
Spread of infection from middle ear and mastoid to the
A computed tomography (CT) scan of the mastoid and
petrous part of temporal bone is called petrositis. It may
swelling of the neck may establish the diagnosis.
be associated with acute coalescent mastoiditis, latent
Treatment mastoiditis or chronic middle ear infections.
(a) Cortical mastoidectomy for coalescent mastoidi-
tis with careful exploration of the tip for a fistulous Pathology
opening into the soft tissues of the neck. Like mastoid, petrous bone may be of three types: pneu-
(b) Drainage of the neck abscess through a separate inci- matized with air cells extending to the petrous apex, dip-
sion and putting a drain in the dependent part. loic containing only marrow spaces and sclerotic. Pneu-
(c) Administration of intravenous antibiotics guided by matization of petrous apex occurs in only 30% of cases
the culture and sensitivity report of the pus taken at with cells extending from the middle ear or mastoid to
the time of surgery. the petrous apex. Usually two cell tracts are recognized:
1. Posterosuperior tract which starts in the mastoid and
4. MEATAL ABSCESS (LUC ABSCESS). In this case, pus
runs behind or above the bony labyrinth to the pe-
breaks through the bony wall between the antrum and
trous apex; some cells even pass through the arch of
external osseous meatus. Swelling is seen in deep part of
superior semicircular canal to reach the apex.
bony meatus. Abscess may burst into the meatus.
2. Anteroinferior tract which starts at the hypotympa-
num near the eustachian tube runs around the cochlea
5. BEHIND THE MASTOID (CITELLI’S ABSCESS). Abscess
to reach the petrous apex.
is formed behind the mastoid more towards the occipi-
tal bone (compare postauricular mastoid abscess which Infective process runs along these cell tracts and reaches
forms over the mastoid). Some authors consider abscess of the petrous apex. Pathological process is similar to that of co-
the digastric triangle, which is formed by tracking of pus alescent mastoiditis forming epidural abscess at the petrous
from the mastoid tip, as the Citelli’s abscess. apex involving cranial nerve VI and trigeminal ganglion.

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88 SECTION I — Diseases of Ear

Clinical Features Granulation tissue surrounding the nerve is removed but

Gradenigo syndrome is the classical presentation, and con- if it actually invades the nerve sheath, it is left in place. If
sists of a triad of (i) external rectus palsy (VIth nerve pal- a segment of the nerve has been destroyed by the granu-
sy), (ii) deep-seated ear or retro-orbital pain (Vth nerve lation tissue, resection of nerve and grafting are better left
involvement) and (iii) persistent ear discharge. It is un- to a second stage when infection has been controlled and
common to see the full triad these days. fibrosis has matured.
Persistent ear discharge with or without deep-seated
pain in spite of an adequate cortical or modified radical D. LABYRINTHITIS
mastoidectomy also points to petrositis.
Fever, headache, vomiting and sometimes neck rigid- There are three types of labyrinthitis:
ity may also be associated. Some patients may get facial 1. Circumscribed labyrinthitis
paralysis and recurrent vertigo due to involvement of fa- 2. Diffuse serous labyrinthitis
cial and statoacoustic nerves. 3. Diffuse suppurative labyrinthitis
Diagnosis of petrous apicitis requires both CT scan and
MRI. CT scan of temporal bone will show bony details of Circumscribed Labyrinthitis (Fistula of
the petrous apex and the air cells while MRI helps to dif- Labyrinth)
ferentiate diploic marrow-containing apex from the fluid There is thinning or erosion of bony capsule of labyrinth,
or pus. usually of the horizontal semicircular canal.
Treatment AETIOLOGY. The causes are:
Cortical, modified radical or radical mastoidectomy is
often required if not already done. The fistulous tract 1. Chronic suppurative otitis media with cholesteatoma
should be found out, which is then curetted and enlarged is the most common cause.
to provide free drainage. Tract of posterosuperior cells 2. Neoplasms of middle ear, e.g. carcinoma or glomus tu-
starts in the Trautmann’s triangle or the attic. Tract of an- mour.
terior cells is situated near the tympanic opening of eus- 3. Surgical or accidental trauma to labyrinth.
tachian tube and passes above the carotid artery, anterior
to the cochlea. In the latter case, radical mastoidectomy CLINICAL FEATURES. A part of membranous labyrinth
is required. is exposed and becomes sensitive to pressure changes.
Suitable intravenous antibacterial therapy should pre- Patient complains of transient vertigo often induced by
cede and follow surgical intervention. Most cases of acute pressure on tragus, cleaning the ear or while performing
petrositis can now be cured with antibacterial therapy Valsalva manoeuvre.
alone. It should be given in initial high doses and con- It is diagnosed by “fistula test” which can be performed
tinued for 4-5 days, even after complete disappearance of in two ways.
symptoms. 1. Pressure on tragus. Sudden inward pressure is applied
on the tragus. This increases air pressure in the ear ca-
nal and stimulates the labyrinth. Patient will complain
C. FACIAL PARALYSIS of vertigo. Nystagmus may also be induced with quick
It can occur as a complication of both acute and chronic component towards the ear under test.
otitis media. 2. Siegel’s speculum. When positive pressure is applied to
ear canal, patient complains of vertigo usually with
Acute Otitis Media nystagmus. The quick component of nystagmus would
Facial nerve is normally well-protected in its bony canal. be towards the affected ear (ampullopetal displace-
Sometimes, the bony canal is dehiscent and the nerve lies ment of cupula).
just under the middle ear mucosa. It is in these cases that Ampullopetal flow of endolymph (as also ampullo-
inflammation of middle ear spreads to epi- and perineu- petal displacement of cupula) whether in rotation, caloric
rium causing facial paralysis. Facial nerve function fully or fistula test causes nystagmus to same side.
recovers if acute otitis media is controlled with systemic If negative pressure is applied, again it would induce
antibiotics. Myringotomy or cortical mastoidectomy may vertigo and nystagmus but this time the quick com-
sometimes be required. ponent of nystagmus would be directed to the (oppo-
site) healthy side due to ampullofugal displacement of
Chronic Otitis Media cupula.
Facial paralysis in chronic otitis media either results from
cholesteatoma or from penetrating granulation tissue. TREATMENT. In chronic suppurative otitis media or
Cholesteatoma destroys bony canal and then causes pres- cholesteatoma, mastoid exploration is often required to
sure on the nerve, further aided by oedema of associated eliminate the cause. Systemic antibiotic therapy should
inflammatory process. Facial paralysis is insidious but be instituted before and after operation to prevent spread
slowly progressive. Treatment is urgent exploration of the of infection into the labyrinth.
middle ear and mastoid. Facial canal is inspected from
the geniculate ganglion to the stylomastoid foramen. If Diffuse Serous Labyrinthitis
granulation tissue or cholesteatoma has entered the bony It is diffuse intralabyrinthine inflammation without pus
canal, the latter is uncapped in the area of involvement. formation and is a reversible condition if treated early.

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 Chapter 12 — Complications of Suppurative Otitis Media 89

AETIOLOGY Pathology
1. Most often it arises from pre-existing circumscribed In acute otitis media, bone over the dura is destroyed by
labyrinthitis associated with chronic middle ear sup- hyperaemic decalcification, while in chronic otitis media
puration or cholesteatoma. it is destroyed by cholesteatoma and in such a case the
2. In acute infections of middle ear cleft, inflammation pus comes to lie directly in contact with dura. Spread of
spreads through annular ligament or the round window. infection can also occur by venous thrombophlebitis; in
3. It can follow stapedectomy or fenestration operation. this case, bone over the dura remains intact. An extra-
dural abscess may lie in relation to dura of middle or pos-
CLINICAL FEATURES. Mild cases complain of vertigo and terior cranial fossa or outside the dura of lateral venous
nausea but in severe cases, vertigo is worse with marked sinus (perisinus abscess). The affected dura may be covered
nausea, vomiting and even spontaneous nystagmus. Quick with granulations or appear unhealthy and discoloured.
component of nystagmus is towards the affected ear.
As the inflammation is diffuse, cochlea is also affected Clinical Features
with some degree of sensorineural hearing loss.
Most of the time extradural or perisinus abscesses are
Serous labyrinthitis, if not checked, may pass onto
asymptomatic and silent, and are discovered accidentally
suppurative labyrinthitis with total loss of vestibular and
during cortical or modified radical mastoidectomy.
cochlear function.
However, their presence is suspected when there is:
TREATMENT 1. Persistent headache on the side of otitis media.
• Medical 2. Severe pain in the ear.
1. Patient is put to bed, his head immobilized with af- 3. General malaise with low-grade fever.
fected ear above. 4. Pulsatile purulent ear discharge.
2. Antibacterial therapy is given in full doses to con- 5. Disappearance of headache with free flow of pus from
trol infection. the ear (spontaneous abscess drainage).
3. Labyrinthine sedatives, e.g. prochlorperazine Diagnosis is made on contrast-enhanced CT or MRI.
(Stemetil) or dimenhydrinate (Dramamine), are giv-
en for symptomatic relief of vertigo. Treatment
4. Myringotomy is done if labyrinthitis has followed
1. CORTICAL OR MODIFIED RADICAL OR RADICAL MAS-
acute otitis media and the drum is bulging. Pus is
TOIDECTOMY. It is often required to deal with the causa-
cultured for specific antibacterial therapy.
tive disease process. Extradural abscess is evacuated by
• Surgical. Cortical mastoidectomy (in acute mastoiditis)
removing overlying bone till the limits of healthy dura
or modified radical mastoidectomy (in chronic middle
are reached. Cases where bony plate of tegmen tympani
ear infection or cholesteatoma) will often be required
or sinus plate is intact but there is suspicion of an abscess,
to treat the source of infection. Medical treatment
the intact bony plate is deliberately removed to evacuate
should always precede surgical intervention.
any collection of pus.
Diffuse Suppurative Labyrinthitis
This is diffuse pyogenic infection of the labyrinth with 2. AN ANTIBIOTIC COVER. should be provided for a mini-
permanent loss of vestibular and cochlear functions. mum of 5 days and patient closely observed for any fur-
ther complications, such as sinus thrombosis, meningitis
AETIOLOGY. It usually follows serous labyrinthitis, pyo- or brain abscess.
genic organisms entering through a pathological or surgi-
cal fistula. B. SUBDURAL ABSCESS
CLINICAL FEATURES. There is severe vertigo with nausea This is collection of pus between dura and arachnoid.
and vomiting due to acute vestibular failure. Spontane-
ous nystagmus will be observed with its quick component Pathology
towards the healthy side. Patient is markedly toxic. There Infection spreads from the ear by erosion of bone and
is total loss of hearing. Relief from vertigo is seen after dura or by thrombophlebitic process in which case inter-
3-6 weeks due to adaptation. vening bone remains intact. Pus rapidly spreads in sub-
dural space and comes to lie against the convex surface
TREATMENT. It is same as for serous labyrinthitis. Rarely, of cerebral hemisphere causing pressure symptoms. With
drainage of the labyrinth is required, if intralabyrinthine time, the pus may get loculated at various places in sub-
suppuration is acting as a source of intracranial complica- dural space.
tions, e.g. meningitis or brain abscess.
Clinical Features
Signs and symptoms of subdural abscess are due to (i) me-
II. INTRACRANIAL COMPLICATIONS ningeal irritation, (ii) thrombophlebitis of cortical veins
OF OTITIS MEDIA of cerebrum and (iii) raised intracranial tension.
A. EXTRADURAL ABSCESS
1. MENINGEAL IRRITATION. There is headache, fever
It is collection of pus between the bone and dura. It may (102 °F or more), malaise, increasing drowsiness, neck
occur both in acute and chronic infections of middle ear. rigidity and positive Kernig’s sign.

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90 SECTION I — Diseases of Ear

2. CORTICAL VENOUS THROMBOPHLEBITIS. Veins over Lumbar puncture and CSF examination establish the
the cerebral hemisphere undergo thrombophlebitis lead- diagnosis. CSF is turbid, cell count is raised and may even
ing to aphasia, hemiplegia and hemianopia. There may reach 1000/mL with predominance of polymorphs; pro-
be Jacksonian type of epileptic fits which may increase to tein level is raised, sugar is reduced and chlorides are di-
give a picture of status epilepticus. minished.
CSF is always cultured to find the causative organisms
3. RAISED INTRACRANIAL TENSION. There is papilloede- and their antibiotic sensitivity.
ma, ptosis and dilated pupil (IIIrd nerve involvement),
and involvement of other cranial nerves. CT scan or MRI Treatment
is required for diagnosis. MEDICAL. Medical treatment takes precedence over sur-
gery.
Treatment Antimicrobial therapy directed against aerobic and an-
Lumbar puncture should not be done as it can cause her- aerobic organisms should be instituted. Culture and sen-
niation of the cerebellar tonsils. It is a neurological emer- sitivity of CSF will further aid in the choice of antibiotics.
gency. A series of burr holes or a craniotomy is done to Corticosteroids combined with antibiotic therapy
drain subdural empyema. Intravenous antibiotics are ad- further helps to reduce neurological or audiological com-
ministered to control infection. Once infection is under plications.
control, attention is paid to causative ear disease which
may require mastoidectomy. SURGICAL. Meningitis following acute otitis media may
require myringotomy or cortical mastoidectomy. Menin-
C. MENINGITIS * gitis following chronic otitis media with cholesteatoma
will require radical or modified radical mastoidectomy.
It is inflammation of leptomeninges (pia and arachnoid) Surgery is undertaken as soon as general condition of
usually with bacterial invasion of CSF in subarachnoid patient permits. It may be done urgently, if there has been
space. It is the most common intracranial complication no satisfactory response to medical treatment.
of otitis media. It can occur in both acute and chronic
otitis media. In infants and children, otogenic meningitis
D. OTOGENIC BRAIN ABSCESS
usually follows acute otitis media while in adults it is due
to chronic middle ear infection. Fifty per cent of brain abscesses in adults and twenty-
five per cent in children are otogenic in origin. In adults,
Mode of Infection abscess usually follows chronic suppurative otitis media
Blood-borne infection is common in infants and children; with cholesteatoma, while in children, it is usually the
in adults, it follows chronic ear disease, which spreads by result of acute otitis media. Cerebral abscess is seen twice
bone erosion or retrograde thrombophlebitis. In the latter as frequently as cerebellar abscess.
case, it may be associated with an extradural abscess or
granulation tissue. Route of Infection
In one-third of the patients with meningitis, another Cerebral abscess develops as a result of direct extension of
intracranial complication may coexist. middle ear infection through the tegmen or by retrograde
thrombophlebitis, in which case the tegmen will be in-
Clinical Features tact. Often it is associated with extradural abscess.
Symptoms and signs of meningitis are due to (i) presence Cerebellar abscess also develops as a direct extension
of infection, (ii) raised intracranial tension, and (iii) me- through the Trautmann’s triangle or by retrograde throm-
ningeal and cerebral irritation. Their severity will vary bophlebitis. This is often associated with extradural ab-
with the extent of disease. scess, perisinus abscess, sigmoid sinus thrombophlebitis
or labyrinthitis.
1. There is rise in temperature (102-104 °F) often with
chills and rigors. Bacteriology
2. Headache.
Both aerobic and anaerobic organisms are seen. Aero-
3. Neck rigidity.
bic ones include pyogenic staphylococci, Streptococcus
4. Photophobia and mental irritability.
pneumoniae, Streptococcus haemolyticus, Proteus mirabilis,
5. Nausea and vomiting (sometimes projectile).
Escherichia coli and Pseudomonas aeruginosa. Common
6. Drowsiness which may progress to delirium or coma.
among the anaerobic ones are the Peptostreptococcus and
7. Cranial nerve palsies and hemiplegia.
Bacteroides fragilis. Haemophilus influenzae is rarely seen.
Examination will show (i) neck rigidity, (ii) positive
Kernig’s sign (extension of leg with thigh flexed on abdo- Pathology
men causing pain), (iii) positive Brudzinski’s sign (flexion Brain abscess develops through four stages.
of neck causes flexion of hip and knee), (iv) tendon reflex-
es are exaggerated initially but later become sluggish or 1. STAGE OF INVASION (INITIAL ENCEPHALITIS). It often
absent and (v) papilloedema (usually seen in late stages). passes unnoticed as symptoms are slight. Patient may
have headache, low-grade fever, malaise and drowsiness.
Diagnosis
CT or MRI with contrast will help to make the diagnosis. 2. STAGE OF LOCALIZATION (LATENT ABSCESS). There are
It may also reveal another associated intracranial lesion. no symptoms during this stage. Nature tries to localize

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 Chapter 12 — Complications of Suppurative Otitis Media 91

the pus by formation of a capsule. The stage may last for

several weeks.

3. STAGE OF ENLARGEMENT (MANIFEST ABSCESS). Ab-

scess begins to enlarge. A zone of oedema appears round
the abscess and is responsible for aggravation of symp-
toms. Clinical features at this stage are due to:
(a) Raised intracranial tension.
(b) Disturbance of function in the cerebrum or cerebel-
lum, causing focal symptoms and signs.

4. STAGE OF TERMINATION (RUPTURE OF ABSCESS). An

expanding abscess in the white matter of brain ruptures
into the ventricle or subarachnoid space resulting in fatal
meningitis.

Clinical Features
Brain abscess is often associated with other complica-
tions, such as extradural abscess, perisinus abscess, men-
ingitis, sinus thrombosis and labyrinthitis, and thus the
clinical picture may be overlapping.
Clinical features can be divided into:
1. those due to raised intracranial tension.
2. those due to area of brain affected. They are the local- Figure 12.6. CT scan showing left-sided cerebellar abscess.
izing features.

1. SYMPTOMS AND SIGNS OF RAISED INTRACRANIAL

TENSION
(a) Headache. Often severe and generalized, worse in the (v) Pupillary changes and oculomotor palsy. It indicates
morning. transtentorial herniation.
(b) Nausea and vomiting. The latter is usually projectile. (b) Cerebellar abscess (Figure 12.6)
Seen more often in cerebellar lesions. (i) Headache involves suboccipital region and may
(c) Level of consciousness. Lethargy, which progresses to be associated with neck rigidity.
drowsiness, confusion, stupor and finally coma. (ii) Spontaneous nystagmus is common and irregular
(d) Papilloedema is absent in early cases. Appears late and generally to the side of lesion.
when raised intracranial tension has persisted for (iii) Ipsilateral hypotonia and weakness.
2-3 weeks. Appears early in cerebellar abscess. (iv) Ipsilateral ataxia. Patient staggers to the side of lesion.
(e) Slow pulse and subnormal temperature. (v) Past-pointing and intention tremor can be elicited
by finger nose test.
2. LOCALIZING FEATURES (vi) Dysdiadochokinesia. Rapid pronation and supi-
(a) Temporal lobe abscess nation of the forearm shows slow and irregular
(i) Nominal aphasia. If abscess involves dominant movements on the affected side.
hemisphere, i.e. left hemisphere in right-handed
persons, patient fails to tell the names of com- Investigations
mon objects such as key, pen, etc. but can dem- 1. SKULL X-RAYS. are useful to see midline shift, if pineal
onstrate their use. gland is calcified, and also reveals gas in the abscess cav-
(ii) Homonymous hemianopia. This is due to pressure ity. They have been replaced by CT scan.
on the optic radiations. Visual field, opposite to
the side of lesion, is lost. It can be elicited by con- 2. CT SCAN. is the single most important means of inves-
frontation test, by standing in front of the patient tigation and helps to find the site and size of an abscess
and comparing his visual field with that of the ex- (Figure 12.7). It also reveals associated complications such
aminer, or by perimetry. The defect is usually in as extradural abscess, sigmoid sinus thrombosis, etc. MRI
the upper, but sometimes in the lower quadrants. has further improved the diagnosis.
(iii) Contralateral motor paralysis. In the usual upward
spread of abscess, face is involved first followed 3. X-RAY MASTOIDS OR CT SCAN. of the temporal bone
by the arm and leg. Inward spread, towards in- for evaluation of associated ear disease.
ternal capsule, involves the leg first followed by
the arm and the face. 4. LUMBAR PUNCTURE. Great care should be exercised
(iv) Epileptic fits. Involvement of uncinate gyrus caus- while doing lumbar puncture because of the risk of con-
es hallucinations of taste, and smell and invol- ing. CSF will show some rise in pressure, increase in pro-
untary smacking movements of lips and tongue. tein content but normal glucose level. White cell count
Generalized fits may occur. of CSF is raised but is much less than seen in cases of

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92 SECTION I — Diseases of Ear

E. LATERAL SINUS THROMBOPHLEBITIS

*
(SYN. SIGMOID SINUS THROMBOSIS) =
It is an inflammation of inner wall of lateral venous sinus
with formation of an intrasinus thrombus.

Aetiology
It occurs as a complication of acute coalescent mastoidi-
tis, masked mastoiditis or chronic suppuration of middle
ear and cholesteatoma.

Pathology
The pathological process can be divided into the follow-
ing stages:

1. FORMATION OF PERISINUS ABSCESS. Abscess forms in

relation to outer dural wall of the sinus. Overlying bony
dural plate may have been destroyed by coalescent bone
erosion or cholesteatoma. Sometimes, it remains intact
Figure 12.7. CT scan of right-sided otogenic cerebral abscess. when route of infection was by thrombophlebitic process.

2. ENDOPHLEBITIS AND MURAL THROMBUS FORMATION.

Inflammation spreads to inner wall of the venous sinus
meningitis. CSF contains polymorphs or lymphocytes de- with deposition of fibrin, platelets and blood cells leading
pending on the acuteness of lesion. to thrombus formation within the lumen of sinus.

