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Ménière’s disease (or syndrome, since its cause is unknown) was first described by French physician Prosper Ménière in 1861. It is a balance disorder of the inner ear.
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Symptoms
The symptoms of Meniere's are variable; not all sufferers experience the same symptoms. However, four symptoms are considered to comprise so-called "classic Meniere's":
- periodic episodes of rotary vertigo (the abnormal sensation of movement) or dizziness
- fluctuating, progressive, unilateral (in one ear) or bilateral (in both ears) hearing loss, often in the lower frequency ranges
- unilateral or bilateral tinnitus (the perception of noises, often ringing, roaring, or whooshing), sometimes variable
- a sensation of fullness or pressure in one or both ears
Meniere's often begins with one symptom, and gradually progresses. A diagnosis may be made in the absence of all four classic symptoms.
Attacks of vertigo can be severe, incapacitating, and unpredictable. In some patients, attacks of vertigo can last for hours or days, and may be accompanied by an increase in the loudness of tinnitus and temporary hearing loss in the affected ear(s). Hearing may improve after an attack, but often becomes progressively worse. Vertigo attacks are sometimes accompanied by nausea, vomiting, and sweating.
Some sufferers experience what are informally known as "drop attacks" -- a sudden, severe attack of dizziness or vertigo that causes the sufferer, if not seated, to fall. Some patients may find it impossible to get up for some time, until the attack passes or medication takes effect. There is also the risk of injury from falling.
In addition to low frequency hearing loss, sounds can seem tinny or distorted, and patients can experience unusual sensitivity to loud noises. Some sufferers also experience nystagmus, or uncontrollable rhythmical and jerky eye movements, usually in the horizontal plane.
Other symptoms include so-called "brain fog" (temporary loss of short term memory, forgetfulness, and confusion), deafness, exhaustion and drowsiness, headaches, vision problems, and depression.
Cause
The exact cause of Ménière's disease is not known, but it is believed to be related to swelling of the endolymphatic sac, part of the vestibular system of the inner ear, which is responsible for the body's sense of balance. The symptoms may occur in the presence of a middle ear infection, head trauma or an upper respiratory tract infection, or by using aspirin, smoking cigarettes or drinking alcohol. They may be further exacerbated by excessive consumption of caffeine and even salt in some patients. The diagnosis is usually established by clinical findings and medical history. However, a detailed neurological examination, audiometry and even head magnetic resonance imaging (MRI) scan can be performed to exclude a tumour of the cranial nerve VIII (vestibulocochlear nerve) which would cause similar symptoms.
Treatment
Initial treatment is aimed at dealing with immediate symptoms, and so will vary from patient to patient. Doctors may recommend training or other methods for dealing with tinnitus, stress reduction, hearing aids to deal with hearing loss, and medication to alleviate nausea and symptoms of vertigo.
Most patients are advised to adopt a low-sodium diet, typically one to two grams (1000-2000mg) at first, but diets as low as 400mg are not uncommon. Patients are advised to avoid caffeine, alchohol and tobacco, all of which can aggravate symptoms of Meniere's. Some recommend avoiding Aspartame. Patients are often prescribed a mild diuretic (sometimes vitamin B6).
Treatments aimed at lowering the pressure within the inner ear include antihistamines, anticholinergics, steroids, and diuretics.
Surgery of the semicircular canals or the vestibular nerve is very rarely performed in some untreatable and most severe cases. Another treatment is chemical labyrinthectomy, in which a drug (such as Gentamycin) that "kills" the vestibular apparatus is injected into the inner ear. These radical treatments eliminate vertigo, but they also eliminate the patient's normal sense of balance, and so are used only as a last resort.
Progression
Meniere's sometimes begins with one or two of the classic symptoms, but with time the others normally develop as well. Attacks of vertigo can become worse and more frequent over time, resulting in loss of employment, loss of the ability to drive, and inability to travel. Some patients become largely housebound. Hearing loss can become more profound and may become permanent. Some patients become deaf in the affected ear. Tinnitus can also worsen over time. Some patients with unilateral symptoms, as many as fifty percent by some estimates, will develop symptoms in both ears. Some of these will become totally deaf.
Yet the disease may end spontaneously and never repeat again. Some sufferers find that after eight to ten years their vertigo attacks gradually become less frequent and less severe; in some patients they disappear completely. In some patients, symptoms of tinnitus will also disappear, and hearing will stabilize (though usually with some permanent loss). In some patients, however, symptoms may labe unresponsive to medications.
Famous sufferers
Alan B. Shepard, the first American astronaut, was diagnosed with Ménière’s disease in 1964, grounding him after only one brief spaceflight. Several years later, surgery (which was then at the experimental stage) was performed, allowing Shepard to fly to the Moon on Apollo 14.
See also
External links
- NIH information (http://www.nidcd.nih.gov/health/balance/meniere.asp)nl:Ziekte van Ménière