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SNHL
Saisuree Nivatwongs
ENT-PMK
Sensorineural hearing loss
Introduction
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History
Physical examination
Auditory testing
Vestibular testing
Laboratory testing
Radiographic testing
Sensorineural hearing loss
Etiology
• Development & Hereditary
disorder
• Infectious disorder
• Pharmacologic disorder
• Trauma
• Neurologic disorder
• Vascular & Hematologic
disorder
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Immune disorder
Bone disorder
Neoplasms
Endocrine & Metabolic
disorder
• Disorder of unknown
etiology
**Sudden Sensorineural hearing loss**
History
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Unilateral / Bilateral
Chronicity
Tinnitus / Vertigo
Otalgia / Otorrhea
Headache
Eye symptoms
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Underlying disease
Ototoxic drugs
Hx of trauma
Noise exposure
Family Hx
Physical Examination
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Weber / Rinne test
Otoscopy
Cranial nerve
Stigmata of associated disease
Generally no abnormality**
Auditory Testing
• Conventional
audiometry
• Tympanometry
• Acoustic reflex
threshold
• Auditory brainstem
response
• Electrocochleography
• Otoacoustic emission
Clinical evaluation
Laboratory testing
• Fluorescent treponemal
antibody absorption test :
FTA-ABS
• Microhemagglutination test
for Treponema pallidum :
MHA-TP
• Venereal disease research
laboratory : VDRL
• Routine hematologic studies
• Routine metabolic studies
Vestibular testing
• Adjunct in selected patients
Radiographic testing
• MRI with Gadolinium 
Retrocochlear hearing loss?
• Computed tomography 
Labyrinthine abnormality?
Etiology
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Development & Hereditary disorder
Infectious disorder
Pharmacologic disorder
Trauma
Neurologic disorder
Vascular & Hematologic disorder
Immune disorder
Bone disorder
Neoplasms
Disorder of unknown etiology
Development & Hereditary
disorder
• Waardenburg syndrome
• Large vestibular aqueduct syndrome
• Usher syndrome
• Alport syndrome
Etiology
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Development & Hereditary disorder
Infectious disorder
Pharmacologic disorder
Trauma
Neurologic disorder
Vascular & Hematologic disorder
Immune disorder
Bone disorder
Neoplasms
Disorder of unknown etiology
Infectious disorder
Labyrinthitis
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Serous labyrinthitis
Abnormal process within the
labyrinth
Endolymphatic hydrops
Hearing loss and vestibular
dysfunction
Permanent or transient
Sudden onset of sensorineural
hearing loss and acute vertigo
Viral labyrinthitis is common
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Suppurative labyrinthitis
Bacterial invasion of the inner
ear
Profound hearing loss and acute
vertigo
Caused by a fistula between the
middle ear and the labyrinth
Alternatively, the route of
invasion can be meningogenic
Most common etiology of
deafness associated with
meningitis
Infectious disorder
Herpes zoster oticus
• Varicella-zoster infection
• Most commonly associated
with facial paralysis
• HL and vertigo can occur
Infectious disorder
Measles
• Not uncommon cause of
deafness in children
• Bilateral HL
• Moderate-to-profound
HL
• Vestibular function can
be similarly affected
Mumps
• Paramyxovirus infection
• Unilateral SNHL
• Unilateral deafness in
otherwise healthy
children
• Sudden deafness in
adult  Subclinical
mumps infection in those
without previous
immunity
Infectious disorder
Cytomegalovirus
• Common cause of
congenital and
progressive HL in
children
• Sudden SNHL in adults
• Hearing loss associated
with AIDS may
represent reactivation
of latent CMV infections
Infectious disorder