Treatment 3. OBLITERATION OF SINUS LUMEN AND INTRASINUS

MEDICAL. High doses of antibiotics are given parenter- ABSCESS. Mural thrombus enlarges to occlude the sinus
ally. As the infection is often mixed, antibiotics may be lumen completely. Organisms may invade the thrombus
combined. Chloramphenicol and third generation ceph- causing intrasinus abscess which may release infected
alosporins are usually effective. Bacteroides fragilis, an emboli into the blood stream causing septicaemia.
obligate anaerobe, often seen in brain abscess, responds
to metronidazole. Aminoglycoside antibiotics, e.g. gen- 4. EXTENSION OF THROMBUS. Though central part of
tamicin, may be required if infection suspected is pseu- thrombus breaks down due to intrasinus abscess, throm-
domonas or proteus. Culture of discharge from the ear botic process continues both proximally and distally.
may be helpful in the choice of antibiotic. Proximally, it may spread to confluence of sinuses and
Raised intracranial tension can be lowered by dexa- to superior sagittal sinus or cavernous sinus, and distally,
methasone, 4 mg i.v. 6 hourly or mannitol 20% in doses into mastoid emissary vein, to jugular bulb or jugular
of 0.5 g/kg body weight. vein.
Discharge from the ear should be treated by suction
clearance and use of topical ear drops.
Bacteriology
In acute infections, haemolytic streptococcus, pneumo-
NEUROSURGICAL. Abscess is approached through a ster- coccus or staphylococcus are common. These days, ma-
ile field. Options include: (i) repeated aspiration through jority of cases of thrombophlebitis are seen in chronic
a burr hole, (ii) excision of abscess and (iii) open inci- infection with cholesteatomas, and the organisms found
sion of the abscess and evacuation of pus. The choice of are Bacillus proteus, Pseudomonas pyocyaneus, Escherichia
surgical procedure is left to the judgement of the neu- coli and Staphylococci.
rosurgeon. If abscess is treated by aspiration, it should
be followed by repeat CT or MRI scans to see if it di- Clinical Features
minishes in size. An expanding abscess, or one that does 1. HECTIC PICKET-FENCE TYPE OF FEVER WITH RIGORS.
not decrease in size, may require excision. Pus recovered This is due to septicaemia, often coinciding with release
from the abscess should be cultured and its sensitivity of septic emboli into blood stream. Fever is irregular hav-
discovered. Penicillin can be instilled into the abscess ing one or more peaks a day. It is usually accompanied
after aspiration. by chills and rigors. Profuse sweating follows fall of tem-
perature. Clinical picture resembles malaria but lacks
OTOLOGIC. Associated ear disease which caused the brain regularity.
abscess needs attention. Acute otitis media might have re- In between the bouts of fever, patient is alert with a
solved with the antibiotics given for the abscess. Chronic sense of well-being. Patients receiving antibiotics may not
otitis media would require radical mastoidectomy to re- show this picture.
move the irreversible disease and to exteriorize the infect-
ed area. Surgery of the ear is undertaken only after the ab- 2. HEADACHE. In early stage, it may be due to perisinus
scess has been controlled by antibiotics and neurosurgical abscess and is mild. Later, it may be severe when intracra-
treatment. nial pressure rises due to venous obstruction.

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 Chapter 12 — Complications of Suppurative Otitis Media 93

3. PROGRESSIVE ANAEMIA AND EMACIATION 5. Cavernous sinus thrombosis. There would be chemo-
sis, proptosis, fixation of eyeball and papilloedema.
4. GRIESINGER’S SIGN. This is due to thrombosis of mas- 6. Otitic hydrocephalus, when thrombus extends to sag-
toid emissary vein. Oedema appears over the posterior ittal sinus via confluence of sinuses.
part of mastoid.
Treatment
5. PAPILLOEDEMA. Its presence depends on obstruction 1. INTRAVENOUS ANTIBACTERIAL THERAPY. Choice of
to venous return. It is often seen when right sinus (which antibiotic will depend on sensitivity of organism and
is larger than left) is thrombosed or when clot extends to tolerance of the patient. Antibiotic can be changed af-
superior sagittal sinus. Fundus may show blurring of disc ter culture and sensitivity report is available. Antibiotics
margins, retinal haemorrhages or dilated veins. Fundus should be continued at least for a week after the opera-
changes may be absent when collateral circulation is good. tion, which is invariably required.

6. TOBEY-AYER TEST. This is to record CSF pressure by 2. MASTOIDECTOMY AND EXPOSURE OF SINUS. A com-
manometer and to see the effect of manual compression plete cortical or modified radical mastoidectomy is per-
of one or both jugular veins. formed, depending on whether sinus thrombosis has
Compression of vein on the thrombosed side produces complicated acute or chronic middle ear disease. Sinus
no effect while compression of vein on healthy side pro- bony plate is removed to expose the dura and drain the
duces rapid rise in CSF pressure which will be equal to perisinus abscess.
bilateral compression of jugular veins. An infected clot or intrasinus abscess may be present
and must be drained. In such cases, sinus dura is already
7. CROWE-BECK TEST. Pressure on jugular vein of healthy destroyed or may appear unhealthy and discoloured with
side produces engorgement of retinal veins (seen by oph- granulations on its surface. Dura is incised and the infect-
thalmoscopy) and supraorbital veins. Engorgement of ed clot and abscess drained. Before incision in the dura,
veins subsides on release of pressure. sinus is packed, above and below, by inserting a pack be-
tween the bone and dura of sinus to control bleeding.
8. TENDERNESS ALONG JUGULAR VEIN. This is seen when Healthy red clot beyond the abscess at either end of
thrombophlebitis extends along the jugular vein. There sinus should not be disturbed. Pack is removed 5-6 days
may be associated enlargement and inflammation of jug- postoperatively and wound secondarily closed.
ular chain of lymph nodes and torticollis.
3. LIGATION OF INTERNAL JUGULAR VEIN. It is rarely
Investigations required these days. It is indicated when antibiotic and
surgical treatment have failed to control embolic phe-
1. BLOOD SMEAR. is done to rule out malaria. nomenon and rigors, or tenderness and swelling along
jugular vein is spreading.
2. BLOOD CULTURE. is done to find causative organisms.
Culture should be taken at the time of chill when organ- 4. ANTICOAGULANT THERAPY. It is rarely required and
isms enter the blood stream. Repeated cultures may be used when thrombosis is extending to cavernous sinus.
required to identify the organisms.
5. SUPPORTIVE TREATMENT. Repeated blood transfu-
3. CSF EXAMINATION-CSF. is normal except for rise in sions may be required to combat anaemia and improve
pressure. It also helps to exclude meningitis. patient’s resistance.
4. X-RAY MASTOIDS. may show clouding of air cells (acute
mastoiditis) or destruction of bone (cholesteatoma). F. OTITIC HYDROCEPHALUS
It is characterized by raised intracranial pressure with nor-
5. IMAGING STUDIES. Contrast-enhanced CT scan can mal CSF findings. It is seen in children and adolescents
show sinus thrombosis by typical delta sign. It is a trian- with acute or chronic middle ear infections.
gular area with rim enhancement and central low density
area is seen in posterior cranial fossa on axial cuts. MR Mechanism
imaging better delineates thrombus. “Delta sign” may Lateral sinus thrombosis accompanying middle ear infec-
also be seen on contrast-enhanced MRI. MR venography tion causes obstruction to venous return. If thrombosis
is useful to assess progression or resolution of thrombus. extends to superior sagittal sinus, it will also impede the
function of arachnoid villi to absorb CSF. Both these fac-
6. CULTURE AND SENSITIVITY. of ear swab. tors result in raised intracranial tension.

Complications Clinical Features

1. Septicaemia and pyaemic abscesses in lung, bone, SYMPTOMS
joints or subcutaneous tissue. 1. Severe headache, sometimes intermittent, is the pre-
2. Meningitis and subdural abscess. senting feature. It may be accompanied by nausea and
3. Cerebellar abscess. vomiting.
4. Thrombosis of jugular bulb and jugular vein with 2. Diplopia due to paralysis of VIth cranial nerve.
involvement of IXth, Xth and XIth cranial nerves. 3. Blurring of vision due to papilloedema or optic atrophy.

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94 SECTION I — Diseases of Ear

SIGNS Treatment
1. Papilloedema may be 5-6 diopters, sometimes with The aim is to reduce CSF pressure to prevent optic atrophy
patches of exudates and haemorrhages. and blindness. This is achieved medically by acetazolamide
2. Nystagmus due to raised intracranial tension. and corticosteroids and repeated lumbar puncture or place-
3. Lumbar puncture. CSF pressure exceeds 300 mm H2O ment of a lumbar drain. Sometimes, draining CSF into the
(normal 70-120 mm H2O). It is otherwise normal in peritoneal cavity (lumboperitoneal shunt) is necessary.
cell, protein and sugar content and is bacteriologically Middle ear infection may require antibiotic therapy
sterile. and mastoid exploration to deal with sinus thrombosis.

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 one or more
foci of ingularly laid
is
Replaced by
bone the

Chapter 13 spongy
layer of Normal Sony
obi
Otosclerosis (Syn. Otospongiosis) capsule .

8 do of bory
Labint
fenestram lying in front of the oval window—the site of
ANATOMY OF LABYRINTH
predilection for stapedial type of otospongiosis.
It may be pertinent to review the anatomy of the laby- Heredity. About 50% of otosclerotics have positive fam-
rinth and introduce the terminology often used to de- ily history; rest are sporadic. Genetic studies reveal that
scribe it: it is an autosomal dominant trait with incomplete pen-
etrance and a variable expressivity.
1. Otic labyrinth. Also called membranous labyrinth or Race. White races are affected more than black Ameri-
endolymphatic labyrinth. It consists of utricle, saccule, cans. It is common in Indians but rare among Chinese
cochlea, semicircular ducts, endolymphatic duct and and Japanese.
sac. It is filled with endolymph. Sex. Females are affected twice as often as males but in
2. Periotic labyrinth or perilymphatic labyrinth (or India, otosclerosis seems to predominate in males.
space). It surrounds the otic labyrinth and is filled Age of onset. Hearing loss usually starts between 20 and
with perilymph. It includes vestibule, scala tympani, 30 years of age and is rare before 10 and after 40 years.
scala vestibuli, perilymphatic space of semicircular ca- Effect of other factors. Hearing loss due to otosclerosis
nals and the periotic duct, which surrounds the endo- may be initiated or made worse by pregnancy. Similarly,
lymphatic duct of otic labyrinth. deafness may increase during menopause, after an acci-
3. Otic capsule. It is the bony labyrinth. It has three dent or a major operation.
layers. The disease may be associated with osteogenesis im-
a. Endosteal. The innermost layer. It lines the bony perfecta with history of multiple fractures. The triad of
labyrinth. symptoms of osteogenesis imperfecta, otosclerosis and
b. Enchondral. Develops from the cartilage and later os- blue sclera is called van der Hoeve syndrome. Lesions of otic
sifies into bone. It is in this layer that some islands capsule seen in osteogenesis imperfecta are histologically
of cartilage are left unossified that later give rise to indistinguishable from those of otosclerosis and both are
otosclerosis. due to genes encoding type I collagen.
c. Periosteal. Covers the bony labyrinth. Viral infection. Electron microscopic and immunohis-
Otic capsule or the bony labyrinth ossifies from 14 tochemical studies have shown RNA related to measles
centres, the first one appears in the region of cochlea at virus. It is likely that otosclerosis is a viral disease as has
16 weeks and the last one appears in the posterolateral been suggested for Paget’s disease.
part of posterior semicircular canal at 20th week.
*
TYPES OF OTOSCLEROSIS z

1. STAPEDIAL OTOSCLEROSIS. Stapedial otosclerosis caus-

OTOSCLEROSIS
ing stapes fixation and conductive deafness is the most
Otosclerosis, more aptly called otospongiosis, is a primary common variety. Here lesion starts just in front of the
disease of the bony labyrinth. In this, one or more foci oval window in an area called “fissula ante fenestram.”
of irregularly laid spongy bone replace part of normally This is the site of predilection (anterior focus). Lesion may
dense enchondral layer of bony otic capsule. Most often, start behind the oval window (posterior focus), around
otosclerotic focus involves the stapes region leading to the margin of the stapes footplate (circumferential), in the
-

stapes fixation and conductive deafness. However, it may footplate but annular ligament being free (biscuit type).
involve certain other areas of the bony labyrinth where it Sometimes, it may completely obliterate the oval window
may cause neurosensory loss or no symptoms at all. niche (obliterative type) (Figure 13.1 ).

2. COCHLEAR OTOSCLEROSIS. Cochlear otosclerosis in-

AETIOLOGY volves region of round window or other areas in the otic
The exact cause of otosclerosis is not known; however, the capsule, and may cause sensorineural hearing loss prob-
-

following facts have been documented. ably due to liberation of toxic materials into the inner
Anatomical basis. Bony labyrinth is made of enchondral
-
ear fluid.
bone which is subject to little change in life. But some-
times, in this hard bone, there are areas of cartilage rests 3. HISTOLOGIC OTOSCLEROSIS. This type of otosclerosis
which due to certain nonspecific factors are activated to remains asymptomatic and causes neither conductive nor
-

form a new spongy bone. One such area is the fissula ante sensorineural hearing loss.
-

95

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 96 SECTION I — Diseases of Ear
unit ospect OH
op
·
verlig Wit

Figure 13.1. Types of stapedial otosclerosis. (A) Anterior focus. (B) Posterior focus. (C) Circumferential. (D) Biscuit type (thick plate).
(E) Obliterative.
Scan to play Otosclerosis and Its Management.

PATHOLOGY 3. Tuning fork tests show negative Rinne (i.e. BC > AC)
=>
first for 256 Hz and then 512 Hz and still later, when
Grossly, otosclerotic lesion appears chalky white, greyish stapes fixation is complete, for 1026 Hz. Weber test will
-
or yellow. Sometimes, it is red in colour due to increased be lateralized to the ear with greater conductive loss.
vascularity, in which case, the otosclerotic focus is active Absolute bone conduction may be normal. It is de-
and rapidly progressive. creased in cochlear otosclerosis with sensorineural loss.
Microscopically, spongy bone appears in the normally
dense enchondral layer of otic capsule. In immature ac- Pure tone audiometry shows loss of air conduction, more
tive lesions, there are numerous marrow and vascular for lower frequencies.
spaces with plenty of osteoblasts and osteoclasts and a Bone conduction is normal. In some cases, there is
-

lot of cement substance which stains blue (blue mantles)

-

a dip in bone conduction curve. It is different at differ-

with haematoxylin-eosin stain. Mature foci show less ent frequencies but maximum at 2000 Hz and is called
vascularity and laying of more bone and more of fibrillar Carhart’s notch (5 dB at 500 Hz, 10 dB at 1000 Hz, 15 dB
substance than cementum, and is stained red. at 2000 Hz and 5 dB at 4000 Hz) (Figure 13.2). Carhart’s
willi notch disappears after successful stapedectomy.
SYMPTOMS parade 2 Mixed hearing loss is not uncommon in otosclerosis.
There is loss in bone conduction with air-bone gap.
1. HEARING LOSS. This is the presenting symptom and Speech audiometry reveals normal discrimination
& usually starts in twenties. It is painless and progressive score except in those with cochlear involvement.
with insidious onset. Often it is bilateral conductive type.

2. PARACUSIS WILLISII. An otosclerotic patient hears bet-

-

ter in noisy than in quiet surroundings. This is because a

normal person will raise his voice in noisy surroundings.

3. TINNITUS. It is more commonly seen in cochlear oto-

-

sclerosis and in active lesions.

4. VERTIGO. It is an uncommon symptom.

-

5. SPEECH. Patient has a monotonous, well-modulated

soft speech.
-

SIGNS
1. Tympanic membrane is quite normal and mobile. Some-
times, a reddish hue may be seen on the promontory
through the tympanic membrane (Schwartze sign). This
is indicative of active focus with increased vascularity. Figure 13.2. Otosclerosis left ear. Note dip at 2000 Hz in bone
2. Eustachian tube function is normal. conduction (Carhart’s notch).

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 Chapter 13 — Otosclerosis (Syn. Otospongiosis) 97

SELECTION OF PATIENTS FOR STAPES SURGERY. Hear-

ing threshold for air conduction should be 30 dB or
worse. (It is this level when patient starts feeling socially
handicapped.)
Average air-bone gap should be at least 15 dB with
Rinne negative for 256 and 512 Hz.
Speech discrimination score should be 60% or more.

Contraindications to Stapes Surgery

1. The only hearing ear.
2. Associated Ménière’s disease. When there is history of
vertigo with clinical evidence of Ménière’s disease in
an otosclerotic patient, there are more chances of sen-
sorineural hearing loss after stapedectomy.
Figure 13.3. (A) Before removal of stapes. (B) Stapes removed and 3. Young children. Recurrent eustachian tube dysfunc-
replaced by a teflon piston. tion is common in children. It can displace the pros-
thesis or cause acute otitis media. Also the growth of
otosclerotic focus is faster in children leading to reclo-
Tympanometry may be normal in early cases but later sure of oval window.
shows a curve of ossicular stiffness. Stapedial reflex be- 4. Professional athletes, high construction workers, di-
comes absent when stapes is fixed (see p. 26). vers and frequent air travellers. Stapes surgery has the
risk to cause postoperative vertigo and/or dizziness
DIFFERENTIAL DIAGNOSIS and thus interfere with their profession; or frequent
air pressure changes may damage the hearing or cause
Otosclerosis should be differentiated from other causes severe vertigo.
of conductive deafness particularly serous otitis media, 5. Those who work in noisy surroundings. After stapedec-
adhesive otitis media, tympanosclerosis, attic fixation of tomy, they would be more vulnerable to get sensori-
head of malleus, ossicular discontinuity or congenital sta- neural hearing loss due to noise trauma.
pes fixation. 6. Otitis externa, tympanic membrane perforation and
exostosis are relative contraindications. Stapedectomy
TREATMENT can be done after they have been treated first for above
conditions. Similarly, stapedectomy is avoided during
MEDICAL. There is no medical treatment that cures oto- pregnancy.
sclerosis. Sodium fluoride has been tried to hasten the
maturity of active focus and arrest further cochlear loss, The operation is preferably done under local anaesthesia.
but controversies exist and this treatment is not recom-
mended generally. Steps of Stapedectomy (Figure 13.5)
1. Meatal incision and elevation of the tympanomeatal
SURGICAL. Stapedectomy/stapedotomy with a placement of flap.
prosthesis is the treatment of choice. Here the fixed oto- 2. Exposure of stapes area. This may require removal of
sclerotic stapes is removed and a prosthesis inserted be- posterosuperior bony overhang of the canal.
tween the incus and oval window (Figure 13.3). Prosthe- 3. Removal of stapes superstructure.
sis employed may be a teflon piston, stainless steel piston, 4. Creation of a hole in the stapes footplate (stapedoto-
platinum–teflon or titanium–teflon piston (Figure 13.4). my) or removal of a part of footplate (stapedectomy).
In 90% of patients, there is good improvement in hearing 5. Placement of prosthesis.
after stapedectomy. 6. Repositioning the tympanomeatal flap.

Complications of Stapedectomy
1. Tear of tympanomeatal flap and later perforation of
tympanic membrane
2. Injury to chorda tympani with taste disturbance par-
ticularly if opposite chorda was earlier injured
3. Incus dislocation
4. Injury to facial nerve
5. Vertigo
a. Early in postoperative period (intraoperative trau-
ma, serous labyrinthitis, long prosthesis)
b. Late due to perilymph fistula and benign paroxys-
mal positional vertigo
6. Perilymph fistula/granuloma
7. Conductive loss
Figure 13.4. Stapes prostheses. (A) Teflon piston. (B) Platinum– a. Short prosthesis
teflon piston. (C) Titanium–teflon piston. b. Loose prosthesis

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 98 SECTION I — Diseases of Ear

·
incisio
Raind
·
flap ~
-

mand
Lary
-

PS -

-
Figure 13.5. Steps of stapedectomy (see text).

c. Displacement of prosthesis Stapes mobilization is no longer done these days as it

d. Incus erosion (late) gives temporary results; refixation being quite common.
8. Sensorineural hearing loss Lempert’s fenestration operation is almost outdated now.
a. Intraoperative trauma Here an alternative window is created in the lateral semi-
b. Labyrinthitis circular canal to function for the obliterated oval window.
c. Perilymph fistula/granuloma It has the disadvantage of a postoperative mastoid cavity
9. Dead ear and an inherent hearing loss of 25 dB which cannot be
corrected.
Two per cent of patients undergoing this operation
may suffer sensorineural loss. Slowly progressive high fre-
HEARING AID. Patients who refuse surgery or are unfit for
quency loss is seen in long-term follow-up. One in 200
surgery can use hearing aid. It is an effective alternative.
patients may get a totally “dead” ear.

