Meniérè’s Disease

Meniérè’s disease is a clinical syndrome that consists of four symptoms:

1. Episodes of severe, incapacitating vertigo lasting on the order of several minutes to a few hours (usually 30 minutes to 8 hours or so). The vertigo is usually a sensation of spinning, but can also be a feeling of being pushed or pulled (pulsion).  In rare forms of Meniérè’s disease, patients experience sudden “drop-attacks” which cause them to fall suddenly to the ground without warning and without loss of consciousness (the so-called otolithic crisis of Tumarkin). These attacks last only for a few seconds, but because of their unpredictability and severity are potentially the most devastating amongst all forms of Meniérè’s disease.
2. Fluctuating, slowly progressive hearing loss— the hearing loss is of a “sensorineural” type, arising in the inner ear. The hearing classically will worsen during a vertigo attack, and may improve after resolution of the acute symptoms.
3. Episodic tinnitus (abnormal perception of sound in the ear; usually a roaring, buzzing or ringing)— there is frequently a baseline tinnitus in the ear, but this typically worsens temporarily with a vertigo attack.
4. Aural fullness— a sensation of plugging or clogging in the ear that worsens when a vertigo attack begins.

As emphasized above, more important than the presence of these 4 symptoms in a single patient, is the pattern in which they occur.  Many patients with ear problems will have one or all of these symptoms at some point.  Patients with Meniérè’s will have all of them (or at least 2-3 of them) come on together in distinct episodes.

Meniérè’s attacks are usually very distinct. Patients with Meniérè’s disease will typically remember the first attack they had, and can catalogue each of the distinct episodes as they occur.  This differs from many other types of vertigo and balance disorders in which the symptoms are more vague and the episodes less distinct. In between the episodes, most Meniérè’s patients feel well, though they can have significant disability from the uncertainty of when the next attack will come on.

What causes Meniérè’s disease?

The root cause of Meniérè’s disease is unknown, though the symptoms are thought to be produced by an increase in the fluid pressure in the inner ear, i.e “endolymphatic hydrops.”  In all likelihood, a variety of insults to the ear can lead to endolymphatic hydrops as their common final pathway, thereby producing symptoms akin to those of Meniérè’s disease. When the inciting cause of hydropic symptoms is identified, then the proper descriptor is Meniérè’s syndrome or delayed endolymphatic hydrops.  When the symptoms develop spontaneously, with no identifiable cause, it is termed Meniérè’s disease.

What is the natural history of Meniérè’s disease?

 At least half of all patients with newly diagnosed Meniérè’s disease will have remission of their symptoms in the first few years. There is some evidence suggesting that prompt initiation of treatment can prevent progression to a more long-term course. When the disease persists, progressive inner ear damage results in worsening hearing in the affected ear, but a decrease in the frequency and severity of vertigo attacks. Tumarkin crises (drop-attacks) may ensue in the end-stages of the disease, and are an indication for prompt intervention to prevent serious injury.

 What is the treatment for Meniérè’s disease?

 The mainstay of treatment is directed towards attempts to decrease the fluid pressure in the inner ear. This is done by aggressive salt-restriction, sometimes in combination with a diuretic (“water pill”). A diuretic alone will not overcome the inner ear’s ability to retain salt, so this medication should be reserved for patients in whom salt-restriction alone is insufficient. It is important not to decrease salt intake too much, as sodium is an essential mineral for the body to function.  However, in practice this is not too much of a concern since most people find any sodium restriction to be a greater challenge than over-restriction. The goal is to reduce your daily sodium intake to 1500-2000 milligrams. This involves more than not sprinkling salt on your food.  It requires diligence in precisely measuring your sodium intake from all sources by inspecting package labels and kitchen habits.  Restaurant eating must usually be limited since it is difficult to accurately quantify sodium intake in that setting, and the foods are typically highly salted.

Some guidelines for maintaining a low-salt diet are as follows:

1.   Do not add salt to food or cooking. If this is too difficult at first, try halving the amount of salt you add to recipes and at the table.  If you slowly work your way down it will be much easier.  Also, potassium containing salt substitutes are okay, and may be used if desired.

2.   Restrict salt (sodium) intake to between 1500 milligrams (mg) to 2000 mg daily

3.      Avoid high-salt (sodium) foods (see table)

4.      Drink 6-8 glasses of fresh (unsoftened) water per day

Table: Guidelines for a low-salt diet

During the severe, episodic attacks medications may be used to suppress the vertigo and nausea.  Diazepam (Valium) works well. Another oral drug that is commonly used is meclizine (Antivert).  Both of these are sedating. One problem with these medications for an acute attack is that if nausea is severe they can be impossible to keep it down.  In this circumstance antihistamine suppositories such as promethazine (Phenergan) are very useful. It is important to reserve these vestibular suppressants for the acute attacks of vertigo. When used long-term they impair the body’s ability to recover from inner ear injuries, and can produce chronic imbalance.

Surgery for Meniérè’s disease

When medical therapy fails to control the vertigo associated with Meniérè’s disease, surgical intervention should be considered. Surgical options should be divided into those that preserve residual hearing in the affected ear, and those that destroy it.  The latter are typically more reliable in their ability to control vertigo, but should only be undertaken if the residual hearing is minimal or not useful, and if the other ear has useful hearing and is not expected to become more severely affected. There are many other considerations that go into choosing what type of procedure is best for each person.  Some of the more commonly performed procedures are: chemical perfusion of the inner ear (“Gentamicin injection;” this can be performed in the office and is easily repeated if need be), endolymphatic sac surgery, vestibular nerve section and transmastoid labyrinthectomy. The pros and cons of each of these procedures should be discussed in detail with the physician who will perform the treatment so as to choose the option that is best for each individual.

Table: Comparison of procedures commonly used to control vertigo

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