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In 1861, the French physician, Prosper Mèniére, described a condition
that now bears his name. Meniere's disease is a disorder of the inner
ear that causes episodes of vertigo, ringing in the ears (tinitus),
a feeling of fullness or pressure in the ear, and fluctuating hearing
loss.
A typical attack of Meniere's disease is preceded by fullness in one
or both ears. Hearing fluctuation or changes in tinnitus may also precede
an attack. A Meniere's disease episode generally involves severe vertigo
(spinning), imbalance, nausea, and vomiting. The average attack lasts
two to four hours. Following a severe attack, most people find that
they are exhausted and must sleep for several hours.
Meniere's disease episodes may occur in clusters in which several
attacks occur within a short period of time. In others, years may pass
between episodes. Between the acute attacks, most people are free of
symptoms or note mild imbalance and tinnitus.
Meniere's disease affects roughly 0.2 percent of
the population. In two-thirds of the cases, Meniere's disease is
confined to one ear, while in the other third, both ears are involved.
In most cases, a progressive hearing loss occurs in the affected ear(s).
Although an acute attack can be incapacitating, the disease itself is
not fatal.
An acute attack of Meniere's disease is believed to result from fluctuating
pressure of the fluid inside of the inner ear. In some cases, the endolymphatic
duct may be obstructed by scar tissue, or may be narrow from birth. Abnormally
enlarged fluid pathways into the ear such as the vestibular aqueduct or
cochlear aqueduct may also be associated with Meniere's disease-like symptoms.
Recently attention has been focused on the immunologic function of
the endolymphatic sac -- may contribute to a substantial percentage of Meniere's disease. However,
for the most part the underlying cause of Meniere's
disease is unknown. It is most often attributed to viral infections
of the inner ear, head injury, a hereditary predisposition, or food
allergy.
The periodic admixture of perilymph and endolymph frequently kills hair
cells in the inner ear. This is a gradual process occurring over years,
but frequently results in unilateral deafness. Cochlear (hearing) hair
cells are the most sensitive. Vestibular hair cells seem more resilient.
Mechanical disruption is also likely an effect with dilation of the utricle
and saccule of the ear being a well known pathological finding. This may
result in the gradual onset of a chronic unsteadiness, even when patients
are not having attacks, and is also a reasonable explanation for periodic
attacks of benign paroxysmal positional
vertigo (BPPV). Finally, it also seems likely that there may be rupture
of the suspensory system for the membranous labyrinth. No evidence exists
that Meniere's disease kills the cochleovestibular nerve (see Kitamura
et al, 1997).
Studies have shown that the disease affects approximately
200 out of 100,000 people. The majority of people with Meniere's disease
are more than 40 years old, with equal distribution between males and
females.
Currently no cure is available for Meniere's disease, but there are ways
to manage the condition and help you control symptoms.
Diagnosis is based on a combination of the correct set of symptoms (usually
episodic dizziness and hearing disturbance), hearing tests which document
that hearing is reduced after an attack, and then gets better, and exclusion
of alternative causes. The process of diagnosis usually includes hearing
testing, an electronystagmography (ENG) test, several blood tests, and
a magnetic resonance imaging (MRI) scan of the head.
- meclizine (for example, Antivert and Bonine), chewable
- lorazepam (for example, Ativan and Loraz/Intensl) 0.5 mg, can be taken under the tongue
- promethazine (for example, Phenergan and Promethacon) (orally or suppository)
- prochlorperazine (for example, Compazine and Cotranzine) (orally or suppository)
- dexamethasone (for example, Decadron and Decarex) 4 mg orally for four days
During an acute attack, lay down on a firm surface. Stay as motionless
as possible, with your eyes open and fixed on a stationary object. Do
not try to drink or sip water, as you'd be very likely to vomit. Stay
like this until the severe vertigo (spinning) passes, then get up SLOWLY.
After the attack subsides, you'll probably feel very tired and need to
sleep for several hours.
If vomiting persists and you are unable to drink fluids for longer
than 24 hours (12 hours for children), contact your doctor. He or she
can prescribe nausea medication, and/or vestibular suppressant medication.
Your physician may want to see you. Meclizine (for example, Antivert and Bonine) and lorazepam (for example, Ativan and Loraz/Intensl) are commonly used
vestibular suppressant medications and prochlorperazine (for example, Compazine and Cotranzine) or
promethazine (for example, Phenergan and Promethacon) are commonly used medications for nausea.