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 Chapter 14
Facial Nerve and Its Disorders

ANATOMY AND FUNCTIONS brainstem at pontomedullary junction, travels through

OF FACIAL NERVE posterior cranial fossa and enters the internal acoustic
meatus. At the fundus of the meatus (lateral most part of
Facial nerve runs from pons to parotid. It is a mixed nerve meatus), the nerve enters the bony facial canal, traverses
having motor and a sensory root. The latter is also called the temporal bone and comes out of the stylomastoid fo-
the nerve of Wrisberg and carries secretomotor fibres to ramen. Here it crosses the styloid process and divides into
the lacrimal gland and salivary glands, and brings fibres terminal branches. The course of the nerve (Figure 14.2)
of taste and general sensation. Thus there are two effer- can thus be divided into three parts.
ent and two afferent pathways. Components of the facial
nerve include: 1. INTRACRANIAL PART. From pons to internal acoustic
1. Special visceral efferent forms the motor root and meatus (15–17 mm).
supplies all the muscles derived from the second
branchial arch, i.e. all the muscles of facial expression, 2. INTRATEMPORAL PART. From internal acoustic mea-
auricular muscles (now vestigial), stylohyoid, posterior tus to stylomastoid foramen. It is further divided into:
belly of digastric and the stapedius. (a) Meatal segment (8–10 mm). Within internal acoustic
2. General visceral efferent supplies secretomotor fibres meatus.
to lacrimal, submandibular and sublingual glands and (b) Labyrinthine segment (4.0 mm). From fundus of mea-
the smaller secretory glands in the nasal mucosa and tus to the geniculate ganglion where nerve takes a
the palate. turn posteriorly forming a “genu.” The nerve in the
3. Special visceral afferent brings taste from the anterior labyrinthine segment has the narrowest diameter
two-thirds of tongue via chorda tympani and soft and (0.61–0.68 mm) and the bony canal in this segment
hard palate via greater superficial petrosal nerve. Taste is also the narrowest. Thus oedema or inflammation
is carried to the nucleus of tractus solitarius. can easily compress the nerve and cause paralysis.
4. General somatic afferent brings general sensation This is also the shortest segment of the nerve.
from the concha, posterosuperior part of external ca- (c) Tympanic or horizontal segment (11.0 mm). From genic-
nal and the tympanic membrane. These fibres account ulate ganglion to just above the pyramidal eminence.
for vesicular eruption in herpes zoster infection of the It lies above the oval window and below the lateral
geniculate ganglion. It also brings proprioceptive sen- semicircular canal.
sation from the facial muscles. (d) Mastoid or vertical segment (13.0 mm). From the pyra-
mid to stylomastoid foramen. Between the tympan-
NUCLEUS OF FACIAL NERVE ic and mastoid segments is the second genu of the
nerve.
Motor nucleus of the nerve is situated in the pons. It re-
ceives fibres from the precentral gyrus. Upper part of the 3. EXTRACRANIAL PART. From stylomastoid foramen to
nucleus which innervates forehead muscles receives fibres the termination of its peripheral branches.
from both the cerebral hemispheres, while the lower part
of nucleus which supplies lower face gets only crossed
fibres from one hemisphere. The function of forehead
BRANCHES OF FACIAL NERVE
is preserved in supranuclear lesions because of bilateral 1. GREATER SUPERFICIAL PETROSAL NERVE. It arises from
innervation. Facial nucleus also receives fibres from the geniculate ganglion and carries secretomotor fibres to lac-
thalamus by alternate routes and provides involuntary rimal gland and the glands of nasal mucosa and palate.
control to facial muscles. The emotional movements
such as smiling and crying are thus preserved in supra- 2. NERVE TO STAPEDIUS. It arises at the level of second
nuclear palsies because of these fibres from the thalamus genu and supplies the stapedius muscle.
(Figure 14.1).
3. CHORDA TYMPANI. It arises from the middle of vertical
COURSE OF FACIAL NERVE segment, passes between the incus and neck of malleus,
and leaves the tympanic cavity through petrotympanic
Motor fibres take origin from the nucleus of VIIth nerve, fissure. It carries secretomotor fibres to submandibular
hook round the nucleus of VIth nerve and are joined by and sublingual glands and brings taste from anterior two-
the sensory root (nerve of Wrisberg). Facial nerve leaves the thirds of tongue.

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100 SECTION I — Diseases of Ear

6. MUSCULAR BRANCHES. To stylohyoid and posterior

belly of digastric.

7. PERIPHERAL BRANCHES. The nerve trunk, after cross-

ing the styloid process, forms two divisions, an upper
temporofacial and a lower cervicofacial, which further di-
vide into smaller branches. These are the temporal, zygo-
matic, buccal, mandibular and cervical and together form
pes anserinus (goose-foot). They supply all the muscles of
facial expression.

BLOOD SUPPLY OF FACIAL NERVE

It is derived from four blood vessels: (i) Anterior-inferior
cerebellar artery supplies the nerve in cerebellopontine
angle; (ii) labyrinthine artery, branch of anterior-inferior
cerebellar artery, which supplies the nerve in internal
Figure 14.1. Forehead receives bilateral innervation and is thus auditory canal; (iii) superficial petrosal artery, a branch of
saved in supranuclear paralysis. Emotional movements controlled by
middle meningeal artery, which supplies geniculate gan-
thalamo-nuclear fibres are also preserved.
glion and the adjacent region; and (iv) stylomastoid artery,
branch of posterior auricular artery, which supplies the
mastoid and tympanic segment. All the arteries form an
external plexus which lies in the epineurium and feeds a
deeper intraneural internal plexus (Figure 14.3).

SURGICAL LANDMARKS OF FACIAL NERVE

For middle ear and mastoid surgery
1. Processus cochleariformis. It demarcates the genicu-
late ganglion which lies just anterior to it. Tympanic
segment of the nerve starts at this level.
2. Oval window and horizontal canal. The facial nerve
runs above the oval window (stapes) and below the
horizontal canal.
3. Short process of incus. Facial nerve lies medial to the
short process of incus at the level of aditus.
4. Pyramid. Nerve runs behind the pyramid and the pos-
terior tympanic sulcus.
5. Tympanomastoid suture. In vertical or mastoid seg-
ment, nerve runs behind this suture.
6. Digastric ridge. The nerve leaves the mastoid at the
anterior end of digastric ridge.
For parotid surgery (Figure 14.4)
1. Cartilaginous pointer. The nerve lies 1 cm deep and
slightly anterior and inferior to the pointer. Cartilagi-
nous pointer is a sharp triangular piece of cartilage of
the pinna and “points” to the nerve.
2. Tympanomastoid suture. Nerve lies 6–8 mm deep to
this suture.
3. Styloid process. The nerve crosses lateral to styloid
Figure 14.2. (A) Course of facial nerve. Intratemporal part consists of
process.
four segments: meatal (1), labyrinthine (2), tympanic (3) and mastoid 4. Posterior belly of digastric. If posterior belly of di-
(4). (B) Branches of facial nerve on face. gastric muscle is traced backwards along its upper bor-
der to its attachment to the digastric groove, nerve is
found to lie between it and the styloid process.
4. COMMUNICATING BRANCH. It joins auricular branch of
vagus and supplies the concha, retroauricular groove, pos-
VARIATION AND ANOMALIES OF FACIAL
terior meatus and the outer surface of tympanic membrane.
NERVE (FIGURE 14.5)
5. POSTERIOR AURICULAR NERVE. It supplies muscles of 1. Bony dehiscence. This is the most common anom-
pinna, occipital belly of occipitofrontalis and communi- aly. Dehiscence (absence of bony cover) occurs
cates with auricular branch of vagus. most commonly in tympanic segment over the oval

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 Chapter 14 — Facial Nerve and Its Disorders 101

window. It also occurs near the region of geniculate

ganglion or in the region of retrofacial mastoid cells.
A dehiscent nerve is prone to injury at the time of
surgery or gets easily involved in mastoid and middle
ear infections.
2. Prolapse of nerve. The dehiscent nerve may prolapse
over the stapes and make stapes surgery or ossicular
reconstruction difficult.
3. Hump. The nerve may make a hump posteriorly near
the horizontal canal making it vulnerable to injury
while exposing the antrum during mastoid surgery.
4. Bifurcation and trifurcation. The vertical part of
facial nerve divides into two or three branches, each
occupying a separate canal and exiting through indi-
vidual foramen.
5. Bifurcation and enclosing the stapes. The nerve
divides proximal to oval window—one part passing
above and the other below it and then reuniting.
6. Between oval and round windows. Just before oval
window the nerve crosses the middle ear passing be-
tween oval and round windows.
Anomalies of the nerve are more common in congeni-
tal ears; utmost care should be taken while operating cas-
es of microtia or other congenital conditions of the ear.

STRUCTURE OF NERVE
From inside out, a nerve fibre consists of axon, myelin
sheath, neurilemma and endoneurium. A group of nerve
fibres is enclosed in a sheath called perineurium to form a
fascicle and the fascicles are bound together by epineurium
(Figure 14.6).

Figure 14.3. Blood supply of facial nerve. (1) Cerebellopontine an- SEVERITY OF NERVE INJURY
gle: Anterior-inferior cerebellar artery. (2) Internal auditory canal:
Labyrinthine artery. (3) Geniculate ganglion and adjacent facial nerve: Degree of nerve injury will determine the regeneration of
Superficial petrosal. (4) Mastoid segment: Stylomastoid artery. Thus nerve and its function. Earlier nerve injuries were divided
both carotid and vertebrobasilar systems supply the nerve and meet into:
at labyrinthine segment.
1. Neurapraxia, a conduction block, where flow of axo-
plasm through the axons was partially obstructed.
2. Axonotmesis—injury to axons.
3. Neurotmesis—injury to nerve.
Sunderland classified nerve injuries into five degrees of
severity based on anatomical structure of the nerve and
this classification is now widely accepted.
1°= Partial block to flow of axoplasm; no morphologi-
cal changes are seen. Recovery of function is complete
(neurapraxia).
2°= Loss of axons, but endoneurial tubes remain intact.
During recovery, axons will grow into their respective
tubes, and the result is good (axonotmesis).
3°= Injury to endoneurium. During recovery, axons of
one tube can grow into another. Synkinesis can occur
(neurotmesis).
4°= Injury to perineurium in addition to above. Scarring
will impair regeneration of fibres (partial transection).
5°= Injury to epineurium in addition to above (complete
nerve transection).
The first three degrees are seen in viral and inflamma-
Figure 14.4. Surgical landmarks of the facial nerve in parotid tory disorders while fourth and fifth are seen in surgical
surgery. or accidental trauma to the nerve or in neoplasms.

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102 SECTION I — Diseases of Ear

Figure 14.5. Variations and abnormalities in the course of facial nerve. (A) Normal, (B) bony dehiscence, (C) hump posteriorly (near the second
genu), (D) bifurcation, (E) trifurcation, (F) bifurcating and reuniting round the oval window and (G) the nerve passing between the oval and
round windows.

Figure 14.6. Structure of a nerve. (A) Cross section of nerve. (B) Structure of a nerve fibre, longitudinal and cross-sectional views.

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 Chapter 14 — Facial Nerve and Its Disorders 103

ELECTRODIAGNOSTIC TESTS Thus ENoG and EMG are complimentary and help to
prognosticate in cases of facial paralysis and in deciding
These tests are useful to differentiate between neurapraxia the procedure for reanimation, i.e. nerve substitution ver-
and degeneration of the nerve. They also help to predict sus muscle transposition or sling operation.
prognosis and indicate time for surgical decompression
of the nerve.

1. MINIMAL NERVE EXCITABILITY TEST. The nerve is CAUSES OF FACIAL PARALYSIS

stimulated at steadily increasing intensity till facial twitch
is just noticeable. This is compared with the normal side. The cause may be central or peripheral. The peripheral le-
There is no difference between the normal and paralyzed sion may involve the nerve in its intracranial, intratem-
side in conduction block. In other injuries, where degener- poral or extratemporal parts. Peripheral lesions are more
ation sets in, nerve excitability is gradually lost. When the common and about two-thirds of them are of the idio-
difference between two sides exceed 3.5 m amp, the test pathic variety (Table 14.1).
is positive for degeneration. Degeneration of fibres cannot
be detected earlier than 48–72 h of its commencement. A. IDIOPATHIC
2. MAXIMAL STIMULATION TEST (MST). This test is simi- 1. Bell’s Palsy
lar to the minimal nerve excitability test but instead of Sixty to seventy-five per cent of facial paralysis is due to
measuring the threshold of stimulation, the current level Bell’s palsy. It is defined as idiopathic, peripheral facial pa-
which gives maximum facial movement is determined ralysis or paresis of acute onset. Both sexes are affected with
and compared with the normal side. Response is visually
graded as equal, decreased or absent. Reduced or absent
response with maximal stimulation indicates degenera- TABLE 14.1 CAUSES OF FACIAL PARALYSIS
tion and is followed by incomplete recovery.
• Central
3. ELECTRONEURONOGRAPHY (ENOG). It is a sort of • Brain abscess
evoked electromyography. The facial nerve is stimulated at • Pontine gliomas
the stylomastoid foramen and the compound muscle ac- • Poliomyelitis
• Multiple sclerosis
tion potentials are picked up by the surface electrodes. Su-
• Intracranial part (cerebellopontine angle)
pramaximal stimulation is used to obtain maximal action
• Acoustic neuroma
potentials. The responses of action potentials of the para- • Meningioma
lyzed side are compared with that of the normal side on • Congenital cholesteatoma
similar stimulation and thus percentage of degenerating • Metastatic carcinoma
fibres is calculated. Studies reveal that degeneration of 90% • Meningitis
occurring in the first 14 days indicates poor recovery of • Intratemporal part
function. Faster rate of degeneration occurring in less than • Idiopathic
14 days has a still poorer prognosis. ENoG is most useful – Bell palsy
between 4 and 21 days of the onset of complete paralysis. – Melkersson syndrome
• Infections
4. ELECTROMYOGRAPHY (EMG). This tests the motor – Acute suppurative otitis media
activity of facial muscles by direct insertion of needle – Chronic suppurative otitis media
electrodes usually in orbicular oculi and orbicularis oris – Herpes zoster oticus
– Malignant otitis externa
muscles and the recordings are made during rest and vol-
• Trauma
untary contraction of muscle. – Surgical: Mastoidectomy and stapedectomy
In a normal resting muscle, biphasic or triphasic po- – Accidental: Fractures of temporal bone
tentials are seen every 30–50 ms. • Neoplasms
In a denervated muscle, spontaneous involuntary ac- – Malignancies of external and middle ear
tion potentials called fibrillation potentials are seen. They – Glomus tumour
appear 14–21 days after denervation. With regeneration – Facial nerve neuroma
of the nerve after injury, polyphasic reinnervation potentials – Metastasis to temporal bone (from cancer of breast,
replace fibrillation potentials. They appear 6–12 weeks bronchus, prostate)
prior to clinical evidence of facial function and thus pro- • Extracranial part
• Malignancy of parotid
vide the earliest evidence of recovery.
• Surgery of parotid
Voluntary contraction causes motor discharge. Dimin- • Accidental injury in parotid region
ished or no response to voluntary contraction is seen af- • Neonatal facial injury (obstetrical forceps)
ter nerve injury. • Systemic diseases
Electromyography is useful in planning reanimation • Diabetes mellitus
procedures. Presence of normal or polyphasic potentials • Hypothyroidism
after 1 year of injury indicates that reinnervation is taking • Uraemia
place and there is no need for reanimation procedure. If • Polyarteritis nodosa
fibrillation potentials are seen, it indicates intact motor • Wegener’s granulomatosis
end plates but no evidence of reinnervation and need for • Sarcoidosis (Heerfordt’s syndrome)
• Leprosy
nerve substitution. Electrical silence indicates atrophy of
• Leukaemia
motor end plates and need for muscle transfer procedures • Demyelinating disease
rather than nerve substitution.

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104 SECTION I — Diseases of Ear

equal frequency. Any age group may be affected though (stapedial paralysis) or loss of taste (involvement of chor-
incidence rises with increasing age. A positive family his- da tympani). Paralysis may be complete or incomplete.
tory is present in 6–8% of patients. Risk of Bell palsy is Bell palsy is recurrent in 3–10% of patients.
more in diabetics (angiopathy) and pregnant women (re-
tention of fluid).
-
DIAGNOSIS. Diagnosis is always by exclusion. All other
known causes of peripheral facial paralysis should be ex-
AETIOLOGY cluded. This requires careful -history, complete otological
1. VIRAL INFECTION. Most of the evidence supports the and head and neck examination, X-ray studies, blood
tests such as total count, peripheral smear, sedimentation
-

viral aetiology due to herpes simplex, herpes zoster or the

-

rate, blood sugar and serology.

-

Epstein–Barr virus. Other cranial nerves may also be in-

volved in Bell palsy which is thus considered a part of the Nerve excitability tests are done daily or on alternate
total picture of polyneuropathy. days and compared with the normal side to monitor
2. VASCULAR ISCHAEMIA. It may be primary or second- nerve degeneration.
ary. Primary ischaemia is induced by cold or emotional Localizing the site of lesion (topodiagnosis) helps in
stress. Secondary ischaemia is the result of primary ischae- establishing the aetiology and also the site of surgical de-
mia which causes increased capillary permeability lead- compression of nerve, if that becomes necessary.
ing to exudation of fluid, oedema and compression of
TREATMENT
microcirculation of the nerve.
GENERAL
3. HEREDITARY. The fallopian canal is narrow because of -O
1. Reassurance.
hereditary predisposition and this makes the nerve suscepti- Y
2. Relief of ear pain by analgesics.
ble to early compression with the slightest oedema. Ten per
3. Care of the eye as outlined on p. 108. Eye must be pro-
cent of the cases of Bell palsy have a positive family history. Z

tected against exposure keratitis.

4. AUTOIMMUNE DISORDER. T-lymphocyte changes have =
4. Physiotherapy or massage of- the facial muscles gives
been observed. -

psychological support to the patient. It has not been

CLINICAL FEATURES (FIGURES 14.7 AND 14.8 A,B ). shown to influence recovery. Active facial movements
Onset is sudden. Patient is unable to close his eye. On at- are encouraged when there is return of some move-
tempting to close the eye, eyeball turns up and out (Bell ment to the facial muscles.
phenomenon). Saliva dribbles from the angle of mouth. MEDICAL MANAGEMENT
Face becomes asymmetrical. Tears flow down from the
eye (epiphora). Pain in the ear may precede or accompany • Steroids.
=
Their utility has not been proved beyond
the nerve paralysis. Some complain of noise intolerance doubt in carefully controlled studies. Prednisolone is
-
the drug of choice. If patient reports within 1 week,
the adult dose of prednisolone is 1 mg/kg/day divided
into morning and evening doses for 5 days. Patient is
seen on the fifth day. If paralysis is incomplete or is
recovering, dose is tapered during the next 5 days. If
paralysis remains complete, the same dose is contin-
ued for another 10 days and thereafter tapered in next
5 days (total of 20 days). Contraindications to use of
steroids include pregnancy, diabetes, hypertension,
peptic ulcer, pulmonary tuberculosis and glaucoma.
Steroids have been found useful to prevent incidence
of synkinesis, crocodile tears and to shorten the recov-
ery time of facial paralysis. Steroids can be combined
with acyclovir for Herpes zoster oticus or Bell palsy.
• Other drugs. Vasodilators, vitamins, mast cell inhibitors
- -

Figure 14.7. Facial paralysis left side. Compare it with normal side. and antihistaminics have not been found useful.

Figure 14.8. Bell’s palsy left side: (A) Adult. (B) Child.
Scan to play Bell’s Palsy.

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 Chapter 14 — Facial Nerve and Its Disorders 105

Figure 14.9. Ramsay–Hunt syndrome. Note facial palsy and small vesicles in the concha of the right side.

*
SURGICAL TREATMENT. Nerve decompression relieves
pressure on the nerve fibres and thus improves the micro-
circulation of the nerve. Vertical and tympanic segments
of nerve are decompressed. Some workers have suggested
total decompression including labyrinthine segment by
postaural and middle fossa approach.

PROGNOSIS. Eighty-five to ninety per cent of the patients

recover fully. Ten to fifteen per cent recover incomplete-
ly and may be left with some stigmata of degeneration.
Recurrent facial palsy may not recover fully. Prognosis is
good in incomplete Bell palsy (95% complete recovery)
and in those where clinical recovery starts within 3 weeks
of onset (75% complete recovery).

2. Melkersson Syndrome
It is also an idiopathic disorder consisting of a triad of
facial paralysis, swelling of lips and fissured tongue. Pa-
ralysis may be recurrent. Treatment is the same as for Bell
palsy.
• Recurrent facial palsy. Recurrent facial palsy is seen Figure 14.10. (A) A longitudinal fracture runs along the axis of pe-
in Bell palsy (3–10% cases), Melkersson syndrome, trous pyramid. Typically, it starts at the squamous part of temporal
bone, runs through the roof of the external ear canal and middle ear
diabetes, sarcoidosis and tumours. Recurrent palsy on
towards the petrous apex and to the foramen lacerum. (B) Transverse
the same side may be caused by a tumour in 30% of
fracture. It runs across the axis of petrous. Typically, it begins at the
cases. foramen magnum, passes through occipital bone, jugular fossa and
• Bilateral facial paralysis. Simultaneous bilateral fa- petrous pyramid, ending in the middle cranial fossa. It may pass me-
cial paralysis may be seen in Guillain-Barré syndrome, dial, lateral or through the labyrinth.
sarcoidosis, sickle cell disease, acute leukaemia, bulbar
palsy, leprosy and some other systemic disorders. C. TRAUMA
1. Fractures of Temporal Bone
B. INFECTIONS Fractures of temporal bone may be longitudinal, trans-
Herpes Zoster Oticus (Ramsay–Hunt verse or mixed (Figures 14.10 and 14.11). Facial palsy is
O seen more often in transverse fractures (50%). Paraly-
Syndrome)
sis is due to intraneural haematoma, compression by a
There is facial paralysis along with vesicular rash in the
bony spicule or transection of nerve. In these cases, it
O externalauditory canal and pinna (Figure 14.9). There
is important to know whether paralysis was of immedi-
may also be anaesthesia of face, giddiness and hearing
ate or delayed onset. Delayed onset paralysis is treated
impairment due to involvement of Vth and VIIIth nerves.
- conservatively like Bell palsy while immediate onset pa-
Treatment is the same as for Bell palsy.
ralysis may require surgery in the form of decompres-
Infections of Middle Ear (see p. 89) sion, re-anastomosis of cut ends or cable nerve graft
Malignant Otitis Externa (see p. 55) (Table 14.2).

