Download Etiology

SENSORINEURAL
HEARING LOSS IN ADULTS
นพ.อัครรัตน์ ศาสตร์สูงเนิน
อาจารย์ที่ปรึ กษา
รศ.สมชาย ศรี ร่มโพธิ์ทอง
28 ธันวาคม 2549
Sensorineural hearing loss
Introduction
Clinical evaluation
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28-Dec-2006
History
Physical examination
Auditory testing
Vestibular testing
Laboratory testing
Radiographic testing
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Sensorineural hearing loss
Etiology
• Development &
Hereditary disorder
• Infectious disorder
• Pharmacologic
disorder
• Trauma
• Neurologic disorder
• Vascular &
Hematologic disorder
28-Dec-2006
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Immune disorder
Bone disorder
Neoplasms
Endocrine & Metabolic
disorder
• Disorder of unknown
etiology
**Sudden Sensorineural hearing loss**
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Clinical evaluation
History
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Unilateral / Bilateral
Chronicity
Tinnitus / Vertigo
Otalgia / Otorrhea
Headache
Eye symptoms
28-Dec-2006
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Underlying disease
Ototoxic drugs
Hx of trauma
Noise exposure
Family Hx
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Clinical evaluation
Physical examination
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28-Dec-2006
Weber / Rinne test
Otoscopy
Cranial nerve
Stigmata of associated disease
Generally no abnormality**
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Clinical evaluation
Auditory testing
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28-Dec-2006
Conventional audiometry
Tympanometry
Acoustic reflex threshold
Auditory brainstem response
Electrocochleography
Otoacoustic emission
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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
28-Dec-2006
Vestibular testing
• Adjunct in selected patients
Radiographic testing
• MRI with Gadolinium 
Retrocochlear hearing loss?
• Computed tomography 
Labyrinthine abnormality?
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Etiology
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28-Dec-2006
Development & Hereditary disorder
Infectious disorder
Pharmacologic disorder
Trauma
Neurologic disorder
Vascular & Hematologic disorder
Immune disorder
Bone disorder
Neoplasms
Disorder of unknown etiology
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Development & Hereditary disorder
Large vestibular aqueduct
syndrome
Nonsymdromic
hereditary HL
• Not associated with
hereditory abnormalities
• Genetic factor
• ~ 90%  Recessive
fashion
28-Dec-2006
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Inner ear dysplasia
Enlarged vestibular aqueduct
Normal to profound HL
Asymmetric
Anacusis with fluctuation
Progressive HL
Well demonstrated on CT
imaging of temporal bone
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Development & Hereditary disorder
Waardenburg syndrome
• Autosomal-dominant
1) Dystopia canthrum (Lateral
displacement of the media
canthi)
2) Broad nasal root
3) Confluence of the
medialportions of the
eyebrows
4) Partial or total
heterochromia iridis
5) A white forelock
6) Sensorinearal hearing loss
• Vary hearing loss
• Unilateral / Bilateral
28-Dec-2006
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Development & Hereditary disorder
Alport syndrome
• Autosomal-dominant
• More common in
woman
• Much severe in men
(55-75%  ESRD)
• Interstitial nephritis
• SNHL
• Ocular manifestations
• Progressive hearing
loss
28-Dec-2006
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Development & Hereditary disorder
Usher syndrome
• Autosomal-recessive
• Retinitis pigmentosa &
SNHL
• Type I ~85%
 Profound HL
 Absent vestibular
response
 Retinitis pigmentosa
28-Dec-2006
Stereocilia are arranged
in three tiers atop a hair
cell.
Tip links connecting
shorter stereocilia to their
taller neighbors.
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Etiology
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28-Dec-2006
Development & Hereditary disorder
Infectious disorder
Pharmacologic disorder
Trauma
Neurologic disorder
Vascular & Hematologic disorder
Immune disorder
Bone disorder
Neoplasms
Disorder of unknown etiology
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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
28-Dec-2006
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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
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Infectious disorder
Herpes zoster oticus
• Varicella-zoster infection
• Most commonly associated
with facial paralysis
• HL and vertigo can occur
28-Dec-2006
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Infectious disorder
Measles
• Not uncommon cause
of deafness in children
• Bilateral HL
• Moderate-to-profound
HL
• Vestibular function can
be similarly affected
28-Dec-2006
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Infectious disorder
Mumps
• Paramyxovirus
infection
• Unilateral SNHL
• Unilateral deafness in
otherwise healthy
children
• Sudden deafness in
adult  Subclinical
mumps infection in
those without previous
immunity
28-Dec-2006
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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
28-Dec-2006
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Infectious disorder
Rocky Mountain
spotted fever
• Tick-borne infection
• Rickettsia rickettsii
• Headache, fever,
myalgias, and
petechial rash
• Systemic vasculitis
• Progressive SNHL
• Vasculitis involving the
auditory system
28-Dec-2006
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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)
28-Dec-2006
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Infectious disorder
Lyme disease
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Tick-borne infection
Borrelia burgdorferi
Facial paralysis
Possible etiology of
SNHL in endemic
areas
28-Dec-2006
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Etiology
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28-Dec-2006
Development & Hereditary disorder
Infectious disorder
Pharmacologic disorder
Trauma
Neurologic disorder
Vascular & Hematologic disorder
Immune disorder
Bone disorder
Neoplasms
Disorder of unknown etiology
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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
28-Dec-2006
Ototopical preparations
 Neomycin, Gentamicin, and
Tobramycin-containing
 Cochlear or vestibular
ototoxicity
 Avoid the use of
aminoglycoside-containing
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
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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
28-Dec-2006
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
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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
28-Dec-2006
Nonsteroidal
antiinflammatory drugs
 Naproxen, Ketoralac &
Piroxicam
 Ototoxicity resulting from
use of NSAIDs is rare
 Only reversible physiologic
changes, without major
morphologic changes
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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
28-Dec-2006
Erythromycin
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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
28-Dec-2006
 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
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Pharmacologic disorder
Vincristine and
vinblastine
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The vinca alkaloids
Potent neurotoxicity
Peripheral neuropathy
Cranial neuropathies,
ataxia, and hearing loss
