AUDITORY SYSTEM Four major divisions of auditory system –

Function
• Focus: (CD, Figure 3.4.1)
- Anatomy and function of the ear

- Sound transfer function of ear:

When sound is conducted into the ear, how
sound is affected by that process

I. Outer ear (CD, Figure 3.4.2)

• Three parts of outer ear

1) Pinna
2) Ear canal
3) Ear drum

• Major function of outer ear 1) protection

2) amplification
3) sound localization

Four major divisions of auditory system -

Anatomy

1. The outer ear

- pinna
- ear canal
- eardrum

2. The middle ear

- three ossicle bones;
(malleus, incus, stapes)
- two major muscles
(stapedial muscle, tensor
tympani)
- Eustachian tube

3. The inner ear

- cochlea (hearing)
- vestibular system (balance)

4. The central auditory system

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 I.
(1) Pinna (BinaI. Outer ear:
(3) ear drum (CD, Figure 3.4.5)
Major function of outer ear
: As a boundary between outer
and middle ear
: Vibrates in response to sound

II. Middle ear

• Three main parts of middle ear

(1) Three Ossicle bones:

- Malleus(1), Incus(3), Stapes(6)
Function: Impedance matching

(1) Pinna (Spec

(2) Two muscles
- Stapedial muscle(5)
- Tensor tympani(9)
Function: Protection

(3) Eustachian tube(8)

Function: Equalizer of air pressure

II. Middle ear (3) Eustachian Tube

(1) Pinna
• Function of ossicles
- 99.9% sound is reflected due to high
impedance of fluid in the cochlea (0.1% sound is
only passed = - 30 dB sound loss from air - fluid
I. Outer ear: impedance mismatch)
(2) Ear canal (CD, Figure 3.4.4)
ear gain

• Three parts of outer ear - Middle ear bones overcome the loss of
1) Pinna sound by increasing sound pressure (+34dB)
2) Ear canal => Impedance matching
3) Ear drum • Three mechanisms for impedance
• Major function of outer ear 1) matching
protection 1) Area ratio of the ear drum to the stapes

• Anotia
2) amplification footplate (20:1)
3) sound localization => 20 log (20/1) = +26dB SPL

Right
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 * Basic concept: p = f/a
2) Lever action of the ossicles (1.3:1)
=> 20 log(1.3/1) = +2 dB SPL
3) Buckling of ear drum
( x 2 pressure increase
=> 20 log(2/1) = +6dB SPL

III. Inner ear – Inner hair cells (IHC) & Outer

hair cells (OHC)
Inner hair cells: produce sensation of hearing
Outer hair cells: modify BM response and act as
amplification system

II. Middle ear - Impedance matching

In total, 20 x 1.3 x 2 increase in pressure by
middle ear and ear drum ( + 34 dB SPL).
It works for mismatched impedance
(99.9% sound loss = apx - 30 dB)

IV.
]

III. Inner ear (CD, Figure 3.4.11)

Tonot
• Twp parts of inner ear
1) Cochlea (Hearing)
- Scala vestibuli
- Scala media
- Scala tympani
2) Vestibular system (balance)
• Major function of inner ear
1) Hearing
(It transmits sound to
neural impulse and gives
resonant frequency)
2) Balance Outer ear:
Acoustic energy, in the form of sound waves,
passes pinna, ear canal. Sound waves hit the ear
drum, causing it to vibrate like a drum.
Middle ear:

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It sets three ossicle bones (malleus, incus,
stapes) into motion, changing acoustic energy
• SENSORI-NEURAL
to mechanical energy. These middle ear • MIXED
bones mechanically amplify sound and
compensate mismatched impedance. • NON-ORGANIC
Inner ear and Central auditory nervous
system: CONDUCTIVE HEARING LOSS
When the stapes moves in and out of the oval • Occurs from a dysfunction of the outer or
window of the cochlea, it creates a fluid middle ear
motion, hydrodynamic energy. It causes
membranes in the Organ of Corti to shear
• Can usually be treated with medicine or
surgery
against the hair cells.
This creates an electrochemical signal • A deficit of loudness only
which is sent via the auditory nerve to the Characteristics of Conductive Loss:
brain.
• Maintain soft speaking voice

