By :-

Dr. Trilok Guleria

JR ENT-HNS
 Impairment in the ability to perceive sound stimulus is
termed as hearing loss.

 3 types:
1. Conductive : due to defect in the mechanical transmission
of sound to the sensory apparatus of the ear.

2. Sensorineural : due to defect in the sensory apparatus of the

ear.

3. Mixed
 Hearing loss due to defect in the sensory apparatus -
cochlea (sensory).

 Or in the pathways of conduction of nerve impulses to

the brain (neural).

 Neural causes can be

 Peripheral: 8th nerve.

 Central: auditory pathway or cortex.

 Normal appearance of external ear and tympanic membrane
on examination.
 Positive Rinne’s test : AC>BC.
 Weber’s lateralized to better ear.
 Bone conduction decreased.
 Normal air bone gap on PTA.
 Hearing loss can exceed 60 dB.
 Congenital: present at birth, due to anomalies of
inner ear or due to pre or perinatal factors.

 Acquired: acquired later in life due to trauma, drug

intake or disorders affecting the ear.
 Presbycusis.

 Trauma to inner ear, 8th nerve or auditory

pathways.

 Infections of inner ear – labyrinthitis.

 Noise induced.
 Meniere’s disease.

 Acoustic neuroma.

 Secondary to systemic disorders: DM,

Hypothyroidism, CKD, Multiple sclerosis,
Autoimmune disorders.

 Idiopathic Sudden sensorineural hearing loss.

 First described by De Klevn in 1944.

 Definition : Sudden sensorineural hearing loss is defined as

30 dB sensorineural hearing loss in at least 3 contiguous
frequencies over a period of less than 3 days (72 hours).

 Can be unilateral or bilateral (1%).

 Usually accompanied by tinnitus (70%) or vertigo (40%).

 Overall incidence of 5-20 / 1,00,000 population.
 In 1976, Shaia and Sheehy studied the average age
of incidence of SSNHL in 1220 cases:
 Under 30 - 13%
 30-39 - 13%
 40-49 - 21%
 50-59 - 22%
 60-69 - 18%
 70+ 13%
 Multiple factors are implicated in the
etiopathogenesis of Sudden SNHL.

 90 % idiopathic.

 5 - 10 % specific causes.
 Viral infections
 Bacterial infections
 Vascular causes
 Autoimmune
 Hypoxia
 Metabolic causes
 Ototoxicity
 Traumatic
 Neural
 Neoplastic
 Meniere’s disease
 Psychogenic
 Idiopathic
 Viral infection causes Sudden SNHL by causing
cochleitis.
 Mumps
 Measles
 CMV
 Human immunodeficiency virus
 Rubella
 Chicken pox
 Herpes zoster
 Infectious mononucleosis (EBV)
  Viruses reach the inner ear by blood stream affecting
stria vascularis

endolymph

Organ of corti
Eg :- Measels ,Mumps ,CMV

 The direct proof of invasion of viruses like Herpes

zoster , Herpes simplex ,Rubella ,Influenza ,HIV, EBV
in the labyrinth is not known but they are clinically
known to cause hearing loss.
 More severe in adolescent and adults.
 Causes encephalitis and meningitis.
 Inflammatory changes in the stria vascularis of the
cochlea.
 Leads to unilateral, variable degree of SNHL.
 Common in children.
 Labyrinthine involvement causes abrupt bilateral
hearing loss along with the measles rash.
 Tinnitus and vertigo are present in 70 %.
 SNHL is the most common sequela of congenital
rubella infection (58%).
 Most often seen when maternal rubella infection
occurs within the first 16 weeks of pregnancy.
 Vestibular function is spared .
 Hearing loss typically manifests in the first 6 to 12
months of life, although it can present at birth .
 Audiograms often show a flat, uniform mild to
severe SNHL, but isolated high-frequency hearing
loss has been reported
 Due to bacterial labyrinthitis as a complication of
CSOM or iatrogenic or spread through
hematological route.
 The membranous labyrinth is totally destroyed
causing the SNHL
 Causative bacteria involved in labyrinthitis

