HEARING LOSS

Classification
CONDUCTIVE HEARING LOSS AND
ITS MANAGEMENT
Any disease process which interferes with the conduction of sound to reach cochlea causes
conductive hearing loss.
The lesion may lie in the external ear and tympanic mem-brane, middle ear or ossicles up to
stapediovestibular joint.
The characteristics of conductive hearing loss are:
1. Negative Rinne test, i.e. BC > AC.
2. Weber lateralized to poorer ear.
3. Normal absolute bone conduction.
4. Low frequencies affected more.
5. Audiometry shows bone conduction better than air conduction with air-bone gap. Greater the air-
bone gap, more is the conductive loss.
6. Loss is not more than 60 dB.
7. Speech discrimination is good.
Aetiology

Congenital and acquired

AVERAGE HEARING LOSS SEEN IN
DIFFERENT LESIONS OF
CONDUCTIVE APPARATUS
Management
Tympanoplasty
• It is an operation to
• (i) eradicate disease in the middle ear and
• (ii) to reconstruct hearing mechanism. It may be combined with
mastoidectomy if disease process so demands.
• Type of middle ear reconstruction depends on the damage present in the
ear. The procedure may be limited only to repair of tympanic membrane
(myringoplasty), or to reconstruction of ossicular chain (ossiculoplasty), or
both (tympanoplasty).
• Reconstructive surgery of the ear has been greatly facilitated by
development of operating microscope, microsurgical instruments and
biocompat-ible implant materials.
Types of tympanoplasty
Myringoplasty
• . It is repair of tympanic membrane.
1. Graft materials of choice are temporalis fascia or the perichondrium taken
from the patient.
2. Sometimes, homografts such as dura, vein, fascia or cadaver tympanic
membrane are also used.
3. Repair can be done by two techniques—the underlay or the overlay.
4. In the underlay technique, margins of perforation are freshened and the graft
placed medial to perforation or tympanic annulus, if large, and is supported by
gelfoam in the middle ear.
5. In the overlay technique, the graft is placed lateral to fibrous layer of the
tympanic membrane after carefully removing all squamous epithelium from the
lateral surface of tympanic membrane remnant.
Ossicular reconstruction.
• Ossicles are essential for transmission of sound from tympanic
membrane to labyrinth.
• Several types of prosthesis are available to replace ossicles
depending on the ossicular defects .
• Autograft ossicles can be sculptured to bridge the gap. Homograft
preserved ossicles with or without tympanic membrane have been
used but are difficult to procure and have danger of transmission of
disease.
Types of prosthesis
SENSORINEURAL HEARING LOSS
AND ITS MANAGEMENT
• Sensorineural hearing loss (SNHL) results from lesions of the cochlea, VIIIth nerve or central
auditory pathways. It may be present at birth (congenital) or start later in life (acquired).
• The characteristics of sensorineural hearing loss are:
• 1. A positive Rinne test, i.e. AC > BC.
• 2. Weber lateralized to better ear.
• 3. Bone conduction reduced on Schwabach and absolute bone conduction tests.
• 4. More often involving high frequencies.
• 5. No gap between air and bone conduction curve on audiometry.
• 6. Loss may exceed 60 dB.

• 7. Speech discrimination is poor.

• 8. There is difficulty in hearing in the presence of noise
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
• Acquired It appears later in life.
• The cause may be genetic or nongenetic.
• The genetic hearing loss may manifest late (delayed onset) and may affect only the hearing, or be a part of a larger syndrome affecting
other systems of the body as well (syndromal).

Common causes of acquired SNHL include:

1. Infections of labyrinth—viral, bacterial or spirochaetal
2. Trauma to labyrinth or VIIIth nerve, e.g. fractures of temporal bone or concussion of the labyrinth or the ear surgery
3. Noise-induced hearing loss
4. 4.3 Ototoxic drugs
5. 5. Presbycusis
6. 6. Ménière’s disease
7. 7. Acoustic neuroma
8. 8. Sudden hearing loss
9. 9. Familial progressive SNHL 10. Systemic disorders, e.g. diabetes, hypothyroidism, kidney disease, autoimmune disorders, multiple
sclerosis, blood dyscrasias.
 DIAGNOSIS
• 1. history.
• It is important to know whether disease is congenital or acquired,
stationary or progressive, associated with other syndromes or not,
involvement of other members of the family and possible aetiologic
factors.medicine.