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106 SECTION I — Diseases of Ear

Figure 14.11. Longitudinal fracture of the temporal bone right side. (A) CT scan showing the fracture line. (B) Fracture line as seen during the
operation (arrow).

TABLE 14.2 DIFFERENCES IN LONGITUDINAL AND TRANSVERSE FRACTURES OF TEMPORAL BONE

Longitudinal Transverse
Frequency More common (80%) Less common (20%)
Type of injury Parietal blow Occipital blow
Fracture line Runs parallel to long axis of petrous pyramid. Runs across the petrous. Starts at foramen magnum
Starts at squamous part of temporal bone to or jugular foramen towards the foramen
end at foramen lacerum spinosum
Bleeding from ear Common, due to injury to tegmen and Absent because tympanic membrane is intact.
tympanic membrane Haemotympanum may be seen
Cerebrospinal fluid otorrhoea Present, often mixed with blood Absent or unmanifested
Structures injured Tegmen, ossicles and tympanic membrane Labyrinth or CN VIII
Hearing loss Conductive Sensorineural
Vertigo Less often; due to concussion Severe, due to injury to labyrinth or CN VIII
Facial paralysis Less (20%), delayed onset. Nerve is injured Most common (50%). Immediate onset. Injury to
in tympanic segment, distal to geniculate nerve in meatal or labyrinthine segment proximal
ganglion to geniculate ganglion.

2. Ear or Mastoid Surgery (a) Anatomical knowledge of the course of facial nerve,
Facial nerve is injured during stapedectomy, tympanoplasty possible variations and anomalies and its surgical
or mastoid surgery. Paralysis may be immediate or delayed landmarks. Cadaver dissections should be an impor-
and treatment is the same as in temporal bone trauma. tant part of the training in ear surgery.
Sometimes, nerve is paralyzed due to pressure of packing (b) Always working along the course of nerve and never
on the exposed nerve and this should be relieved first. across it.
Operative injuries to facial nerve can be avoided if at- (c) Constant irrigation when drilling to avoid thermal in-
tention is paid to the following: jury. Use diamond burr when working near the nerve.

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 Chapter 14 — Facial Nerve and Its Disorders 107

(d) Gentle handling of the nerve when it is exposed,

avoiding any pressure of instruments on the nerve.
(e) Not to remove any granulations that penetrate the nerve.
(f) Using magnification; never to work on facial nerve
without an operating microscope.

3. Parotid Surgery and Trauma to Face

Facial nerve may be injured in surgery of parotid tumours
or deliberately excised in malignant tumours. Accidental
injuries in the parotid region can also cause facial paraly-
sis. Application of obstetrical forceps may also result in
facial paralysis in the neonate due to pressure on the ex-
tratemporal part of nerve.

D. NEOPLASMS
1. Intratemporal Neoplasms
Carcinoma of external or middle ear, glomus tumour,
rhabdomyosarcoma and metastatic tumours of temporal
bone, all result in facial paralysis. Facial nerve neuroma
occurs anywhere along the course of nerve and produces
paralysis of gradual or sudden onset. It is treated by ex- Figure 14.12. Topographical localization of the VIIth nerve lesions.
cision and nerve grafting. High-resolution CT scan and (A) Suprageniculate or transgeniculate lesion. Secretomotor fibres to
gadolinium-enhanced MRI is very useful for facial nerve the lacrimal gland leave at the geniculate ganglion and are interrupted
tumour. in lesions situated at/or proximal to the geniculate ganglion. (B) Su-
prastapedial lesions cause loss of stapedial reflex and taste but preserve
2. Tumours of Parotid lacrimation. (C) Infrastapedial lesions cause loss of taste but preserve
stapedial reflex and lacrimation. (D) Infrachordal lesions cause loss of
Facial paralysis with tumour of the parotid almost always
facial motor function alone.
implies malignancy (see Tumours of salivary glands). Scan to play Anatomy and Functions of Facial Nerve.

E. SYSTEMIC DISEASES AND FACIAL

PARALYSIS A lesion outside the temporal bone, in the parotid area,
affects only the motor functions of nerve. It may some-
Peripheral facial paralysis is mostly of idiopathic variety but times be incomplete as some branches of the nerve may
always needs exclusion of diabetes, hypothyroidism, leu- not be involved in tumour or trauma.
kaemia, sarcoidosis, periarteritis nodosa, Wegener’s granu-
lomatosis, leprosy, syphilis and demyelinating disease.
TOPODIAGNOSTIC TESTS FOR LESIONS IN
INTRATEMPORAL PART (FIGURE 14.12 )
LOCALIZATION OF FACIAL LESION The following tests are useful in finding the site of lesion
in paralysis of lower motor neuron.
A. CENTRAL FACIAL PARALYSIS
It is caused by cerebrovascular accidents (haemorrhage, 1. SCHIRMER TEST. It compares lacrimation of the two
-

thrombosis or embolism), tumour or an abscess. It causes sides. A strip of filter paper is hooked in the lower fornix
paralysis of only the lower half of face on the contralat- of each eye and the amount of wetting of strip measured.
eral side. Forehead movements are retained due to bilat- Decreased lacrimation indicates lesion proximal to the
eral innervation of frontalis muscle. Involuntary emo- geniculate ganglion as the secretomotor fibres to lacrimal
tional movements and the tone of facial muscles are also gland leave at the geniculate ganglion via greater superfi-
retained. cial petrosal nerve.

2.-STAPEDIAL REFLEX. Stapedial reflex is lost in lesions

B. PERIPHERAL FACIAL PARALYSIS
above the nerve to stapedius. It is tested by tympanometry.
All the muscles of the face on the involved side are para-
lyzed. Patient is unable to frown, close the eye, purse the 3. TASTE TEST. It can be measured by a drop of salt
-
lips or whistle. or sugar solution placed on one side of the protruded
A lesion at the level of nucleus is identified by associated tongue, or by electrogustometry. Impairment of taste in-
paralysis of VIth nerve. dicates lesion above the chorda tympani.
A lesion at cerebellopontine angle is identified by the pres-
ence of vestibular and auditory defects and involvement 4. SUBMANDIBULAR SALIVARY FLOW TEST. It also meas-
-

of other cranial nerves such as Vth, IXth, Xth and XIth. ures function of chorda tympani. Polythene tubes are
A lesion in the bony canal, from internal acoustic meatus passed into both Wharton ducts and drops of saliva
to stylomastoid foramen, can be localized by topodiag- counted during one minute period. Decreased salivation
nostic tests. shows injury above the chorda.

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108 SECTION I — Diseases of Ear

COMPLICATIONS FOLLOWING FACIAL

PARALYSIS
Peripheral facial paralysis due to any cause may result in
any of the following complications:

1. INCOMPLETE RECOVERY. Facial asymmetry persists.

Eye cannot be closed resulting in epiphora. A weak oral
sphincter causes drooling and difficulty in taking food.

2. EXPOSURE KERATITIS. Eye cannot be closed, tear film

from the cornea evaporates causing dryness, exposure
keratitis and corneal ulcer. This is worse when tear pro-
duction is also affected. It can be prevented by use of arti-
ficial tears (methylcellulose drops) every 1–2 h, eye oint-
ment and proper cover for the eye at night.
Temporary tarsorrhaphy may also be indicated. Eye
closure can also be improved by using gold-weight im-
plant sutured to the tarsal plate deep to levator palpebrae
muscle. Figure 14.13. Hemifacial spasm. Note all the facial muscles and
platysma in the spasm. Picture taken during paroxysm of clonic con-
tractions.
3. SYNKINESIS (MASS MOVEMENT). When the patient
wishes to close the eye, corner of mouth also twitches or
vice versa. It is due to cross innervation of fibres; there is
no treatment. is acoustic neuroma, congenital cholesteatoma or glomus
tumour. Many cases of hemifacial spasm are due to irrita-
4. TICS AND SPASMS. They are the result of faulty regen- tion of the nerve because of a vascular loop at the cerebel-
eration of fibres. Involuntary movements are seen on the lopontine angle. Microvascular decompression through
affected side of the face. posterior fossa craniotomy has met with high success rate
in these cases. Idiopathic type has been treated by selec-
5. CONTRACTURES. They result from fibrosis of atrophied tive section of the branches of facial nerve in the parotid
muscles or fixed contraction of a group of muscles. They or by puncturing the facial nerve with a needle in its tym-
affect movements of face but facial symmetry at rest is panic segment.
good. Botulinum toxin has been used in the affected muscle.
It blocks the neuromuscular junction by preventing re-
6. CROCODILE TEARS (GUSTATORY LACRIMATION). There lease of acetylcholine.
is unilateral lacrimation with mastication. This is due to
faulty regeneration of parasympathetic fibres which now 2. BLEPHAROSPASM. Twitchings and spasms are limited
supply lacrimal gland instead of the salivary glands. It to orbiculars oculi muscles on both sides. The eyes are
can be treated by section of greater superficial petrosal closed due to muscle spasms causing functional blind-
nerve or tympanic neurectomy. ness. The cause is uncertain, but probably lies in the basal
ganglia. It is treated by selective section of nerves supply-
7. FREY’S SYNDROME (GUSTATORY SWEATING). There is ing muscles around the eye on both sides.
sweating and flushing of skin over the parotid area during Botulinum-A toxin injected into the periorbital mus-
mastication. It results from parotid surgery. cles gives relief for 3–6 months. Injection can be repeated,
if necessary.
8. PSYCHOLOGICAL AND SOCIAL PROBLEMS. Drooling
during eating and drinking and impairment of speech
cause social problems.
SURGERY OF FACIAL NERVE
1. DECOMPRESSION. The nerve may be compressed by
HYPERKINETIC DISORDERS oedema, haematoma or a fractured bone in its intratem-
OF FACIAL NERVE poral part. The bony canal is exposed and uncapped. The
sheath of nerve is also slit to relieve pressure due to oede-
They are characterized by involuntary twitching of facial ma or intraneural haematoma.
muscles on one or both sides.
2. END-TO-END ANASTOMOSIS. This is done when the
1. HEMIFACIAL SPASM. It is characterized by repeated, gap between severed ends of the nerves is only a few mil-
uncontrollable twitchings of facial muscles on one side limetres. It is a suitable procedure for extratemporal part
(Figure 14.13). It is of two types (i) essential or idiopathic, of the nerve. There should not be any tension in the ap-
where cause is not known and (ii) secondary, where cause proximated ends.

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 Chapter 14 — Facial Nerve and Its Disorders 109

3. NERVE GRAFT (CABLE GRAFT). When the gap between movements of facial muscles, but at the expense of atro-
severed ends cannot be closed by end-to-end anastomo- phy of tongue on that side. However, disability of tongue
sis, a nerve graft is more suitable than extensive rerout- due to atrophy is not so severe and patient adjusts to the
ing or mobilization of nerve. Nerve graft is taken from difficulty in chewing and articulation after a few weeks.
greater auricular, lateral cutaneous nerve of thigh or the
sural nerve. In the bony canal, the graft may not require 5. PLASTIC PROCEDURES. They are used to improve cos-
any suturing. metic appearance when nerve grafting is not feasible or
has failed. The procedures include facial slings, face lift
4. HYPOGLOSSAL-FACIAL ANASTOMOSIS. Hypoglossal operation or slings of masseter and temporalis muscle.
nerve is anastomosed to the severed peripheral end of the The latter also gives some movement to face in addition
facial nerve. It improves the muscle tone and permits some to symmetry.

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 Chapter 15
·

Ne
Ménière’s Disease
S

Nystagmus
T
Ataxia
Ménière’s disease, also called endolymphatic hydrops, is a 3. ALLERGY. The offending allergen may be a foodstuff
disorder of the inner ear where the endolymphatic sys- or an inhalant. In these cases, inner ear acts as the “shock
tem is distended with endolymph. It is characterized by organ” producing excess of endolymph. Nearly 50% of
(i) vertigo, (ii) sensorineural hearing loss, (iii) tinnitus and patients with Ménière’s disease have concomitant inhal-
(iv) aural fullness. ant and/or food allergy.
It is possible that Ménière’s disease is multifactorial,
resulting in the common end point of endolymphatic hy-
PATHOLOGY drops with classical presentation.

The main pathology is distension of endolymphatic sys- 4. SODIUM AND WATER RETENTION. Excessive amounts
tem, mainly affecting the cochlear duct (scala media) of fluid are retained leading to endolymphatic hydrops.
and the saccule, and to a lesser extent the utricle and
semicircular canals. The dilatation of cochlear duct is 5. HYPOTHYROIDISM. About 3% of cases of Ménière’s
such that it may completely fill the scala vestibuli; there disease are due to hypothyroidism. Such cases benefit
is marked bulging of Reissner’s membrane, which may from thyroid replacement therapy.
even herniate through the helicotrema into the apical
part of scala tympani (Figure 15.1). The distended sac-
6. AUTOIMMUNE AND VIRAL AETIOLOGIES have also been
cule may come to lie against the stapes footplate. The
suggested on the basis of experimental, laboratory and
utricle and saccule may show outpouchings into the
clinical observations.
semicircular canals.

AETIOLOGY CLINICAL FEATURES

The main pathology in Ménière’s disease is distension of Age and sex. Disease is commonly seen in the age group of
endolymphatic system due to increased volume of endo- 35–60 years. Males are affected more than females. Usu-
lymph. This can result either from increased production ally, disease is unilateral but the other ear may be affected
of endolymph or its faulty absorption or both. Normally, after a few years.
endolymph is secreted by stria vascularis, fills the mem- Cardinal symptoms of Ménière’s disease are (i) episodic
branous labyrinth and is absorbed through the endolym- vertigo, (ii) fluctuating hearing loss, (iii) tinnitus and (iv)
phatic sac (see p. 11 for inner ear fluids). sense of fullness or pressure in the involved ear.
The exact cause of Ménière’s disease is not yet known.
Various theories have been postulated (Figure 15.2). 1. VERTIGO. It comes in attacks. The onset is sudden. Pa-
tient gets a feeling of rotation of himself or his environ-
1. DEFECTIVE ABSORPTION BY ENDOLYMPHATIC SAC. ment. Sometimes, there is feeling of “to and fro” or “up
Normally, endolymph is carried by the endolymphatic and down” movement. Attacks come in clusters, with pe-
duct to the sac where it is absorbed. Defective absorp- riods of spontaneous remission lasting for weeks, months
tion by the sac may be responsible for raised endolymph or years. Usually, an attack is accompanied by nausea and
pressure. Experimental obstruction of endolymphatic sac vomiting with ataxia and nystagmus. Severe attacks may
and its duct also produces hydrops. Ischaemia of sac has be accompanied by other symptoms of vagal disturbances
been observed in cases of Ménière’s disease undergoing such as abdominal cramps, diarrhoea, cold sweats, pallor
sac surgery, indicating poor vascularity and thus poor ab- and bradycardia. Usually, there is no warning symptom of
sorption by the sac. Distension of membranous labyrinth an oncoming attack of vertigo but sometimes the patient
leads to rupture of Reissner’s membrane and thus mixing may feel a sense of fullness in the ear, change in character
of perilymph with endolymph, which is thought to bring of tinnitus or discomfort in the ear which herald an attack.
about an attack of vertigo. Some cases of Ménière’s disease show Tullio phenomenon.
It is a condition where loud sounds or noise produce ver-
2. VASOMOTOR DISTURBANCE. There is sympathetic over- tigo and is due to the distended saccule lying against the
activity resulting in spasm of internal auditory artery and/ stapes footplate. This phenomenon is also seen when there
or its branches, thus interfering with the function of coch- are three functioning windows in the ear, e.g. a fenestra-
lear or vestibular sensory neuroepithelium. This is respon- tion of horizontal canal in the presence of a mobile stapes.
sible for deafness and vertigo. Anoxia of capillaries of stria
vascularis also causes increased permeability, with transu- 2. HEARING LOSS. It usually accompanies vertigo or may
dation of fluid and increased production of endolymph. precede it. Hearing improves after the attack and may be

111

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112 SECTION I — Diseases of Ear
.
otoscopy
o N

If
· .

surfore
O
laudiamet
O

Figure 15.1. (A) Normal cochlear duct. (B) Cochlear duct is distended with endolymph pushing the Reissner’s membrane into scala vestibuli.

* #
EXAMINATION
Y V
1. OTOSCOPY. No abnormality is seen in the tympanic
membrane.
V ~
* 2. NYSTAGMUS. It is seen only during acute attack. The
quick component of nystagmus is towards the unaffected
Y
ear.
* Y
Y 3. TUNING FORK TESTS. They indicate sensorineural hear-
ing loss. Rinne test is positive, absolute bone conduction is
E reduced in the affected ear and Weber is lateralized to the
better ear.
Figure 15.2. Aetiologic factors and symptomatology of Ménière’s
disease (endolymphatic hydrops).
INVESTIGATIONS
normal during the periods of remission. This fluctuating
nature of hearing loss is quite characteristic of the disease. 1. PURE TONE AUDIOMETRY. There is sensorineural hear-
-

With recurrent attacks, improvement in hearing during ing loss. In early stages, lower frequencies are affected and
remission may not be complete; some hearing loss being the curve is of rising type. When higher frequencies are
added in every attack leading to slow and progressive de- involved curve becomes flat or a falling type (Figure 15.3).
terioration of hearing which is permanent.
• Distortion of sound. Some patients complain of distort-
ed hearing. A tone of a particular frequency may ap-
pear normal in one ear and of higher pitch in the other
leading to diplacusis. Music appears discordant.
• Intolerance to loud sounds. Patients of Ménière’s disease
cannot tolerate amplification of sound due to recruitment
phenomenon. They are poor candidates for hearing aids.

3. TINNITUS. It is low-pitched roaring type and is ag-

gravated during acute attacks. Sometimes, it has a hiss-
ing character. It may persist during periods of remission.
Change in intensity and pitch of tinnitus may be the
warning symptom of attack.

4. SENSE OF FULLNESS OR PRESSURE. Like other symp-

toms, it also fluctuates. It may accompany or precede an
attack of vertigo.

5. OTHER FEATURES. Patients of Ménière’s disease often Figure 15.3. (A) Audiogram in early Ménière’s disease. Note: Hear-
show signs of emotional upset due to apprehension of ing loss is sensorineural and more in lower frequencies—the rising
the repetition of attacks. Earlier, the emotional stress was curve. As the disease progresses, middle and higher frequencies get
considered to be the cause of Ménière’s disease. involved and audiogram becomes flat or falling type (B & C).

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 Chapter 15 — Ménière’s Disease 113

TABLE 15.1 RESULTS OF VARIOUS TESTS TO DIFFERENTIATE A COCHLEAR FROM A RETROCOCHLEAR LESION
Normal Cochlear lesion Retrocochlear lesion
Pure tone audiogram Normal Sensorineural hearing loss Sensorineural hearing loss
Speech discrimination score 90–100% Below 90% Very poor
Roll over phenomenon Absent Absent Present
Recruitment Absent Present Absent
SISI score 0–15% Over 70% 0–20%
Threshold tone decay test 0–15 dB Less than 25 dB Above 25 dB
Stapedial reflex Present Present Absent
Stapedial reflex decay (page 107) Normal Normal Abnormal
BERA Normal interval between wave I & V Normal interval between wave I & V Wave V delayed or absent

BERA, brainstem evoked response audiometry.

~
2. -
SPEECH AUDIOMETRY. Discrimination score is usually 5. CALORIC TEST. It shows reduced response on the af-
55–85% between the attacks but discrimination ability is fected side in 75% of cases. Often, it reveals a canal pa-
much impaired during and immediately following an at- resis on the affected side (most common) but sometimes
tack. there is directional preponderance to healthy side or a
V combination of both canal paresis on the affected side
3. SPECIAL AUDIOMETRY TESTS. They indicate the and directional preponderance on the opposite side.
cochlear nature of disease and thus help to differenti-
ate from retrocochlear lesions, e.g. acoustic neuroma 6. GLYCEROL TEST. Glycerol is a dehydrating agent.
(Table 15.1). When given orally, it reduces endolymph pressure and
thus causes an improvement in hearing.
(a) Recruitment test is positive.

O
Patient is given glycerol (1.5 mL/kg) with an equal
(b) SISI (short increment sensitivity index) test. SISI score is
amount of water and a little flavouring agent or lemon
better than 70% in two-thirds of the patients (normal
juice. Audiogram and speech discrimination scores are
15%).
co
(c) Tone decay test. Normally, there is decay of less than
20 dB.
recorded before and 1–2 h after ingestion of glycerol. An
improvement of 10 dB in two or more adjacent octaves
Y or gain of 10% in discrimination score makes the test
positive. There is also improvement in tinnitus and in the
4. ELECTROCOCHLEOGRAPHY. It shows changes diagnos-
sense of fullness in the ear. The test has a diagnostic and
tic of Ménière’s disease. Normally, ratio of summating po-
prognostic value. These days, glycerol test is combined
tential (SP) to action potential (AP) is 30%. In Ménière’s
with electrocochleography.
disease, SP/AP ratio is greater than 30% (Figure 15.4).

Electwachler VARIANTS OF MÉNIÈRE’S DISEASE D

1. COCHLEAR HYDROPS. Here, only the cochlear symp-
toms and signs of Ménière’s disease are present. Vertigo is
absent. It is only after several years that vertigo will make
its appearance. It is believed that in these cases, there is
block at the level of ductus reuniens, thereby confining
the increased endolymph pressure to the cochlea only
(Figure 15.5).

2. VESTIBULAR HYDROPS. Patient gets typical attacks of

episodic vertigo while cochlear functions remain normal.
It is only with time that a typical picture of Ménière’s

Figure 15.4. Electrocochleography. (A) Normal ear. (B) Ear with Mé-
nière’s disease. Voltage of summating potential (SP) is compared with
that of action potential (AP). Normally SP is 30% of AP. This ratio is
enhanced in Ménière’s disease. Figure 15.5. Left membranous labyrinth.

