HEARING LOSS

 Hearing Loss is when a person is not able to hear

as well as someone with normal hearing – hearing
thresholds of 20db or better in both ears
DEGREE OF HEARING LOSS

• MILD 26db-40db
• MODERATE 41db-55db
• MODERATELY SEVERE 56db-70b
• SEVERE 71db-91db
• PROFOUND >91db
CLASSIFICATION
 CONGENITAL HL
• Many hereditary conditions produce hearing loss at birth or later
due to secondary degeneration of inner ear structures.
• Mostly occur as recessive conditions and skip generations within
family

HEREDITARY CAUSES OTHER CAUSES

 Waardenburg’s syndrome  Congenital cholesteatoma

(progressive hearing loss +
colored and widest eyes)  Fixation of malleus

 Usher’s Syndrome  Fixation of Stapes footplate

(retinitis pigmentosa)
 Ossicular discontinuity
 Alport's Syndrome
(kidney disease)
Syndromes associated with hearing loss
CONDUCTIVE HL
SENSORINEURAL Hearing Loss
SENSORY DEAFNESS NEURAL DEAFNESS

 Age related HL  Acoustic Neuroma

 Acoustic trauma  Viral infections of auditory
 Viral infections of inner ear nerve
  Infections or inflammation of
Meniere’s disease
brain covering – Meningitis
 Ototoxicity
 Brain tumor
 Stroke
 FUNCTIONAL OR NONORGANIC HL

 Inability to hear because of psychological causes with no

underlying organic lesions

1. MALINGERING - conscious origin, to gain advantage

2. HYSTERICAL - unconscious origin, organ of hearing is normal

3. PSYCOSOMATIC - unconscious origin, there may be an organic

change in auditory apparatus resulting from mental causes
OTHER FORMS OF HL
 ASSESMENT OF HEARING LOSS

TUNING FORK TESTS

Rinne's
Weber's
Absolute Bone Conduction
 Absolute Bone Conduction

Intepretation

Normal - Conductive HL

Short - SNHL
 PURE TONE AUDIOMETRY

 Used to asses the hearing threshold levels of an individual,

Enabling determination of the degree,
type and configuration of hearing loss

NORMAL
• both air and bone conduction
will be superimposed at each test
frequency between 0 to 10db

• AC threshold not > 25 dB

BC threshold not > 25 dB
 Conductive HL

• AC threshold > 25 dB
• BC threshold < 25 dB
• A-B gap > 10 dB
• Maximal CHL is 60 dB
 SNHL
• AC threshold > 25 dB
• BC threshold > 25 dB
• A-B gap < 10 dB
• AC and BC threshold
below normal and similar
• No ABG
 Mixed HL

• AC threshold >25dB
• BC threshold > 25 dB
• A-B gap > 10 dB
 IMPEDENCE AUDIOMETRY
• Tympanometry is a test of middle ear functioning.

• It looks at the flexibility (compliance) of the eardrum to

changing air pressures, indicating how effectively
sound is transmitted into the middle ear.

• This objective test also allows us to view the functioning

of the Eustachian Tube, the upper auditory pathways
and the reflex contraction from the middle ear muscles.

• Impedance testing is crucial in distinguishing a

conductive loss from a sensorineural hearing loss.
 A Type

• Suggests normal
middle ear functioning

• Peak is
between +/- 100 daPa

• Compliance from
0.3-1.5 ml
 AD Type

• highly compliant
middle ear system

• Middle ear pressure

Normal

• Compliance is increased
more than 1.5 ml

• Seen in : lax or thin TM

Ossicular discontinuity
 AS Type

• less compliant
middle ear system

• Middle ear pressure

Normal

• Compliance is reduced
less than 0.3 ml

• Seen in ossicular fixation

Otosclerosis , tympanoscelrosis
 B Type
• Minimal or no movement of
Tympanic membrane at all, hence
there is no identifiable peak

• Normal ear canal volume

• Seen in
middle ear involvement
from fluid (middle ear effusion)
Perforated TM
Occluded ear canal
 TYPE C

Suggests Eustachian Tube dysfunction

(often seen just before or after effusion)

• Middle ear pressure

Negative

• Compliance from 0.3-1.5 ml

• Seen in
Eustachian tube dysfunction
 BERA
(Brain Stem Evoked Response
Audiometry)
• Hearing examination performed on children aged 1 to
3 years.

• Objective test to understand the transmission of

electrical waves from the VIII cranial nerve to the
brainstem, in response to click sounds given through
the ear.

• BERA is generally used to identify any pathology in the

vestibulocochlear nerve or the brainstem.

• The test is recommended for infants who are at a high

risk for hearing loss and in whom conventional
audiometry cannot be performed
wave form
 Interpretation

Following parameters are measured:

• Amplitude of the wave – indicative of the number of
neurons firing
• Latency – speed of transmission
• Time interval between peaks
• The difference in wave V latency of one ear compared to
the other.