 Haemophilus influenza
 Neisseria meningitidis
 Streptococcus species
 Staphylococcus species
 Proteus species
 Syphilis can cause Sudden SNHL and also lead to
fluctuating SNHL.
 Can be congenital or acquired.
 Congenital syphilis can cause profound and usually
bilateral loss.
 Sudden SNHL may be unilateral or bilateral . Later
is usually symmetrical in high frequencies.
 Vertebrobasilar insufficiency
 Sickle cell disease
 Leukemia
 Polycythemia
 Macroglobulinemia
 Cardiopulmonary bypass
 The arterial supply to the cochlea is such that the
basal turn is fed first by the main cochlear artery
with the cochlear apex fed last.
 Based on this anatomy one would expect occlusion of
the labyrinthine artery to cause both vestibular and
auditory symptoms which is supported by
histopathologic findings as describe above.
 In addition, one would expect temporary occlusion
in blood flow to affect low frequency areas of the
cochlea first as these areas are the most distal in
terms of blood supply.
 Cochleovestibular blood supply may be affected by
circulatory disorders such as embolic phenomenon,
thrombosis, vasospasm, and hypercoagulable or high
viscosity states resulting in SSNHL
 The underlying pathophysiology can be explained by
the occurrence of sudden anoxic injury to the
cochlea.
 The cochlea is extraordinarily intolerant of blood
supply disruptions.
 Suga and co-workers performed experimental
embolizations of cochlear vessels and showed loss of
cochlear action potentials within 60 seconds.
 Autoimmune inner ear disease
 Ulcerative colitis
 Relapsing polychondritis
 Systemic Lupus erythematosus ( SLE )
 Polyarteritis nodosa
 Cogan’s syndrome
 Wegener’s granulomatosis
 The pathogenesis of immune-mediated sensorineural
deafness and vestibular dysfunction are unclear, but
are presumed to include: vasculitis of vessels
supplying the inner ear, autoantibodies directed
against inner ear antigenic epitopes, or cross-
reacting antibodies.

 Autoimmune hearing loss implies that inner ear

proteins are recognized immunologically as foreign
or non-self
 An autoimmune disease of the cornea and vestibulo-
auditory apparatus.
 Triad of autoimmune keratitis, vertigo and SNHL.
 SNHL is progressive in nature after initial
symptoms.
 Hearing fluctuates with disease exacerbations and
remissions
 Majority develop bilateral deafness (67%)
 McCabe first described autoimmune inner ear
disease (AIED) in 1979.
 The clinical picture of AIED usually consists of
rapidly progressive bilateral sensorineural hearing
loss usually in the absence of other systemic
manifestations.
 50% of patients may complain of dizziness.
 The symptoms often progress over weeks or months
but can also present as sudden hearing loss
 Breaks in the membranous labyrinth
 Intracochlear
 Oval and/or round window
 History – inciting event
 Blow to the head :Temporal bone fracture , Inner ear
concussion
 Otologic surgery
 Sneezing
 Bending over
 Lifting a heavy object
 Exposure to sudden changes in barometric pressure
:Flying, SCUBA diving
 It is seen in acoustic trauma.
 Due to sudden loud noise of over 140 dB.
 Rupture of Reissner’s membrane, damage to organ
of corti, hair cells and mixing of perilymph &
endolymph occurs due to the loud noise leading to
hearing loss.
 May be accompanied by vertigo.
 Hypoxia to the brain causes direct cell death of the
cochlear or nerve cells leading to SNHL.
 Hypercapnia also causes SNHL by deactivation of
enzyme carbonic anhydrase which may participate
in the generation of the endocochlear potential.
 Acute and chronic renal failure.
 Alport syndrome: renal disease with hearing loss.
 Diabetes mellitus: mainly due to neuropathy.
Insidious onset.
 Hyperlipidemia
 Hypothyroidism
 Drugs and chemicals which damage the inner ear are
called ototoxic.
 They cause sensorineural hearing loss,tinitis, vertigo.
1.AMINOGLYCOSIDES
 Streptomycin,Gentamycin,Tobramycin are primarily
VESTIBULOTOXIC.
 Destroy type1 hair cells of crista ampullaris.

 In large doses can damage cochlea.