• Excellent speech discrimination when

speech is loud enough
• Typically either low frequency or flat
hearing loss (equal at all frequencies)

CAUSES OF CONDUCTIVE HEARING LOSS:

Outer Ear:
– Occlusion/foreign body
– Congenital Atresia
– External Otitis
Causes of Conductive Hearing Loss: Middle Ear
Middle ear:
• Otitis Media
• TM Perforation
• Cholesteatoma
• Ossicular fixation
DIAGNOSTIC TEST FOR AUDITORY ACUITY – Otosclerosis
SYMPTOMS OF EAR DISEASES • Ossicular Disarticulation
• Blocked Eustachian Tube, reduced
1.TUNING FORK TESTS middle ear pressure, TM retraction and
> RINNE TEST eventual effusion
> WEBER TEST
2. WHISPER VOICE TEST TREATMENT: CONDUCTIVE HEARING
3. AUDIOMETRY LOSSES
PURE TONE • Conductive hearing losses are due to
SPEECH AUDIOMETRY problems that occur in the outer and middle ear
4. TYMPANOGRAM OR IMPEDANCE which are usually temporary and/or treatable with
AUDIOMETRY antibiotics or surgery.
5. OCULOVESTIBULAR TEST/ CALORIC ICE
WATER TEST • For those few people who have
uncorrectable conductive hearing losses, hearing
SYMPTOMS OF EAR DISEASES aids are of significant benefit as sound remains
• DEAFNESS clear if it is made loud enough.
• PAIN
• DISCHARGE SENSORI-NEURAL HEARING LOSSES
• VERTIGO • Dysfunction of the inner ear or auditory
• TINNITUS nerve, usually permanent and untreatable
• Results in loudness deficit and distorted
TYPES OF HEARING LOSS: hearing.
• CONDUCTIVE • Nerve endings in cochlea or nerve
pathways are damaged.
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• Message does not effectively reach the
brain.
• Middle ear structures are intact.
Characteristics of SNHL:
• Inappropriately loud voice
• Tinnitus
• High frequency loss common, but any
configuration possible
• Speech sounds distorted
• Background noise makes listening more
difficult
• Hearing aids may help
• Amount of loss varies from person to
person
• Risk of noise-induced progression stops
if no longer in noise exposed, but aging
invariably worsens loss
– For most, aging effects aren’t
significant before age 50+
CAUSES OF SENSORI-NEURAL HEARING
LOSS:
• Genetics/Congenital
• Disease
– Mumps, Measles
– Meningitis, CMV
• Ototoxic drugs
• Head trauma
• Presbycusis
• Meniere’s Disease
• Acoustic Neuroma
• Ototoxin Exposure
Characteristics of NIHL:
(noise induced hearing loss)
• Loss can be sudden, as with acoustic
trauma from an explosion.
• More often a gradual onset that may go
unnoticed.
– NIHL also known as noise induced
permanent threshold shift (NIPTS), typically
takes years of exposure, gradual erosion of
hearing that eventually affects
communication.
Classic Symptoms of
NIHL:
A notch or drop in hearing at 4000 Hz.
Generally affects 3000-6000 Hz range first,
then notch becomes deeper & wider
• Typically bilateral and symmetrical
• Tinnitus common
• Reduced speech comprehension,
particularly in background noise. Why?
l Vowels are low frequency sounds that
carry 90% of speech energy (I can hear you
talking….)
l Consonants are higher frequency sounds
that carry most of the meaning of speech. NIHL
begins in high frequencies.
(But I can’t understand what you are saying.)
The “4 P’s”
Noise induced hearing loss is:
• Painless
•
•
Progressive
Permanent
• Preventable
TREATMENT:
• Sensori-neural hearing loss is due to
problems that occur in the inner ear and are
almost always permanent and untreatable.
• Hearing aids will benefit most people with
sensori-neural loss, but results can vary.
MIXED HEARING LOSS:
• Combination of conductive (outer or
middle ear) disorder and sensori-neural hearing
loss.
• Treatment may be available for the
conductive portion; however, the sensori-neural
portion will remain.
• Causes can be unrelated (for example,
NIHL plus TM rupture), or related (for example
cochlear otosclerosis).
NON-ORGANIC HEARING LOSS
• Non-Organic:
– No medical or physical reason for hearing
loss, may be voluntary or involuntary
• Malingering:
– Consciously faking or exaggerating a
hearing impairment, often for monetary or other
personal gain, to escape assignments or
responsibilities,
or as an anti-establishment
gesture
NON-ORGANIC HEARING LOSS
Symptoms that should alert you to malingering:
• Substantial, equal hearing loss at all
frequencies or no response to pure tones at all in
one or both ears
• Inconsistent results, or markedly different
than prior results
– Unilateral “deafness” without significant
medical history unlikely
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• Exaggerated attention to test, may
press on earphones, difficulty hearing you call
them back for testing or to your directions
(normal voice level is around 60 dB), but can
hear you when your back is turned or when no
visual cues
• Patient history may provide clues to
non-organic behavior if nearing retirement, or
pending discipline or deployment
• Psychogenic Hearing Loss -
Unconscious development of a non-organic
hearing loss – a compensatory protective
device, a psychogenic problem (the patient
believes the impairment is real)

