OTOSCLEROSIS

OTOSCLEROSIS

Sandipan naskar
Sandipan naskar
CNMCH
CNMCH
INTRODUCTION
INTRODUCTION
 Worldwide, the number of patients suffering from
 Worldwide, the number of patients suffering from
otosclerosis has declined considerably.
otosclerosis has declined considerably.

 Cause for rejoicing / an unique predicament to modern-

 Cause for rejoicing / an unique predicament to modern-
day otologist.
day otologist.

 We (young modern-day otologists) will need to

 We (young modern-day otologists) will need to
overcome our own learning curve before being able to
overcome our own learning curve before being able to
perform the surgery reliably and deliver consistently
perform the surgery reliably and deliver consistently
good results.
good results.

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DEFINITION:
DEFINITION:
Otosclerosis is a localized hereditary metabolic
Otosclerosis is a localized hereditary metabolic
disorder affecting endochondral bone of the otic
disorder affecting endochondral bone of the otic
capsule that is characterized by disordered resorption
capsule that is characterized by disordered resorption
and deposition of bone.
and deposition of bone.
This process occurrs in two phases:
This process occurrs in two phases:
(1) active phase as characterized by bone resorption
(1) active phase as characterized by bone resorption
(spongiosis)
(spongiosis)
(2) phase of remission characterized by bone deposition
(2) phase of remission characterized by bone deposition
(sclerosis).
(sclerosis).
It is unique to human temporal bone;
It is unique to human temporal bone;
Normal inhibition of bone remodelling is lost resulting in foci of
Normal inhibition of bone remodelling is lost resulting in foci of
bone remodelling
bone remodelling

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HISTORY
HISTORY
First described by Valsalva (early 18th thcentury)
First described by Valsalva (early 18 century)

VonTroltsch named the final inactive stage as

VonTroltsch named the final inactive stage as
‘otosclerosis’ (1869)
‘otosclerosis’ (1869)
Siebenmann disignated the active stage as ‘otospongiosis’ (1912)
Siebenmann disignated the active stage as ‘otospongiosis’ (1912)
Toynbee: described different types of stapes fixation and oval window
Toynbee: described different types of stapes fixation and oval window
involvement
involvement

Politzer first recognised it as primary bone disease

Politzer first recognised it as primary bone disease

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EPIDEMIOLOGY
EPIDEMIOLOGY
 RACE:
 RACE:
There appears to be a definite racial predisposition.
There appears to be a definite racial predisposition.

Caucasians (most common)

Caucasians (most common)
Southeast Asian (less common)
Southeast Asian (less common)
Native American (less common)
Native American (less common)
African (rare)
African (rare)

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 PREVALENCE:
 PREVALENCE:
The exact incidence remains unclear and next to
The exact incidence remains unclear and next to
impossible to determine.
impossible to determine.
prevalence of clinically apparent otosclerosis0.3% to 0.5% of
prevalence of clinically apparent otosclerosis0.3% to 0.5% of
general population (Shambaugh)
general population (Shambaugh)

prevalence of histologic otosclerosis is 10% to 12%

prevalence of histologic otosclerosis is 10% to 12%
of which only 1% become clinically apparent.
of which only 1% become clinically apparent.

Many authors have noted that the prevalence otosclerosis

Many authors have noted that the prevalence otosclerosis
has declined steeply in recent times.
has declined steeply in recent times.

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 AGE OF ONSET:
 AGE OF ONSET:
It can range from 10 to 50years of age, however, it can
It can range from 10 to 50years of age, however, it can
also occur in older people.
also occur in older people.
the incidence of presumptive clinical otosclerosis
the incidence of presumptive clinical otosclerosis
increases with age.
increases with age.

 GENDER:
 GENDER:
Not a genetically sex-linked characteristic disease; thus,
Not a genetically sex-linked characteristic disease; thus,
a ratio of 1:1 would have been expected.
a ratio of 1:1 would have been expected.
Indeed, histologically the distribution is equal
Indeed, histologically the distribution is equal
clinically Male:Female = 1:2
clinically Male:Female = 1:2
progress to involve both ear in 85-90%
progress to involve both ear in 85-90%

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 OTOSCLEROSIS AND PREGNANCY:
 OTOSCLEROSIS AND PREGNANCY:
There are many reports that associate the onset of hearing
There are many reports that associate the onset of hearing
loss caused by otosclerosis and the onset of pregnancy.
loss caused by otosclerosis and the onset of pregnancy.
some of them mentioned-
some of them mentioned-
pregnency aggravates the hearing loss.
pregnency aggravates the hearing loss.
in bilateral stapedial otosclerosis the incidence is 33% after one
in bilateral stapedial otosclerosis the incidence is 33% after one
pregnency and 64% after 6 pregnencies.
pregnency and 64% after 6 pregnencies.
Reason – unknown ;
Reason – unknown ;
Hormonal factors may be responsible.
Hormonal factors may be responsible.