 Neomycin, Kanamycin, Amikacin, Sisomycin and

Dihydrostreptomycin COCHLEOTOXIC.
 Destroy outer hair cells, starting at basal coil and
progressing up to apex of cochlea.
 2.Diuretics
 Ethacrynic acid, furosemide and bumetanide cause
EDEMA and CYSTIC CHANGES in stria
vascularis
3.Salicylates
4.Antimalarials(Quinine)
 It cause vasoconstriction in the small vessels of the
cochlea and stria vascularis.
5.Cytotoxic drugs
Cause cochlear damage. Mainly affect outer hair cells
of cochlea.
 Drugs :-Cisplatin, Carboplatin, Vinca alkaloids.
6.Miscellaneuos
 Desferoxamine:- High freq hearing loss due to outer
hair cell damage
 Mercury and its compounds

 Gold

 Lead

 Arsenic

 Aniline dyes

 Alcohol

 Tobacco

 Marijuana
 Meningitis
 Multiple sclerosis
 Sarcoidosis
 Friedreich's ataxia
 Amyotrophic lateral sclerosis
 Vogt-Koyanagi-Harada syndrome
 Xeroderma pigmentosum
 Acoustic neuroma
 Leukemia
 Multiple Myeloma
 Metastasis to internal auditory canal
 Meningeal carcinomatosis
 Also known as acoustic neuroma or 8th nerve tumour.

 Benign, encapsulated, slow growing tumour of the

cerebello pontine angle.

 Arises from the Schwann cells around the vestibular

nerve in the internal auditory canal.

 Expands to erode the canal and involve the other cranial

nerves like 5th, 9th, 10th and 11th.
 Progressive Unilateral SNHL with tinnitus – main
symptoms.
 Poor speech perception more than the audiometric
loss.
 Late or absent vestibular symptoms.
 Involvement of other cranial nerves.
 Brainstem, cerebellar involvement and features of
raised ICT.
 High frequency SNHL.
 Poor speech discrimination.
 Roll over phenomenon.
 SISI : 0 -20 % suggestive of nerve deafness.
 TDT : retrocochlear lesion.
 Absent or diminished responses in caloric tests.
 MRI with contrast is gold standard for diagnosis.
 Also called endolymphatic hydrops.
 Attacks of SNHL followed by periods of normal hearing.
 Increased volume of endolymph.
 Due to –
 Increased production by stria vascularis.
 Decreased absorption by endolyphatic sac.
 Both.
 Characterized by –
 Vertigo.
 Fluctuating SNHL.
 Tinnitus.
 Aural fullness.
 Possible:
 no definitive episode.
 Episodic vertigo without hearing loss
 Probable:
 One definitive episode of vertigo
 Hearing loss recorded in PTA at least once
 Tinnitus or aural fullness
 Definite:
 Two or more episodes of vertigo lasting 20 mins.
 Hearing loss recorded in PTA at least once
 Tinnitus or aural fullness
 Certain: definite plus HPE confirmation
 Pure tone audiometry
 Sensorineural type of graph.
 Initially, only lower freq affected  rising graph.
 As higher freq are affected, flat graph downward
sloping graph.
 Speech audiometry
 Speech discrimination score is between 55 -85 %.
 Even lower during acute attacks.
 Other tests
 Recruitment test positive.
 SISI score of over 70%.
 Tone decay test shows decay of about 20 dB.
 These indicate a cochlear pathology.
 Caloric tests
 Cold caloric test shows reduced or absent response on
the affected side.
 Signifies canal paresis of the affected side.
 Electrocochleography
 Measures the electrical activity of cochlea and the
auditory nerve.
 Measures : CM: cochlear microphonics, SP: summating
potential, AP: action potential.
 In Meniere's disease, basilar membrane becomes taut
causing increase in summating potential.
 SP:AP ratio increases.
 Meniere’s: > 30%.
 Best obtained in acute phase.
 Glycerol dehydration test
 10dB improvement in 2 or more adjecent octaves or
10% improvement in SDS after 1-2 hours is
considered test positive.
 Tinnitus and aural fullness also improves.
 Test can be combined with transtympanic electro –
cochleography.
 Acetazolamide test
 Acetazolamide 500 mg in aqueous solution is
injected intravenously over one minute.
 Electrocochleogram monitored continuously for 45
minutes.
 Improvement is seen within 10 -15 minutes.
 Idiopathic SSNHL is a diagnosis of exclusion.
 SSNHL is termed as idiopathic after ruling out all
the known causes of SSNHL.
 History
 Clinical examination
 Otological examination
 Audiometric evaluation
 Blood work up
 Radiological examination
 Detailed history to rule out possible cause:
 Symptoms
 Unilateral/ bilateral
 Onset and progression
 Severity
 History of URTI
 History of acoustic / direct trauma
 History of ototoxicity
 History of systemic disorders
 History of surgery
 History of previous treatment
 History of similar complaints in family
 General examination: to rule out systemic
pathologies
 Neurological examination to rule out cranial nerve
involvement as in acoustic neuroma
 Cardiovascular evaluation to rule out
thromboembolism
 Otoscopy to examine tympanic membrane and EAC
to look for lesions.
 To rule out any features of labyrinthitis secondary
to CSOM.
 Tuning fork tests to differentiate SNHL and CHL.
Rinne’s , Weber, ABC & Schwabach’s.
 Cold caloric test and Dix- Hallpike test to examine
vestibular function.
 Pure tone audiometry: to diagnose and quantify the
degree of SNHL.
 Special tests for hearing to differentiate cochlear
from retro-cochlear pathology.
 Speech audiometry
 SDS
 TDT
 SISI
 Objective tests: to evaluate the exact hearing
thresholds and to identify malingerers if any.
 Oto-acoustic emissions
 Impedence audiometry.
 Brain stem evoked response audiometry.
 Complete blood counts with ESR: rule out
Leukemia, Infections.
 Blood sugar: to rule out DM.
 Renal function tests: to rule out renal disorders.
 Lipid profile: to rule out hyperlipidemia which is
common in hypertensives.
 Thyroid function tests: to rule out hypothyroidism.
 VDRL test: to rule out syphilis.
 HIV test.
 Coagulation profile: to rule out hypercoaguable
state.
 Auto-antibodies : to rule out Autoimmune Diseases.
 CT Imaging of temporal region – to rule out
labyrinthitis or other complications of CSOM.
 MRI to rule out CP angle tumours or other intra
cranial causes such as demyelination, typically seen
in multiple sclerosis, and small vessel ischaemic
changes.
 Multimodal treatment.
 Most cases are idiopathic.
 Investigations to find any specific cause.
 Treatment should be started early for better results.
 Patient should be admitted for treatment.
 Vasodilators