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Endolymphati shunt decomp of endolymph Soo
Chapter 15 — Ménière’s Disease 115

Diazepam (Valium or Calmpose) 5–10 mg may be giv- and the wick is passed through it. When soaked with a
en intravenously. It has a tranquillizing effect and also drug, the wick delivers the drug to the round window to
suppresses the activity of medial vestibular nucleus. be absorbed into the inner ear. It has been used to deliver
In some patients, acute attack can be stopped by atro- steroids in sudden deafness and gentamicin to destroy
pine, 0.4 mg, given subcutaneously. vestibular labyrinth in Ménière’s disease.
5. Vasodilators: Carbogen (5% CO2 with 95% O2) is a
Tgood cerebral vasodilator
labyrinthine circulation.
and its inhalation improves
D. SURGICAL TREATMENT O
It is used only when medical treatment fails.

C. MANAGEMENT OF CHRONIC PHASE 1. CONSERVATIVE PROCEDURES. They are used in cases

When patient presents after the acute attack, the treat- where vertigo is disabling but hearing is still useful and
needs to be preserved. They are:
ment consists of:
Y
(a) Decompression of endolymphatic sac.
↓;
1. Vestibular sedatives. Prochlorperazine (Stemetil)
2 10 mg, thrice a day, orally for two months and then (b) Endolymphatic shunt operation. A tube is put, connect-
reduced to 5 mg thrice a day for another month. ing endolymphatic- sac with subarachnoid space, to
drain excess endolymph.
2. Vasodilators. Betahistine (Vertin) 8–16 mg, thrice (c) Sacculotomy (Fick’s operation). It is puncturing the
-a day, given orally, also increases labyrinthine blood
flow by releasing histamine in the body.
E saccule with a needle through stapes footplate. A dis-
tended saccule lies close to stapes footplate and can
3. Diuretics. Sometimes, diuretic furosemide, 40 mg be easily penetrated. Cody’s tack procedure consists
Ttablet, taken on alternate days with potassium supple- of placing a stainless steel tack through the stapes
ment helps to control recurrent attacks, if not con- footplate. The tack would cause periodic decompres-
trolled by vasodilators or vestibular sedatives. Thi- sion of the saccule when it gets distended. Both these
azide diuretics (hydrochlorothiazide), 12.5 mg daily operations were claimed to have shown good results
can also be used. but they could not be reproduced by others and thus
abandoned. Cochleosacculotomy is another similar
4. Propantheline bromide (Probanthine), 15 mg, thrice procedure in which, instead of saccule, cochlear duct
a day, can be given alone or in combination with vas- is punctured and drained into the perilymph (otic-
odilator and is quite effective. However, they are not periotic shunt). The procedure is performed with a
preferred by many due to side effects. curved needle passed through the round window to
5. Elimination of allergen. Sometimes, a food or inhal- puncture cochlear duct. j =
ant allergen is responsible for such attacks. It should be (d) Section of vestibular nerve. The nerve is exposed by ret-
-
found and eliminated or desensitization done. rosigmoid or middle cranial fossa approach and se-
lectively sectioned. It controls vertigo but preserves
6. Hormones. Investigations should be directed to find hearing.
any endocrinal disorder such as hypothyroidism, and (e) Ultrasonic destruction
-
of vestibular labyrinth. Cochlear
appropriate replacement therapy given. Control of function is preserved.
stress by change in lifestyle is important to prevent re-
current attacks. 2. DESTRUCTIVE PROCEDURES. They totally destroy
About 80% of the patients can be effectively managed cochlear and vestibular function and are thus used only
by medical therapy alone. when cochlear function is not serviceable.
• Labyrinthectomy. Membranous labyrinth is completely
-
Intratympanic gentamicin therapy destroyed either by opening through the lateral semi-
(chemical labyrinthectomy) circular canal by transmastoid route or through the
oval window by a transcanal approach. This gives relief
Gentamicin is mainly vestibulotoxic. It has been used in
from the attacks of vertigo.
daily or biweekly injections into the middle ear. Drug is
absorbed through the round window and causes destruc-
tion of the vestibular labyrinth. Total control of vertigo 3. INTERMITTENT LOW-PRESSURE PULSE THERAPY
spells has been reported in 60–80% of patients with some [MENIETT DEVICE THERAPY (FIGURE 15.6)]. It is ob-
relief from symptoms in others. Hearing loss, sometimes served that intermittent positive pressure delivered to
severe and profound, has been reported in 4–30% of pa- inner ear fluids brings relief from the symptoms of Mé-
tients treated with this mode of therapy. nière’s disease. Not only there is improvement in vertigo,
tinnitus and ear fullness, but hearing may also improve.
Intermittent positive pressure waves can be delivered
Microwick through an instrument called Meniett device which has
It is a small wick made of polyvinyl acetate and meas- been approved by FDA. A prerequisite
-
for such a therapy
is to perform a myringotomy and insert a ventilation↑
-

ures 1 mm × 9 mm. It is meant to deliver drugs from

-
external canal to the inner ear and thus avoid repeated in- tube so that the device when coupled to the external ear
-

tratympanic injections. It requires a tympanostomy tube canal can deliver pressure waves to the round window
(grommet) to be inserted into the tympanic membrane membrane via the ventilation tube. Pressure waves pass

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116 SECTION I — Diseases of Ear

minf &

deve
Figure 15.6. Mechanism of intermittent low pressure pulse therapy. Pressure waves pass through ventilation tube (1) to round window mem-
brane (2) and transmitted to perilymph (yellow) and compress endolymphatic labyrinth (blue) to redistribute endolymph pressure to sac (3) and
blood vessels (4).

through the perilymph and cause reduction in endo- Patient can self-administer the treatment at home.
lymph pressure by redistributing it through various com- It may require a few months before complete remission
munication channels such as the endolymphatic sac or of disease is obtained. Meniett device therapy has been
the blood vessels (Figure 15.6). Some believe they regulate recommended for patients who have failed medical treat-
secretion of endolymph by the stria vascularis. ment and the surgical options are being considered.

&
Labepitting
O

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 Chapter 16
Tumours of External Ear

Of all the cases of ear carcinoma, 85% occur on the pinna, 5. HAEMANGIOMAS. They are congenital tumours often
10% in the external canal and 5% in the middle ear. seen in childhood. Other parts of face and neck may also
Tumours of the external ear may arise from the pinna be involved. They are of two types:
or external auditory canal (Table 16.1).
(a) Capillary haemangioma. It is a mass of capillary-sized
blood vessels and may present as a “port-wine stain.”
It does not regress spontaneously.
TUMOURS OF AURICLE (b) Cavernous haemangioma (also called strawberry tu-
mour). It consists of endothelial-lined spaces filled
BENIGN TUMOURS with blood. It increases rapidly during the first year
but regresses thereafter and may completely disap-
1. PREAURICULAR SINUS OR CYST. This results from faulty pear by the fifth year.
union of hillocks of the first and second branchial arch- (c) Vascular malformation. See Figure 16.2A–C.
es during the development of pinna. Preauricular sinus
presents as a small opening in front of the crus of helix. 6. PAPILLOMA (WART). It may present as a tufted growth
It has a branching tract lined by squamous epithelium or flat grey plaque and is rough to feel. It is viral in origin.
which when blocked results in a retention cyst. Patient Treatment is surgical excision or curettage with cauteriza-
usually presents with a cyst which is infected. Surgery is tion of its base.
indicated if there is unsightly swelling or infection. Cyst
or sinus tract must be excised completely to avoid recur- 7. CUTANEOUS HORN. It is a form of papilloma with
rence. heaping up of keratin and presents as horn-shaped tu-
mour. It is often seen at the rim of helix in elderly people.
2. SEBACEOUS CYST. Common site is postauricular sulcus Treatment is surgical excision.
or below and behind the ear lobule. Treatment is total
surgical excision. 8. KERATOACANTHOMA. It is a benign tumour clinically
resembling a malignant one. It presents as a raised nodule
3. DERMOID CYST. Usually presents as a rounded mass with a central crater. Initially, it grows rapidly but slowly
over the upper part of mastoid behind the pinna. regresses leaving a scar. Treatment is excision biopsy.

4. KELOID. It often follows trauma such as piercing the ear

lobule for ornaments or a surgical incision (Figure 16.1).
There is a genetic susceptibility. Black races are more of-
ten affected. Keloid presents as a pedunculated tumour.
Treatment is surgical excision with injection of triamci-
nolone into the surgical site or immediate postoperative
radiation of 300 rads.

TABLE 16.1 TUMOURS OF EXTERNAL EAR

Pinna External ear canal
• Benign • Benign
• Preauricular cyst or sinus • Osteoma
• Sebaceous cyst • Exostosis
• Dermoid cyst • Ceruminoma
• Keloid • Sebaceous adenoma
• Haemangioma • Papilloma
• Papilloma • Malignant
• Cutaneous horn • Squamous cell carcinoma
• Keratoacanthoma • Basal cell carcinoma
• Neurofibroma • Adenocarcinoma
• Malignant • Malignant ceruminoma
• Squamous cell carcinoma • Melanoma
• Basal cell carcinoma
• Melanoma
Figure 16.1. Keloid following piercing of an ear lobule for an earring.

117

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118 SECTION I — Diseases of Ear

Figure 16.2. Venolymphatic malformation of the pinna in a child (A) patient, (B) during operation and (C) the excised specimen.

often with en bloc removal of parotid gland and cervical

lymph nodes.

2. BASAL CELL CARCINOMA. The common sites are the

helix and the tragus. It is more common in men beyond
50 years of age. It presents as a nodule with central crust,
removal of which results in bleeding. Ulcer has a raised or
beaded edge. Lesion often extends circumferentially into
the skin but may penetrate deeper, involving cartilage or
bone. Lymph node metastases usually do not occur.
Treatment. Superficial lesions, not involving cartilage,
can be irradiated and cosmetic deformity avoided. Le-
sions involving cartilage may require surgical excision as
in cases of squamous cell carcinoma.

3. MELANOMA. It may occur anywhere over the auricle. It

is more common in men of light complexion who are ex-
posed to sun. Metastases are seen in 16–50% of the cases.
Treatment. Superficial melanoma, less than 1 cm in di-
ameter, situated over the helix, is managed by wedge re-
Figure 16.3. Squamous cell carcinoma of pinna. section and primary closure.
Superficial melanoma, larger than 1 cm, infiltrative
melanomas, melanoma of posterior auricular surface or
9. NEUROFIBROMA. It presents as a nontender, firm swell- concha and all recurrent melanomas are treated by resec-
ing and may be associated with von Recklinghausen dis- tion of pinna, parotidectomy and radical neck dissection.
ease. Treatment is surgical excision, if tumour occludes
ear canal or presents a cosmetic problem.
TUMOURS OF EXTERNAL AUDITORY
MALIGNANT TUMOURS CANAL
BENIGN TUMOURS
1. SQUAMOUS CELL CARCINOMA. The site of predilection
is the helix (Figure 16.3). It may present as a painless nod-
ule or an ulcer with raised everted edges and indurated 1. OSTEOMA. It arises from cancellous bone and presents
base. Metastases to regional lymph nodes occur very late. as a single, smooth, bony, hard, pedunculated tumour,
Disease is more common in males in their fifties who had often arising from the posterior wall of the osseous mea-
prolonged exposure to direct sunlight. Fair-complexioned tus, near its outer end (Figure 16.4). Treatment is surgical
people are more prone. removal by fracturing through its pedicle or removal with
Treatment. Small lesions with no nodal metastases a drill.
are excised locally with 1 cm of healthy area around
it. Larger lesions of the pinna or those coming within 2. EXOSTOSES. They are multiple and bilateral, often pre-
1 cm of external auditory canal and lesions with nodal senting as smooth, sessile, bony swellings in the deeper
metastases may require total amputation of the pinna, part of the meatus near the tympanic membrane. They

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 Chapter 16 — Tumours of External Ear 119

regularly followed up. Some of the ceruminomas are ma-

lignant and if there is any suspicion of malignancy on
histology, postoperative radiotherapy should be given.

4. SEBACEOUS ADENOMA. It arises from sebaceous glands

of the meatus and presents as a smooth, skin-covered
swelling in the outer meatus. Treatment is surgical exci-
sion.

5. PAPILLOMA. Similar to the one seen on the pinna.

MALIGNANT TUMOURS

1. SQUAMOUS CELL CARCINOMA. Most often, it is seen in

cases of long-standing ear discharge. It may arise primar-
Figure 16.4. An osteoma arising from the anterior wall of right ily from the meatus or be a secondary extension from the
external auditory canal.
middle ear carcinoma.
Presenting symptoms are blood staining of hitherto
mucopurulent or purulent discharge and severe earache.
arise from compact bone. Exostosis is often seen in per- Examination may show an ulcerated area in the mea-
sons exposed to entry of cold water in the meatus as in tus or a bleeding polypoid mass or granulations. Facial
divers and swimmers. Males are affected three times more nerve may be paralyzed because of local extension of dis-
than females. ease through posterior meatal wall or its spread into the
Treatment. When small and asymptomatic, no treat- middle ear. Regional lymph nodes (preauricular, postau-
ment is necessary. Larger ones, which impair hearing or ricular, infra-auricular and upper deep cervical) may be
cause retention of wax and debris, may be removed with involved.
high speed drill to restore normal sized meatus. Exostoses Treatment is en bloc wide surgical excision with post-
may extend deeply and lie in close relation to the facial operative radiation.
nerve. Therefore, use of gouge and hammer should be
avoided. 2. BASAL CELL AND ADENOCARCINOMAS. They can rarely
arise from the meatus. Clinical picture is similar to that of
3. CERUMINOMA. It is a tumour of modified sweat glands squamous cell variety. Diagnosis is made only on biopsy.
which secrete cerumen. It presents as a smooth, firm, Treatment is wide surgical excision and postoperative ra-
skin-covered polypoid swelling in outer part of the mea- diation.
tus, generally attached to the posterior or inferior wall. It
obstructs the meatus leading to retention of wax and de- 3. MALIGNANT CERUMINOMA. Malignant type is twice as
bris. Malignant type outnumbers the benign by 2:1 ratio. common as benign.
Treatment. Tumour has a tendency to recur, there-
fore wide surgical excision should be done and patient 4. MALIGNANT MELANOMA. Rare tumour.

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 Chapter 17
Tumours of Middle Ear and Mastoid

CLASSIFICATION Spread of Glomus Tumour

1. Tumour may initially fill the middle ear and later per-
Tumours of middle ear and mastoid can be divided into:
forate through the tympanic membrane to present as a
1. Primary tumours vascular polyp.
2. It may invade labyrinth, petrous pyramid and the
(a) Benign: Glomus tumour
mastoid.
(b) Malignant: Carcinoma, sarcoma
3. It may invade jugular foramen and the base of skull,
2. Secondary tumours causing IXth to XIIth cranial nerve palsies.
4. By spread through eustachian tube, it may present in
(a) From adjacent areas, e.g. nasopharynx, external mea-
the nasopharynx.
tus and the parotid.
5. It may spread intracranially to the posterior and mid-
(b) Metastatic, e.g. from carcinoma of bronchus, breast,
dle cranial fossae.
kidney, thyroid, prostate and gastrointestinal tract.
6. Metastatic spread to lungs and bones is rare, but seen
in 4% of cases. Metastatic lymph node enlargement

GLOMUS TUMOUR
O can also occur.

It is the most common benign neoplasm of middle ear CLINICAL FEATURES

and is so-named because of its origin from the glomus In 90% of cases, symptoms pertain to the ear.
bodies. The latter resemble carotid body in structure and
are found in the dome of jugular bulb or on the promon- 1. WHEN TUMOUR IS INTRATYMPANIC. Earliest symp-
tory along the course of tympanic branch of IXth cranial toms are hearing loss and tinnitus. Hearing loss is con-
nerve (Jacobson’s nerve). The tumour consists of paragan- ductive and slowly progressive. Tinnitus is pulsatile and
glionic cells derived from the neural crest. of swishing character, synchronous with pulse and can be
temporarily stopped by carotid pressure.
AETIOLOGY AND PATHOLOGY Otoscopy shows a red reflex through intact tympanic
membrane. “Rising sun” appearance is seen when tumour
The tumour is often seen in the middle age (40–50 years). arises from the floor of middle ear. Sometimes, tympanic
Females are affected five times more. membrane appears bluish and may be bulging.
It is a benign, nonencapsulated but extremely vascu- “Pulsation sign” (Brown sign) is positive, i.e. when ear
lar neoplasm. Its rate of growth is very slow and several canal pressure is raised with Siegel’s speculum, tumour
years may pass before there is any change from the initial pulsates vigorously and then blanches; reverse happens
symptoms. Tumour is locally invasive. with the release of pressure.
Microscopically, it shows masses or sheets of epithelial
cells which have large nuclei and a granular cytoplasm. 2. WHEN TUMOUR PRESENTS AS A POLYP. In addition
There is abundance of thin-walled blood sinusoids with to hearing loss and tinnitus, there is history of profuse
no contractile muscle coat, accounting for profuse bleed- bleeding from the ear either spontaneously or on at-
ing from the tumours. tempts to clean it.
For purposes of diagnosis and treatment, two types are Dizziness or vertigo and facial paralysis may appear.
differentiated. Earache is less common than in carcinoma of the external
and middle ear, and helps to differentiate them from it.
1. GLOMUS JUGULARE. They arise from the dome of jug- Otorrhoea may occur due to secondary infection and
ular bulb, invade the hypotympanum and jugular fora- the condition may simulate chronic suppurative otitis
men, causing neurological signs of IXth to XIIth cranial media with polyp.
nerve involvement. They may compress jugular vein or Examination reveals a red, vascular polyp filling the
invade its lumen. meatus. It bleeds readily and profusely on manipulation
or at biopsy.
2. GLOMUS TYMPANICUM. They arise from the promon-
tory of the middle ear and cause aural symptoms, some- 3. CRANIAL NERVE PALSIES. This is a late feature ap-
times with facial paralysis. pearing several years after aural symptoms. IXth to XIIth

121

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122 SECTION I — Diseases of Ear

cranial nerves may be paralyzed. There is dysphagia and 7. BIOPSY. Preoperative biopsy of the tumour for diagno-
hoarseness with unilateral paralysis of the soft palate, sis is never done. Clinical and radiologic features are very
pharynx (IX, X) and vocal cord (X) with weakness of the characteristic to make diagnosis. Tumour is very vascular
trapezius and sternomastoid muscles (XI) and atrophy of and bleeds profusely. There is also likelihood of injuring
half of tongue (XII). the high jugular bulb or aberrant internal carotid artery if
Tumour may present as a mass over the mastoid or in diagnosis is mistaken.
the nasopharynx.
Signs of intracranial involvement may also occur.
TREATMENT
4. AUDIBLE BRUIT. At all stages, auscultation with steth- It consists of:
oscope over the mastoid may reveal systolic bruit.
Some glomus tumours secrete catecholamines and pro- 1. Surgical removal.
duce symptoms like headache, sweating, palpitation, hy- 2. Radiation.
pertension and anxiety, and require further investigations. 3. Embolization.
4. Combination of the above techniques.
5. RULE OF 10S. Remember that 10% of the tumours are
Surgical approaches to glomus tumours
familial, 10% multicentric (occurring in more than one site)
and up to 10% functional, i.e. they secrete catecholamines. 1. TRANSCANAL APPROACH. Suited for limited glomus
tympanicum tumour where entire circumference of the
tumour is visible, only tympanotomy will suffice to gain
DIAGNOSIS access to the tumour.
In addition to thorough history and physical examina-
tion, the patient is checked-up to find out the extent 2. HYPOTYMPANIC APPROACH. Suited for tumours limit-
of tumour, other associated glomus tumours and serum ed to promontory with extension to hypotympanum but
levels of catecholamines or their breakdown products in not into the mastoid. A superiorly based tympanomeatal
urine (vanillylmandelic acid, metanephrine, etc.). Inves- flap is raised by postauricular approach. Bony inferior
tigations include: tympanic ring is drilled away to see the lower limit of
tumour.
1. COMPUTED TOMOGRAPHY (CT) SCAN HEAD. Using
bone window, 1 mm thin sections are cut. It helps to dis- 3. EXTENDED FACIAL RECESS APPROACH. Used for glo-
tinguish glomus tympanicum from the glomus jugulare mus tympanicum extending into mastoid but not into
tumour by identification of caroticojugular spine which the jugular bulb. If extensive, modified radical operation
is eroded in the latter. CT scan also helps to differenti- is done.
ate it from the aberrant carotid artery, high or dehiscent
jugular bulb. 4. MASTOID-NECK APPROACH. Used for glomus jugulare
tumours not extending to internal carotid artery, poste-
2. MRI. It shows soft tissue extent of tumour. Magnetic rior cranial fossa or neck.
resonance angiography and venography further help to
delineate invasion of jugular bulb and vein or compres- 5. INFRATEMPORAL FOSSA APPROACH OF FISCH. Used for
sion of the carotid artery. large glomus jugulare tumours.