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 TYPES OF OTOSCLEROSIS:
 TYPES OF OTOSCLEROSIS:

1. Histologic otosclerosis
1. Histologic otosclerosis
2. Fenestral / Clinical otosclerosis
2. Fenestral / Clinical otosclerosis
3. Cochlear otosclerosis
3. Cochlear otosclerosis
4. Malignant otosclerosis
4. Malignant otosclerosis
5. Far advanced otosclerosis
5. Far advanced otosclerosis

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 HISTOLOGIC OTOSCLEROSIS:
 HISTOLOGIC OTOSCLEROSIS:
o a finding on microscopic examination of temporal
o a finding on microscopic examination of temporal
bones.
bones.

o location of the otosclerotic changes is such that it

o location of the otosclerotic changes is such that it
generally does not involve the stapes bone, the
generally does not involve the stapes bone, the
stapediovestibular joint, or the cochlear endosteum.
stapediovestibular joint, or the cochlear endosteum.

o therefore asymptomatic.
o therefore asymptomatic.

o diagnosed only by post-mortem examination of the

o diagnosed only by post-mortem examination of the
temporal bone.
temporal bone.

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“Histologic” Otosclerotic Focus
“Histologic” Otosclerotic Focus

Stapes
Stapes
Footplate
Footplate

Cochlea
Cochlea

Vestibule
Vestibule

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 “Histologic” Otosclerotic Focus
“Histologic” Otosclerotic Focus
Cochlea
Cochlea

Stapes
Stapes
Footplate
Footplate

Vestibule
Vestibule

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 FENESTRAL (Clinical) OTOSCLEROSIS:
 FENESTRAL (Clinical) OTOSCLEROSIS:

o an otosclerotic lesion that involves the stapes bone and

o an otosclerotic lesion that involves the stapes bone and
/or the stapediovestibular joint.
/or the stapediovestibular joint.

o clinically manifested by a conductive hearing impairment

o clinically manifested by a conductive hearing impairment

o also defined ‘presumptive clinical otosclerosis’

o also defined ‘presumptive clinical otosclerosis’
(British National Study of Hearing)
(British National Study of Hearing)

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Clinical
Clinical
Otosclerotic
Otosclerotic
Foci
Foci

Stapes
Stapes
Footplate
Footplate

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Page  15
 COCHLEAR OTOSCLEROSIS:
 COCHLEAR OTOSCLEROSIS:

o the otosclerotic lesion invades the cochlear endosteum

o the otosclerotic lesion invades the cochlear endosteum

o usually reserved for the occurrence of pure

o usually reserved for the occurrence of pure
sensorineural hearing loss due to otosclerosis
sensorineural hearing loss due to otosclerosis
without any conductive component.
without any conductive component.

o hearing loss is usually bilateral and symmetrical

o hearing loss is usually bilateral and symmetrical

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 Otosclerotic Foci
Otosclerotic Foci

saccule
saccule

utricle
utricle

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Otosclerotic Foci
Otosclerotic Foci

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 “MALIGNANT” (OBLITERATIVE) OTOSCLEROSIS:
 “MALIGNANT” (OBLITERATIVE) OTOSCLEROSIS:

o active otosclerosis involving both oval and round

o active otosclerosis involving both oval and round
windows and most of the bony labyrinth
windows and most of the bony labyrinth
o initially by mixed hearing loss
o initially by mixed hearing loss

o profound sensorineural hearing

relentlessly loss
progress to
o profound sensorineural hearing loss
o both windows are obliterated by the otosclerotic focus
o both windows are obliterated by the otosclerotic focus

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RW
RW

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Page  21
 FAR ADVANCED OTOSCLEROSIS (FAO):
 FAR ADVANCED OTOSCLEROSIS (FAO):

o defined as no measurable air or bone conduction

o defined as no measurable air or bone conduction
or
or
o air conduction no better than 95 dB and bone conduction
o air conduction no better than 95 dB and bone conduction
at 55 dB to 60 dB at one frequency only.
at 55 dB to 60 dB at one frequency only.

