HEARING LOSS

DR SRAVYA M V
SECOND YEAR
MS SALAKYATANTRA
GAVC TPRA
Anatomy of ear

• External ear

i. Auricle / pinna

ii. External acoustic canal

iii. Tympanic membrane

• Middle ear
Ossicles
 Bony labyrinth

Membranous labyrinth

Inner ear
Hearing loss

• Impairment of hearing & its severity may vary from mild to severe / profound

• Deafness - little / no hearing at all

WHO - “deaf” - those individuals whose hearing impairment is so severe that they are
unable to benefit from any type of amplification

• HL > 90 dB in the better ear (profound impairment) / total loss of hearing in both ears
CLASSIFICATION

Hearing Loss

Organic Nonorganic
• Psychosomatic
• Conductive
• Malingering
• Sensorineural
• Hysterical
• Sensory (cochlear)
• Neural
• Peripheral (VIIIth nerve)

• Central (Central auditory pathways)

• Type of loss ( conductive , sensory , neural)

• Location of the problem ( external ear ,middle ear , cochlea , auditory nerve, central)

• Mode of onset ( sudden / insidious )

• Rate of progression

• Degree of loss ( mild , moderate , severe )

• Etiology

• Bilateral / unilateral
DEGREE OF HEARING LOSS (WHO CLASSIFICATION)

On the basis of PTA

1. Mild 26–40 dB

2. Moderate 41–55 dB

3. Moderately severe 56–70 dB

4. Severe 71–91 dB

5. Profound > 91 dB

6. Total Normal

No apparent impairment of hearing from 0 - 25 dB

Hearing threshold in better Degree of impairment Ability to understand
ear 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 - 91 Severe Can understand only

shouted / amplified speech

Above 91 Profound Usually cannot understand

even amplified speech
CONDUCTIVE HEARING LOSS
• Caused by any disease process which interferes with the conduction of sound to reach
cochlea
• The lesion may lie in the external ear & tympanic membrane, middle ear / ossicles up to
stapedio vestibular joint
Aetiology
Congenital Acquired

Meatal atresia External ear – any obstruction in ear canal

Fixation of stapes footplate Wax , foreign body , furuncle, acute inflammatory swelling benign/malignant
tumour ,atresia of canal
Fixation of malleus head Middle ear

Ossicular discontinuity Perforation of TM – traumatic / infective

Congenital cholesteatoma Fluid in middle ear – acute otitis media, serous otitis media , hemotympanum

Mass in middle ear – benign / malignant tumours

Disruption of ossicles – trauma to ossicular chain, CSOM, cholesteatoma

Fixation of ossicles – otosclerosis , tympanosclerosis , adhesive otitis media

Eustachian tube blockage – retracted tympanic membrane , serous otitis media

Characteristics
1. Negative Rinne test 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

Average hearing loss in different lesions of conductive apparatus
1. Complete obstruction of ear canal 30 dB

2. Perforation of tympanic membrane 10–40 dB

3. Ossicular interruption with intact drum 54 dB

4. Ossicular interruption with perforation 38 dB

5. Malleus fixation 10–25 dB

6. Closure of oval window 60 dB

Management

Medical / surgical
1. Removal of canal obstructions - impacted wax, foreign body, osteoma / exostosis,
keratotic mass, benign / malignant tumours / meatal atresia

2. Removal of fluid - Myringotomy with / without grommet insertion

3. Removal of mass from middle ear - Tympanotomy & removal of small middle ear
tumours / cholesteatoma behind intact TM

4. Stapedectomy - oto sclerotic fixation of stapes footplate

5. Tympanoplasty - Repair of perforation, ossicular chain / both

6. Hearing aid - where surgery is not possible, refused / has failed

SENSORINEURAL HEARING LOSS
• Results from lesions of the cochlea, VIIIth nerve / central auditory pathway

• Congenital/acquired
Characteristics
1. A positive Rinne test AC > BC

2. Weber lateralized to better ear

3. Bone conduction reduced on Schwabach & absolute bone conduction tests

4. More often involving high frequencies

5. No gap between air & bone conduction curve on audiometry

6. Loss may exceed 60 dB

7. Speech discrimination is poor

8. Difficulty in hearing in the presence of noise

Aetiology

• Congenital
• Present at birth
• Result of anomalies of the inner ear / damage to the hearing apparatus by
prenatal / perinatal factors
A. Prenatal causes

1. Infant factors
• Genetic / nongenetic causes

• Anomalies – nonsyndromic/syndromic

• May involve only the membranous labyrinth / both the membranous & bony
labyrinths
(a) Scheibe dysplasia