Medications Used Between Attacks
- Diuretics
- triamterene/hydrochlorothiazide (for example, Dyazide and Triamter/HCTZ)
- Vestibular Suppressants
- lorazepam (for example, Ativan and Loraz/Intensl) 0.5 mg twice a day or as needed
- clonazepam (Klonapin) 0.5 mg twice a day or as needed
- diazepam (for example, Valium and Valrelease) 2 mg twice a day or as needed
- meclizine (for example, Antivert and Bonine) 12.5 mg to 25 mg as needed
- Calcium Channel Blockers
- verapamil (for example, Calan and Covera-HS)
- flunarizine/cinnarizine
- nimodipine (Nimotop)
- Steroids (rarely)
- dexamethasone (for example, Decadron and Decarex)
- prednisone
- Immune suppressants
Between attacks, medication may be prescribed to help regulate the fluid
pressure in your inner ear, thereby reducing the severity and frequency
of the Meniere's disease episodes. Triamterene/hydrochlorothiazide (for example, Dyazide and Triamter/HCTZ) is the most common medication for this purpose. Neptazine can be used when response to triamterene/hydrochlorothiazide (for example, Dyazide and Triamter/HCTZ) isn't adequate. Verapamil (for example, Calan and Covera-HS) (typical dose: 120 SR) sometimes
reduces the frequency of attacks. Some physicians prescribe Histamine injections. Prednisone or other steroids (for example,
dexamethasone) are occasionally helpful in short bursts. Vestibular
suppressants such as meclizine (for example, Antivert and Bonine) or lorazepam (for example, Ativan and Loraz/Intensl) are used on an as-needed basis.
Many new medications are available that have
not been extensively tested and can be tried under the supervision of
your doctor. In addition, some unusual medications
exist that are either considered alternative or are available only outside
the U.S. that might be worth considering.
The hydrops diet will probably be recommended.
This is an important part of treatment for virtually all patients with
Meniere's disease. Experience has shown that STRICT adherence to this
dietary regimen will result in significant improvement in symptoms for
most patients.
The purpose of treatment between attacks is to prevent or reduce the
number of episodes and decrease the chances of further hearing loss.
A permanent tinnitus (ringing in the ears) or a progressive hearing
loss may be the consequence of long-term Meniere's disease. Hearing
aids may be necessary.
In extremely severe cases, treatments that deaden the inner ear such as
gentamicin injections or surgery may be considered .This is a last resort for persons who have severe,
disabling attacks. We favor the use of gentamicin for most instances in
which destructive treatments are being considered. Injections
of gentamicin are given through the ear drum, through a small tube. This
procedure allows one to treat one side alone, without affecting the other.
Typically about four injections are given, during a thirty day period.
Dizziness may reoccur one year later, requiring another series. Although
intratympanic steroids injections have also been recently used to treat
Meniere's disease by Shea, the consensus is that this treatment has not
been clearly proven effective or to have a reasonable scientific basis.
Alternatively, a surgical treatment is used in which the vestibular
nerve is clipped. This operation, called a vestibular neurectomy or
vestibular nerve section is very effective in eliminating vertigo. Again,
at our institution, Dr. Alan Micco has experience with this surgery.
Another operation, called a labyrinthectomy is recommended for persons
who have lost all usable hearing or in whom vestibular nerve section
is considered too dangerous.
A third procedure, the endolymphatic shunt procedure, is used by some
doctors to relieve pressure in the inner ear. Unfortunately, according
to most studies, the shunt procedure does not appear to be better than
doing nothing. This may be because the shunt can easily get plugged
up. We do not recommend this procedure for our patients. Shunt surgery
is still somewhat controversial, and some authors continue to feel that
there is a place for it (Pensak and Friedman, 1998).
Surgical treatments have not been shown to preserve hearing to any
greater extent than medical treatments (Kinney et al, 1996). A review
of surgical treatment has recently been published by LaRouere (see references).
Since the acute symptoms of Meniere's disease are episodic, it is important
to explain to your family and friends what might happen when you have
an attack. Then, if the symptoms occur when they are present, they will
understand, be able to help you, and not be overly frightened.