Vitamins Diuretics

Combined
therapy

Corticosteroids Plasma
Expanders
 Patient should be admitted and advised bed rest.
 Especially in case membrane rupture is suspected as
the cause for SSNHL.
 Intermittent oxygen inhalation at 4-6 liters per minute
for 15 minutes every 6 hourly.
 Provide more oxygen to the nerve tissue by increasing
the perilymph oxygenation.
 Carbogen (5% CO2 + 95% O2) inhalation – Increases
the partial pressure of O2 in perilymph . CO2 is a known
potent vasodilator of the vestibulocochlear vasculature,
resulting in increased blood flow.
 Improvement in hypoxia induced SSNHL.
 Oxygen therapy in the form of hyperbaric oxygen has
also shown good results.
 Treatment of choice when the loss is retro-cochlear,
and are the only effective treatment.
 Prednisolone in tapered doses over a period of 3
weeks.
 Proton pump inhibitors given along with steroids.
 Intratympanic injection of steroids is being tried
alternative to oral steroids as it has shown to
penetrate the inner ear effectively in animal studies.
 Like xanthinol nicotinate, glycerol.
 Betahistine : 16mg three times a day
 Glyceryl Trinitrate / Nitroglycerine patch
 Helps to relieve vasospasm.
 Improves blood supply to the nerve tissue.
 Glycerol increases the cochlear and cerebral blood
flow significantly after intravenous administration
 A course of antiviral drug – Valcivir, Acyclovir,
Famciclovir.
 Valcivir : 1 gm three times a day for a week.
 Acyclovir : 800 mg four times a day for a week.
 Famciclovir : 500 mg three times a day for a week
 To treat viral infection if any.
 Stellate ganglion block : blocks sympathetic activity
and results in vasodilatation of the vertebral artery.
Effective within 2 weeks of onset of SSNHL.
 Exploration of the middle ear with repair of an
inner ear fistula is recommended in patients with
a clear history of sudden hearing loss associated
with diving, straining, altitude change, or recent
otologic surgery.
 The role of surgery in patients who do not
improve with non-surgical therapy remains
controversial.
 Anticoagulants like heparin in case thrombo-
embolism is suspected.
 Low molecular weight dextran: to treat
hypercoaguable states. Increases capillary blood flow
by hypervolaemic haemodilution and by decreasing
factor VIII; this results in increased cardiac output
and tissue blood flow. Contraindicated in patients
with cardiac failure and bleeding disorders.
 Thyroxine supplementation: in case of SSNHL
due to hypothyroid state.
 Treatment or control of DM, HTN and
hyperlipidemia.
 Treatment of BACTERIAL LABYRINTHITIS
 Antibiotic is given based on cultural sensitivity
results.
 Should consist of broad-spectrum antibiotic.
Treatment of Traumatic causes
Strict bed rest
HOB elevated 30 degrees.
Avoid straining or hard nose blowing
+/- stool softeners
 If the patient has improvement, 6 more weeks of modified
physical activity should be followed.
 If no improvement is seen after five days, surgical therapy
including middle ear exploration with patching of the
perilymphatic fistula should be performed.
 Bed Rest