3. CT HEAD AND MRI COMBINED. together provide an 6. TRANSCONDYLAR APPROACH. Used for tumours
excellent preoperative guidance in the differential diag- extending towards foramen magnum. Usually they are
nosis of petrous apex lesions. recurrent glomus jugulare tumours. It gives approach to
craniocervical junction with exposure of occipital con-
4. FOUR-VESSEL ANGIOGRAPHY. It is necessary when CT dyle and jugular tubercle.
head shows involvement of jugular bulb, carotid artery or Radiation treatment does not cure the tumour but
intradural extension. It also helps to delineate any other may reduce its vascularity and arrest its growth. Radia-
glomus tumour (as they may be multiple), find the feed- tion is used for inoperable tumours, residual tumours,
ing vessels or embolization of tumour if required. recurrences after surgery or for older individuals where
extensive skull base surgery is not indicated.
5. BRAIN PERFUSION AND FLOW STUDIES. They are nec- Embolization is used to reduce the vascularity of
essary when tumour is pressing on internal carotid artery. tumour before surgery or is the sole treatment in the in-
If the case needs surgery, brain perfusion and adequacy of operable patients who have received radiation.
contralateral internal carotid artery and circle of Willis can
be assessed. If needed, xenon blood flow and isotope stud-
ies are done for precise blood flow, and the risk of stroke CARCINOMA OF MIDDLE EAR
and need for surgical replacement of internal carotid artery. AND MASTOID
6. EMBOLIZATION. In large tumours, embolization of It is a rare condition, there being one case in 20,000 new
feeding vessels 1–2 days before operation helps to reduce patients examined, but it is the commonest primary mid-
blood loss. dle ear malignancy.

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 Chapter 17 — Tumours of Middle Ear and Mastoid 123

AETIOLOGY
It affects age group of 40–60 years and is slightly more
common in females. Most cases (75%) have associated
long-standing ear discharge. Chronic irritation may be
the causative factor in such cases. Some cases are seen
in radical mastoid cavities. Primary carcinoma of mastoid
air cells is also seen in radium dial painters.

PATHOLOGY
Tumour may arise primarily from middle ear or be an ex-
tension of carcinoma of the deep meatus. Squamous cell
variety is by far the most common. Adenocarcinoma may
occasionally be seen; it arises from the glandular elements
of middle ear.
Figure 17.1. A 4-year-old child with rhabdomyosarcoma of the right
SPREAD OF TUMOUR. To begin with, carcinoma destroys middle ear and mastoid. He also had facial palsy on the same side.
ossicles, facial canal, internal ear, jugular bulb, carotid ca-
nal or deep bony meatus and mastoid. It may spread in pe-
trous pyramid towards its apex. Dura is usually resistant. Radiotherapy alone is given as a palliative measure
It may spread to the parotid gland, temporomandibular when tumour involves cranial nerves (IXth to XIIth) or
joint, infratemporal fossa and down the eustachian tube spreads into the cranial cavity or the nasopharynx.
to nasopharynx. Lymph node enlargement occurs late.

CLINICAL FEATURES SARCOMAS

Patient often presents with clinical picture simulating
chronic suppurative otitis media. However, the following • RhabdomyosaRcoma. It is a rare tumour, mostly af-
features in age group of 40–60 years may arouse suspicion fecting children. It arises from the embryonic muscles
of malignancy: tissue or the pluripotential mesenchyme. In early stages,
1. Chronic foul-smelling discharge especially when blood it mimics chronic suppurative otitis media with ear dis-
stained. charge, polyp or granulations. Facial palsy occurs early
2. Pain which is usually severe and comes at night. (Figure 17.1). Diagnosis is made only on biopsy. Prognosis
3. Facial palsy. is poor. A combination of radiation and chemotherapy is
4. Friable, haemorrhagic granulations or polyp. the treatment of choice. Surgery is done in selected local-
5. Appearance of or increase in hearing loss or vertigo. ized lesions.

• otheR saRcomas. Osteosarcoma, lymphoma, fibrosar-

DIAGNOSIS coma and chondrosarcoma are rare. Distant metastases
Definitive diagnosis is made only on biopsy. Extent of dis- are seen in the lungs or bone. Prognosis is poor.
ease is judged by clinical and radiological examination.
CT scan and angiography are useful in the assessment of
disease. SECONDARY TUMOURS
Tumours of external auditory meatus, parotid gland or
TREATMENT nasopharynx may invade middle ear cleft either through
A combination of surgery and radiotherapy gives better the preformed pathways or bone erosion.
results. Surgery consists of radical mastoidectomy, sub- Sometimes, temporal bone is the site of distant me-
total or total petrosectomy depending on the extent of tastases in advanced cases of carcinoma of the breast,
tumour. bronchus, prostate, kidney or gastrointestinal tract.

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 intracanaliclar
Chapter 18 small size
-
medi
Acoustic Neuroma large
size

Acoustic neuroma is also known as vestibular schwanno- Progressive unilateral sensorineural hearing loss, often
ma, neurilemmoma or eighth nerve tumour. accompanied by tinnitus, is the presenting symptom in major-
ity of cases. There is marked difficulty in understanding
speech, out of proportion to the pure tone hearing loss.
INCIDENCE This feature is characteristic of acoustic neuroma. Some
patients may get sudden hearing loss.
Acoustic neuroma constitutes 80% of all cerebellopontine Vestibular symptoms are imbalance or unsteadiness.
angle tumours and 10% of all the brain tumours. True vertigo is seldom seen.

3. CRANIAL NERVE INVOLVEMENT

PATHOLOGY
• Vth nerve. This is the earliest nerve to be involved.
It is a benign, encapsulated, extremely slow-growing tu- There is reduced corneal sensitivity, numbness or par-
mour of the VIIIth nerve. Microscopically, it consists of aesthesia of face. Involvement of this nerve indicates
elongated spindle cells with rod-shaped nuclei lying in that the tumour is roughly 2.5 cm in diameter and
rows or palisades. Bilateral tumours are seen in patients occupies the cerebellopontine angle.
with neurofibromatosis. • VIIth nerve. Sensory fibres are affected early. There is
hypoaesthesia of posterior meatal wall (Hitzelberger’s
sign), loss of taste (as tested by electrogustometry) and
ORIGIN AND GROWTH OF TUMOUR reduced lacrimation on Schirmer test. Motor fibres are
more resistant and are affected late. Delayed blink re-
The tumour almost always arises from the Schwann cells of flex may be an early manifestation.
the vestibular, but rarely from the cochlear division of VIIIth • IXth and Xth nerves. There is dysphagia and hoarseness
nerve within the internal auditory canal (Figure 18.1). As it due to palatal, pharyngeal and laryngeal paralysis.
expands, it causes widening and erosion of the canal and • Other cranial nerves. XIth and XIIth, IIIrd, IVth and
then appears in the cerebellopontine angle. Here, it may VIth are affected when tumour is very large.
grow anterosuperiorly to involve Vth nerve or inferiorly
to involve the IXth, Xth and XIth cranial nerves. In later 4. BRAINSTEM INVOLVEMENT. There is ataxia, weak-
stages, it causes displacement of brainstem, pressure on ness and numbness of the arms and legs with exagger-
cerebellum and raised intracranial tension (Figure 18.2). ated tendon reflexes. They are seen when long motor and
The growth of the tumour is extremely slow and the his- sensory tracts are involved.
tory may extend over several years.
5. CEREBELLAR INVOLVEMENT. Pressure symptoms on
cerebellum are seen in large tumours. This is revealed
CLASSIFICATION by finger-nose test, knee-heel test, dysdiadochokinesia,
ataxic gait and inability to walk along a straight line with
Depending on the size, the tumour is classified as: tendency to fall to the affected side.
1. Intracanalicular (when it is confined to internal audi-
tory canal) 6. RAISED INTRACRANIAL TENSION. This is also a late
2. Small size (up to 1.5 cm) feature. There is headache, nausea, vomiting, diplopia
3. Medium size (1.5–4 cm) due to VIth nerve involvement and papilloedema with
4. Large size (over 4 cm) blurring of vision.

CLINICAL FEATURES INVESTIGATIONS AND DIAGNOSIS

Vo Attempts should be made to diagnose the tumour in its
1. AGE AND SEX. Tumour is mostly seen in age group of otological phase when it is still intracanalicular. This
40–60 years. Both sexes are equally affected. is possible when all cases of unilateral sensorineural
hearing loss with tinnitus or imbalance are carefully
2. COCHLEOVESTIBULAR SYMPTOMS. They are the earli- evaluated.
est symptoms when tumour is still intracanalicular and
are caused by pressure on cochlear or vestibular nerve fi- 1. AUDIOLOGICAL TESTS. See Table 15.1 for difference be-
bres or on the internal auditory artery. tween cochlear and retrocochlear lesions.

125

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(a) Pure tone audiometry will show sensorineural hear- 5. RADIOLOGICAL TESTS
ing loss, more marked in high frequencies. (a) Plain X-rays (transorbital, Stenver’s, Towne’s and sub-
(b) Speech audiometry shows poor speech discrimina- mentovertical views) give positive findings in 80% of
tion and this is disproportionate to pure tone hear- patients. However, small intracanalicular tumours are
ing loss. Roll-over phenomenon, i.e. reduction of dis- not detected.
crimination score when loudness is increased beyond (b) Computed tomography (CT) scan. A tumour that pro-
a particular limit is most commonly observed. jects even 0.5 cm into the posterior fossa can be de-
(c) Recruitment phenomenon is absent. tected by a CT scan. If combined with intrathecal
(d) Short Increment Sensitivity Index (SISI) test will show air, even the intrameatal tumour can be detected. CT
a score of 0–20% in 70–90% of cases. scan has replaced earlier methods of pneumoenceph-
(e) Threshold tone decay test shows retrocochlear type alography and myodil meatography.
of lesion. (c) MRI with gadolinium contrast. It is superior to CT scan
and is the gold standard for diagnosis of acoustic neu-
2. STAPEDIAL REFLEX DECAY TEST. (see p. 27). roma. Intracanalicular tumour, of even a few milli-
metres, can be easily diagnosed by this method.
3. VESTIBULAR TESTS. Caloric test will show diminished (d) Vertebral angiography. This is helpful to differentiate
or absent response in 96% of patients. When tumour is acoustic neuroma from other tumours of cerebello-
very small, caloric test may be normal. pontine angle when doubt exists.

4. NEUROLOGICAL TESTS. Complete examination of 6. EVOKED RESPONSE AUDIOMETRY (BERA). It is very

cranial nerves, cerebellar functions, brainstem signs of useful in the diagnosis of retrocochlear lesions. In the
pyramidal and sensory tracts should be done. Fundus is presence of VIIIth nerve tumour, a delay of > 0.2 ms in
examined for blurring of disc margins or papilloedema. wave V between two ears is significant (see p. 28).

7. CSF EXAMINATION. Protein level is raised. Lumbar

puncture is usually avoided.
Important tests for work-up of acoustic neuroma are
given below:
• Pure tone audiometry.
• Speech discrimination score.
• Roll-over curve.
• Stapedial reflex decay.
Figure 18.1. Inner aspect of lateral end of internal auditory canal
• Evoked response audiometry.
with structures passing through different areas. • MRI with contrast.

Figure 18.2. Acoustic neuroma and its expansion. (A) Intracanalicular. (B) Tumour extending into cerebellopontine angle. (C) Tumour pressing
on CN V. (D) Very large tumour pressing on CN V, IX, X, XI, and brainstem and cerebellum.

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DIFFERENTIAL DIAGNOSIS TABLE 18.1 TUMOURS OF CEREBELLOPONTINE

ANGLE
Acoustic neuroma should be differentiated from the
• Acoustic neuroma
cochlear pathology (i.e. Ménière’s disease) and other cer- • Meningioma
ebellopontine angle tumours, e.g. meningioma, primary • Epidermoid (cholesteatoma)
cholesteatoma and arachnoidal cyst (Table 18.1). • Arachnoid cyst
• Schwannoma of other cranial nerves (e.g. CN V >VII > IX, X, XI)
• Aneurysm
• Glomus tumour
TREATMENT
• Metastasis
SURGERY
Surgical removal of the tumour is the treatment of choice.
X-knife or Gamma knife surgery. It is a form of stereotac-
Surgical approach will depend upon the size of tumour.
tic radiotherapy where radiation energy is converged on
The various approaches are:
the tumour, thus minimizing its effect on the surround-
1. Middle cranial fossa approach. ing normal tissue. This causes arrest of the growth of the
2. Translabyrinthine approach. tumour and also reduction in its size. It can be used in
3. Suboccipital (retrosigmoid) approach. patients who refuse surgery or have contraindications to
4. Combined translabyrinthine-suboccipital approach. surgery or in those with a residual tumour.
X-knife surgery is done through linear accelerator and
gamma knife through a Cobalt-60 source.
RADIOTHERAPY
Cyber knife. It is an improvement over the above. It is
Conventional radiotherapy by external beam has no role in totally frameless and more accurate. It uses real-time im-
the treatment of acoustic neuromas due to low tolerance age guidance technology through computer-controlled
of the central nervous system to radiation. robotics.

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 Chapter 19
The Deaf Child

Children with profound (> 90 dB loss) or total deafness fail (e) Mondini dysplasia. Only basal coil is present or cochlea
to develop speech and have often been termed deaf-mute is 1.5 turns. There is incomplete partition between the
or deaf and dumb. However, these children have no defect scalae due to absence of osseous spiral lamina. Condi-
in their speech producing apparatus. The main defect is tion is unilateral or bilateral. This deformity may be
deafness. They have never heard speech and therefore do seen in Pendred, Waardenburg, branchio-oto-renal,
not develop it. In lesser degrees of hearing loss, speech Treacher-Collins and Wildervanck syndromes.
does develop but is defective. The period from birth to (f) Enlarged vestibular aqueduct. Vestibular aqueduct is en-
5 years of life is critical for the development of speech and larged (> 2 mm), endolymphatic sac is also enlarged
language, therefore, there is need for early identification and can be seen on T2 MRI. It causes early onset sen-
and assessment of hearing loss and early rehabilitation in sorineural hearing loss which is progressive. Vertigo
infants and children. It was observed that children whose may be present. Perilymphatic fistula may occur.
hearing loss was observed and managed before 6 months (g) Semicircular canal malformations. Both superior and later-
of age had higher scores of vocabulary, better expressive al or only lateral semicircular canal malformations may
and comprehensive language skills than those diagnosed be seen. They can be identified on imaging techniques.
and managed after 6 months of age emphasizing the im-
portance of early identification and treatment. 2.MATERNALFACTORS
(a) Infections during pregnancy.
(b) Drugs during pregnancy.
(c) Radiation to mother in the first trimester.
AETIOLOGY
(d) Other factors.
Hearing loss in a child may develop from causes before Syndromes commonly associated with hearing loss are
birth (prenatal), during birth (perinatal) or thereafter given in Table 19.1.
(postnatal).
(a) Infections during pregnancy. Infections which affect the
developing fetus are toxoplasmosis, rubella, cytomeg-
A. PRENATAL CAUSES aloviruses, herpes type 1 and 2 and syphilis. Remem-
They may pertain to the infant or the mother. ber mnemonic, TORCHES.
(b) Drugs during pregnancy. Streptomycin, gentamicin, to-
1. INFANT FACTORS. An infant may be born with inner ear bramycin, amikacin, quinine or chloroquine, when
anomalies due to genetic or nongenetic causes. Anoma- given to the pregnant mother, cross the placental bar-
lies may affect inner ear alone (nonsyndromic) or may rier and damage the cochlea. Thalidomide not only
form part of a syndrome (syndromic). affects ear but also causes abnormalities of limbs,
Anomalies affecting the inner ear may involve only heart, face, lip and palate.
the membranous labyrinth or both the membranous and (c) Radiation to mother in the first trimester.
bony labyrinths. They include: (d) Other factors. Nutritional deficiency, diabetes, toxae-
mia and thyroid deficiency. Maternal alcoholism is
(a) Scheibe dysplasia. It is the most common inner ear anom- also teratogenic to the developing auditory system.
aly. Bony labyrinth is normal. Superior part of membra-
nous labyrinth (utricle and semicircular ducts) is also B. PERINATAL CAUSES
normal. Dysplasia is seen in the cochlea and saccule;
hence also called cochleosaccular dysplasia. It is inherited They relate to causes during birth or in early neonatal
as an autosomal recessive nonsyndromic trait. period. They are as follows.
(b) Alexander dysplasia. It affects only the basal turn of
membranous cochlea. Thus only high frequencies are 1. ANOXIA. It damages the cochlear nuclei and causes
affected. Residual hearing is present in low frequencies haemorrhage into the ear. Placenta praevia, prolonged
and can be exploited by amplification with hearing aids. labour, cord round the neck and prolapsed cord can all
(c) Bing-Siebenmann dysplasia. There is complete absence cause fetal anoxia.
of membranous labyrinth. 2. PREMATURITY AND Low BIRTH WEIGHT. Born before
(d) Michel aplasia. There is complete absence of bony and term or with birth weight less than 1500 g (3.3 lb).
membranous labyrinth. Even the petrous apex is ab-
sent but external and middle ears may be completely 3. BIRTH INJURIES. e.g. forceps delivery. They may cause
unaffected. No hearing aids or cochlear implantation intracranial haemorrhage with extravasation of blood
can be used. into the inner ear.

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TABLE 19.1 SYNDROMES ASSOCIATED WITH HEARING LOSS

Type of hearing Onset (Congenital/
Syndrome Features loss delayed) Type of inheritance
1. Waardenburg • White forelock Unilateral or bilateral Congenital AD
syndrome • Heterochromia iridis (Figure 19.2) SNHL
(Figure 19.1) • Vitiligo
• Dystopia canthorum
2. Usher syndrome • Retinitis pigmentosa SNHL Delayed AR
• Night blindness
3. Jervell and Lange- • Repeated syncopal attacks SNHL Congenital AR
Nielson syndrome • Prolonged QT interval in ECG
4. Pendred syndrome • Goitre (nontoxic) usually evident SNHL Congenital AR
before puberty
• Perchlorate discharge test shows
defect in organic binding of iodine
5. Alport syndrome • Hereditary progressive Progressive SNHL Delayed AD or X-linked
glomerulonephritis
• Corneal dystrophy
6. Treacher-Collins • Antimongoloid palpebral fissures Conductive Congenital AD
syndrome • Coloboma of lower lid
(mandibulofacial • Hypoplasia of mandible and malar
dysostosis) bones
• Microtia pinna and meatal atresia
• Malformed malleus and incus (stapes
normal)
7. Crouzon syndrome • Frog eyes (exophthalmos with Conductive or Congenital AD
(craniofacial divergent squint) mixed
dysostosis) • Hypertelorism
• Parrot-beak nose
• Mandibular prognathism
• Premature closure of cranial sutures
with mental retardation
8. Apert syndrome • Syndactyly Conductive (Stapes AD
• All other features of Crouzon fixation)
syndrome
9. Klippel-Feil • Short neck SNHL or mixed Congenital AR
syndrome • Fused cervical vertebrae
• Spina bifida
• Atresia of ear canal
10. Wildervanck • Klippel-Feil syndrome SNHL Congenital X-linked
syndrome • SNHL
• CN VI Paralysis
11. Branchio-oto-renal • Branchial fistulas/cysts Conductive or Congenital AD
syndrome • Malformed pinnae with preauricular mixed
pits or sinuses
• Renal abnormalities
12. Stickler syndrome • Small jaw Conductive or SNHL Delayed AD
• Cleft palate (Pierre-Robin sequence)
• Myopia ≥ retinal detachment
• Cataract
• Juvenile onset arthritis
13. Van der Hoeve • Osteogenesis imperfecta with history Conductive, SNHL Delayed AD
syndrome of fractures or mixed (like
• Blue sclera otosclerosis)
• Hearing loss (delayed onset)
14. Pierre-Robin • Micrognathia SNHL Conductive AD
sequencea • Glossoptosis loss
• Cleft palate
• Often part of Stickler syndrome
15. Goldenhar • Facial asymmetry Mixed or conductive Congenital AD or sporadic Extra
syndrome (Facio- • Low set ears, atresia of ear canal chromosome
auriculo-vertebral • Cardiac abnormalities Multifactorial
dysplasia) or (oculo- • Preauricular tags/pits (genetic and
auriculo-vertebral • Hemivertebrae in cervical region environmental)
[OAV] syndrome) • Epibulbar dermoid
• Coloboma of upper lid

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TABLE 19.1 SYNDROMES ASSOCIATED WITH HEARING LOSS (CONT.)

Type of hearing Onset (Congenital/
Syndrome Features loss delayed) Type of inheritance
16. Down syndrome • Microcephaly Conductive Congenital Extra chromosome
(Trisomy 21) • Mental retardation/delayed
development
• Short stature
• Epicanthal folds
• Stenosis of ear canal
• High incidence of serous otitis media
• Atlanto-axial instability

SNHL, sensorineural hearing loss; AD, autosomal dominant; AR, autosomal recessive.
aIt is called a sequence, not a syndrome, because multiple anomalies result in sequence from a single primary abnormality, i.e. micrognathia which leads

to glossoptosis which in turn causes cleft palate.

4. NEONATAL JAUNDICE. Bilirubin level greater than

20 mg% damages the cochlear nuclei.

5. NEONATAL MENINGITIS

6. SEPSIS

7. TIME SPENT IN NEONATAL ICU

8. OTOTOXIC DRUGS. used for neonatal meningitis or

septicaemia.

C. POSTNATAL CAUSES

1. GENETIC. Though deafness is genetic, it manifests later

in childhood or adult life. Deafness may occur alone as in
familial progressive sensorineural deafness or in association
with certain syndromes, e.g. Alport, Klippel-Feil, Hurler, etc.

2. NONGENETIC. They are essentially same as in adults

Figure 19.1. Waardenburg syndrome. Note white forelock, hetero-
and include:
chromia iridis and depigmentation of skin. (a) Viral infections (measles, mumps, varicella, influen-
za), meningitis and encephalitis.
(b) Secretory otitis media.
(c) Ototoxic drugs.
(d) Trauma, e.g. fractures of temporal bone, middle ear
surgery or perilymph leak.
(e) Noise-induced deafness.