CENTRAL HEARING LOSS

• Occurring within central nervous
system (cortex, brainstem, or ascending Otitis Externa
auditory pathways) as opposed to peripheral
organs of hearing (cochlea and middle ear) • Bacterial infection of external auditory
• Often associated with other canal
neurological disorders (multiple sclerosis, • Categorized by time course
tumors)
• Sometimes confused with non-organic
– Acute
hearing loss due to vague symptoms or – Subacute
inappropriate test behavior – Chronic
•

Otitis exter
Always requires diagnostic work-up by
an audiologist, otologist, and/or neurologist; Acute Otitis Externa (AOE)
patient usually hears WNL for pure tones
• “swimmer’s ear”
IN SUMMARY…. • Preinflammatory stage
• Conductive Hearing Loss:
– Usually low frequency or flat, affects • Acute inflammatory stage
outer and/or middle ear, usually temporary - or – Mild
at least medically or surgically treatable.
• Sensori-neural Hearing Loss: – Moderate
– Often high frequency, affects inner ear, – Severe
usually permanent.
• Mixed Hearing Loss: AOE: Preinflammatory Stage
Usually affects both high and low freqs,
–
both conductive and sensori-neural
• Edema of stratum corneum and plugging
components, but only conductive portion of apopilosebaceous unit
treatable. • Symptoms: pruritus and sense of fullness
• Non-Organic Hearing Loss:
– Typically display a flat loss or total • Signs: mild edema
deafness in one ear, but may exaggerate a • Starts the itch/scratch cycle
true loss, may (rarely) be involuntary but AOE: Mild to Moderate Stage
usually malingering is involved. Prior test
results are your best clue. • Progressive infection
• Central Hearing Loss: • Symptoms
– Hearing for pure tones often normal, – Pain
problem is between cochlea and cortex
– Increased pruritus
(receptor cells OK but a transmission or
processing problem). • Signs
– Erythema
– Increasing edema
– Canal debris, discharge
AOE: Severe Stage
• Severe pain, worse with ear movement
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• Signs • Hearing loss (if lesion occludes canal)
– Lumen obliteration Furunculosis: Signs
– Purulent otorrhea • Edema
– Involvement of periauricular soft tissue • Erythema
AOE: Treatment
• Tenderness
• Most common pathogens: P.
• Occasional fluctuance
aeruginosa and S. aureus
Furunculosis: Treatment
• Four principles
• Local heat
– Frequent canal cleaning
– Topical antibiotics
• Analgesics

– Pain control • Oral anti-staphylococcal antibiotics

– Instructions for prevention • Incision and drainage reserved for

Chronic Otitis Externa (COE)
• Chronic inflammatory process
• Persistent symptoms (> 2 months)
• Bacterial, fungal, dermatological
etiologies
COE: Symptoms
• Unrelenting pruritus
• Mild discomfort
localized abscess
• IV antibiotics for soft tissue extension
Otomycosis
• Fungal infection of EAC skin
• Primary or secondary
• Most common organisms: Aspergillus and
Candida
Otomycosis: Symptoms
• Often indistinguishable from bacterial OE