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DISTRIBUTION OF OTOSCLEROTIC LESIONS
DISTRIBUTION OF OTOSCLEROTIC LESIONS
anterior to the oval window (80-95%) Most common
anterior to the oval window (80-95%)
fissula ante fenestrum
fissula ante fenestrum
round window niche (about 30%)
round window niche (about 30%)
apical medial wall of the cochlear labyrinth (about 15 %)
apical medial wall of the cochlear labyrinth (about 15 %)
stapes footplate (about 12%)
stapes footplate (about 12%)
posterior to the oval window (5-10%)
posterior to the oval window (5-10%)

walls of the internal auditory canal

walls of the internal auditory canal
around the vestibular and cochlear aqueducts
around the vestibular and cochlear aqueducts
around the semicircular canals Less
around the semicircular canals
around malleus and incus
around malleus and incus Commonly

involved

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HISTOPATHOLOGY OF OTOSCLEROSIS
HISTOPATHOLOGY OF OTOSCLEROSIS

 Otosclerosis is characterized by the following:

 Otosclerosis is characterized by the following:
1. Bone resorption
1. Bone resorption
2. New bone formation
2. New bone formation
3. Vascular proliferation
3. Vascular proliferation
4. Connective tissue stroma.
4. Connective tissue stroma.

 The otic capsule contains small regions of immature cartilaginous

 The otic capsule contains small regions of immature cartilaginous
tissue called the “globuli interossei.” This may be the loci of the
tissue called the “globuli interossei.” This may be the loci of the
earliest lesions of otosclerosis.
earliest lesions of otosclerosis.

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Can be divided into :
Can be divided into :
early phase / spongiotic phase
early phase / spongiotic phase
late phase / sclerotic phase
late phase / sclerotic phase

Early Phase:
Early Phase:
The lesion consists of—
The lesion consists of—
histiocytes
histiocytes
osteoblasts
osteoblasts
osteocytes
osteocytes
osteoclasts
osteoclasts

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First Stage:
First Stage:
resorption of enchondral bone around blood vessels by
resorption of enchondral bone around blood vessels by
histiocytes and osteoclasts enlargement of perivascular
histiocytes and osteoclasts enlargement of perivascular
spaces
spaces
Second stage:
Second stage:
widening of the vascular channels and dialatation of the
widening of the vascular channels and dialatation of the
microcirculation
microcirculation
Third stage:
Third stage:
deposition of cellular fibrous connective tissue within this enlarged
deposition of cellular fibrous connective tissue within this enlarged
perivascular space
perivascular space

reticular cells and fibroblasts within the connective tissue are

reticular cells and fibroblasts within the connective tissue are
transformed into osteoblasts and osteocytes
transformed into osteoblasts and osteocytes

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these osteoblasts lay down immature basophilic bone, rich in
these osteoblasts lay down immature basophilic bone, rich in
ground substance and deficient in collagen;
ground substance and deficient in collagen;
the osteocytes themselves becomes active and elongated;
the osteocytes themselves becomes active and elongated;
microfoci of otospongiosis forms and they fuse to form large foci.
microfoci of otospongiosis forms and they fuse to form large foci.

Fourth stage:
Fourth stage:
Resorption and deposition of immature bone goes on continuously
Resorption and deposition of immature bone goes on continuously
within an otosclerotic focus.
within an otosclerotic focus.

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So, histologically an active focus is indicated by—
So, histologically an active focus is indicated by—

o Areas of nonosseous tissues that demonstrate

o Areas of nonosseous tissues that demonstrate
increased cellularity and vascularity
increased cellularity and vascularity

o Areas of bone resorption and/or bone deposition

o Areas of bone resorption and/or bone deposition

o Increased vascularity, thickening and fibrosis of the

o Increased vascularity, thickening and fibrosis of the
overlying mucosa
overlying mucosa

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 Late Phase:
Late Phase:
the newly laid down immature bone is replaced by osseous tissue
the newly laid down immature bone is replaced by osseous tissue
containing more collagen and less ground substance
containing more collagen and less ground substance

formation of dense sclerotic bone in areas of previous bone

formation of dense sclerotic bone in areas of previous bone
resorption ; the vascular channels become narrowed
resorption ; the vascular channels become narrowed

the otosclerotic focus is larger in volume than the bone it replaces

the otosclerotic focus is larger in volume than the bone it replaces
and thus it causes thickening of the structure it involves.
and thus it causes thickening of the structure it involves.