Noise Stool
exposure Softeners

Elevate
Head Stress

Alcohol
 COGAN’S SYNDROME
 The cornerstone of therapy is corticosteroids: topical for
IK and oral for vestibulo-auditory involvement.
 Most authors suggest using prednisolone 1mg/kg for 2-
4 weeks with a subsequent rapid taper for cases of
complete resolution and slow taper for those with
incomplete response.
 AIED
 Prednisone 1mg/kg/day for 4 weeks followed by a slow
taper if the patient responds.
 If the patient relapses on the taper, high dose prednisone
and if continued recurrence occurs with tapering, a
cytotoxic agent such as methotrexate (MTX) dosage of
7.5-15 mg weekly with folic acid, or cyclophosphamide .
Vestibular Schwannoma
Treatment depends upon size of tumour.
Various modalities are surgery, steriotactic surgery.
Meniere’s Disease
• General measures
•Vestibular sedatives: Dimenhydramine , Promethazine , Prochlorperazine
•Vasodilators: inhalation of carbogen, histamine drip.
•Diuretics.
Criteria for ablative procedures
•Only in definite Meniere’s disease.
•Ablative procedure done in long standing, unilateral Meniere's
disease with no evidence of disease in contralateral ear at time of
procedure.
•Advanced age is a relative contraindication.
 Recovery in case of SSNHL is very less unless
treated aggressively as early as possible.
 There is usually residual hearing loss of varying
level even after treament.
 Rehabilitation of hearing impairment can be done
using :
 Hearing aids – when residual loss is not profound.
 Cochlear implant – when recovery is minimal and
profound hearing impairment is present. Results are
better as the patient is post lingual.
 Published series report spontaneous recovery
rates for patients with SSNHL range from 47%
to 63%.
 Patients in whom there is no change within 2
weeks are unlikely to show much recovery.
 Four variables have been shown to affect recovery
from ISSNHL
• Time since onset
• Audiogram type
• Vertigo
• Age
 Patients with ISSNHL have an overall better chance
at hearing recovery if:
 They receive treatment within the first 7 to 14 days
of ISSNHL. Sooner the patient is seen and therapy
initiated, the better the recovery.
 Those under 15years and over 60 years have
significantly poorer recovery rates
 Patients with severe vertigo have significantly
worse outcomes than patients with no symptoms of
vertigo
 Patients with profound hearing loss significantly
decreased recovery rates as compared to mild to
moderate degree of hearing loss
 SSNHL is subjective sensation of hearing
impairment in one or both ears developing within 72
hours and a decrease in hearing of more than or
equal to 30 decibels (dB), on 3 consecutive frequency
in comparison to normal ear on audiometry
 Most patients with SSNHL cannot be given a cause
for their diagnosis.
 Highest incidence in 50-60 yrs. old
 Recovery usually begins within two weeks of onset
 SSNHL is considered to be a true otologic
emergency, given the observation that there is less
recovery of hearing when treatment is delayed.
 Thorough History, Examination & Investigations
 Rule out specific known causes.
 Corticosteroid therapy is the current standard of
care.
 Rehabilitate those whose hearing does not improve.