EVALUATION OF A DEAF CHILD

FINDING THE CAUSE
This may require a detailed history of prenatal, perinatal or
postnatal causes, family history, physical examination and
certain investigations depending on the cause suspected.
1. Suspicion of hearing loss. Hearing loss is suspected if
(i) the child sleeps through loud noises unperturbed or
fails to startle to loud sounds, (ii) fails to develop speech
at 1–2 years. A partially hearing child may have a de-
fective speech and perform poorly in school and be la-
belled mentally retarded. It is essential that all children
Figure 19.2. Heterochromia iridis. Iris showing different colours. at risk for hearing loss should be screened and followed.

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132 SECTION I — Diseases of Ear

TABLE 19.2 METHODS OF HEARING ASSESSMENT which may persist for 3–4 days. They are normal even
IN INFANTS AND CHILDREN when VIIIth nerve is nonfunctional. Thus can be used
in the diagnosis of neuropathy of VIIIth nerve.
• Neonatal screening procedures (b) ABRs are generated in response to sound stimulus pre-
• ABR/OAEs
sented to the ear and picked up from the scalp. With
• Arousal test
• Auditory response cradle
a response of 30–35 dB nHL, the infant who passes
• Behaviour observation audiometry the test and the hearing is considered normal. Infants
• Moro’s reflex who fail these tests are followed up with repeat tests.
• Cochleopalpebral reflex
Arousal test. A high-frequency narrow band noise is
• Cessation reflex
presented for 2 s to the infant when he is in light sleep. A
• Distraction techniques (6–18 months)
• Conditioning techniques (7 months – 2 years) normal hearing infant can be aroused twice when three
• Visual reinforcement audiometry such stimuli are presented to him.
• Play audiometry (2–5 years) Auditory response cradle is a screening device for new-
• Objective tests borns, where baby is placed in a cradle and his behaviour
• ABR (trunk and limb movement, head jerk and respiration) in
• Otoacoustic emissions response to auditory stimulation are monitored by trans-
• Impedance audiometry ducers. It can screen babies with moderate, severe or pro-
found hearing loss.

2. Risk factors for hearing loss in children (Recom- 2. BEHAVIOUR OBSERVATION AUDIOMETRY. Auditory sig-
mendations of Joint Committee on Infant Hearing— nal presented to an infant produces a change in behav-
updated to 1994). iour, e.g. alerting, cessation of an activity, widening of
(a) Family history of hearing loss. eyes or facial grimacing. Moro’s reflex is one of them and
(b) Prenatal infections (TORCHES). consists of sudden movement of limbs and extension of
(c) Craniofacial anomalies including those of pinna head in response to sound of 80–90 dB. In cochleopalpebral
and ear canal. reflex, the child responds by a blink to a loud sound. In
(d) Birth weight less than 1500 g (3.3 lbs). cessation reflex, an infant stops activity or starts crying in
(e) Hyperbilirubinaemia requiring exchange transfusion. response to a sound of 90 dB.
(f) Ototoxic medications included but not limited
to aminoglycosides used in multiple courses or in 3. DISTRACTION TECHNIQUES. are used in children
combination with loop diuretics. 6–7 months old. The child at this age turns his head to
(g) Bacterial meningitis. locate the source of sound. In this test, the child is seated
(h) 1Apgar score of 0–4 at 1 min or 0–6 at 5 min. in his mother’s lap, an assistant distracts the child’s atten-
(i) Mechanical ventilation for 5 days or longer. tion while the examiner produces a sound from behind or
(j) Stigmata or other findings associated with a from one side to see if the child tries to locate it. Sounds
syndrome known to include sensorineural and/or used are high frequency rattle (8 kHz), low-frequency
conductive hearing loss. hum, whispered sound as “S, S, S”, xylophone, warbled
tones or narrow band noise (500–4000 Hz).
ASSESSMENT OF HEARING IN INFANTS
AND CHILDREN 4. CONDITIONING TECHNIQUES
(a) Visual reinforcement audiometry (VRA). It is a condition-
Assessment of auditory function in neonates, infants and
ing technique in which child is trained to look for an
children demands special techniques. They are grouped
auditory stimulus by turning his head. This behaviour
under the following heads (Table 19.2):
is reinforced by a flashing light or an animated toy.
This test helps to determine the hearing threshold us-
1. SCREENING PROCEDURES. They are employed to test
ing standard audiometric techniques. The auditory
hearing in “high-risk” infants and are based on infant’s
stimulus is delivered by headphones or better still by
behavioural response to the sound signal. It is now ob-
insert earphones which are accepted better and are
served that 95% of children with one or more risk factors
also light weight. Test is well-suited between the de-
have normal hearing. On the contrary, 50% of children
velopmental age of 6 months to 2 years.
with sensorineural hearing loss had no risk factor. This
(b) Play audiometry. The child is conditioned to perform
leads to a programme of universal neonatal screening for
an act such as placing a marble in a box, putting a ring
early detection.
on a post or putting a plastic block in a bucket each
Two important tests are to study otoacoustic emissions
time he hears a sound signal. Each correct performance
(OAEs) and auditory brainstem responses (ABR).
of the act is reinforced with praise, encouragement or
(a) OAEs are generated at outer hair cells and can be reward. Ear specific thresholds can be determined by
picked up from the external ear as the energy pro- standard audiometric techniques. This test can be used
duced by them travels in reverse direction from outer in children with developmental age of 2–4 or 5 years.
hair cells → ossicles → tympanic membrane → ear (c) Speech audiometry. The child is asked to repeat the
canal where it is picked up. OAEs are absent if outer names of certain objects or to point them out on the
hair cells in the cochlea are nonfunctional or there is pictures. The voice can be gradually lowered. In this
middle ear effusion or canal debris due to meconium way, hearing level and speech discrimination can be

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 Chapter 19 — The Deaf Child 133

tested. The test can also be used to examine the child’s

MANAGEMENT
expressive ability when he is asked to name the toys
like horse, duck or objects like cup, plate, etc. It is essential to know the degree and type of hearing loss,
and other associated handicaps such as blindness or men-
5. OBJECTIVE TESTS tal retardation and whether hearing loss is prelingual (be-
(a) Evoked response audiometry. fore development of speech) or postlingual. Aetiology of
(i) Electrocochleography. It can measure auditory sen- hearing loss remains obscure in about half the cases.
sitivity to within 20 dB. But it is an invasive pro- Aims of habilitation of any hearing-impaired child are
cedure requiring placement of electrodes through development of speech and language, adjustment in soci-
the tympanic membrane. ety and useful employment in a vocation.
(ii) Auditory brainstem response. It is not a direct test
of hearing but correlates highly with the pure- 1. PARENTAL GUIDANCE. It is a great emotional shock
tone thresholds. Identifiable waveforms in ABR for parents to learn that their child is deaf. They should
are generally present 10–20 dB above behavioural be dealt with sympathetically, so as to accept the child.
threshold. ABR provides an ear-specific informa- They should be told of child’s disability and how to
tion as sound stimulus can be presented to each care for it. Habilitation of the deaf demands a lot from
ear separately by headphones or ear inserts. It is an parents: care and periodic replacement of hearing aid,
objective test and can be done under sedation as change of ear-moulds as child grows, follow-up visits for
the latter has no effect on ABR. ABR is used both as re-evaluation, education at home and the selection of
a screening test and as a definitive hearing assess- vocation.
ment test in children. In a screening test, a response
to a click stimulus of less than 40 dB nHL or less 2. HEARING AIDS. Most deaf children have a small but
is the criterion of passing the test. To find hearing useful portion of residual hearing which can be exploited
threshold in an infant, ABR tracing is obtained first by amplification of sound. Hearing aids should be pre-
at higher sound stimulus and then gradually low- scribed as early as possible. If necessary, binaural aids, one
ered till wave V is just identifiable but repeatable. for each ear, can be used. Hearing aids help to develop
(b) Otoacoustic emissions (see p. 29). Transient evoked lip-reading also.
emissions (TEOAEs) are absent in ears where hear-
ing loss exceeds 30 dB. Distortion product emissions 3. COCHLEAR IMPLANTS (SEE P. 138)
(DPOAEs) are absent when hearing loss exceeds 50 dB.
(c) Impedance audiometry. Normally, stapedius muscle 4. DEVELOPMENT OF SPEECH AND LANGUAGE. Communi-
contracts reflexly in response to a sound of 70–100 dB cation is a two way process, depending on the receptive
HL and this reflex can be recorded. Absence of acous- and expressive skills. Reception of information is through
tic reflex indicates middle ear disorder, retrocochlear visual, auditory or tactile faculties while expression is
hearing loss or severe to profound SNHL. Used with through oral or written speech or the manual sign lan-
behaviour audiometry, acoustic reflexes are useful guage. In the hearing impaired, auditory faculty is poor or
component to cross-check. Absence of acoustic reflex, totally absent (Figure 19.3). Thus, for proper communica-
but a normal tympanometry with parental concern tion, there is need either to improve hearing through am-
for hearing loss suggests possibility of SNHL of severe plification of the residual hearing or cochlear implants;
to profound degree. Absence of acoustic reflex but an and in the absence of the feasibility of developing the au-
abnormal tympanogram generally indicates conduc- ditory faculty, one has to develop visual or tactile means
tive loss. Since ABR and OAEs provide more informa- of communication.
tion, use of acoustic reflexes in assessment of paediat-
(a) Auditory-oral communication. This is the method used
ric testing has declined.
by a normal person and is the best way of communica-
OAEs and ABR have been used both in screening pro- tion. In the deaf, it can be used in those with moderate
grammes and in hearing evaluation in infants and children. to severe hearing loss or those who are postlingually

Figure 19.3. The faculties of a hearing-impaired person which can be utilized for receptive and expressive skills in communication.

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134 SECTION I — Diseases of Ear

deaf. Hearing aids are provided to augment auditory sternum and the vibrations of speech are perceived
reception. At the same time, training is also imparted through tactile sensation.
in speech reading, i.e. to read movements of lips, face,
and natural gestures of hand and body. Expressive 5. EDUCATION OF THE DEAF. There are residential and day
skill is encouraged through oral speech. schools for the hearing impaired. Some children with mod-
(b) Manual communication. It makes use of the sign lan- erate hearing loss can be integrated into schools for the nor-
guage or finger-spelling method but has the disadvan- mal hearing children with preferential seating in the class.
tage that abstract ideas are difficult to express and Radio hearing aids have revolutionized education of
general public does not understand it. the deaf. In this device, the microphone and transmitter
(c) Total communication. It uses all modalities of sensory are worn by the teacher and the receiver and amplifier
input, i.e. auditory, visual, tactile and kinaesthetic. by the child. With this system, the child can hear the
Such children are taught to develop oral speech, lip- teacher’s voice better, without being disturbed by envi-
reading and sign language. All children with prelin- ronmental noises.
gual severe to profound deafness, should undergo
training in this form of communication. Vibrotactile 6. VOCATIONAL GUIDANCE. The deaf are sincere and good
aids are useful for those who are totally deaf and also workers. Given the opportunity, commensurate with their
blind. These aids are attached to the child’s hand or ability, they can be usefully employed in several vocations.

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 Chapter 20
Rehabilitation of the Hearing Impaired

All hearing-impaired individuals need some sort of au- with a tubing and an earmould. It is useful for slight
ral rehabilitation for communication. The various means to moderate cases of hearing loss particularly the high
available to them are: frequency ones.
3. Spectacle types. It is a modification of the “behind-
1. Instrumental devices
the-ear” type and the unit is housed in the auricular
(a) Hearing aids
part of the spectacle frame. It is useful to persons who
(i) Conventional hearing aids
need both eye glasses for vision and a hearing aid. It is
(ii) Bone-anchored hearing aids
not very popular now.
(iii) Implantable hearing aids (vibrant soundbridge)
4. In-the-ear (ITE) types. The entire hearing aid is
(b) Implants
housed in an earmould which can be worn in the ear.
(i) Cochlear implants
It is useful for mild to moderate hearing losses with flat
(ii) Auditory brainstem implants
configuration. They are very popular because of their
(c) Assistive devices for the deaf
cosmetic appeal.
2. Training
5. Canal types (ITC and CIC). The hearing aid is so small
(a) Speech (lip) reading
that the entire aid can be worn in the ear canal with-
(b) Auditory training
out projecting into the concha. For using this aid, it is
(c) Speech conservation
required that the ear canal should be large and wide,
and patient should have the dexterity to manipulate
the minute controls in the aid. It is useful for mild
I. INSTRUMENTAL DEVICES to moderate cases of hearing loss of high frequency
(1–4 kHz).
A. HEARING AIDS Two types are available: in the canal (ITC) and another
Conventional Hearing Aids still smaller and invisible type, completely in the canal
(CIC).
A hearing aid is a device to amplify sounds reaching the
ear. Essentially, it consists of three parts: (i) a microphone,
INDICATIONS FOR HEARING AID. Any individual who has
which picks up sounds and converts them into electri-
a hearing problem that cannot be helped by medical or
cal impulses, (ii) an amplifier, which magnifies electrical
surgical means is a candidate for hearing aid.
impulses and (iii) a receiver, which converts electrical im-
pulses back to sound. This amplified sound is then carried 1. Sensorineural hearing loss, which interferes with day-
through the earmould to the tympanic membrane. to-day activities of a person. Hearing aid may not suit
all such persons because of the intolerable distortion of
TYPES OF HEARING AIDS sound in some, particularly in those with recruitment.
AIR CONDUCTION HEARING AID. In this, the amplified 2. Deaf children should be fitted with hearing aid as ear-
sound is transmitted via the ear canal to the tympanic ly as possible for development of speech and learning.
membrane. In severely deaf children, binaural aids (one for each
BONE CONDUCTION HEARING AID. Instead of a receiver, ear and individually fitted) are more useful. Training
it has a bone vibrator which snugly fits on the mastoid in lip reading is given simultaneously.
and directly stimulates the cochlea. This type of aid is 3. Conductive deafness. Most of such persons can be
especially useful in persons with actively draining ears, helped by surgery but hearing aid is prescribed when
otitis externa or atresia of the ear canal when ear inserts surgery is refused or not feasible or has failed.
cannot be worn.
Most of the aids are air conduction type. They can be: FITTING A HEARING AID. While fitting a hearing aid,
consideration is given to:
1. Body-worn types. Most common type (Figure 20.1A );
microphone and amplifier along with the battery are 1. Degree of hearing loss.
in one case worn at the chest level while receiver is 2. Configuration of hearing loss (type of frequencies af-
situated at the ear level. This type of aid allows high fected).
degree of amplification with minimal feedback. It is 3. Type of hearing loss (conductive or sensorineural).
useful in severely deaf persons or children with con- 4. Presence of recruitment.
genital deafness. 5. Uncomfortable loudness level.
2. Behind-the-ear (BTE) types. Here microphone, am- 6. Age and dexterity of patient.
plifier, receiver and battery are all in one unit which 7. Condition of the outer and middle ear.
is worn behind the ear. It is coupled to the ear canal 8. Cosmetic acceptance of the aid.
135

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Figure 20.1. Various types of hearing aids. (A) Body-worn. (B) Behind-the-ear type. (C) Spectacle type. (D) In-the-ear type.
Scan to play Hearing Aids.

9. Type of earmould.
10. The type of fitting, whether it is monoaural (one aid
only), binaural (one aid for each ear), binaural with
y-connection (one aid but two receivers, one for each
ear) or the contralateral routing of signals type.
CROS (CONTRALATERAL ROUTING OF SIGNALS). In this
type, microphone is fitted on the side of the deaf ear and
the sound thus picked up is passed to the receiver placed
in the better ear. This is useful for persons with one ear
severely impaired and helps in sound localization coming
from the side of the deaf ear. Now bone-anchored hearing
aids (see infra) are being preferred for single-sided deaf-
ness and have replaced the use of CROS aids.

Bone-anchored Hearing Aid (BAHA)

Bone-anchored hearing aid is a type of hearing aid which is Figure 20.2. Appearances when sound processor is attached to
based on the principle of bone conduction. It is primarily abutment.
suited to people who have conductive hearing loss, unilateral
hearing loss and those with mixed hearing loss who cannot BAHA has three components: (i) titanium fixture (ii) ti-
otherwise wear “in the ear” or “behind the ear” hearing aids. tanium abutment and (iii) sound processor (Figure 20.3).
Bone-anchored hearing aids use a surgically implant- The titanium fixture is surgically embedded in the skull
ed abutment to transmit sound by direct conduction bone with abutment exposed outside the skin. The titani-
through bone to the cochlea, bypassing the external au- um fixture bonds with the surrounding tissue in a process
ditory canal and middle ear (Figure 20.2). called osseointegration. The sound processor is attached to

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 Chapter 20 — Rehabilitation of the Hearing Impaired 137

TABLE 20.1 INDICATIONS FOR BAHA

1. When air-conduction (AC) hearing aid cannot be used:
• Canal atresia, congenital or acquired, not amenable to
treatment.
• Chronic ear discharge, not amenable to treatment.
• Excessive feedback and discomfort from air-conduction
hearing aid.
2. Conductive or mixed hearing loss, e.g. otosclerosis and
tympanosclerosis where surgery is contraindicated.
3. Single-sided hearing loss.

Figure 20.3. Bone-anchored hearing aid (BAHA).

1. Piezoelectric devices. Piezoelectric devices operate by
passing an electric current into a piezoceramic crystal,
which changes its volume and thereby produce a vi-
the abutment once osseointegration is complete which bratory signal. This piezoelectric transducer in turn is
usually takes 2–6 months after implantation. The BAHA coupled to the ossicles and drives the ossicular chain
device transmits vibrations to the external abutment by vibration.
which further vibrates the skull and cochleae.
Examples of such devices are Envoy, middle-ear trans-
CANDIDACY PROFILE. Bone-anchored hearing aids can
ducer (MET or also called otologic device), Rion and
be used in:
totally integrated cochlear amplifier (TICA).
1. People who have chronic inflammation or infection 2. Electromagnetic hearing devices. Electromagnetic
of the ear canal and cannot wear standard “in the ear” hearing devices function by passing an electric current
air-conduction hearing aids. into a coil, which creates a magnetic flux that drives an
2. Children with malformed or absent outer ear and ear adjacent magnet. The small magnet is attached to one
canals as in microtia or canal atresia. of the ossicles of the middle ear to convey vibrations to
3. Single-sided deafness (see Table 20.1). the cochlea.
In the past, the contralateral routing of signal (CROS) An example of such a device is the vibrant soundbridge
hearing aid was the only option available for rehabilita- device (previously known as the Symphonix device; now
tion of patients with single-sided deafness. Poor perfor- being manufactured by MED-EL).
mance and aesthetic considerations limited the use of VIBRANT SOUNDBRIDGE DEVICE. The vibrant sound-
CROS aids. The BAHA device can now be implanted on bridge is a semi-implantable device made of two compo-
the side of the deaf ear, and it transmits the sound by nents: an internal and an external. The internal component
means of bone conduction to the contralateral cochlea. is called vibrating ossicular prosthesis (VORP) and is made
The BAHA is fixed on the deaf side and collects sound up of three parts: the receiver, floating mass transducer
waves to transmit to healthy cochlea of the other side. (FMT) and a conductor link between the two. FMT is con-
This process eliminates the head-shadow effect and al- nected to the incus (Figures 20.4 and 20.5).
lows for hearing from both sides of the head. The BAHA The external component is called the audio proces-
substantially improves speech recognition in quiet and in sor which is worn behind the ear. The audio processor
noise compared with the CROS aids.
SURGERY. The surgery is typically performed in a sin-
gle stage in adults. About 3 months are allowed for osse-
ointegration before the sound processor can be attached.
A two-stage procedure is recommended in children in
whom the fixture is placed into the bone in the first stage.
After about 6 months to allow for osseointegration, a
second-stage operation is done to connect the abutment
through the skin to the fixture.
Complications of BAHA are few and may include oc-
casional failure to osseointegrate the implant and local
infections and inflammation at the implant site.

Implantable Hearing Aids

Implantable middle ear hearing aids represent a new
category of hearing devices that work on a direct drive
principle. Rather than delivering acoustic energy into
the external auditory canal (as with traditional hearing
aid systems), direct drive middle ear implant systems use
mechanical vibrations delivered directly to the ossicular
chain, while leaving the ear canal completely open.
Implantable middle ear devices are generally available
in two types: Figure 20.4. Vibrant soundbridge middle ear implant.