 Peaks I , III and V most useful

 Find out interpeak latencies of I-III , III-V and I-V
 Disorders will produce delays
 Bera - interpretation

• Wave I : delayed/absent indicate cochlear lesion

• Wave V : delayed/absent indicate upper brainstem

lesion

• Wave I-III : prolongation may indicate lower brainstem

lesion

• Wave III-V : prolongation may indicate upper

brainstem lesion

• Wave I-V : prolongation may indicate whole brainstem

lesion
 OAE
(Oto Acoustic Emission)
• Are actually soft sounds generated by the movement of structures
(outer hair cells) in the cochlea

• Stimulation is sent in through middle ear, emission occurs within

cochlea, sound then must travel BACK OUT through the middle
ear, external ear and be recorded by microphone of the device
 OAE - USE

• Sensitive measure of outer hair cell function

• Early identification and diagnosis of auditory dysfunction in

pediatric and adult

• Screening in new born

• Confirm soundfield results in toddlers

• In adults to confirm malingering

 MANAGEMENT OF HEARING LOSS
-CONDUCTIVE HL
• Removal of canal obstructions, e.g. impacted wax, foreign body,
osteoma or exostosis, keratotic mass, benign or malignant
tumors, or meatal atresia.

• Removal of fluid. Myringotomy with or without grommet

insertion.

• Removal of mass from middle ear. Tympanotomy and removal

of small middle ear tumors or cholesteatoma behind intact
tympanic membrane.
 MANAGEMENT OF HEARING LOSS
-CONDUCTIVE HL
• Stapedectomy, as in osteosclerotic fixation of stapes footplate.

• Tympanoplasty. Repair of perforation, ossicular chain or both.

• Hearing aid. In cases, where surgery is not possible, refused or

has failed
 MANAGEMENT OF HEARING LOSS
-SNHL
• Early detection of SNHL is important as measures can be taken to
stop its progress, reverse it or to start an early rehabilitation
programme, so essential for communication.

• Syphilis of the inner ear is treatable with high doses of penicillin

and steroids with improvement in hearing.

• Serous labyrinthitis can be reversed by attention to middle ear

infection.

• Early management of Meniere's disease can prevent further

episodes of vertigo and hearing loss.
MANAGEMENT OF HEARING LOSS
-SNHL

• Ototoxic drugs should be used with care and discontinued if

causing hearing loss. In many such cases, it may be possible to
regain hearing, total or partial, if the drug is stopped.

• Noise- induced hearing loss can be prevented from further

deterioration if the person is removed from the noisy
surroundings.
 REHABILITATION OF IMPAIRED HEARING
- Hearing Aids
CONVENTIONAL HA

• A hearing aid is a device to amplify sounds reaching the ear.

Essentially, it consists of three parts:
(i) a microphone, which picks up sounds and converts them into
electrical impulses

(ii) an amplifier, which magnifies electrical impulses and

(iii) a receiver, which converts electrical impulses back to sound.

This amplified sound is then carried through the earmould to the
tympanic membrane.
 Hearing Aids
Types
• Air conduction hearing aid- In In this, the amplified sound
is transmitted via the ear canal to the tympanic membrane.

• Bone conduction hearing aid- Instead of a receiver, it has

a bone vibrator which snugly fits on the mastoid and directly
stimulates the cochlea.
In people with actively draining ears, otitis externa or atresia of
the ear canal when ear inserts cannot be worn.
 TYPES OF HEARING AIDS

•
 INDICATIONS OF HEARING AIDS
• Any individual who has a hearing problem that cannot be helped
by medical or surgical means is a candidate for hearing aid.

1. Sensorineural hearing loss - which interferes with day-to- day

activities of a person.

2. Deaf children should be fitted with hearing aid as early as

possible for development of speech and learning. In severely
deaf children, binaural aids.

3. Conductive deafness - Most of such persons can be helped by

surgery but refused or not feasible or has failed.
 HEARING AIDS
- IMPLANTABLE
BONE ANCHORED HEARING AID
• Based on the principle of bone conduction.

• It is primarily suited to people who have conductive hearing

loss, unilateral hearing loss and those with mixed hearing loss
who can- not otherwise wear “in the ear” or “behind the ear”
hearing aids.

• Bone-anchored hearing aids use a surgically implanted

abutment to transmit sound by direct conduction through bone
to the cochlea, bypassing the external auditory canal and
middle ear
• COMPONENTS
(i) titanium fixture

(ii) titanium abutment

(iii) sound processor

 INDICATIONS OF BAHA

• Candidacy profile.
1. People who have chronic inflammation or infection of
the ear canal and cannot wear standard “in the ear” air-
conduction hearing aids.

2. Children with malformed or absent outer ear and ear

canals as in microtia or canal atresia.

3. Single-sided deafness
 COCHLEAR IMPLANT
• Electronic device that can provide useful hearing and improved
communication abilities for persons who have severe to
profound sensorineural hearing loss and who cannot benefit
from hearing aids.

