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What Is Meniere’s Disease:

Meniere’s disease, also known as morbus meniere or Meniere’s syndrome, is an uncommon disorder of the inner ear. Modern medicine does not know why this disease develops or what actually causes it, but we do have a large amount of information about how the disorder progresses and ways to treat it. Since the exact cause is unknown (doctors say it is an idiopathic disease), there is no known cure for this disorder. The treatments are intended to reduce or prevent the symptoms (feelings or sensations produced by the disease) that are associated with Meniere’s disease: vertigo, (a particular form of dizziness), hearing loss, tinnitus (ringing in the ear), and a feeling of fullness in the ear (like the ear has to "pop," but can not). These four symptoms make up the cardinal features of Meniere’s disease, for it is defined as a disorder in which the patient suffers from two to four of these symptoms and no other cause has been found to produce these symptoms. Medically, Meniere’s disease is a "diagnosis of exclusion"; when every other cause for these symptoms has been ruled out, then the patient must be suffering from Meniere’s disease. If another reason can be found to account for the symptoms, the patient does not have Meniere’s.

Meniere’s disease is named after Dr Prosper Meniere, who in 1861 first described a peculiar disorder that he had seen a few times in which patients suffered from irregular episodes of dizziness, hearing loss, and ringing in their ears. These attacks came and went, and in between the patients did not have any symptoms. Since then, people who have all three of the above symptoms are said to have classic Meniere’s disease; it was much later on that other physicians noted that many Meniere’s sufferers reported a feeling of fullness in their ear at times. Although the disorder tends to affect only one ear, it can be occasionally seen to involve both ears, particularly with hearing loss.

Patients who do not have the three main symptoms are said to have "variant forms" of Meniere’s; they have at least two of the main symptoms, but not all three. Perhaps they never have ringing in their ears or do not notice any hearing loss, or their symptoms do not go away (in classic Meniere’s, patients have minimal or no symptoms between attacks). Some people may get the hearing loss, dizziness, and fullness, but not experience the whirling attacks of vertigo; others may get the vertigo and hearing loss but not the tinnitus.

Since Meniere’s disease usually has an irregular pattern of severity, different sufferers may experience different patterns of the disease or different intensity of symptoms. Some people develop minimal hearing loss, where as others may have almost complete deafness for a period of time in one ear. Most patients suffer symptoms in only one ear, or in one ear more than the other, but a few may have symptoms (bilateral Meniere’s) in both ears at the same time, or at differing times. There is no consistent pattern to Meniere’s: some people may always have a slight tinnitus and get rare, severe attacks of disabling vertigo; others may experience attacks monthly, weekly, and even daily. Attacks may vary in severity or may always hit with the same intensity. Quite often, Meniere’s will go into remission, when all symptoms or some of the symptoms may disappear for periods of time, often for years at a time, only to return in the future. Often, after many years, the disorder disappears, but the person may be left with some permanent hearing loss; this is commonly known as "burned-out" Meniere’s.

Meniere’s disease is often unpredictable, but some patients seem to develop a pattern for their Meniere’s and can even predict when they may be prone to an attack. Many people find that certain foods, activities, or situations seem to bring on an attack of their symptoms. These events are called triggers because they may trigger an episode of Meniere’s. One common collection of triggers can be seen in Table 1.

Because there is so much variability with the disease and there is no one test that can prove or "make" the diagnosis of Meniere's, the American Academy of Otolaryngology/Head and Neck Surgery, the group of physicians that specialize in treating disorders of the ears, nose, and throat (ENT physicians), has developed medical guidelines as to what is required for a diagnosis of Meniere’s disease. The most recent version of the guidelines for diagnosis of Meniere’s was published in 1995 and can be seen in Table 2.

Other disorders of the body or ear may produce some or all of the symptoms of Meniere’s disease. They are said to be "mimics" of this disorder. Two of the more interesting mimics are acoustic neuroma and autoimmune inner ear disease. Since these may have specific treatments unique to each disorder, often a physician will do special tests or examinations to determine if any of these other disorders can be causing the patient’s symptoms. These will be mentioned further on, under " differential diagnosis ."

How common is Meniere’s disease? No one actually knows for sure, since the disease is not reported in every case to any government agency or authority. One large study in Sweden estimated that there is one new case of Meniere’s per year for every 2163 people or 46 cases per 100,000 per year (0.046% of the population develops Meniere’s every year). A study in Great Britain estimated that Meniere’s disease occurs much more commonly, one case per year per 636 people, or 0.16% (four times higher than the Swedish estimate). Since the disorder lasts an average of 25 years, there are about 25 people who already suffer from Meniere’s for each new case per year. This would mean that in a country like the United States, if it had a similar rate of occurrence, there would be between 2,400,000 and 7,500,000 people who suffer from Meniere’s at any point in time. The disease seems to prefer starting in people between 30 and 50 years of age, but has been known to start in people younger or older.

 

Table 1:
Common Triggers for Meniere’s Attacks

(Note that not everybody responds to the same triggers or in the same way. This is a list of common events/situations/foods that often lead to Meniere’s attacks in some, but not all, Meniere’s patients.)

  • Stress
  • Salty foods
  • Alcohol
  • Caffeine
  • Allergies
  • Sugar
  • Menstrual cycle/menstruation
  • Pregnancy
  • Orgasm
  • Barometric pressure changes
  • Visual stimuli such as parallel vertical lines (nystagmus inducers)


Table 2
1995 Revised American Academy of Otolaryngology/ Head and Neck Surgery Diagnostic Criteria for Meniere’s Disease

  • Essential feature
    - Sensorineural hearing loss documented on at least one occasion
  • Certain Meniere’s
    - Histopathologic confirmation and the "Definite Meniere’s" criteria
  • Definite Meniere’s
    - Two or more attacks of vertigo lasting more than 20 minutes
    - Audiometrically documented hearing loss
    - Tinnitus or aural fullness
    - Other causes excluded
  • Probable Meniere’s
    - At least one vertigo episode
    - Audiometrically documented hearing loss
    - Tinnitus or aural fullness
    - Other causes excluded
  • Possible Meniere’s
    - Episodic vertigo without documented hearing loss
    - Sensorineural hearing loss, fluctuating or fixed, with disequilibrium but without definite episodes of vertigo
    - Other causes excluded

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