Meniere’s Disease

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❖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.
 Dilated membranous labyrinth
Normal membranous labyrinth in Meniere's disease (Hydrops)
➢Possible Causes
◼ Anatomical- ◼ Viral-serum IgE to
abnormalities herpes simples virus
◼ Genetic-autosomal types I and II,
dominant Epstein-Barr virus and
◼ Immunological-
CMV
immune complex ◼ Vascular-associated
deposition with migraines
◼ Metabolic-potassium
intoxication
 Age Distribution and Incidence
In the US: 50% of patients have a positive family history.
The estimated prevalence is 150 cases per 100,000 population

40’s and 50’s

Women>Men
 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)

◼ 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.
Treatment Cont’d

◼ 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.
Treatment Cont’d

◼ 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.
Treatment Cont’d

◼ 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.
Treatment Cont’d

◼ 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.
Treatment Cont’d

◼ 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
surgical procedures Cont’d

◼ 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 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.
surgical procedures Cont’d

◼ 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:
1. endolymphatic sac decompression or shunt
2. vestibular nerve section
3. Labyrinthectomy
4. transtympanic medication perfusion.
surgical procedures Cont’d

◼ 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%.
surgical procedures Cont’d

◼ 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.
surgical procedures Cont’d

◼ 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.
Labyrinthectomy Cont’d

◼ 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.
surgical procedures Cont’d

◼ 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.
Treatment Cont’d

◼ 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.
Diet Cont’d

◼ 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.
Treatment Cont’d

◼ 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.
Prognosis Cont’d

◼ 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.