• 2. seVerity oF deaFness (mild, moderate, moderately seVere,

seVere, pr,oFound or total). This can be found out on audiometry.
• 3. Type oF audiogram.
• Whether loss is high frequency, low frequency, mid-frequency or flat
type.
• 4. site oF lesion. i.e. cochlear, retrocochlear or central.
• 5. laboratory tests. They depend on the aetiology suspected, e.g. X-
rays or CT scan of temporal bone for evidence of bone destruction
(congenital cholesteatoma, glomus tumour, middle ear malignancy
or acoustic neuroma), blood counts (leukaemia), blood sugar
(diabetes), serology for syphilis, thyroid functions (hypothyroidism),
kidney function tests, etc.
Management
• 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.
• Hearing loss of hypothyroidism can be reversed with replacement therapy.
• Serous labyrinthitis can be reversed by attention to middle ear infection.
• Early management of Ménière’s disease 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.
• 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.
SPECIFIC FORMS OF HEARING LOSS
• 1. INFLAMMATION OF LABYRINTH

• Viral labyrinthitis.
• Viruses usually reach the inner ear by blood stream affecting stria
vascularis and then the endolymph and organ of Corti. Measles,
mumps and cytomegaloviruses are well-documented to cause
labyrinthitis.
• Several other viruses, e.g. rubella, herpes zoster, herpes simplex,
influenza and Epstein–Barr are clinically known to cause deafness
but direct proof of their invasion of labyrinth is lacking.
• Bacterial.
• Bacterial infections reach labyrinth through the middle ear
(tympanogenic) or through CSF (meningogenic).
• Sensorineural hearing loss following meningitis is a well-known
clinical entity.
• Bacteria can invade the labyrinth along nerves, vessels, cochlear
aqueduct or the endolymphatic sac. Membranous labyrinth is totally
destroyed
• syphilitic.
• Sensorineural hearing loss is caused both by congenital and acquired syphilis.
Congenital syphilis is of two types: the early form, manifesting at the age of 2 or
the late form, manifesting at the age of 8–20 years. Syphilitic involvement of the
inner ear can cause:
• A) Sudden sensorineural hearing loss, which may be unilateral or bilateral. The latter
is usually symmetrical in high frequencies or is a flat type.
• (b) Ménière’s syndrome with episodic vertigo, fluctuating hearing loss, tinnitus and
aural fullness—a picture simulating Ménière’s disease.
• © Hennebert’s sign. A positive fistula sign in the absence of a fistula. This is due to
fibrous adhesions between the stapes footplate and the membranous labyrinth.
• (d) Tullio phenomenon in which loud sounds produce vertigo.
• Diagnosis of otosyphilis can be made by other clinical evidence of
late acquired or congenital syphilis (interstitial keratitis, Hutchinson’s
teeth, saddle nose, nasal septal perforation and frontal bossing) and
the laboratory tests. Fluorescent treponema-absorption test (FTA-
ABS) and venereal disease research laboratory (VDRL) or rapid plasma
reagin (RPR) tests from CSF are useful to establish the diagnosis.
• Treatment of otosyphilis includes i.v. penicillin and steroids.
B. FAMILIAL PROGRESSIVE
SENSORINEURAL HEARING LOSS
• C. OTOTOXICITY
• 1. Aminoglycoside antibiotics
• Streptomycin, gentamicin and tobramycin are primarily vestibulotoxic.
They selectively destroy type I hair cells of the crista ampullaris but,
administered in large doses, can also damage the cochlea.
• Neomycin, kanamycin, amikacin, sisomycin and dihydrostreptomycin
are cochleotoxic. They cause selective destruction of outer hair
cells, starting at the basal coil and progressing onto the apex of
cochlea.
• Patients particularly at risk are those:
• (a) having impaired renal function,
• (b) elderly people above the age of 65,
• (c) concomitantly receiving other ototoxic drugs,
• (d) who have already received aminoglycoside antibiotics,
• (e) who are receiving high doses of ototoxic drugs with high serum
level of drug, and
• (f) who have genetic susceptibility to aminoglycosides. Here the
antibiotic binds to the ribosome and interferes with protein synthesis,
thus causing death of the cochlear cells.
• . 2. diuretics.
• Furosemide, bumetanide and ethacrynic acid are called loop
diuretics as they block transport of sodium and chloride ions in the
ascending loop of Henle.
• They are known to cause oedema and cystic changes in the stria
vascularis of the cochlear duct.
• In most cases, the effect is reversible but permanent damage may
occur. Hearing loss may be bilateral and symmetrical or sometime
sudden in onset.
• 3.salicylates.
• Symptoms of salicylate ototoxicity are tinnitus and bilateral
sensorineural hearing loss particularly affecting higher frequencies.
Site of lesion testing indicates cochlear involvement, but light and
electron microscopy have failed to show any morphologic changes in
the hair cells. Possibly they interfere at enzymatic level.
• Hearing loss due to salicylates is reversible after the drug is
discontinued. SNHL has also been noted with other NSAIDs, e.g.
naproxen, piroxicam and ketorolac but is reversible.
• 4.quinine.
• Ototoxic symptoms due to quinine are tinnitus and sensorineural
hearing loss, both of which are reversible. Higher doses may cause
permanent loss.
• The symptoms generally appear with prolonged medication but may
occur with smaller doses in those who are susceptible. Congenital
deafness and hypoplasia of cochlea have been reported in children
whose mothers received this drug during the first trimester of
pregnancy.
• Ototoxic effects of quinine are due to vasoconstriction in the small
vessels of the cochlea and stria vascularis.
• 5. chloroquine and hydroxychloroquine. Effect is similar to that of
quinine and cause reversible SNHL. Sometimes permanent deafness
can result.