Syphilis
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Congenital or acquired syphilis
80%  Symptomatic neurosyphilis
HL in syphilis  Meningolabyrinthitis
Syphilitic HL Indistinguishable from
Ménière’s disease
• Hennebert’s sign (a positive fistula test
without middle ear disease)
• Tullio’s phenomenon (vertigo or
nystagmus on exposure to highintensity sound)
Etiology
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Development & Hereditary disorder
Infectious disorder
Pharmacologic disorder
Trauma
Neurologic disorder
Vascular & Hematologic disorder
Immune disorder
Bone disorder
Neoplasms
Disorder of unknown etiology
Pharmacologic disorder
Aminoglycosides
 Streptomycin, Kanamycin,
Neomycin, Amikacin, Gentamicin,
Tobramycin, and Netilmycin
 Death of the hair cell
 Different patterns of ototoxicity
with different aminoglycosides
 Unilateral or asymmetric
 Reversibility of the HL
 Risk factors
(1) presence of renal disease
(2) longer duration of therapy
(3) increased serum levels
(4) advanced age
(5) concomitant administration of
other ototoxic drugs
Ototopical preparations
 Neomycin, Gentamicin, and
Tobramycin-containing
 Cochlear or vestibular
ototoxicity
 Avoid the use of aminoglycosidecontaining topical preparations in
uninflamed ears with tympanic
membrane perforations
 Ingredients of ototopical
preparations also have ototoxic
potential
 Polymyxin B, Propylene glycol,
Acetic acid, Antifungal agents
Pharmacologic disorder
Loop diuretics
 Effect by blocking sodium and
water reabsorption in the
proximal loop of Henle
 Reversible SNHL
 Bilateral and symmetric
 Sudden in onset
 Alteration of endolymphatic ion
concentration and
endocochlear potential
 Risk factors
(1) Renal failure
(2) Rapid infusion
(3) Aminoglycoside
administration
Antimalarials
 Quinine  Tinnitus, SNHL, &
Visual disturbances
 Chincinonism  Tinnitus,
headache, nausea, and disturbed
vision
 Quinine appears to be primarily
on hearing and usually is
transient
 Permanent hearing loss may
occur with large doses or in
sensitive patients
Pharmacologic disorder
Salicylates
 Aspirin  Tinnitus and
reversible SNHL
 HL  Dose-dependent
 Moderate-to-severe range
 SNHL, loss of otoacoustic
emissions, reduced cochlear
action potentials
 Alteration of the “tips” of
auditory nerve fiber tuning
curves
 Alteration in turgidity and
motility of outer hair cells
Nonsteroidal
antiinflammatory drugs
 Naproxen, Ketoralac &
Piroxicam
 Ototoxicity resulting from
use of NSAIDs is rare
 Only reversible physiologic
changes, without major
morphologic changes
Pharmacologic disorder
Vancomycin
 Almost received Vancomycin &
loop diuretics or
aminoglycosides
 Ototoxicity  Intravenously
 Permanent or transient SNHL
 Excreted by the kidney
 Renal failure  Vancomycin
half-life  Increase
ototoxicity
 Itself ototoxic  Unclear
Erythromycin
Uncommon
Partially
Intravenously
Reversible on
discontinuation
 No reports
- Newer macrolide
- Clarithromycin
- Azithromycin
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Pharmacologic disorder
Cisplatinum
Nitrogen mustards
 Antineoplastic agents
 Mechlorethamine has Serious
ototoxicity
 Limited use  Severe toxic
 Shrinkage of the organ of
Corti
 Loss of inner and outer hair
cells
 Cell-cycle nonspecific
cancer chemotherapeutic
agent
 Dose-limiting SNHL
 Adults (25% to 86%)
 Children (84% to 100%)
 Bilateral / Irreversible
 Tinnitus or vertigo
 HL  Dose-related
 Progressive outer hair
cell loss
 Inner hair cells, neural
structures and the stria
vascularis are affected
Pharmacologic disorder
Vincristine and
vinblastine
The vinca alkaloids
Potent neurotoxicity
Peripheral neuropathy
Cranial neuropathies,
ataxia, and hearing loss
 Loss of hair cells and
primary auditory
neurons