(b) Alexander dysplasia

(c) Bing–Siebenmann dysplasia

(d) Michel aplasia

(e) Mondini dysplasia

(f) Enlarged vestibular aqueduct

(g) Semicircular canal malformations

2. Maternal factors
(a) Infections during pregnancy - TORCHES

(b) Drugs during pregnancy - Streptomycin, gentamicin, tobramycin, amikacin, quinine /

chloroquine

(c) Radiation to mother in the first trimester

(d) Other factors

Nutritional deficiency

diabetes

toxaemia

thyroid deficiency

Maternal alcoholism - teratogenic to the developing auditory system

B. Perinatal causes

Causes during birth / in early neonatal period

1. Anoxia - Damages the cochlear nuclei & causes haemorrhage into the ear - Placenta praevia,
prolonged labour, cord round the neck & prolapsed cord

2. Prematurity & low birth weight (<1500 g)

3. Birth injuries - forceps delivery - intracranial haemorrhage with extravasation of blood into the inner
ear

4. Neonatal jaundice - Bilirubin > 20 mg% damages the cochlear nuclei

5. Neonatal meningitis
6. Sepsis
7. Time spent in neonatal ICU
Acquired

• Appears later in life

• Genetic/nongenetic

• The genetic hearing loss may manifest late (delayed onset) & may affect only
the hearing / be a part of a larger syndrome
Common causes
1. Infections of labyrinth—viral,
bacterial / spirochaetal
2. Trauma to labyrinth / VIIIth nerve
- fractures of temporal bone /
concussion of the labyrinth / the
6. Ménière’s disease
7. Acoustic neuroma
8. Sudden hearing loss
9. Familial progressive SNHL
10. Systemic disorders - diabetes,

ear surgery hypothyroidism, kidney disease,

autoimmune disorders, multiple sclerosis,
3. Noise-induced hearing loss
blood dyscrasias
4. Ototoxic drugs

5. Presbycusis
Diagnosis
1. History
2. Severity of deafness
3. Type of audiogram
4. Site of lesion
5. Laboratory tests
Management

• Early detection

• Syphilis of the inner ear

high doses of penicillin & steroids with improvement in hearing

• Hearing loss of hypothyroidism

reverses with replacement therapy

• Serous labyrinthitis
reversed by attention to middle ear infection
• Early management of Ménière’s disease
prevent further episodes of vertigo & hearing loss

• SNHL due to perilymph fistula

surgical correction by sealing the fistula in the oval / round window with fat

• Ototoxic drugs
should be used with care & discontinued if causing hearing loss

• Noise induced hearing loss

prevented from further deterioration - if the person is removed from the noisy surroundings

• Rehabilitation of hearing impaired with hearing aids & other devices

SPECIFIC FORMS OF HEARING LOSS

A. INFLAMMATIONS OF LABYRINTH
• Viral, bacterial / syphilitic

1. Viral labyrinthitis
• Viruses reach the inner ear by blood stream affecting stria vascularis & then the
endolymph & organ of Corti
• Measles, mumps & cytomegaloviruses

• Rubella, herpes zoster, herpes simplex, influenza & Epstein–Barr are clinically known to
cause deafness
2. Bacterial
• Reach labyrinth through the middle ear (tympanogenic) / through CSF (meningogenic)

3. Syphilitic
• Congenital & acquired

• Congenital syphilis – 2 types

• Early form - manifesting at the age of 2

• Late form - manifesting at the age of 8–20 years

(a) Sudden SNHL - unilateral / bilateral

(b) Ménière’s syndrome with episodic vertigo, fluctuating hearing loss, tinnitus & aural
fullness

(c) Hennebert’s sign - positive fistula sign in the absence of a fistula

Due to fibrous adhesions b/w the stapes footplate & the membranous labyrinth

d) Tullio phenomenon - loud sounds produce vertigo

Diagnosis of otosyphilis
• Other clinical evidence of late acquired / congenital syphilis & the laboratory tests

• Fluorescent treponema-absorption test (FTA-ABS)

• Venereal disease research laboratory (VDRL)

• Rapid plasma reagin (RPR) tests from CSF

Treatment
• I V penicillin

• Steroids
B. FAMILIAL PROGRESSIVE SNHL
• Genetic disorder

• Progressive degeneration of the cochlea

• Starting in late childhood / early adult life

• Hearing loss is bilateral with flat / basin-

shaped audiogram but an excellent speech
discrimination
C. OTOTOXICITY

• Drugs & chemicals - damage the inner ear & cause SNHL, tinnitus & vertigo

1. Aminoglycoside antibiotics

• Streptomycin, gentamicin & tobramycin - primarily vestibulotoxic

• Selectively destroy type I hair cells of the crista ampullaris

• In large doses - damage the cochlea

• Neomycin, kanamycin, amikacin, sisomycin & dihydrostreptomycin – cochleotoxic

• Selective destruction of outer hair cells, starting at the basal coil & progressing onto the apex
of cochlea
Patients at risk,
(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