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138 SECTION I — Diseases of Ear

Figure 20.5. Components of the vibrating ossicular prosthesis (VORP).

contains a microphone that picks up sound from the en- of the inherent issues of conventional hearing aids such
vironment and transmits it across the skin by radiofre- as occlusion, feedback, discomfort and wax related prob-
quency waves to the internal receiver. lems. One major advantage of direct drive devices is the
CANDIDACY PROFILE. Appropriate candidates for di- ability to provide improved sound quality to the hearing-
rect drive middle ear hearing devices include adults aged impaired subjects particularly in noisy environments.
18 years and older with moderate-to-severe sensorineural
hearing loss. Candidates should have experience of using
traditional hearing aids and should have a desire for an
B. IMPLANTS
alternative hearing system. Cochlear Implants
Often, patients who are interested in seeking direct
A cochlear implant is an electronic device that can provide
drive middle ear hearing devices have experienced dissat-
useful hearing and improved communication abilities for
isfaction regarding the sound quality of their current hear-
persons who have severe to profound sensorineural hear-
ing aids. Other problems these patients feel with these
ing loss and who cannot benefit from hearing aids.
aids are discomfort due to the occlusion effect of the ca-
A cochlear implant works by producing meaningful
nal, wax occluding hearing aid mould and wax impaction
electrical stimulation of the auditory nerve where degen-
of the external auditory canal, inability to wear traditional
eration of the hair cells in the cochlea has progressed to a
hearing aids due to sensitive ear canal skin and the inabil-
point such that amplification provided by hearing aids is
ity to overcome acoustic feedback issues (see Table 20.2 for
no longer effective. Various cochlear implants are shown
disadvantages of conventional hearing aids).
in Figures 20.6–20.8.
PROCEDURE. The internal device is surgically implant-
ed. The procedure is conducted under general anaesthe-
COMPONENTS AND FUNCTIONING OF A COCHLEAR IM-
sia. The receiver of the implant is positioned under the
PLANT (FIGURE 20.9). A cochlear implant has an external
skin over the mastoid bone via a standard cortical mas-
and internal component.
toidectomy and posterior tympanotomy approach; the
ossicular chain is visualized and the FMT is attached to 1. External component. It consists of an external speech
the long process of the incus. The middle ear structures processor and a transmitter. The speech processor may
are not modified. Therefore, there is no significant impact be body worn or behind the ear type; the latter being
on the residual hearing of the patient. preferred.
Six to eight weeks after the procedure, the patient is
fitted with the external audio processor that attaches
magnetically to the back of the ear. The processor is then
programmed.
ADVANTAGES. A direct drive system provides mechani-
cal energy directly to the ossicles, bypassing the ear ca-
nal and the tympanic membrane. This eliminates many

TABLE 20.2 DISADVANTAGES OF CONVENTIONAL

HEARING AIDS
• Cosmetically unacceptable due to visibility.
• Acoustic feedback.
• Spectral distortion.
• Occlusion of external auditory canal.
• Collection of wax in the canal and blockage of insert.
• Sensitivity of canal skin to earmoulds.
Figure 20.6. MED-EL cochlear implants. (A) MED-EL C-401. (B) So-
• Problem to use in discharging ears.
nata model with ear level speech processor.

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 Chapter 20 — Rehabilitation of the Hearing Impaired 139

continuous interleaved sampling(CIS), spectral peak

(SPEAK) and advanced combination encoder (ACE).
The electrical impulses are sent from the processor to
the transmitting coil which in turn sends the signal to
the surgically implanted receiver/stimulator via radiofre-
quency. The receiver/stimulator decodes the signal and
transmits it to the electrode array. Current day implants
are multichannel processors with the electrode having a
linear array of electrode contacts used to deliver multiple
channels of current to different places along the basilar
membrane. The electrode array which has been placed
in the scala tympani of the cochlea stimulates the spiral
ganglion cells. The auditory nerve is thus stimulated and
Figure 20.7. Nucleus cochlear implant (Cochlear Corporation) with
sends these electrical pulses to the brain which are finally
ear level speech processor. interpreted as sound.
CANDIDACY PROFILE. Cochlear implants may be used
both in children and adults. The following criteria help
define candidacy for cochlear implantation:
1. Bilateral severe to profound sensorineural hearing loss.
2. Little or no benefit from hearing aids.
3. No medical contraindication for surgery.
4. Realistic expectation.
5. Good family and social support toward habilitation.
6. Adequate cognitive function to be able to use the device.
Candidates with such hearing impairment may be de-
fined as prelingual or postlingual depending on wheth-
er they were deafened before or after the acquisition of
speech and language.
In children who have hearing impairment at birth or
early in childhood, early intervention with hearing aids
Figure 20.8. Advanced bionics cochlear implant system. or a cochlear implant is vital for auditory stimulus. Audi-
tory deprivation, i.e. lack of auditory stimulus in the early
2. Internal component. It is surgically implanted and developmental period causes degeneration in the central
comprises the receiver/stimulator package with an elec- auditory pathways. This will limit the benefit in terms of
trode array. speech and language acquisition following cochlear im-
plantation.
Sound is picked up by the microphone in the speech
processor. The speech processor analyses and codes OUTCOMES OF COCHLEAR IMPLANTATION. Factors that
sounds into electrical pulses. The processor uses a variety predict a successful clinical outcome are:
of coding strategies to deliver meaningful speech param-
eters from the acoustic stimulus to the nerve. Examples of 1. Previous auditory experience (postlingual patients or
such strategies are simultaneous analogue strategy (SAS), prior use of hearing aids).

Figure 20.9. Principle of cochlear implant.

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140 SECTION I — Diseases of Ear

2. Younger age at implantation (especially for prelingual children who may have disabilities other than hearing
children). loss. This may provide information that is important
3. Shorter duration of deafness. when counselling parents about expectations following
4. Neural plasticity within the auditory system. cochlear implantation.
Multichannel implants are the standard today and per-
form much better than single-channel devices. Postlin- SURGERY. The principle of cochlear implant surgery is to
gual children or adults achieve very good benefit. They place the electrode array within the scala tympani of the
develop the ability to recognize speech with no or mini- cochlea. This allows the electrodes to be in close proxim-
mal lip reading or visual cues. They eventually can also ity to the spiral ganglion cells and their dendrites (that lie
use the telephone. in the modiolus and osseous spiral lamina of the cochlea,
Prelingually deafened children also develop good respectively).
speech understanding and language acquisition over a pe- Surgery is carried out under general anaesthesia and is
riod of time. This can take a couple of years and requires similar to mastoid surgery. Once the patient is positioned,
constant auditory-verbal training. Early age at implanta- prepped and draped, the position of the device is marked
tion ensures better results and children can be implanted and the incision planned. Flaps are elevated carefully so
at 12 months of age. as not to disrupt the blood supply. Usually, a two-layered
Prelingually deafened adults with no or little prior approach is chosen utilizing a flap of skin and subcuta-
auditory experience obtain very limited benefit from neous tissue, followed by a second layer of musculoperi-
cochlear implantation. They will however obtain sound osteal flap. A pocket is created under the second flap and
awareness. a well or recess is drilled in the bone to house the re-
ceiver/stimulator.
There are broadly two surgical techniques to approach
EVALUATION. Thorough evaluation of the patient is very
the cochlea for implantation: (i) The facial recess approach
critical in the selection of candidates for a cochlear im-
where a simple cortical mastoidectomy is done first and
plant. The main purpose is to determine if the patient is
the short process of the incus and the lateral semicircular
medically and audiologically suitable for an implant. It
canal are identified. The facial recess is opened by perform-
also helps the clinicians to predict and counsel the family
ing a posterior tympanotomy. The stapes, promontory
regarding the expected outcomes following the procedure.
and round window niche are identified. Cochleostomy
Medical evaluation through detailed history and
is then performed anteroinferior to the round window
physical examination is necessary to confirm fitness for
membrane to a diameter of 1.0–1.6 mm depending on
a general anaesthetic. The necessary preanaesthetic tests
the electrode to be used. (ii) The pericanal techniques where
will be required to be carried out. All candidates must be
a tympanomeatal flap is elevated to perform a cochleos-
fully vaccinated against meningitis (particularly Haemo-
tomy either by endaural or postaural approach. In the
philus influenzae type B, Pneumococcus and in some areas
pericanal techniques a bony tunnel is drilled along the
Meningococcus).
external canal towards the middle ear. The examples of
Imaging of the temporal bone, cochlea, auditory nerve
pericanal techniques include the Veria and suprameatal re-
and brain is carried out using CT and MRI. This is re-
cess approach.
quired to provide an image of the structure of the cochlea
The device is placed in the “well” created and is se-
and help identify any anomalies or pathology that may
cured with ties. The electrode array is gently and gradu-
complicate the implantation process.
ally inserted through the cochleostomy till complete in-
Audiological evaluation may include some or all of the
sertion has been achieved. Electrophysiological testing is
following depending on the age of the patient:
carried out to check that the electrode impedances and
• Pure tone audiogram telemetry responses are satisfactory.
• Speech discrimination tests The wound is closed in layers and a mastoid bandage
• Tympanometry applied.
• Otoacoustic emissions (OAE)
• Auditory brainstem responses (ABR) POSTOPERATIVE MAPPING (PROGRAMMING) OF DEVICE
• Auditory steady state responses (ASSR) AND HABILITATION. Activation of the implant is done
3–4 weeks after implantation. Following this the implant
A hearing aid trial and evaluation is mandatory in deter- is “programmed” or “mapped.” Mapping is done on a reg-
mining the candidacy for cochlear implantation. This may ular basis during postoperative rehabilitation to fine-tune
include aided free-field sound detection thresholds, as well the processor and get the best performance as the patient
as aided speech perception and discrimination scores. gets used to hearing with the implant.
Speech and language evaluation is required to assess the (Re) Habilitation is an essential part for those who
child’s communicative status and to determine any de- have undergone cochlear implantation. All patients need
velopmental language or articulation disorders. This will auditory-verbal therapy. In auditory-verbal therapy, the
also form a baseline for further evaluations postimplanta- emphasis is laid on making the child listen and speak like
tion to help assess progress and identify areas of deficit in a normal person rather than use lip reading and visual
speech perception. This in turn would aid in the program- cues. Learning to listen takes time and requires concerted
ming of the patient’s device. efforts from the patient, the family and the person pro-
Psychological evaluation is performed where there may viding habilitation services.
be concerns regarding the cognitive status or mental Table 20.3 summarizes the complications of cochlear
function of the patient. This is also important to identify implant surgery.

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TABLE 20.3 COMPLICATIONS OF COCHLEAR signal or relay the signal to an area closer to the individ-
IMPLANT SURGERY ual. A “hearing dog” is one such simple device. The dog
is trained to bark loudly at the sound of a doorbell or cry
Early complications Late complications
of a baby to alert his master. It is a helpful companion for
• Facial paralysis • Exposure of device and the hearing impaired.
• Wound infection extrusion For people with severe to profound or total deafness,
• Wound dehiscence • Pain at the site of implant even these devices which produce extra loud sound may
• Flap necrosis • Migration/displacement of
not be useful. They need assistive signalling devices where
• Electrode migration device
• Device failure • Late device failure
the sound (as of doorbell, telephone, alarm clock, baby
• CSF leak • Otitis media crying) is changed into a light signal or vibrations. Alarm
• Meningitis clock with flashing lights or those devices which produce
• Postoperative dizziness/ strong vibrations to awaken the individual or even shake
vertigo his bed are also available.

3. Telecommunication Devices
A telephone amplifier can be attached to the hand set of
Auditory Brainstem Implant (ABI) a telephone, residential or public, to amplify the sound.
This implant is designed to stimulate the cochlear nu- A telephone coupler is a device that can be connected to
clear complex in the brainstem directly by placing the the telephone and the signal produced is picked up by the
implant in the lateral recess of the fourth ventricle. Such hearing aid.
an implant is needed when CN VIII has been severed in For the profoundly or totally deaf individuals, telecom-
surgery of vestibular schwannoma. In these cases, cochlear munication devices for the deaf (TDDs) can be used. They
implants are obviously of no use. In unilateral acoustic convert typed message into sounds that can be transmit-
neuroma, ABI is not necessary as hearing is possible from ted over the standard telephone lines, and at the other
the contralateral side but in bilateral acoustic neuromas end another TDD converts these sound signals back into
as in NF2, rehabilitation is required by ABI. typewritten messages. Email and short message services
Brainstem implant is similar to “Nucleus” multichan- (SMS) on mobile phones have made life easier for the
nel cochlear implant except that the multielectrode ar- hearing impaired.
ray is attached to a Dacron mesh, which is placed on the Closed-caption television decoder can be attached to
brainstem. Receiver/stimulator has a removable magnet television sets to provide them cues to enjoy news, mov-
so that MRI can be safely performed in such cases if need ies and other programmes.
arises.
ABIs help in communication, awareness and recogni-
tion of environmental sounds; however, they are not as
efficient as multichannel cochlear implants. Only limited II. TRAINING
numbers of such implants have been performed in the
world and are under constant technological develop- A. SPEECH READING
ments. Earlier called lip-reading, it is an integrated process to
understand speech by studying movements of lips, facial
C. ASSISTIVE DEVICES expression, gestures and the probable context of conver-
sation. The skill of speech reading is not only useful for
Hearing-impaired persons should enjoy life as best as nor- the totally deaf but also useful for those hearing-impaired
mally hearing persons do. For this, devices are needed to individuals who have high-frequency loss and difficulty
help him to listen in special difficult situations, warn him in hearing in noisy surroundings.
of danger signals and help him to telecommunicate with
his family and friends who are far away from him. These
devices can thus be divided into three groups: B. AUDITORY TRAINING
1. Assistive Listening Devices and Systems It enhances listening skill and is used with speech read-
ing. The patient is exposed to various listening situations
They are not hearing aids but devices which help the
with different degrees of difficulty and taught selectively
hearing impaired to listen efficiently in the presence of
to concentrate on speech sounds.
background noise, over the telephone, in auditoriums or
Auditory training is useful for those using hearing aids
theatres. They may be used by the person individually or
and cochlear implants.
are meant for a group.
According to the technology used, they are grouped as
hard-wired system, induction loops, AM (amplitude mod- C. SPEECH CONSERVATION
ulation), FM (frequency modulation) or infrared signals.
In sudden, severe or profound hearing loss, the person
2. Alerting Devices loses the ability to monitor his own speech production.
A hearing-impaired person may not hear a telephone or a As a result, defects arise in articulation, resonance, pitch
doorbell, a baby crying in another room, an alarm clock and the volume of voice. Speech conservation aims to
or the noise of a smoke detector. Alerting devices are use- educate such a person to use his tactile and propriocep-
ful in such situations. They produce an extra loud sound tive feedback systems to monitor his speech production.

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 Chapter 21
Otalgia (Earache)

Pain in the ear can be due to causes occurring locally in (b) Oral cavity. Benign or malignant ulcerative lesions
the ear or referred to it from remote areas. of oral cavity or tongue.
(c) Temporomandibular joint disorders. Bruxism, osteo-
arthritis, recurrent dislocation and ill-fitting den-
I. LOCAL CAUSES ture.
(d) Sphenopalatine neuralgia.
1. EXTERNAL EAR. Furuncle, impacted wax, otitis ex- 2. Via IXth cranial nerve
terna, otomycosis, myringitis bullosa, herpes zoster and (a) Oropharynx. Acute tonsillitis, peritonsillar abscess,
malignant neoplasms. tonsillectomy. Benign or malignant ulcers of soft
palate, tonsil and its pillars.
2. MIDDLE EAR. Acute otitis media, eustachian tube ob- (b) Base of tongue. Tuberculosis or malignancy.
struction, mastoiditis, extradural abscess, aero-otitis me- (c) Elongated styloid process.
dia and carcinoma middle ear. 3. Via Xth cranial nerve. Malignancy or ulcerative le-
sion of vallecula, epiglottis, larynx or laryngopharynx
and oesophagus.
II. REFERRED CAUSES 4. Via C2 and C3 spinal nerves. Cervical spondylosis, in-
juries of cervical spine and caries spine.
As ear receives nerve supply from Vth (auriculotempo-
ral branch), IXth (tympanic branch) and Xth (auricular
branch) cranial nerves; and from C2 (lesser occipital) and III. PSYCHOGENIC CAUSES
C2 and C3 (greater auricular), pain may be referred from
When no cause has been discovered, pain may be func-
these remote areas (Figure 21.1).
tional in origin but the patient should be kept under ob-
1. Via Vth cranial nerve servation with periodic re-evaluation.
(a) Dental. Caries tooth, apical abscess, impacted mo- Otalgia is a symptom. It is essential to find its cause be-
lar, malocclusion and Costen syndrome.1 fore specific treatment can be instituted.

Figure 21.1. Referred causes of otalgia. Pain is referred via CN V (teeth, oral cavity, TM joint, anterior two-thirds of tongue), C2,3 (cervical spine),
CN IX (tonsil, base of tongue, elongated styloid process) and CN X (vallecula, pyriform fossa or larynx).
143

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 Chapter 22
Tinnitus

Tinnitus is ringing sound or noise in the ear. The character- TABLE 22.1 CAUSES OF TINNITUS
istic feature is that the origin of this sound is within the pa-
Subjective Tinnitus Objective Tinnitus
tient. Usually, it is unilateral but may also affect both ears.
It may vary in pitch and loudness and has been variously • Otologic • Vascular
described by the patient as roaring, hissing, swishing, rus- • Impacted wax • AV shunts
tling or clicking type of noise. Tinnitus is more annoying in • Fluid in middle ear – Congenital AV
• Acute otitis media malformations
quiet surroundings, particularly at night, when the mask-
• Chronic otitis media – Glomus tumour of
ing effect of ambient noise from the environment is lost.
• Ménière’s disease middle ear
• Presbycusis • Arterial bruit
• Noise-induced hearing – Carotid aneurysm
TYPES OF TINNITUS loss – Carotid stenosis
• Idiopathic sudden SNHL – Vascular loop pressing
Two types of tinnitus are described: • Acoustic neuroma on VIIIth nerve in
• Metabolic internal auditory canal
1. Subjective, which can only be heard by the patient. • Hypothyroidism – High-riding carotid
2. Objective, which can even be heard by the examiner • Hyperthyroidism artery
with the use of a stethoscope. • Obesity – Persistent stapedial
• Hyperlipidaemia artery
• Vitamin deficiency • Venous hum
(e.g. B12) – Dehiscent jugular bulb
CAUSES OF TINNITUS (TABLE 22.1) • Neurologic • Patulous eustachian tube
Subjective tinnitus may have its origin in the external ear, • Head injury (labyrinthine • Palatal myoclonus
concussion) • Idiopathic stapedial or tensor
middle ear, inner ear, VIIIth nerve or the central nervous
• Temporal bone fractures tympani myoclonus
system. Systemic disorders like anaemia, arteriosclerosis, • Whiplash injury • Dental
hypertension and certain drugs may act through the inner • Multiple sclerosis • Clicking of TM joint
ear or central auditory pathways. In the presence of con- • Postmeningitic
ductive hearing loss, the patient may hear abnormal noises • Brain haemorrhage
in the head during eating, speaking or even respiration. • Brain infarct
Objective tinnitus is seen less frequently. Vascular • Cardiovascular
lesions, e.g. glomus tumour or carotid artery aneurysm • Hypertension
cause swishing tinnitus synchronous with pulse. It can • Hypotension
be temporarily abolished by pressure on the common ca- • Anaemia
• Cardiac arrhythmias
rotid artery. Venous hum can sometimes be stopped by
• Arteriosclerosis
pressure on the neck veins. • Pharmacologic
Tinnitus synchronous with respiration may occur due • Certain drugs used by the
to abnormally patent eustachian tube. Palatal myoclonus patient
produces clicking sound due to clonic contraction of the • All ototoxic drugs
muscles of soft palate and can be easily diagnosed. Clonic • Psychogenic
contraction of muscles of middle ear (stapedius and ten- • Anxiety
sor tympani) may cause tinnitus which is often difficult • Depression
to diagnose.
Sometimes, tinnitus is psychogenic and no cause can
be found in the ear or central nervous system.
When no cause is found, management of tinnitus in-
Tinnitus should be differentiated from auditory hallu-
cludes:
cinations in which a person hears voices or other organ-
ized sounds like that of music. It is seen in psychiatric 1. Reassurance and psychotherapy. Many times the patient
disorders. has to learn to live with tinnitus.
2. Techniques of relaxation and biofeedback.
3. Sedation and tranquillizers. They may be needed in ini-
TREATMENT OF TINNITUS tial stages till patient has adjusted to the symptom.
4. Masking of tinnitus. Tinnitus is more annoying at bed-
Tinnitus is a symptom and not a disease. Where possible, time when the surroundings are quite. Use of a fan,
its cause should be discovered and treated. Sometimes, loudly clicking clock or a similar device may mask the
even the treatment of cause may not alleviate tinnitus. tinnitus and help the patient to go to sleep. Use of a

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146 SECTION I — Diseases of Ear

hearing aid, in persons with hearing loss, not only im- 2. Habituation of tinnitus. It is blocking the tinnitus-
proves hearing but also provides a masking effect. related neuronal activity to reach level of conscious-
ness. With this therapy patients suffering from
Tinnitus maskers can be used in patients who have
tinnitus lose awareness of tinnitus and also do not get
no hearing loss. They are worn like a hearing aid. Use of
annoyed even when they do have tinnitus.
tinnitus masker for a short time may provide, in some
individuals, a symptom-free period for several hours due Therapy consists of two major components: (i) coun-
to the phenomenon of residual inhibition. selling and (ii) sound therapy.
Counselling. It is important to educate the patient
TINNITUS INSTRUMENT about tinnitus, its mechanism of generations, perception
of tinnitus at subcortical and cortical levels and the plas-
It is a combination of a hearing aid and a masker in one ticity of brain which can habituate any sensory stimuli.
device. Looks like a hearing aid. Both hearing aid and Limbic system (emotions) and autonomic system (body
masking device have independent volume controls. reactions) are the primary sources of negative reactions to
tinnitus, i.e. sleep disturbance, inability to concentrate,
TINNITUS RETRAINING THERAPY (TRT) annoyance, anxiety and depression and not the tinnitus
per se.
Jastreboff from University of Maryland described a neu-
Sound therapy. Patient is exposed to environmental
rophysiologic model for generation of tinnitus and the
sounds, music radio, television, or use of hearing aids (in
basis for habituation therapy. It presumes that tinnitus
case he suffers from hearing loss). In general, he should
does not cause as much annoyance as the emotional reac-
avoid silent environment. To produce external sound for
tions generated from the limbic and autonomic systems.
habituation, sound generators are used which produce
His therapeutic model aims to attenuate connections be-
continuous low-level, broad-band noise for at least 8 h
tween auditory, limbic and autonomic nervous systems
a day. Sound, here is used not for masking the tinnitus
and thus create tinnitus habituation. It occurs at two levels.
but is adjusted to remain at a low level, for habituation.
1. Habituation of reaction. It is uncoupling of brain and TRT needs a long period of 18–24 months but gives a
body from negative reactions to tinnitus. significant improvement in more than 80% of patients.

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