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Eflornithine
 Drug treatment of
trypanosomiasis
 Some Pneumocystis
carinii pneumonia,
Cryptosporidiosis,
Leishmaniasis, and
Malaria
 Cause major and doserelated SNHL
Pharmacologic disorder
Deferoxamine
 Iron-chelating agent
 Auditory and visual
neurotoxicity
 Particularly with larger
doses in younger
patients
 The SNHL is reversible
in some patients when
the dosage is reduced
Lipid-lowering drugs
 Wallerian-like
degeneration
 High doses of HMG-CoA
reductase inhibitors
 Optic& vestibulocochlear
nerve degeneration
 No clinically significant
effect on vision or hearing
Etiology
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Development & Hereditary disorder
Infectious disorder
Pharmacologic disorder
Trauma
Neurologic disorder
Vascular & Hematologic disorder
Immune disorder
Bone disorder
Neoplasms
Disorder of unknown etiology
Trauma
Head injury
 Blunt head injury alone
 Concussive injury of the
labyrinth
 Labyrinthine injury  SNHL
 Temporal bone fracture
 Labyrinthine concussion
 Longitudinal fractures
 Similar to acoustic trauma
 Limited to the high F
 Worse at 4 kilohertz
 Transverse fractures
 Complete loss of
auditory & vestibular
function
 Penetrating injuries
 Subluxation ofthe stapes
into the vestibule
 Profound SNHL
Trauma
Noise-induced HL &
Acoustic trauma
 First published in the 1930s
 Common occupationallyinduced disabilities
 Common in industry
 Caused by excessive noise
exposure
 Temporary SNHL that
recovers over the next 24 to
48 hours
 High intensity & repeated
 Permanent
 Outer hair cell  Most
effect
 More damage in
- High-frequency sound
- Continuous sound
- Pure tones
 Symmetric & Bilateral HL
 Limited to 3 kHz, 4 kHz, and
6 kHz
 Greatest loss  4 kHz
 Progress rapidly in first 10 to
15 years of exposure
Trauma
Noise-induced HL &
Acoustic trauma
 Common patterns  Flat
& downsloping losses
 Acoustic trauma 
Unilateral or asymmetric
 OSHA does not allow
unprotected exposures
greater than 90 dBA
based on an 8 hour/day
time weighted average
(TWA)
 Variability  Age, gender,
race, and coexisting vascular
disease
 No known way to predict
susceptibility to NIHL
 Protection  Earplugs or
earmuffs
 Many hazardous noise
exposures are not
occupational in origin
Trauma
Barotrauma
 Unequalized pressure
differentials between the
middle and external ears
 Occurs during flying or
underwater diving
 Pain, hyperemia and possible
perforation of the tympanic
membrane
 Edema and ecchymosis of the
middle ear mucosa
 Conductive HL may result
Perilymphatic fistula
 Pathologic communication
between the perilymphatic
space of the inner ear and
the middle ear
 Congenital or acquired
 Occur at either the round or
oval windows
Perilymphatic fistula
Congenital
 Occur in the stapes footplate
with labyrinthine anomalies
 Such as Mondini dysplasia
 Communicate with the
subarachnoid space and
result in cerebrospinal fluid
leak and possible meningitis
 Profound hearing loss
Acquired
 Result of
- Barotrauma
- Direct trauma of temporal
- Indirect trauma of temporal
- Complication of stapedectomy
 Sudden SNHL and vertigo after a
head injury, barotrauma, or heavy
lifting or straining
 May be spontaneously
 Diagnosis
 Middle ear exploration
Trauma
Irradiation
 Conventional fractionated
irradiation of the temporal
bone
 Fractionated irradiation 
Limited extent to treat
vestibular schwannoma
 Difficult to determine because
of the limited data available
 Stereotactic irradiation
(“radiosurgery”) for vestibular
schwannoma
 This modality  Risk of
SNHL  High as with
microsurgical removal
Etiology
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Development & Hereditary disorder
Infectious disorder
Pharmacologic disorder
Trauma
Neurologic disorder
Vascular & Hematologic disorder
