Ménière's disease
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| ICD-10 | H81.0 |
|---|---|
| ICD-9 | 386.0 |
| OMIM | 156000 |
| DiseasesDB | 8003 |
| MedlinePlus | 000702 |
| eMedicine | emerg/308 |
Ménière's disease is a disorder of the inner ear that can affect hearing and balance. It is characterized by episodes of dizziness and tinnitus and progressive hearing loss, usually in one ear. It is caused by an increase in volume and pressure of the endolymph of the inner ear. It is named after the French physician Prosper Ménière, who first reported that vertigo was caused by inner ear disorders in an article published in 1861.
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[edit] Symptoms
The symptoms of Ménière's are variable; not all sufferers experience the same symptoms. However, so-called "classic Ménière's" is considered to comprise the following four symptoms:
- 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.
Ménière's often begins with one symptom, and gradually progresses. A diagnosis may be made in the absence of all four classic symptoms.[citation needed]
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.
[edit] Cause
The exact cause of Ménière's disease is not known, but it is believed to be related to endolymphatic hydrops or excess fluid in the inner ear. It is thought that endolymphatic fluid bursts from its normal channels in the ear and flows into other areas causing damage. This may be related to swelling of the endolymphatic sac or other issues in 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 salt in some patients.
[edit] Diagnosis
Many disorders have symptoms similar to Ménière's. The diagnosis is usually established by clinical findings and medical history. However, a detailed oto-neurological examination, audiometry and head magnetic resonance imaging (MRI) scan should be performed to exclude a tumour of the cranial nerve VIII (vestibulocochlear nerve) which would cause similar symptoms. Because there is no definitive test for Ménière's, it is only diagnosed when all other causes have been ruled out.
Ménière's typically begins between the ages of 20 and 50 and is equally prevalent in men and women.
[edit] Treatment
Initial treatment is aimed at both dealing with immediate symptoms and preventing recurrence of symptoms, and so will vary from patient to patient. Doctors may recommend vestibular training, methods for dealing with tinnitus, stress reduction, hearing aids to deal with hearing loss, and medication to alleviate nausea and symptoms of vertigo.
Several environmental and dietary changes are thought to reduce the frequency or severity of symptom outbreaks. 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, alcohol and tobacco, all of which can aggravate symptoms of Ménière's. Some recommend avoiding Aspartame. Patients are often prescribed a mild diuretic (sometimes vitamin B6). Many patients will have allergy testing done to see if they are candidate for allergy desensitization as allergies have been shown to aggravate Ménière's symptoms.
Women may experience increased symptoms during pregnancy or shortly before menstruation, probably due to increased fluid retention.
Lipoflavanoid is also recommended for treatment by some doctors.
Many patients consider fluorescent lighting to be a trigger for symptoms. The plausibility of this can be explained by how important a part vision plays in the overall mechanism of human balance.
Treatments aimed at lowering the pressure within the inner ear include antihistamines, anticholinergics, steroids, and diuretics. A medical device that provides transtympanic micropressure pulses is now showing some promise and is becoming more widely used as a treatment for Ménière's.[1]
Surgery may be recommended if medical management does not control vertigo. Injection of steroid medication behind the eardrum, or surgery to decompress the endolymphatic sac may be used for symptom relief. Permanent surgical destruction of the balance part of the affected ear can be performed for severe cases if only one ear is affected. This can be achieved through chemical labyrinthectomy, in which a drug (such as gentamicin) that "kills" the vestibular apparatus is injected into the middle ear. The nerve to the balance portion of the inner ear can be cut (vestibular neurectomy), or the inner ear itself can be surgically removed (labyrinthectomy). These treatments eliminate vertigo, but because they are destructive, they are used only as a last resort. Typically balance returns to normal after these procedures, but hearing loss may continue to progress.
[edit] Progression
Progression of Ménière's is unpredictable: symptoms may worsen, disappear altogether, or remain the same.
Sufferers whose Ménière's began with one or two of the classic symptoms may develop others with time. 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).
[edit] 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.
Jonathan Swift, author of Gulliver's Travels (1726), is generally thought to have had Ménière’s from age 22.
It has also been suggested that Emily Dickinson was a Ménière's sufferer.
Author and entrepreneur Guy Kawasaki admits to having the illness.
NBA player Steve Francis was diagnosed with the disease in 2002.
Contemporary artist and graphic designer Doc Hammer, of Venture Brothers fame, also has Ménière's syndrome.
Vincent Van Gogh, the Dutch Post-Impressionist, is also believed to have suffered from Ménière's [2]. Some believe "The Starry Night" illustrates his dizziness.
It is has been suggested that Charles Darwin suffered from Ménière’s disease. This idea was based on a common list of symptoms which were present in Darwin's case, such as tinnitus, vertigo, dizziness, motion sickness, vomiting, continual malaise and tiredness. The absence of hearing loss and 'fullness' of the ear (as far as known) excludes however a diagnosis of typical Ménière’s disease. Darwin himself had the opinion that most of his health problems had an origin in his 4-year bout with sea sickness. Later, he could not stand travelling by carriage, and only horse riding would not affect his health. Darwin hunted a lot when he was young and could have damaged his inner ear with the repeated noise of shooting, and one of diagnoses that he received from his physicians at the time was that of "suppressed gout". The source of Darwin's illness, though, is not known for sure. See Charles Darwin's illness for more details.
[edit] See also
Other famous sufferers from Meniere's disease, probably caused by syphillis include: Balzac, Clare, Donizetti, Dostoevsky, Goethe, Gogol, Lamb, Luther, Nietzsche, Maupassant, Pascal, Rousseau, Ruskin, Schumann, Swift, Tchaikovsky, and Van Gogh. (S. Milligan, and A. Clare. "Depression and How to Survive it." London: Ebury, 1993.)
[edit] External links
- NIH information
- UK Ménière's Society
- Online clearing house for Ménière's Information
- Online Ménière's Community
- Vestibular Disorders Association
- Medical Treatment of Ménière's Disease
- Ménière's disease at MayoClinic.com
- The Ménière's Page at The Department of Otolaryngology, Washington University School of Medicine
- Approaching Ménière's Disease Positively
[edit] References
G.P. Rajan et al (2005). "Long-term effects of the Meniett device in Meniere's disease: the Western Australian experience.". The Journal of Laryngology and Otology 119 (5): 391-395.[3]