(f) who have genetic susceptibility to aminoglycosides

antibiotic binds to the ribosome - interferes with protein synthesis- death of the
cochlear cells
2. Diuretics
• Furosemide, bumetanide & ethacrynic acid - loop diuretics - block transport of sodium
& chloride ions in the ascending loop of Henle
• Cause oedema & cystic changes in the stria vascularis of the cochlear duct

• Effect is reversible but permanent damage may occur

• Hearing loss may be bilateral & symmetrical / sometime sudden in onset

3. Salicylates
• Tinnitus & bilateral SNHL particularly affecting higher frequencies

• Reversible after the drug is discontinued

4. Quinine
• Tinnitus & SNHL

• Reversible

• Higher doses - permanent loss

• Due to vasoconstriction in the small vessels of the cochlea & stria vascularis
5. Chloroquine & hydroxychloroquine

6. Cytotoxic drugs
• Nitrogen mustard, cisplatin & carboplatin

• Affect the outer hair cells of the cochlea

7. Deferoxamine (Desferrioxamine)
• Used in the treatment of thalassaemic patients who receive repeated blood
transfusions & in turn have high iron load
• Onset sudden / delayed
8. Miscellaneous
• Isolated cases - erythromycin, ampicillin & chloramphenicol, indomethacin,
phenylbutazone, ibuprofen, tetanus antitoxin, propranolol & propylthiouracil
• Alcohol, tobacco & marijuana

9. Topical ear drops

• By absorption through oval & round windows

• Use of chlorhexidine which was used in the preparation of ear canal before surgery

• Use of ear drops containing aminoglycoside antibiotics - neomycin, framycetin &

gentamicin
D. NOISE TRAUMA
• In boiler makers, iron- and coppersmiths & artillery men

• occupational hazard

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

• Impulse noise - arise from an explosion, gun fire / a powerful cracker & may reach /
cross 140 dB

• Noise level of a gun / rifle may reach 140–170 dB SPL

• Mechanical damage of organ of Corti, tearing of Reissner’s membrane, rupture of hair
cells & allowing mixing of perilymph & endolymph

• A severe blast, in addition, may concomitantly damage the tympanic membrane and
disrupt ossicles further adding conductive loss
2. Noise-induced hearing loss (NIHL)
• Follows chronic exposure to less intense sounds

(a) Temporary threshold shift (TTS)

• Hearing is impaired immediately after exposure to noise

• 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 & duration

(b) Permanent threshold shift (PTS)

• permanent & does not recover at all
The damage caused by noise trauma depends on ,
(i) Frequency of noise - A frequency of 2000–3000 Hz causes more damage than
lower / higher frequencies

(ii) Intensity & 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

(v) Pre-existing ear disease

.
• A noise of 90 dB SPL, 8 h a day for 5 days per week is the maximum safe
limit
• No exposure in excess of 115 dB is to be permitted
• No impulse noise of intensity greater than 140 dB is permitted
• The audiogram in NIHL shows a typical notch, at 4 kHz, both for air and bone conduction

• Symmetrical on both sides

• At this stage- high-pitched tinnitus & difficulty in hearing in noisy surroundings but no

difficulty in day-to-day hearing

• As the duration of noise exposure increases, the notch deepens & also widens to

involve lower & higher frequencies

• Hearing impairment becomes clinically apparent to the patient when the frequencies of

500, 1000 and 2000 Hz (the speech frequencies) are also affected
• NIHL causes damage to hair cells, starting in the basal turn of cochlea

• Outer hair cells affected before the inner hair cells

• Preventable

• Persons who have to work at places where noise is above 85 dB should have pre-

employment & then annual audiograms for early detection

• Ear protectors (ear plugs / ear muffs) should be used where noise levels exceed 85 dB (A).

• They provide protection up to 35 dB

• If hearing impairment has already occurred - rehabilitation

3. Nonauditory effects of noise

• Can affect other systems of the body

• Interferes with rest & sleep causing chronic fatigue & stress

• Through activation of the autonomic nervous system & pituitary– adrenal axis, causes
annoyance & irritability

• Hypertension &peptic ulcer

• Adversely affects task performance where communication through speech is required

• Laryngeal problems - in workers who have to speak loudly in persistently noisy

surroundings
E. AUTOIMMUNE (IMMUNE-MEDIATED) INNER EAR DISEASE
• Progressive bilateral SNHL

• 40 & 50 years

• Equal incidence in both sexes

• 50% - Vestibular symptoms - disequilibrium, motion intolerance, positional / episodic

vertigo

• About 15% - evidence of other autoimmune disorder - ulcerative colitis, systemic lupus,
rheumatoid arthritis / multiple sclerosis
• Bilateral SNHL ≥ 30 dB at any frequency & evidence of progression in at least one ear
on 2 serial audiograms that are done at equal to / less than 3 months apart