Immune disorder
Bone disorder
Neoplasms
Disorder of unknown etiology
Neurologic disorders
Multiple sclerosis
 Multiple areas of CNS
demyelination, inflammation, and
glial scarring
 Age  20 to 30 years
 More common in women
 Cause  Unknown
 4% to 10% of MS  SNHL
 Progressive or sudden
 Bilateral, unilateral, symmetric,
or asymmetric
 Speech discrimination
 Normal or reduced
 Abnormalities of the ABR
 MRI  Periventricular
white-matter
plaques
on T2weighted images
Neurologic disorders
Benign intracranial
hypertension
 Pseudotumor cerebri
 Increased intracranial pressure
 Without evidence of mass lesion,
obstructive hydrocephalus,
intracranial infection, or
hypertensive encephalopathy
 Headache and visual blurring
 Pulsatile tinnitus
 SNHL and vertigo
 More in young, obese women
SNHL  Fluctuating, low-F
Unilateral or bilateral
Vertigo and aural fullness
Diagnosis
 Papilledema
 CSF pressure > 200 mmH2O
 ABR abnormalities
 Management
- Weight loss
- Acetazolamide
- Furosemide
- Lumbar-peritoneal shunting
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Etiology
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Development & Hereditary disorder
Infectious disorder
Pharmacologic disorder
Trauma
Neurologic disorder
Vascular & Hematologic disorder
Immune disorder
Bone disorder
Neoplasms
Disorder of unknown etiology
Vascular and hematologic
disorders
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Migraine
Headache and visual aura
Basilar migraine
 Vertigo, SNHL
 Tinnitus, aural fullness
 Distortion & recruitment
46%  Bilateral, low-F-SNHL
Fluctuated HL
Similarity, between basilar
migraine and Ménière’s Dz.
Drugs in basilar migraine
 No systematic study
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Vertebrobasilar arterial
occlusion
Brainstem syndromes
Anterior inferior cerebellar
artery (AICA)
Occlusion of AICA  SNHL
Thrombosis or embolism
Area infarcted  Inferior pons
Acute AICA infarction
Acute vertigo with N/V
Facial paralysis, SNHL
Tinnitus, gaze paralysis
Loss of pain and temperature
sensation on the face
Ipsilateral Horner’s syndrome
Vascular and hematologic
disorders
Waldenström’s
macroglobulinemia
• Abnormally large amounts of
IgM in the plasma
• Increased blood viscosity
• Subsequent ischemic lesions
• Progressive & sudden SNHL
• SNHL  responded to
alkylating agents or
plasmapheresis
Vascular and hematologic
disorders
Sickle cell anemia
• Incidence of SNHL
• ~ 22% of sickle cell
disease
• Progressive or sudden
• Associated with sickle
cell crises
Vascular and hematologic
disorders
Leukemias & Lymphomas
• SNHL
 Leukemic infiltrates
 Inner ear hemorrhage
 Vascular occlusion
 Labyrinthine ischemia
Etiology
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Development & Hereditary disorder
Infectious disorder
Pharmacologic disorder
Trauma
Neurologic disorder
Vascular & Hematologic disorder
Immune disorder
Bone disorder
Neoplasms
Disorder of unknown etiology
Immune disorders
Cogan’s syndrome
o Attacks of acute nonsyphilitic interstitial
keratitis
o Auditory and vestibular
dysfunction
o Unilateral or bilateral SNHL
o Severe vertigo, nausea,
vomiting, and tinnitus
o Progresses to a profound loss
over months
o Ophthalmologic findings
o If treated  SNHL is
responsive
o Aggressive treatment with
steroids
Immune disorders
Polyarteritis nodosa
o Necrotizing vasculitis of small- and
medium-sized arteries
o Myriad of findings, including weight
loss, fatigue, fever, anorexia,
arthritis, neuropathy, hypertension,
renal failure, abdominal pain, and
SNHL
o Biopsy  Necrotizing vasculitis
o Unilateral or bilateral
o Facial paralysis also may be seen
o Management
- Aggressive doses of steroids
- Immunosuppressive drugs
Immune disorders
Relapsing polychondritis
o An inflammatory reaction in