You may be able to protect yourself from injury if you feel that an
attack is about to begin. Some attacks may occur during the night, so
be sure you have a night light on; you'll be relying more on vision
to help maintain your balance. You will want to make sure that the path
to the bathroom is free of throw rugs, furniture or other obstructions.
Many studies have documented that patients with Meniere's disease
tend to have more psychological disability than the normal population,
possibly including depression and/or anxiety, in reaction to their disease.
It may be necessary to take antidepressants or antianxiety drugs, under
the supervision of an appropriate health care professional.
Hydrops Diet
The fluid-filled hearing and balance structures of the inner ear normally
function independently from the body's overall fluid/blood system. In
a normal inner ear, the fluid is maintained at a constant volume and contains
specific concentrations of sodium, potassium, chloride and other electrolytes.
This fluid bathes the sensory cells of the inner ear and allows them to
function normally. With injury or degeneration of the inner ear structures,
independent control is lost, and the volume and concentrations of the
inner ear fluid fluctuate with changes in the body's fluid/blood. This
fluctuation causes the symptoms of hydrops--pressure or fullness in the
ears, tinnitus (ringing in the ears), hearing loss, dizziness and imbalance.
Your inner ear fluid is influenced by certain substances in your blood
and other body fluids. For instance, when you eat foods that are high
in salt or sugar, your blood level concentration of salt or sugar increases,
and this, in turn, affects the concentration of substances in your inner
ear.
People with certain balance disorders must control the amount of salt
and sugar that is added to food. You must also become aware of the hidden
salts and sugars that foods contain. Limiting or eliminating the use
of caffeine and alcohol will also help to reduce symptoms of dizziness
and ringing in the ears.
The goal of treatment is to provide stable body fluid/blood levels so
that secondary fluctuations in the inner ear fluid can be avoided.
- Distribute your food and fluid intake evenly throughout the day
and from day-to-day. Eat approximately the same amount of food at
each meal and do not skip meals. If you eat snacks, have them at regular
times.
- Avoid eating foods or fluids that have a high salt or sugar content.
High salt or sugar levels in the diet result in fluctuations in the
inner ear fluid pressure and may increase your symptoms. Aim for a
diet high in fresh fruits, vegetables, and whole grains and low in
canned, frozen, or processed foods. A one-gram sodium intake diet
is usually what we recommend.
- Drink adequate amounts of fluid daily. This should include water,
milk, and low-sugar fruit juices (for example, cranberry or cranapple).
Try to anticipate fluid loss that will occur with exercise or heat
and replace these fluids before they are lost. Be cautious about milk
intake -- some individuals have a food allergy and get symptoms from
milk products.
- Avoid caffeine-containing fluids and foods (such as coffee, tea
and chocolate). Caffeine has stimulant properties that may make your
symptoms worse. Caffeine also may make tinnitus louder. Large amounts
of caffeine may trigger migraine (migraine can be difficult to separate
diagnostically from Meniere's disease). Chocolate is also a migraine
trigger.
- Limit your alcohol intake to one glass of beer or wine each day.
Alcohol may trigger migraine-associated vertigo.
- Avoid foods containing monosodium glutamate (MSG). This is often
present in pre-packaged food products and Chinese food. It may increase
symptoms in some patients, possibly because of the link to migraine-associated
vertigo.
- Avoid aspirin (for example, Bayer and Bufferin) and medications that contain aspirin.
Aspirin can cause tinnitus (abnormal noise in the ear).
Nonsteroidal anti-inflammatory agents such as ibuprofen (for example, Advil and Motrin)
or naproxen (for example, Anaprox and Naprelan) should also be avoided when practical.
- Avoid caffeine-containing medications. Caffeine can increase tinnitus
as well as stimulate the problems mentioned above under foods.
- Pay attention to the content of all over-the-counter medications
as well as drugs prescribed by other physicians. Some medications
may increase your symptoms.
- Avoid cigarettes. The nicotine present in cigarettes constricts
blood vessels and can decrease the blood supply to the inner ear,
making your symptoms worse.
Dietitians can help you select a nutritional program that meets your special
needs. They can also suggest ways to prepare your favorite foods for a
restricted-salt and low-sugar diet. With their assistance, you'll find
that modifying your eating habits can help you control the symptoms of
your balance disorder.
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