At the interface of normal and otosclerotic bone two types of

At the interface of normal and otosclerotic bone two types of
changes are seen:
changes are seen:

sharply demarcated line an advancing front that appeared serrated

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Blue Mantle Of Manasse:
Blue Mantle Of Manasse:
o basophilic staining regions that are seen in the otic capsule near
o basophilic staining regions that are seen in the otic capsule near
the otosclerotic foci in the temporal bone that has been stained with
the otosclerotic foci in the temporal bone that has been stained with
hemotoxylin and eosin ( Manasse ; 1922 )
hemotoxylin and eosin ( Manasse ; 1922 )

o
o non-specific histologic changes
non-specific histologic changes
characterised by plexus like projection
characterised by plexus like projection

o
o probably represents bone
probably represents bone
that has been remodelled recently
that has been remodelled recently

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Schwartz’s Sign:
Schwartz’s Sign:
the dialated vessels within the otospongiotic focus forms
the dialated vessels within the otospongiotic focus forms
anastomosis with the vessels of the bony labyrinth
anastomosis with the vessels of the bony labyrinth

as the foci enlarges and reaches the

as the foci enlarges and reaches the
surface mucosa, vascular shunts forms
surface mucosa, vascular shunts forms
between these dialated vessels and the
between these dialated vessels and the
submucosal vessels, resulting in
submucosal vessels, resulting in
hyperemia of the mucosa
hyperemia of the mucosa

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MOLECULAR BIOLOGY

osteoclast

Bone
Bone
Remodelling
Remodelling

osteoblast

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Bone remodeling at a local level is very tightly regulated
Bone remodeling at a local level is very tightly regulated
by a delicate balance among the 3 cytokines:
by a delicate balance among the 3 cytokines:
(1) OPG (osteoprotegrin) {fibroblast > perilymph}
(1) OPG (osteoprotegrin) {fibroblast > perilymph}
(2) RANK {osteoclast}
(2) RANK {osteoclast}
(3) RANK-L{osteoblast}
(3) RANK-L{osteoblast}

RANK-L
RANK-L

RANK
RANK

OPG
OPG

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 In otosclerosis the balance is
In otosclerosis the balance is
tipped in favor of remodeling at
tipped in favor of remodeling at
specific areas within the otic
specific areas within the otic
capsule.
capsule.

osteoclast

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AETIOLOGY
AETIOLOGY

 GENETIC PREDISPOSITION:
 GENETIC PREDISPOSITION:
Otosclerosis represents a heterogeneous group of genetic
Otosclerosis represents a heterogeneous group of genetic
disorders in which different genes may be involved.
disorders in which different genes may be involved.

It is postulated that in response to the different gene variants and

It is postulated that in response to the different gene variants and
environmental factors, the physiologic inhibition of bone turnover in
environmental factors, the physiologic inhibition of bone turnover in
the otic capsule is overruled, which then results in otosclerosis.
the otic capsule is overruled, which then results in otosclerosis.

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o Eight genetic loci OTSC1 to OTSC8 have been published to date
o Eight genetic loci OTSC1 to OTSC8 have been published to date
of which OTSC1 was mapped to 15q25–q26 in an Indian family
of which OTSC1 was mapped to 15q25–q26 in an Indian family

o Association analysis has revealed a significant association between

o Association analysis has revealed a significant association between
defected COL1A1 gene and both familial and sporadic cases of
defected COL1A1 gene and both familial and sporadic cases of
clinical otosclerosis. (association with osteogenesis imperfecta type 1)
clinical otosclerosis. (association with osteogenesis imperfecta type 1)

o Autosomal-dominant transmission with incomplete penetrance in

o Autosomal-dominant transmission with incomplete penetrance in
familial otosclerosis.
familial otosclerosis.

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 MEASLES:
 MEASLES:

There is strong evidence that MeV infection is associated with the

There is strong evidence that MeV infection is associated with the
sporadic form of otosclerosis. (McKenna et al)
sporadic form of otosclerosis. (McKenna et al)

Suggestion of a possible persistent measles virus infection similar

Suggestion of a possible persistent measles virus infection similar
to what occurs in the central nervous system in subacute sclerosing
to what occurs in the central nervous system in subacute sclerosing
panencephalitis (SSPE)
panencephalitis (SSPE)

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 Detection of measles-like structures and antigenicity in active otosclerotic
 Detection of measles-like structures and antigenicity in active otosclerotic
lesions
lesions

 Measles RNA has been found in temporal bone specimen with

 Measles RNA has been found in temporal bone specimen with
otosclerosis . It was also detected in fresh otosclerotic footplate
otosclerosis . It was also detected in fresh otosclerotic footplate
specimens taken at the time of stapedectomy
specimens taken at the time of stapedectomy

 Raised levels of antimeasles antibodies have also been reported in the

 Raised levels of antimeasles antibodies have also been reported in the
perilymph of otosclerotic patients
perilymph of otosclerotic patients

 Epidemiological data reveals a clear and significant decline in the incidence

 Epidemiological data reveals a clear and significant decline in the incidence
of otosclerosis following the introduction of the measles vaccine.)
of otosclerosis following the introduction of the measles vaccine.)