multiple cartilages
o The auricles  1st affected
o Arthritis and eye findings
o HL  Conductive
 Sensorineural
 Mixed HL
o SNHL  Sudden or
progressive
o May be associated with
vestibular disturbances
o Rx
 Steroids
 Immunosuppresive
 Dapsone
Immune disorders
Wegener’s granulomatosis
o Necrotizing granulomatous
vasculitis involving principally
the lungs, airway, and kidneys
o Usually  Conductive HL
o CHL  Involvement of the
eustachian tube or middle ear
o SNHL  If extends into the
inner ear
Immune disorders
Primary autoimmune
inner ear disease
o McCabe  Bilateral SNHL
responsive to
immunosuppressive drugs
o Sudden or progressive HL
o Involves both ears
o Associated with vestibular
symptoms
o Strongly mimic Ménière’s
disease
o Humoral autoimmunity 
Abnormal
o Responsiveness of the HL to
steroids or cytotoxic drugs
 The hallmark
o Used Methotrexate 
Reduce the need for
continued high-dose steroids
Etiology
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Development & Hereditary disorder
Infectious disorder
Pharmacologic disorder
Trauma
Neurologic disorder
Vascular & Hematologic disorder
Immune disorder
Bone disorder
Neoplasms
Disorder of unknown etiology
Bone disorders
Otosclerosis
 Primarily causes
 CHL
 Uncommonly
 Progressive SNHL
 Especially in late disease
 CT images
 Radiolucent area
surrounding the cochlea
 Advanced otosclerosis
 Bilateral profound
mixed hearing loss
Bone disorders
Paget’s disease
 Osteitis deformans
 Most common in older
 ~ 50% of Paget’s disease
 Conductive, SNHL or mixed
 Rarely fixed stapes footplate
 RX
 Calcitonin
 Eidronate disodium
Etiology
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Development & Hereditary disorder
Infectious disorder
Pharmacologic disorder
Trauma
Neurologic disorder
Vascular & Hematologic disorder
Immune disorder
Bone disorder
Neoplasms
Disorder of unknown etiology
Neoplasms
Vestibular schwannoma
 Most common neoplasm
 SNHL
 Originate from 8th CN
 Within the CPA or the IAC
 Approximately 80% of all CPA
neoplasms
 Progressive unilateral SNHL
 Principally the high
frequencies
Neoplasms
Vestibular schwannoma
 Speech discrimination is reduced
out of proportion to the pure tone
thresholds
 Sudden SNHL  10% of patient
 Unilateral or asymmetric tinnitus
 With or without hearing loss
 Mild or severe vestibular symptoms
or may have none
Disorders of unknown
etiology
Presbycusis
 Aging process
 Without other apparent
etiology
 Age-related change 
Stiffness of the basilar
membrane
 30% of aged > 65 years
 Worse for high frequencies
 More severe in men
 Schuknecht  4 types
Presbycusis
 Sensory presbycusis
- Progressively hair
cells loss
- Steeply sloping HFSNHL
 Strial presbycusis
- Atrophy of the stria
vascularis
- Flat audiograms
 Neural presbycusis
- Loss of auditory
nerve fibers
- Reduced speech
discrimination out of
proportion to their
pure tone thresholds
 Cochlear presbycusis
- Mechanical CHL
Disorders of unknown
etiology
Ménière’s disease
 Fluctuant SNHL
 Tinnitus, episodic vertigo, and
aural fullness
 Progresses, gradually or
quickly HL
 Tinnitus  “Buzzing” or
“Roaring”
 Aural fullness  Typically
fluctuates
 Vertigo  Several hours
 After attacks  Fatigued
for 24 hours or more
 Profound loss is rare
 Low F  Commonly
 Bilateral in 30% to 50%
 Endolymphatic spaces
dilatation of the inner ear
Disorders of unknown
etiology
Ménière’s disease
 Vestibular destructive
therapy  No effective
 No therapy  Effective in HL
 Medical therapy
- Sodium-restricted diet
- Diuretic administration
 Lack of an objective
diagnostic test
 Idiopathic endolymphatic
hydrops
 Other pathologic
endolymphatic hydrops
processes
- Syphilis
- Temporal bone trauma
- Serous labyrinthitis
- Stapedectomy
- Autoimmune disease
Thank you for your
attention