• Dryness of canal skin • Pruritus deep within the ear

COE: Signs • Dull pain
• Asteatosis
• Hearing loss (obstructive)
• Dry, flaky skin
• Hypertrophied skin
• Tinnitus
Otomycosis: Signs
• Mucopurulent otorrhea (occasional) • Canal erythema
COE: Treatment
• Mild edema
• Similar to that of AOE
• White, gray or black fungal debris
• Topical antibiotics, frequent cleanings Otomycosis
Otomycosis: Treatment
• Topical Steroids
• Surgical intervention
• Thorough cleaning and drying of canal

– Failure of medical treatment • Topical antifungals

– Goal is to enlarge and resurface the Granular Myringitis (GM)

EAC
• Localized chronic inflammation of pars
Furunculosis tensa with granulation tissue
• Acute localized infection • Toynbee described in 1860

• Lateral 1/3 of posterosuperior canal • Sequela of primary acute myringitis,

previous OE, perforation of TM
• Obstructed apopilosebaceous unit
• Common organisms: Pseudomonas,
• Pathogen: S. aureus Proteus
Furunculosis: Symptoms
• Localized pain GM: Symptoms

• Pruritus • Foul smelling discharge from one ear

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• Often asymptomatic
• Slight irritation or fullness
• No hearing loss or significant pain
GM: Signs
• TM obscured by pus
• “peeping” granulations
• No TM perforations
GM: Treatment
• Careful and frequent debridement
• Topical anti-pseudomonal antibiotics
• Occasionally combined with steroids
• At least 2 weeks of therapy
• May warrant careful destruction of
granulation tissue if no response
Bullous Myringitis
• Viral infection
• Confined to tympanic membrane
• Primarily involves younger children
Bullous Myringitis: Symptoms
• Sudden onset of severe pain
• No fever
• No hearing impairment
• Bloody otorrhea (significant) if rupture
Bullous Myringitis: Signs
• Inflammation limited to TM & nearby
canal
• Multiple reddened, inflamed blebs
• Hemorrhagic vesicles
Bullous Myringitis: Treatment
• Self-limiting
• Analgesics
• Topical antibiotics to prevent secondary
infection
• Incision of blebs is unnecessary
Necrotizing External Otitis(NEO)
• Potentially lethal infection of EAC and
surrounding structures
• Typically seen in diabetics and
immunocompromised patients
• Pseudomonas aeruginosa is the usual
culprit
NEO: History
• Meltzer and Kelemen, 1959
• Chandler, 1968 – credited with naming
NEO: Symptoms
• Poorly controlled diabetic with h/o OE
• Deep-seated aural pain
• Chronic otorrhea
• Aural fullness
NEO: Signs
• Inflammation and granulation
• Purulent secretions
• Occluded canal and obscured TM
• Cranial nerve involvement
NEO: Imaging
• Plain films
• Computerized tomography – most used
• Technetium-99 – reveals osteomyelitis
• Gallium scan – useful for evaluating Rx
• Magnetic Resonance Imaging
NEO: Diagnosis
• Clinical findings
• Laboratory evidence
• Imaging
• Physician’s suspicion
• Cohen and Friedman – criteria from review
NEO: Treatment
• Intravenous antibiotics for at least 4
weeks – with serial gallium scans monthly
• Local canal debridement until healed
• Pain control
• Use of topical agents controversial
• Hyperbaric oxygen experimental
• Surgical debridement for refractory cases
NEO: Mortality
• Death rate essentially unchanged despite
newer antibiotics (37% to 23%)
• Higher with multiple cranial neuropathies
(60%)
• Recurrence not uncommon (9% to 27%)
• May recur up to 12 months after
treatment
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Perichondritis/Chondritis
• Infection of perichondrium/cartilage
• Result of trauma to auricle
• May be spontaneous (overt diabetes)
Perichondritis: Symptoms
• Pain over auricle and deep in canal
• Pruritus
Perichondritis: Signs
• Tender auricle
• Induration
• Edema
• Advanced cases
– Crusting & weeping
– Involvement of soft tissues
Relapsing Polychondritis
• Episodic and progressive inflammation
of cartilages
• Autoimmune etiology?
• External ear, larynx, trachea, bronchi,
and nose may be involved
• Involvement of larynx and trachea
causes increasing respiratory obstruction
Relapsing Polychondritis
• Fever, pain
• Swelling, erythema
• Anemia, elevated ESR
• Treat with oral corticosteroids
Herpes Zoster Oticus
• J. Ramsay Hunt described in 1907