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The measles virus has not been isolated so far
The measles virus has not been isolated so far
from otosclerotic tissue.
from otosclerotic tissue.

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 AUTOIMMUNITY:
 AUTOIMMUNITY:

autoimmune disease with humoral autoimmunity to type II collagen

autoimmune disease with humoral autoimmunity to type II collagen
present in the sites of prediliction
present in the sites of prediliction
o concept is supported by —
o concept is supported by —
Production of lesions in the otic capsule in some
Production of lesions in the otic capsule in some
animals immunized with type II collagen
animals immunized with type II collagen

Elevated circulating antibodies to type II collagen in

Elevated circulating antibodies to type II collagen in
the blood of some patients with otosclerosis
the blood of some patients with otosclerosis
o criticised due to —
o criticised due to —
concurrent joint lesion
concurrent joint lesion
relapsing polychondritis
relapsing polychondritis

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CLINICAL FEATURES
CLINICAL FEATURES
Symptoms:
Symptoms:
o Hearing Loss
o Hearing Loss
Progressive over month to years
Progressive over month to years
Usually bilateral ( unilateral in 15% cases)
Usually bilateral ( unilateral in 15% cases)
Paracusis Willisii (20-78% of patients)
Paracusis Willisii (20-78% of patients)
Patient talks in low voice
Patient talks in low voice
o Tinnitus
o Tinnitus
Occurs in 37-78% of patients
Occurs in 37-78% of patients
Present in the early phase
Present in the early phase
Probably due to abnormal vascularity
Probably due to abnormal vascularity
Pulsetile / roaring / hissing
Pulsetile / roaring / hissing

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o Vestibular Symptoms (10-30%)
o Vestibular Symptoms (10-30%)
Transient attack vertigo are not uncommon
Transient attack vertigo are not uncommon
Due to action of toxic enzymes released by the lesion into
Due to action of toxic enzymes released by the lesion into
the
the
vestibular labyrinth leads to degeneration of SCARPA’S
vestibular labyrinth leads to degeneration of SCARPA’S
GANGLION
GANGLION
Co-existing Meniere's disease
Co-existing Meniere's disease

Dizziness
Dizziness

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Signs:
Signs:
o Otoscopy
o Otoscopy
Normal TM in 90% of cases
Normal TM in 90% of cases
Schwartz’s sign ( Flemingo's flush ) in 10% cases
Schwartz’s sign ( Flemingo's flush ) in 10% cases
o Pneumatic otoscopy
o Pneumatic otoscopy
o To rule out malleus fixation
o To rule out malleus fixation

o Tuning fork test

o Tuning fork test
Initially conductive hearing loss
Initially conductive hearing loss
Gradually becomes mixed or sensorineural
Gradually becomes mixed or sensorineural
Initially at low frequency; later, at high frequency
Initially at low frequency; later, at high frequency

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 Pathology Of Conductive Hearing Impairment:
 Pathology Of Conductive Hearing Impairment:

Appeared to be caused primarily by, narrowing and impairment

Appeared to be caused primarily by, narrowing and impairment
of the annular ligament, especially at the posterior
of the annular ligament, especially at the posterior
stapediovestibular joint space.
stapediovestibular joint space.

The size of the air-bone gap appeared to be determined by the

The size of the air-bone gap appeared to be determined by the
extent and degree of narrowing of the posterior stapediovestibular
extent and degree of narrowing of the posterior stapediovestibular
joint space.
joint space.

bony ankylosis of stepes footplate > AB gap 30 db or more but

bony ankylosis of stepes footplate > AB gap 30 db or more but
not proportional quantitatively
not proportional quantitatively

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 Pathology Of Sensorineural Hearing Impairment:
 Pathology Of Sensorineural Hearing Impairment:

Appears to occur when an otosclerotic focus reaches the

Appears to occur when an otosclerotic focus reaches the
endosteum of the cochlea.
endosteum of the cochlea.
'Hyalinization' of the spiral ligament by releasing cytokines
'Hyalinization' of the spiral ligament by releasing cytokines

 Pathology of vestibular symptoms:

 Pathology of vestibular symptoms:
Damage of the vestibular end organs by soluble toxic
Damage of the vestibular end organs by soluble toxic
substances
substances
liberated by otosclerotic bone
liberated by otosclerotic bone
or
or
Changes in biochemistry of the inner ear fluids
Changes in biochemistry of the inner ear fluids
or
or
Both
Both

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DIAGNOSIS
DIAGNOSIS

Otosclerosis is the presumptive diagnosis of a conductive hearing

Otosclerosis is the presumptive diagnosis of a conductive hearing
impairment with normal mobile tympanic membrane.
impairment with normal mobile tympanic membrane.