• 6. cytotoxic drugs. Nitrogen mustard, cisplatin and carboplatin can

cause cochlear damage. They affect the outer hair cells of the cochlea
• deFeroxamine (desFerrioxamine).
• IT is an iron chelating substance used in the treatment of
thalassaemic patients who receive repeated blood transfusions and in
turn have high iron load.
• Like cisplatin and aminoglycosides, deferoxamine also causes high-
frequency sensorineural hearing loss. Onset of hearing loss is
sudden or delayed. It is permanent but in some cases it can be
reversible when the drug is discontinued. It causes toxicity to
nerves; children are affected more.
NOISE TRAUMA
• 1. acoustic trauma.
• Permanent damage to hearing can be caused by a single brief exposure to
very intense sound without this being preceded by a temporary threshold shift.
• Also called impulse noise, such noise can arise from an explosion, gun fire or a
powerful cracker and may reach or cross 140 dB. Noise level of a gun or rifle
may reach 140–170 dB SPL (sound pressure level).
• Such brief and loud noises mechanically damage organ of Corti, tear Reissner’s
membrane, rupture hair cells and allowing mixing of perilymph and endolymph.
• A severe blast, in addition, may concomitantly damage the tympanic membrane
and disrupt ossicles further adding conductive loss. Impulse noise may be as
brief as 0.2 ms. No impulse noise more than 140 dB (A) is permitted.
• 2. noise-induced hearing loss (nihl).
• 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.
• Amount of TTS depends on the noise—its intensity, frequency and
duration.
• (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.
• 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.
• Nonauditory eFFects oF noise.
• Apart from hear-ing loss, noise can affect other systems of the body. It
interferes with rest and sleep causing chronic fatigue and stress.
Through activation of the autonomic nervous system and pituitary–
adrenal axis, it causes annoyance and irritability.
• Hypertension and peptic ulcer have also been attributed to it. It also
adversely affects task performance where communication through
speech is required. Laryngeal problems have been noticed in
workers who have to speak loudly in persistently noisy surroundings.
E. AUTOIMMUNE (IMMUNE-
MEDIATED) INNER EAR DISEASE
• Immune-mediated inner ear disease (Syn. autoimmune SNHL) causes progressive
bilateral sensorineural hearing loss.
• It occurs between 40 and 50 years with equal incidence in both sexes. Nearly
50% of patients also experience vestibular symptoms like disequilibrium, motion
intolerance, positional or episodic vertigo.
• About 15% of patients have evidence of other autoimmune disorder such as
ulcerative colitis, systemic lupus, rheumatoid arthritis or multiple sclerosis.
• Moscicki et al. defined the condition as: ‘Bilateral SNHL ≥ 30 dB at any
frequency and evidence of progression in at least one ear on two serial
audiograms that are done at equal to or less than 3 months apart. Progression is
defined as threshold shift of ≥ 15 dB at one frequency or 10 dB at two or
more consecutive frequencies or significant change in speech discrimination’.
Investigations
• 1. Audiogram. To establish above criteria, repeated audiograms can be taken at
one month intervals. Audiogram may show loss at high and low frequencies.
• 2. Speech audiogram. Speech discrimination is affected though threshold of pure
tones remains the same.
• 3. Evoked response audiometry. To exclude acoustic neuroma or multiple sclerosis.
• 4. Contrast-enhanced MRI.
• 5. Blood tests to exclude systemic autoimmune disorders. Total and differential
counts, ESR, rheumatoid factor, antinuclear antibodies, C3 and C4 compliment
levels, Raji cell assay for circulating immune complexes. 6. Western blot essay for
anti-Hsp 70 (anti-heat shock protein 70) antibodies. Antigen used in this test is
crude protein extract from bovine renal cells. It is not a specific test for diagnosis
but correlates to both active disease and steroid responsiveness.
Treatment
• Prednisolone 1 mg/kg/day up to a total of 60 mg/day (for adults) for 4 weeks.