• Viral infection caused by varicella
zoster
• Infection along one or more cranial
nerve dermatomes (shingles)
• Ramsey Hunt syndrome: herpes zoster
of the pinna with otalgia and facial paralysis
Herpes Zoster Oticus: Symptoms
• Early: burning pain in one ear,
headache, malaise and fever
• Late (3 to 7 days): vesicles, facial
paralysis
Herpes Zoster Oticus: Treatment
• Corneal protection
• Oral steroid taper (10 to 14 days)
• Antivirals
Erysipelas
• Acute superficial cellulitis
• Group A, beta hemolytic streptococci
• Skin: bright red; well-demarcated,
advancing margin
• Rapid treatment with oral or IV antibiotics
if insufficient response
Perichondritis: Treatment
• Mild: debridement, topical & oral antibiotic
• Advanced: hospitalization, IV antibiotics
• Chronic: surgical intervention with
excision of necrotic tissue and skin coverage
Radiation-Induced Otitis Externa
• OE occurring after radiotherapy
• Often difficult to treat
• Limited infection treated like COE
• Involvement of bone requires surgical
debridement and skin coverage
OTITIS MEDIA
A SPECTRUM OF DISEASE
• acute otitis media
• chronic otitis media with effusion
• atelectasis of the tympanic membrane
• chronic adhesive otitis media
• chronic suppurative otitis media
– tubotympanic (“safe”)
– atticoantral (“unsafe”)
and may be a continuum of disease
ACUTE OTITIS MEDIA
• the presence of a middle-ear effusion
• signs and symptoms of infection
– fever, irritability, pain, otorrhoea
Management of AOM
• Pain relief
• Decongestants (oral/topical) and
antihistamines do not make the eustachian tube
function better
• Decongestants do relieve the symptoms of
a blocked nose
Antibiotic therapy
• Standard spectrum (sensitive to β-
lactamase)
– penicillin, erythromycin, ampicillins
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• Extended spectrum
– amoxicillin/clavulanate,
trimethoprim/sulfamethoxazole
Antibiotic therapy
• Recommended treatment is:
amoxicillin 50mg/kg/day in 3 doses
– Can give up to 100mg/kg/day
– Continue for 5 days
• If no improvement in 2 days change to
amoxicillin/clavulanate
Penicillin allergy
• trimethoprim-sulfamethoxazole
• clindamycin
• ceftriaxone IM, but will often need
continuing oral medication
Antibiotic therapy
• if severe symptoms
- pain
- perforation
(use topical as well, e.g. Ciprofloxacin HC iii
drops tds for 3 days)
• ≤ 2 years of age
• immune deficiency
• follow-up not possible
CHRONIC OTITIS MEDIA WITH EFFUSION
• the presence of a middle ear effusion
• asymptomatic apart from some hearing
loss
CHRONIC SUPPURATIVE OTITIS MEDIA
“deafness and discharge”
• persistent disease
• insidious onset
• severe destruction
• irreversible sequelae
1. tubotympanic disease (“safe”)
central perforation
2. atticoantral disease (“unsafe”)
cholesteatoma
the presence of keratinising squamous
epithelium in the middle ear
PRESENTATIONS OF MIDDLE EAR DISEASE
PAIN
(Otalgia)
DIFFERENTIAL DIAGNOSIS OF EAR PAIN
A. External auditory canal
• trauma ( e.g. from cotton bud abuse)
• auricular haematoma
• foreign body
• otitis externa
• external auditory canal tumour
DIFFERENTIAL DIAGNOSIS OF EAR PAIN
B. Middle ear
• acute otitis media
• bullous myringitis
• chronic suppurative otitis media
• middle ear tumour
DIFFERENTIAL DIAGNOSIS OF EAR PAIN
C. Referred pain
– oropharynx (IXth nerve)
• tonsillitis/post-tonsillectomy
• carcinoma, including posterior tongue
– laryngopharynx (Xth nerve)
• pyriform fossa
– upper molar teeth, TMJ, parotid gland (Vc)
• impacted wisdom teeth
• changes to bite from new dentures
– cervical spine (C2, C3)
• pain is often worse at night
DISCHARGE
(Otorrhoea)
HEARING LOSS
FACIAL PARALYSIS
HEADACHE
VERTIGO
TINNITUS
Meniere’s Disease?
 What is Meniere’s Disease?
• In 1861 Prosper Meniere described a
syndrome characterized by deafness, tinnitus,
and episodic vertigo. He linked this condition to a
disorder of the inner ear.
• In 1938 Hallpike and Cairns described the
underlying pathology of Meniere’s disease as
being endolymphatic hydrops but the precise
etiology still remains elusive.
 Possible Causes
• Anatomical-abnormalities
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• Genetic-autosomal dominant