 Loss of hair cells and
primary auditory neurons
28-Dec-2006
Eflornithine
 Drug treatment of
trypanosomiasis
 Some Pneumocystis
carinii pneumonia,
Cryptosporidiosis,
Leishmaniasis, and
Malaria
 Cause major and doserelated SNHL
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Pharmacologic disorder
Lipid-lowering drugs
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
28-Dec-2006
 Wallerian-like
degeneration
 High doses of HMG-CoA
reductase inhibitors
 Optic& vestibulocochlear
nerve degeneration
 No clinically significant
effect on vision or
hearing
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Etiology
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28-Dec-2006
Development & Hereditary disorder
Infectious disorder
Pharmacologic disorder
Trauma
Neurologic disorder
Vascular & Hematologic disorder
Immune disorder
Bone disorder
Neoplasms
Disorder of unknown etiology
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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
28-Dec-2006
 Transverse fractures
 Complete loss of
auditory & vestibular
function
 Penetrating injuries
 Subluxation ofthe stapes
into the vestibule
 Profound SNHL
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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
28-Dec-2006
 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
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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)
28-Dec-2006
 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
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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
28-Dec-2006
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
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Perilymphatic fistula
Congenital
Acquired
 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
 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
28-Dec-2006
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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
28-Dec-2006
 Stereotactic irradiation
(“radiosurgery”) for
vestibular schwannoma
 This modality  Risk of
SNHL  High as with
microsurgical removal
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Etiology
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28-Dec-2006
Development & Hereditary disorder
Infectious disorder
Pharmacologic disorder
Trauma
Neurologic disorder
Vascular & Hematologic disorder
Immune disorder
Bone disorder
Neoplasms
Disorder of unknown etiology
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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
28-Dec-2006
 Abnormalities of the ABR
 MRI  Periventricular
white-matter plaques
on T2-weighted images
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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
28-Dec-2006
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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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28-Dec-2006
Development & Hereditary disorder
Infectious disorder
Pharmacologic disorder
Trauma
Neurologic disorder
Vascular & Hematologic disorder
Immune disorder
Bone disorder
Neoplasms
Disorder of unknown etiology
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Vascular and hematologic disorders
Vertebrobasilar arterial
occlusion
Migraine
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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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28-Dec-2006
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
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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
28-Dec-2006
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Vascular and hematologic disorders
Sickle cell anemia
• Incidence of SNHL
• ~ 22% of sickle cell
disease
• Progressive or sudden
• Associated with sickle
cell crises
28-Dec-2006
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Vascular and hematologic disorders
Leukemias & Lymphomas
• SNHL
 Leukemic infiltrates
 Inner ear hemorrhage
 Vascular occlusion
 Labyrinthine ischemia
28-Dec-2006
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Etiology
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28-Dec-2006
Development & Hereditary disorder
Infectious disorder
Pharmacologic disorder
Trauma
Neurologic disorder
Vascular & Hematologic disorder
Immune disorder
Bone disorder
Neoplasms
Disorder of unknown etiology
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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
28-Dec-2006
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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
28-Dec-2006
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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
28-Dec-2006
o SNHL  Sudden or
progressive
o May be associated with
vestibular disturbances
o Rx
 Steroids
 Immunosuppresive
 Dapsone
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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
28-Dec-2006
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Immune disorders
Primary autoimmune
inner ear disease
o McCabe  Bilateral
o
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SNHL responsive to
immunosuppressive drugs
Sudden or progressive HL
Involves both ears
Associated with vestibular
symptoms
Strongly mimic Ménière’s
disease
28-Dec-2006
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
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Etiology
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28-Dec-2006
Development & Hereditary disorder
Infectious disorder
Pharmacologic disorder
Trauma
Neurologic disorder
Vascular & Hematologic disorder
Immune disorder
Bone disorder
Neoplasms
Disorder of unknown etiology
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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
28-Dec-2006
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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
28-Dec-2006
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Etiology
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28-Dec-2006
Development & Hereditary disorder
Infectious disorder
Pharmacologic disorder
Trauma
Neurologic disorder
Vascular & Hematologic disorder
Immune disorder
Bone disorder
Neoplasms
Disorder of unknown etiology
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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
28-Dec-2006
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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
28-Dec-2006
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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
28-Dec-2006
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Presbycusis
 Sensory presbycusis
- Progressively hair
cells loss
- Steeply sloping HFSNHL
 Strial presbycusis
- Atrophy of the stria
vascularis
- Flat audiograms
28-Dec-2006
 Neural presbycusis
- Loss of auditory
nerve fibers
- Reduced speech
discrimination out of
proportion to their pure
tone thresholds
 Cochlear presbycusis
- Mechanical CHL
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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
28-Dec-2006
 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
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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
28-Dec-2006
 Other pathologic
endolymphatic hydrops
processes
- Syphilis
- Temporal bone trauma
- Serous labyrinthitis
- Stapedectomy
- Autoimmune disease
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Thank You
28-Dec-2006
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