The 'gold' reference standard of diagnosis is surgery.

The 'gold' reference standard of diagnosis is surgery.

Histological confirmation of otosclerosis is more readily obtained if

Histological confirmation of otosclerosis is more readily obtained if
a total stapedectomy is performed; now that stapedotomy is the
a total stapedectomy is performed; now that stapedotomy is the
favoured surgical procedure, so it is usually valueless.
favoured surgical procedure, so it is usually valueless.

However the diagnosis can be suggested by some investigations

However the diagnosis can be suggested by some investigations

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INVESTIGATIONS
 Pure Tone Audiometry:
A low frequency conductive hearing loss in the early stages

Once the hearing loss reaches the 25 dB stage the patient

demonstrates a conductive hearing loss at all frequencies with a dip
at 2 kHz known as Carhart’s notch

Carhart’s notch-
depression of bone conduction thresholds of approximately 5 dB
at 500 Hz, 10 dB at 1 kHz, 15 dB at 2 kHz, and 5 dB at 4 kHz
mechanical artifact and not truly represent cochlear reserve
Carhart’s effect-

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In late stage mixed type and SN hearing loss occurs

Cookie bite pattern-

mixed type of hearing loss, greatest at mid-frequency and
better hering in low and high frequency

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 Impedance Audiometry:
 Impedance Audiometry:
Static Compliance –
Static Compliance –
Normal static compliance values fall in the range of 0.3 to
Normal static compliance values fall in the range of 0.3 to
1.6 cc. Values < 0.3 cc are indicative of stiffness in the conductive
1.6 cc. Values < 0.3 cc are indicative of stiffness in the conductive
mechanism. Typical ‘As’ type of curve is less common.
mechanism. Typical ‘As’ type of curve is less common.
Acoustic Reflexes –
Acoustic Reflexes –
In fixed stapes – absent reflex
In fixed stapes – absent reflex
Still mobile stapes- diphasic pattern
Still mobile stapes- diphasic pattern

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 Speech Audiometry:
 Speech Audiometry:
Usually speech discrimination score (SDS) is excellent.
Usually speech discrimination score (SDS) is excellent.

 Otoacoustic Emissions:
 Otoacoustic Emissions:
OAEs are not present in otosclerosis.
OAEs are not present in otosclerosis.

 Radiology:
 Radiology:
CT scan / MRI
CT scan / MRI

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HRCT scan
HRCT scan
Active otosclerotic foci were more likely to be identified than inactive
Active otosclerotic foci were more likely to be identified than inactive
foci
foci
Active foci is perceived as “hypodense” area (sensitivity 94%)
Active foci is perceived as “hypodense” area (sensitivity 94%)

Coronal cuts are better for oval window

Coronal cuts are better for oval window
Axial cuts are better for round winow
Axial cuts are better for round winow

Typical of cochlear otosclerosis is the formation of a double ring

Typical of cochlear otosclerosis is the formation of a double ring
effect due to multiple foci coalescing with each other.
effect due to multiple foci coalescing with each other.

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 Cochlear Otosclerosis.
Cochlear Otosclerosis.
The cochlea and semicircular canals are demineralized
The cochlea and semicircular canals are demineralized

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 CT Densitometry:
 CT Densitometry:
Whether the foci is active or inactive
Whether the foci is active or inactive
Response to fluride therapy
Response to fluride therapy

 Single Photon Emission Computed Tomography(SPECT)

 Single Photon Emission Computed Tomography(SPECT)
SPECT allows the detection of pathologic increases of bone
SPECT allows the detection of pathologic increases of bone
metabolism even in the absence of clinical manifestations of the
metabolism even in the absence of clinical manifestations of the
underlying process.
underlying process.

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DIFFERENTIAL DIAGNOSIS
DIFFERENTIAL DIAGNOSIS
o Ossicular discontinuity (CHL 60 dB, Ad tympanogram)
o Ossicular discontinuity (CHL 60 dB, Ad tympanogram)

o Congenital stapes fixation (other congenital anomalies, CHL non

o Congenital stapes fixation (other congenital anomalies, CHL non
progressive)
progressive)

o Malleus head fixation (other congenital anomalies, aural atresia)

o Malleus head fixation (other congenital anomalies, aural atresia)

o Paget’’s disease (diffuse involvement of bony skeleton)

o Paget’’s disease (diffuse involvement of bony skeleton)

o Osteogenesis imperfecta (blue sclera, easy fracture of other

o Osteogenesis imperfecta (blue sclera, easy fracture of other
bone)
bone)

o Superior semicircular canal dehiscence (vertigo, nystagmus with

o Superior semicircular canal dehiscence (vertigo, nystagmus with
loud noise)
loud noise)