• Immunological-immune complex
deposition
Symptoms
Periodic episodes of rotatory vertigo or
dizziness
Fluctuating, progressive, low-frequency
hearing loss
Tinnitus
Fullness/pressure
Diagnosis
• The diagnosis of Meniere disease is
made based on a careful history and physical
exam.
• If the work-up is normal and the classic
symptoms continue, the diagnosis of Meniere
disease is made.
History
• Most important part of the diagnosis
• Pattern of symptoms
• Association between hearing loss,
tinnitus, and vertigo
Physical Examination
• Examination results vary, depending
upon the phase of disease. During remission,
physical examination findings may be
completely normal, particularly if the patient is
symptom free.
• During an acute attack, the patient has
severe vertigo.
• Patients are sometimes diaphoretic and
pale.
• Vital signs may show elevated blood
pressure, pulse, and respiration.
• Spontaneous nystagmus directed toward
affected ear is typical during an acute attack.
Physical Examination (con’t)
• The Romberg test generally shows
significant instability and worsening when the
eyes are closed.
• The Weber tuning fork test usually
lateralizes away from the affected ear.
• The Rinne test usually indicates that air
conduction remains better than bone conduction.
• Complete neurologic evaluation is
important. New-onset vertigo might be an early
sign of stroke, migraine, or brainstem
compression that may require emergent
evaluation and care.
Lab studies
• No lab studies are specific for Meniere
disease.
• A CBC, urinalysis, chemistry panel, and
alcohol and drug screening may be helpful if
other causes are considered.
• If an infectious cause is suspected,
consider blood cultures, urine culture, and a
cerebral spinal fluid (CSF) examination.
Imaging Studies
• Magnetic resonance imaging
- Brain scan should be done to rule
out abnormal anatomy or mass lesions.
Specifically, acoustic neuromas or other
cerebellopontine angle lesions are sought.
Other lesions, such as multiple sclerosis or
Arnold-Chiari malformations, also can be
ruled out.
- Note that mass lesions rarely are
found but are important to exclude.
• CT scans reveal dehiscent superior
semicircular canals and/or widened cochlear and
vestibular aqueducts
Other tests
• Audiometry is particularly helpful to
document present hearing acuity and to detect
future change.
-The patient may not notice a loss at
specific frequencies. Low-frequency or mixed low-
and high-frequency insufficiency may be
observed.
- Typically, the lower frequencies are
affected more severely. This is due to preferential
sensitivity of the apex to the hydrops.
- Multiple hearing tests, which document
fluctuating hearing loss, are helpful in diagnosing
Ménière.
Transtympanic electrocochleography
(ECOG)
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• Transtympanic electrocochleography
(ECOG) specifically detects distortion of the
neural membranes of the inner ear.
• This is presumably due to perilymph
pressure fluctuations and can show evidence
of cochlear involvement.
• ECOG measures the ratio of the
summating potential (probably from the
movement of the basilar membrane) and the
nerve action potential in response to auditory
stimuli. Hydrops is suggested when this ratio is
greater than 35%.
• This is most accurate when Ménière is
active.
Electronystagmography (ENG)
• Electronystagmography (ENG) is a test
of the inner ear function (particularly the
semicircular canals).
• It tests central and peripheral function
and can help localize the site of lesion.
• Typically, Meniere disease causes a
reduced vestibular response in the affected
ear, although response may be increased
secondary to an irritative lesion.
• The direction of the spontaneous
nystagmus during or after an attack of Ménière
is not a reliable indicator of the site of the
lesion. An irritative phase may occur during
the attack (fast phases directed toward
involved ear) followed by a paretic phase (fast
phases directed toward opposite ear).
Differential Diagnosis
• The differential diagnosis is broad and
includes:
perilymph fistula, recurrent
labyrinthitis, otosclerosis, migraine , congenital