• Progression - threshold shift of ≥ 15 dB at one frequency / 10 dB at 2 or more

consecutive frequencies / significant change in speech discrimination
Investigations
1. Audiogram – repeated audiograms - at one month - loss at high & 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 / multiple sclerosis

4. Contrast-enhanced MRI

5. Blood tests to exclude systemic autoimmune disorders –TC,DC, ESR, rheumatoid

factor , ANA, C3 & C4 compliment levels, Raji cell assay for circulating immune
complexes

6. Western blot essay for anti-Hsp 70 (anti-heat shock protein 70) antibodies -
correlates to both active disease & steroid responsiveness
Treatment
• Prednisolone 1 mg/kg/day up to a total of 60 mg/day (for adults) for 4 weeks

• Those who cannot take steroids - methotrexate 15 mg/week for 6–8 weeks

• Alternative to methotrexate - cyclophosphamide - more toxic

• Other treatments - intratympanic steroid injection, systemic IgG injection &

plasmapheresis
F. SUDDEN HEARING LOSS
• 30 dB / more of SNHL over at least 3 contiguous frequencies occurring within a period
of 3 days / less
• Mostly - unilateral

• May be accompanied by tinnitus / temporary spell of vertigo

Aetiology
“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 / thrombosis of labyrinthine / cochlear artery / their

vasospasm. May be associated with diabetes, hypertension, polycythaemia, macroglobinaemia/ 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

Idiopathic
1. Bed rest

2. Steroid therapy Prednisolone 40–60 mg in a single morning dose for 1 week & then tailed

off in a period of 3 weeks. Steroids - anti-inflammatory & relieve oedema - moderate degree

3. Inhalation of carbogen (5% CO2 + 95% O2) - increases cochlear blood flow & improves

oxygenation

4. Vasodilator drugs

5. Low molecular weight dextran - decreases blood viscosity. Contraindicated in cardiac failure

& bleeding disorders

6. Hyperbaric oxygen therapy - raises concentration of oxygen in labyrinthine fluids &

improves cochlear function

7. Low-salt diet & a diuretic

8. Intratympanic steroids therapy - raises the local concentration of steroids in cochlear

fluids - avoid side effects of systemic therapy

Treatment
1. Steroids

2. Inhalation of carbogen

3. Low-salt diet and a diuretic

4. Hyperbaric oxygen

Prognosis
• About half the patients of idiopathic sensorineural hearing loss recover spontaneously
within 15 days
• Chances of recovery - poor after 1 month

• Severe hearing loss & that associated with vertigo - poor prognosis
G. PRESBYCUSIS
• SNHL associated with physiological aging process in the ear

• Manifests at the age of 65 years

• Early - hereditary predisposition, chronic noise exposure / generalized vascular

disease
• 4 pathological types

1. Sensory - degeneration of the organ of Corti, starting at the basal coil &
progressing gradually to the apex
Higher frequencies – affected

Speech discrimination remains good

2. Neural
• Degeneration of the cells of spiral ganglion, starting at the basal coil & progressing to
the apex
• Neurons of higher auditory pathways may also be affected

• high tone loss but speech discrimination is poor & out of proportion to the pure tone
loss
3. Strial / metabolic
• Atrophy of stria vascularis in all turns of cochlea

• Runs in families

• Audiogram - flat , speech discrimination - good

4. Cochlear conductive
• Due to stiffening of the basilar membrane - affecting its movements

• Audiogram - sloping type

• Great difficulty in hearing in the presence of background noise though they may hear
well in quiet surroundings
• Speech being heard but not understood

• Recruitment phenomenon - positive & all the sounds suddenly become intolerable
when volume is raised
• Tinnitus

• Hearing aid

• Curtailment of smoking & stimulants like tea & coffee - decrease tinnitus
NONORGANIC HEARING LOSS (NOHL)
• No organic lesion

• Malingering / psychogenic

• Patient may present with any of the 3 clinical situations

(i) Total hearing loss in both ears

(ii) Total loss in only one ear

(iii) Exaggerated loss in one or both ears

1. High index of suspicion
• Patient makes exaggerated efforts to hear

• Frequently making requests to repeat the question

• Placing a cupped hand to the ear

2. Inconsistent results on repeat pure tone & speech audiometry tests

• Normally, the results of repeat tests are within ±5 dB

• A variation > 15 dB
3. Absence of shadow curve
• Normally, a shadow curve can be obtained while testing bone conduction, if the healthy

ear is not masked - due to transcranial transmission of sound to the healthy ear

• Absence of this curve in a patient complaining of unilateral deafness

4. Inconsistency in PTA & SRT

• Normally, pure tone average (PTA) of 3 speech frequencies (500, 1000 & 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

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 - indicates NOHL

7. Electric response audiometry (ERA)

• Establish hearing acuity of the person to within 5–10 dB of actual thresholds
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