• A cochlear implant works by producing meaningful electrical

stimulation of the auditory nerve where degeneration of the hair
cells in the cochlea has progressed to a point such that
amplification provided by hearing aids is no longer effective.
• Sound is picked up by the microphone in the speech processor. The speech processor analyses and
codes sounds into electrical pulses.
• The processor uses a variety of coding strategies and electrical impulses are sent from the processor to
the transmitting coil which in turn sends the signal to the surgically implanted receiver/stimulator via
radiofrequency.
• The receiver/stimulator decodes the signal and transmits it to the electrode array
• 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 sends these electrical pulses to the brain which are finally
interpreted as sound.
 Candidacy for Cochlear Implant
Cochlear implants may be used both in children and adults.
The following is the criteria:
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

Postoperative Mapping (Programming)
of Device and Habilitation
• Activation of the implant is done 3–4 weeks after implantation.

• Following this the implant is “programmed” or “mapped.”

• Re Habilitation is an essential part for those who have

undergone cochlear implantation.

• All patients need auditory- verbal therapy.

Emphasis is laid on making the child listen and speak like a normal
person rather than use lip reading and visual cues.
 AUDITORY BRAINSTEM IMPLANT
• This implant is designed to stimulate the cochlear nuclear
complex in the brainstem directly by placing the implant in the
lateral recess of the fourth ventricle.
• Such an implant is needed when CN VIII has been severed in
surgery of vestibular schwannoma
• Receiver/stimulator has a removable magnet so that MRI can
be safely performed in such cases if need arises.
 NOISE INDUCED HEARING LOSS
• Hearing loss, in this case, follows chronic exposure to less
intense sounds than seen in acoustic trauma and is mainly a
hazard of noisy occupations.

• (a) Temporary threshold shift (TTS). The hearing is impaired

immediately after exposure to noise but recovers after an
interval of a few minutes to a few hours even up to 2 weeks.

• (b) Permanent threshold shift (PTS). The hearing impairment is

permanent and does not recover at all.
 NOISE INDUCED HEARING LOSS
• The damage caused by noise trauma depends on several
factors:
(i) Frequency of noise. A frequency of 2000–3000 Hz causes more
damage than lower or higher frequencies.

ii) Intensity and duration of noise. As the intensity increases, permissible

time for exposure is reduced.

(iii) Continuous vs interrupted noise. Continuous noise is more harmful.

(iv) Susceptibility of the individual. Degree of TTS and PTS varies in

different individuals.

(v) Pre-existing ear disease

• Ministry of Labour, Govt. of India, Model Rules under Factories
Act
 A noise of 90 dB (A) SPL, 8 h a day for 5 days per week is the
maximum safe limit

 No exposure in excess of 115 dB (A) is to be permitted.

 No impulse noise of intensity greater than 140 dB (A) is

permitted.
 NIHL DIAGNOSIS
• The audiogram in NIHL shows a typical notch, at 4 kHz, both for
air and bone conduction
At this stage, patient complains of high-pitched tinnitus and
difficulty in hearing in noisy surroundings but no difficulty in day-to-
day hearing.

• As the duration of noise exposure

increases, the notch deepens
and also widens to involve
lower and higher frequencies.
 NIHL - MANGEMENT

• PREVENTABLE
 pre-employment and then annual audiograms for early
detection.
 Ear protectors (ear plugs or ear muffs) should be used where
noise levels exceed 85 dB

• If hearing impairment has already occurred:

 Hearing Aids
 Tinnitus retraining therapy
 Pursue Claim – worked >10years in prescribed occupation
 SUDDEN SENSORINEURAL HL

• Defined as 30 dB or more of SNHL over at least three

contiguous frequencies occurring within a period of 3 days or
less.

• Mostly it is unilateral.

• It may be accompanied by tinnitus or temporary spell of

vertigo.
 SUDDEN SENSORINEURAL HL
• idiopathic variety.

• Other etiological factors

1. Infections.
Mumps, herpes zoster, meningitis, encephalitis, syphilis, otitis
media.

2. Trauma.
Head injury, ear operations, noise trauma, baro-trauma

3. Vascular.
Hemorrhage (leukemia), embolism or thrombosis of labyrinthine or
cochlear artery or their vasospasm.
They may be associated with diabetes, hypertension,
polycythemia, macro globin anemia or sickle cell trait.
 SUDDEN SENSORINEURAL HL

4. Ear (otologic).
Ménière’s disease, Cogan’s syndrome, large vestibular aqueduct.

5. Toxic.
Ototoxic drugs, insecticides.

6. Neoplastic.
Acoustic neuroma. Metastases in cerebello-pontine angle,
carcinomatous neuropathy.

7.Miscellaneous.
Multiple sclerosis, hypothyroidism,sarcoidosis.

8. Psychogenic.
 SSNHL - MANAGEMENT
1. Bed rest.
2. Steroid therapy - Prednisolone 40–60 mg in a single
morning dose for 1 week and then tailed off in a period of 3 weeks.
Steroids are anti-inflammatory and relieve oedema.

3. Inhalation of carbogen (5% CO2 + 95% O2) - It increases

cochlear blood flow and improves oxygenation.

4. Vasodilator drugs.
5. Low molecular weight dextran - decreases blood viscosity.

6. Hyperbaric oxygen therapy hyperbaric oxygen raises

concentration of oxygen in labyrinthine fluids and improves
cochlear function
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