ear malformations of many kinds,viral
meningitis, viral encephalitis, neurosyphilis,
stroke, tumors, trauma, autoimmune
disorders, MS, etc.
Treatment
• Medical therapy is both symptomatic
(ie, acute attacks) and prophylactic.
• If Ménière is due to a secondary cause
(ie, Ménière syndrome), primary first-line
management is the diagnosis and treatment of
the primary disease (eg, thyroid disease).
• Vestibulosuppressants (eg, meclizine)
decrease symptoms, but generally only mask
the vertigo by decreasing the brain's response
to vestibular input.
• Diuretics or diuretic-like medications
(eg, hydrochlorothiazide) actually decrease the
fluid pressure load in the inner ear. These
medications help prevent attacks but do not help
once an acute attack has started.
• Anti-inflammatory properties of steroids
are helpful in endolymphatic hydrops. This is
probably due to reduced endolymphatic pressure.
Steroids actually can reverse vertigo, tinnitus,
and hearing loss.
Treatment Cont’d
• Aminoglycosides are a class of antibiotics
that were discovered serendipitously to be
preferentially toxic to the vestibular end organ.
– Destruction of the vestibular end organ
renders the brain insensitive to the fluctuations in
the inner ear pressure during an acute Ménière
attack.
– If given systemically, aminoglycosides
affect both ears.
– Although these drugs can be used to treat
extremely severe bilateral Ménière disease, they
leave the patient with little or no balance
function. The resulting Dandy syndrome, a
complete loss of inner ear function, can be
debilitating.
• During the quiescent phase, medical
treatment of Ménière disease is tailored to each
patient. Lifestyle and dietary changes are usually
the first step. Avoiding trigger substances (eg,
caffeine) alone may be sufficient. Smoking
cessation also is recommended.
• In an acutely vertiginous patient,
management is directed toward vertigo control.
– Intravenous (IV) or intramuscular (IM)
diazepam provides excellent vestibular
suppression and antinausea effects.
– Steroids can be given for anti-
inflammatory effects in the inner ear.
– IV fluid support can help prevent
dehydration and replaces electrolytes.
• Surgical Care:
– Surgical therapy for Ménière disease is
reserved for medical treatment failures and is
otherwise controversial.
– Surgical procedures are divided into 2
major classifications as follows:
• Destructive surgical procedures
• Nondestructive surgical procedures
• Destructive surgical procedures
– Rationale to control vertigo:
Endolymphatic hydrops causes fluid pressure
accumulation within the inner ear, which causes
temporary malfunction and misfiring of the
vestibular nerve. These abnormal signals cause
vertigo. Destruction of the inner ear and/or the
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vestibular nerve prevents these abnormal
signals. As long as the opposite inner ear and
vestibular apparatus function normally, the
brain eventually will compensate for the loss of
one labyrinth.
Destructive surgical procedures Cont’d
• Problems with destructive procedures:
– Destruction of one inner ear depends
on the adequate function of the opposite ear.
Unfortunately, Ménière disease can be
bilateral (7-50%), in which case this method is
contraindicated. Since balance and hearing are
closely intertwined within the labyrinth,
destruction of the balance portion carries a
high risk of hearing loss. Note that destructive
procedures are irreversible and reserved for
severe cases.
• Nondestructive surgical procedures:
– These are directed toward improving
the state of the inner ear. They are less
invasive than destructive procedures and do
not preclude the use of other treatment
modalities. Discussion here is limited to the 4
most generally accepted management
options:
 endolymphatic sac decompression or
shunt
 vestibular nerve section
 Labyrinthectomy
 transtympanic medication perfusion.
• Endolymphatic sac decompression
and/or shunt
– In theory, the endolymphatic sac
procedure decreases endolymph pressure
accumulation by removing the petrous bone,
which encases the endolymph reservoir. This
procedure allows the reservoir sac to expand