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MANAGEMENT
MANAGEMENTOPTIONS
OPTIONS
MEDICAL MANAGEMENT
MEDICAL MANAGEMENT
Directed at the active phase of fenestral otosclerosis, presumed
Directed at the active phase of fenestral otosclerosis, presumed
cochlear otosclerosis
cochlear otosclerosis

 FLUORIDE THERAPY:
 FLUORIDE THERAPY:
Daniel first noted that there is an increase of stapedial otosclerosis
Daniel first noted that there is an increase of stapedial otosclerosis
in areas where the levels of fluoride were low
in areas where the levels of fluoride were low
Shambaugh and Scott first suggested that sodium fluoride in
Shambaugh and Scott first suggested that sodium fluoride in
moderate dosages is very effective only when the otosclerotic focus
moderate dosages is very effective only when the otosclerotic focus
is active.
is active.

Page  55
Fluorides act by:
Fluorides act by:
1. Reducing bone resorption.
1. Reducing bone resorption.
2. Increasing osteoblastic bone formation.
2. Increasing osteoblastic bone formation.
3. Fluorides prevent the release of proteolytic enzymes that are
3. Fluorides prevent the release of proteolytic enzymes that are
cytotoxic to the cochlea.
cytotoxic to the cochlea.
4. Reducing the vascularity of an active foci.
4. Reducing the vascularity of an active foci.

Indications:
Indications:
Patients with progressive sensorineural hearing loss, positive family
Patients with progressive sensorineural hearing loss, positive family
history, radiological sign suggestive of active cochlear otosclerosis.
history, radiological sign suggestive of active cochlear otosclerosis.

Stapedial otosclerosis patients who refused surgery.

Stapedial otosclerosis patients who refused surgery.

Page  56
 Administration:
Administration:
has been used as –
has been used as –
Fluoridation of drinking water
Fluoridation of drinking water
Oral fluorides
Oral fluorides

Results:
Results:
at present there is no evidence that support use of fluoride in either
at present there is no evidence that support use of fluoride in either
forms in management of otosclerosis.
forms in management of otosclerosis.

 BISPHOSPHONATES:
 BISPHOSPHONATES:

Page  57
 HEARING AIDS
HEARING AIDS

may be considered in following situations—

may be considered in following situations—

1. Cannot undergo surgery because of major systemic illnesses.

1. Cannot undergo surgery because of major systemic illnesses.
2. The only hearing ear.
2. The only hearing ear.
3. Inadequate hearing reserve
3. Inadequate hearing reserve
4. Surgery is not elected for by the patient.
4. Surgery is not elected for by the patient.

Page  58
5. Early (mild) conductive hearing loss.
5. Early (mild) conductive hearing loss.
6. During post-stapedectomy rehabilitation
6. During post-stapedectomy rehabilitation
7. As a rescue treatment many years after surgery
7. As a rescue treatment many years after surgery
8. In combination with surgery in FAO
8. In combination with surgery in FAO

BAHA
BAHA
can be a low risk alternative to stapedectomy.
can be a low risk alternative to stapedectomy.

Page  59
SURGERY:
SURGERY:
HISTORICAL PERSPECTIVE
HISTORICAL PERSPECTIVE

 Three distinct phases can be identified in the development of

 Three distinct phases can be identified in the development of
surgery for otosclerosis:
surgery for otosclerosis:
stapedectomy and mobilization of the stapes
stapedectomy and mobilization of the stapes
(in late ninteenth century)
(in late ninteenth century)
the fenestration procedures
the fenestration procedures
(early twentieth century)
(early twentieth century)
mobilization of the stapes revisited, along with stapedectomy and
mobilization of the stapes revisited, along with stapedectomy and
stapedotomy with prostheses
stapedotomy with prostheses
(in the later half of the twentieth century.)
(in the later half of the twentieth century.)

Page  60
 Stapedectomy And Mobilization Of The Stapes
Stapedectomy And Mobilization Of The Stapes
(in late ninteenth century)
(in late ninteenth century)
 Kessel (1878)
 Kessel (1878)
first described his work on the columella of pigeons and stapes of
first described his work on the columella of pigeons and stapes of
dogs and humans.
dogs and humans.
he removed the stapes footplate 'allowing a new membrane to
he removed the stapes footplate 'allowing a new membrane to
form'.
form'.