Sometimes response is late.
• If no response is seen in 4 weeks, steroid is tapered off in 12 days. Responders
continue till a plateau is reached and then continue on maintenance dose of
10–20 mg every other day for about 6 months.
• Side effects and risks of longterm steroid therapy should be kept in mind. Those
who cannot take steroids can be given methotrexate 15 mg/week for 6–8
weeks and if the patient responds, continue it for 6 months.
• If no response is obtained for 6–8 weeks trial, drug is discontinued. Alternative
to methotrexate is cyclophosphamide but it is more toxic. Other treatments
include intratympanic steroid injection, systemic IgG injection and
plasmapheresis.
Sudden hearing loss
Aetiology
Most often the cause of sudden deafness remains obscure, in which case it is called the idiopathic variety. In such
cases, three aetiological factors are considered—viral, vascular or the rupture of cochlear membranes.
Spontaneous perilymph fistulae may form in the oval or round window. Other aetiological factors which cause
sudden deafness and must be excluded are listed below. Remember the mnemonic “In The Very Ear Too No Major
Pathology.”
1. Infections. Mumps, herpes zoster, meningitis, encephalitis, syphilis, otitis media.
2. Trauma. Head injury, ear operations, noise trauma, barotrauma, spontaneous rupture of cochlear membranes.
3. Vascular. Haemorrhage (leukaemia), embolism or thrombosis of labyrinthine or cochlear artery or their
vasospasm. They may be associated with diabetes, hypertension, polycythaemia, macroglobinaemia or sickle cell
trait.
4. Ear (otologic). Ménière’s disease, Cogan’s syndrome, large vestibular aqueduct.
5. Toxic. Ototoxic drugs, insecticides.
6. Neoplastic. Acoustic neuroma. Metastases in cerebellopontine angle, carcinomatous neuropathy
7. Miscellaneous. Multiple sclerosis, hypothyroidism, sarcoidosis. 8. Psychogenic.
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. They have been found useful in idiopathic sudden hearing loss of
moderate degree.
• 3. Inhalation of carbogen (5% CO2 + 95% O2). It increases cochlear blood flow and
improves oxygenation.
• 4. Vasodilator drugs.
• 5. Low molecular weight dextran. It decreases blood viscosity. It is contraindicated
in cardiac failure and bleeding disorders.
• 6. Hyperbaric oxygen therapy. Available only in selected centres, hyperbaric oxygen
raises concentration of oxygen in labyrinthine fluids and improves cochlear function
• 7. Low-salt diet and a diuretic. It is empirical and has same benefit
as in cases of Ménière’s disease.

• 8. Intratympanic steroids therapy. It raises the local concentration

of steroids in cochlear fluids, thus avoiding side effects of systemic
therapy.
Treatment
• Many treatment protocols have been suggested for idiopathic sensorineural
sudden hearing loss but none has shown significant benefit over the
benefit of spontaneous recovery which occurs in 50–60% cases within first 2
weeks.
• None of the drugs, dextran 40, vasodilators, carbogen inhalation (5% CO2
with 95% O2), diatrizoate meglumine, have shown significant benefit.
Generally prescribed medicines include:
• 1. Steroids.
• 2. Inhalation of carbogen.
• 3. Low-salt diet and a diuretic.
• 4. Hyperbaric oxygen.
PRESBYCUSIS
• 1. sensory.
• This is characterized by degeneration of the organ of Corti, starting at
the basal coil and progressing gradually to the apex. Higher
frequencies are affected but speech discrimination remains good.
• 2. neural.
• This is characterized by degeneration of the cells of spiral ganglion,
starting at the basal coil and progressing to the apex. Neurons of
higher auditory pathways may also be affected. This manifests
with high tone loss but speech discrimination is poor and out of
proportion to the pure tone loss.
• 3. strial or metabolic.
• hiss is characterized by atrophy of stria vascularis in all turns of cochlea. In this, the physical and
chemical processes of energy production are affected. It runs in families. Audiogram is flat but
speech discrimination is good.