more freely, thus dissipating pressure. A drain
or valve from the endolymphatic space to
either the mastoid or subarachnoid space can
be inserted as another means of further
reducing pressure.
– Success rates (in terms of controlling
vertigo and stabilizing hearing acuity) with this
procedure are reported at 60-80%.
• Vestibular nerve section
– For patients with useful hearing in the
affected ear, sectioning the diseased
vestibular nerve can be the ultimate solution.
– Although the hearing and balance
functions are housed in one common chamber
within the inner ear, their neural connections
to the brain separate into distinct nerve
bundles as they course through the internal
auditory canal.
– This anatomical separation allows balance
function to be isolated and ablated without
affecting hearing function.
• Labyrinthectomy
– This management option for Ménière
disease has the advantage of a high cure rate
(>95%) and is useful in the patient whose
hearing on the diseased side has been destroyed
already by Ménière disease.
– Labyrinthectomy involves ablation of the
diseased inner ear organs.
– This procedure is less complex than
vestibular nerve section because labyrinthectomy
does not require entry into the cranial cavity.
– Labyrinthectomy is less invasive than
vestibular nerve section.
• This procedure carries less danger of
cerebrospinal fluid leak and meningitis since
craniotomy is not required.
• Like those who undergo vestibular nerve
section, patients require a few days of inpatient
care.
• Accommodation to the surgical loss of one
vestibular apparatus usually takes weeks or
months.
• Vestibular rehabilitation during this time
period is also helpful.
• Transtympanic perfusion of
medication
– Medications for Ménière disease are
applied through a myringotomy within the middle
ear cavity, where they presumably are absorbed
through the round window membrane into the
inner ear.
– Transtympanic perfusion is a relatively
low-risk, simple procedure that applies a high
concentration of medicine with minimal systemic
effects.
• Diet:
– Dietary management is appropriate in
patients not severely affected; patients avoid
substances that may trigger or exacerbate fluid
pressure buildup in the inner ear.
– Similar to managing systemic
hypertension, the goal for Ménière disease is to
reduce the total body fluid volume. This, in turn,
may reduce the inner ear fluid volume.
– Since sodium seems to play a major role in
fluid retention within the inner ear, avoiding salt
(eg, pizza, preserved foods, smoked fish) is
paramount.
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• Consult with a nutritionist to establish a
rigid salt-restricted diet (1.5 g sodium per
day).
• Avoiding other trigger substances (eg,
caffeine, nicotine, alcohol, high-carbohydrate
substances, high-cholesterol/triglyceride
foods) also can help.
• Note that many preserved and smoked
foods contain sodium nitrite, which can
contribute to high sodium content.

• Activity:
– Endolymphatic hydrops does not
preclude regular activity. Exercise is
recommended in moderation.
– Because of the unpredictable nature of
the disease, balance-intensive, dangerous
tasks (eg, especially climbing ladders) should
be avoided.

Prognosis
• Prognosis is variable, since the disease
pattern of exacerbation and remission makes
evaluation of treatment and prognosis difficult
to predict.
– In general, Ménière symptoms tend to
stabilize spontaneously with time. With regard
to vertigo, about half of patients stabilize over
several years.
– Patients tend to "burn out" over time
and with residual poor balance and hearing.

• Ménière disease can be classified into

several stages of progression. Early stages
involve cochlear hydrops, which proceeds to
affect the vestibular system.
– Ménière disease is most bothersome
during these early stages.
– As patients progress to later stages, the
hydrops fills the vestibule so completely that
no further room is available for pressure
fluctuation and the vertigo spells disappear.
– The acute attacks are replaced by
constant imbalance and progressive hearing
loss.

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