 Bucheron (1888) and Miot (1890)

 Bucheron (1888) and Miot (1890)
 Blake (1892) and Jack (1893)
 Blake (1892) and Jack (1893)
performed similar procedures and achieved improved hearing in
performed similar procedures and achieved improved hearing in
more than half of their patients.
more than half of their patients.
.
.

Page  61
But
But
the results were temporary
the results were temporary
effective in early ankylosis
effective in early ankylosis
complications were more (meningitis & death)
complications were more (meningitis & death)

In 6th thInternational Otologic Congress, London (1899) these

In 6 International Otologic Congress, London (1899) these
procedures were condemned.
procedures were condemned.

Page  62
 Fenestration Procedures
Fenestration Procedures
(early twentieth century)
(early twentieth century)
 Holmgren (1930)
 Holmgren (1930)
father of fenestration surgery
father of fenestration surgery

 Sourdille (Holmgren’s student)

 Sourdille (Holmgren’s student)
multi-stage fenestration surgery
multi-stage fenestration surgery
64% stisfactory results
64% stisfactory results

Page  63
 Julius Lempert
 Julius Lempert

popularised
popularised
the single staged
the single staged
fenestration procedure
fenestration procedure

Fenestration surgery provided significant and lasting hearing

Fenestration surgery provided significant and lasting hearing
improvement in large series of patients
improvement in large series of patients

Page  64
 Mobilization Of The Stapes Revisited
Mobilization Of The Stapes Revisited
(in the later half of the twentieth century)
(in the later half of the twentieth century)
 Samuel Rosen (1952)
 Samuel Rosen (1952)

Reintroduced stapes mobilisation,

Reintroduced stapes mobilisation,
unware of earlier work of Miot, Bucheron
unware of earlier work of Miot, Bucheron

Became accepted as the

Became accepted as the
treatment of choice for those
treatment of choice for those
with only partial ankylosis, though re-ankylosis tended to
with only partial ankylosis, though re-ankylosis tended to
develop a few years later.
develop a few years later.

Page  65
 Stapedectomy
Stapedectomy
(in the later half of the twentieth century.)
(in the later half of the twentieth century.)

 John Shea (1956)

 John Shea (1956)

Perfected stapedectomy
Perfected stapedectomy
and introduced the concept of
and introduced the concept of
ossicular continuty by a teflon prsthesis
ossicular continuty by a teflon prsthesis

Within a decade this procedure became the standard operation for

Within a decade this procedure became the standard operation for
treatment of otosclerosis
treatment of otosclerosis

Page  66
In the next three decades this procedure underwent furthur
In the next three decades this procedure underwent furthur
refinement and has been replaced (except in some selected cases)
refinement and has been replaced (except in some selected cases)
by
by
Limited Stapedectomy (only the posterior half of footplate)
Limited Stapedectomy (only the posterior half of footplate)

and more recently

and more recently

Stapedotomy
Stapedotomy

Page  67
 Best surgical candidate:
 Best surgical candidate:

 Previously un-operated ear

 Previously un-operated ear
 Good health
 Good health
 ABG of 25 dB or more
 ABG of 25 dB or more
 Negative Rinne test in 512 Hz
 Negative Rinne test in 512 Hz
 Excellent speech discrimination score
 Excellent speech discrimination score

Page  68
 Contraindication:
 Contraindication:

1. Perforation of ear drum

1. Perforation of ear drum
2. Otitis externa and otitis media
2. Otitis externa and otitis media
3. Meniere’s Disease
3. Meniere’s Disease
4. Age more than 70 yr (relative)
4. Age more than 70 yr (relative)
5. Only hearing ear (except in profound mixed hearing loss who is
5. Only hearing ear (except in profound mixed hearing loss who is
a candidate for cochlear implant)
a candidate for cochlear implant)
6. Contralateral ear has a disease which may threaten hearing in
6. Contralateral ear has a disease which may threaten hearing in
future
future
7. Those requiring intact vestibule for professional or occupational
7. Those requiring intact vestibule for professional or occupational
purpose (air travel, scuba diving, parachuting)
purpose (air travel, scuba diving, parachuting)

Page  69
 Anaesthesia:
 Anaesthesia:

Local vs General
Local vs General

Page  70
SURGICAL STEPS:

12’O clock to 6’O clock (conventional)

Incision:
1’O clock to 7’O clock (better exposure)

Page  71
Elevation Of The Flap:

Page  72
Chorda Tympani Is Dissected Free
Of Fibrous Annulus & manubrium

Flap Along With The Nerve Is

Folded Anteriorly On The Umbo

Page  73
Removal Of Scutum:

Page  74
 Thin plate of scutum is
removed by a currete

Page  75