• 4. cochlear conductiVe.
• This is due to stiffening of the basilar membrane thus affecting its movements. Audiogram is sloping type.
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 phenomenon is positive and all the sounds suddenly become intolerable when volume is
raised. Tinnitus is another bothersome problem and in some it is the only complaint. Patients of
presbycusis can be helped by a hearing aid.
• They should also have lessons in speech reading through visual cues. Curtailment of smoking and
stimulants like tea and coffee may help to decrease tinnitus.
NONORGANIC HEARING LOSS
(NOHL)
• In this type of hearing loss, there is no organic lesion. It is either due to
malingering or is psychogenic.
• 1. high index oF suspicion.
• Suspicion further rises when the patient makes exaggerated efforts to hear,
frequently making requests to repeat the question or placing a cupped
hand to the ear.

• 2. inconsistent results on repeat pure tone and speech audiometry

tests.
• Normally, the results of repeat tests are within ±5 dB. A variation greater
than 15 dB is diagnostic of NOHL.
• 3. absence oF shadow curVe.
• Normally, a shadow curve can be obtained while testing bone
conduction, if the healthy ear is not masked.
• This is due to transcranial transmission of sound to the healthy ear.
Absence of this curve in a patient complaining of unilateral deafness
is diagnostic of NOHL.
• 4. inconsistency in pta and srt.
• Normally, pure tone average (PTA) of three speech frequencies (500,
1000 and 2000 Hz) is within 10 dB of speech reception threshold
(SRT). An SRT better than PTA by more than 10 dB points to NOHL.
• 5. stenger test.
• It can be done with a pair of identical tuning forks or a double-
channel audiometer. Principle involved is that, if a tone of two
intensities, one greater than the other, is delivered to two ears
simultaneously, only the ear which receives tone of greater intensity
will hear it.
• To 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 tuning 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.

• DEFINITION OF DEAF.
• (Ministry of Social Welfare, Government of India— Scheme of Assistance to Hearing
Handicap).
• “The deaf are those in whom the sense of hearing is nonfunctional for ordinary
purposes of life.”
• They do not hear/understand sounds at all even with amplified speech. The cases
included in the category will be those having hearing loss more than 90 dB in the
better ear (profound impairment) or total loss of hearing in both ears.
• The partially hearing are defined as those falling under any one of
the following categories:
Degree of hearing loss
IMPAIRMENT, DISABILITY AND
HANDICAP
• When a disease process strikes an organ or a system it causes an
impairment either in structure or function, but this impairment may
or may not become clinically manifested.
• When impairment affects the ability to perform certain functions in
the range considered normal for that individual it is called
disability.
• The disability further restricts the duties and roles expected from an
individual by society and is called a handicap.
• To exemplify, injury (disease) to the ear may result in hearing
impairment which, depending on its severity, will affect the
individual’s ability to hear and perform certain activities (disability)
and will be termed handicap by the society:

• Disease → Impairment → Disability → Handicap.

DEGREE OF HANDICAP
• Sometimes it is desired to express the impairment and handicap in
terms of percentage for the purposes of compensation. Different
countries and professional bod-ies have adopted their own system to
calculate this percentage.
• In the above calculation only three speech frequencies (500, 1000 and
2000 Hz) are taken into account but it is felt that frequency of 3000 Hz
is important for hearing in the presence of noise and should also be
taken into account.
• American Academy of Ophthalmology and Otolaryngology
recommends and takes into account the average of four frequencies
500, 1000, 2000 and 3000 Hz when calculating the handicap.
• Government of India reserved certain percentage of vacancies in
Group C and D in favour of the physically handicapped and has
extended certain other benefits. It has also recommended the
classification based on percentage of impairment and the test
required to be performed .
• (Brochure on Reservations and Concessions for Physically
Handicapped in Central Govt. Services published by Ministry of
Personnel, Pub-lic Grievances and Pensions, Dept. of Personnel and
Training.) PTO…
Thank you…