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Ménières disease is named after the 19th Century physician who described this condition. Ménières is a disease of adults, often beginning in the thirties or forties. It rarely occurs in children.
Features of Ménières Disease
- Tinnitus or ringing in the ear
- Fluctuating hearing loss
- Pain, pressure or fullness of the ear
- Bouts of vertigo with nausea and vomiting
- Attacks last a few hours to a few days
- Patients usually return to normal between attacks
Ménières disease usually only affects one ear initially. In about one third of patients, however, both ears eventually become involved. In the more chronic phases of the disease, the drop in hearing may not recover between attacks and becomes permanent. Less common, labyrinthine function becomes permanently impaired.
The basic cause of the symptoms of Ménières disease is an increase in the amount and the pressure of the fluid called endolymph, which bathes the inner ear sensory organs within the labyrinth. Ménières disease can be thought of as similar to glaucoma of the eye in which there is also an increase of pressure and fluid.
Ménières disease is also called endolymphatic hydrops. Endolymph is the name of the fluid, and hydrops means swelling. The endolymph fluid is contained within a thin membrane called the membranous labyrinth. Normally, endolymph is continuously made and then reabsorbed so that the pressure remains relatively constant. When the fluid is not adequately reabsorbed, pressure inside the membranous labyrinth increases. This can lead to a ballooning of the membranous labyrinth, which interferes with the normal function of the hair cells that relay information used for hearing and balance. The thin membrane that holds the endolymph may also rupture, causing the fluid to leak out and further interfere with the function of the hair cells. These breaks in the membrane are usually caused by sudden and severe attacks of vertigo and imbalance that can be incapacitating. Fortunately, these broken membranes are repaired rapidly naturally, so that patients usually recover within a day or two.
- Fullness in the ear
- Impaired hearing
- Hearing returns once vertigo begins
- Hearing tests will show low pitch sounds are hardest to hear
- Recruitment – soft sounds are hard to hear, but louder sounds seem too loud
Electrocochleography (ECOG) is a useful diagnostic technique to test inner ear pressure, which is commonly elevated in Ménières disease. Some patients with Ménières may suddenly fall without warning. These events, which may occur early in the course of the disease, are called Tumarkin’s otolithic crises. Fortunately, they often subside on their own. The natural course of Ménières is one of progression, but with extended periods of regression.
The cause of Ménières is unknown, but it can follow other diseases of the inner ear, including:
- Head trauma
- Inner ear infections – often in the distant past
- Viral infections
When there is an identifiable cause for Ménières disease, it is often called Ménières syndrome. There is probably an increased incidence of migraines in patients with this disease. The distinction between migraine associated dizziness and Ménières may be difficult since vertigo and imbalance with hearing symptoms can occur in both.
The treatment for Ménières disease is based upon both treating the symptoms when an attack occurs, and preventing the attacks themselves. Acute attacks of vertigo are treated like any other acute vestibular problem with medications that ease nausea and vomiting or mild sedatives. For loss of hearing, a cortisone-like medication may be administered orally or injected directly into the middle ear.
- Modify diet with rigid salt restriction – this often requires the guidance of a nutritionist
- Avoid foods that may trigger symptoms, such as Monosodium glutamate (MSG) or caffeine
- Avoid nicotine
- Use of a diuretic or water pill may be recommended
- Diamox or Acetazolamide may be used to decrease the production of endolymph fluid and reduce inner ear pressure
For recurrent, incapacitating attacks of vertigo, injections of gentamicin through the ear drum into the middle ear can be used. Gentamicin is an antibiotic that can be toxic to inner ear hair cells, but especially those associated with the vestibular system. These injections can partially destroy labyrinthine function and so decrease the incidence and severity of vertigo attacks without necessarily creating a severe loss of vestibular function. These injections usually do not affect hearing, though this is always a potential unwanted side effect.
Surgical Treatment for Ménières
- Removing the labyrinth, when hearing is already lost
- Cutting the vestibular nerve to the part of the labyrinth that relays vestibular sensation to the brain
These procedures cause a permanent loss of vestibular function .
Because Ménières disease eventually involves both ears in a high percentage of patients, one must be cautious before deciding to produce the permanent loss of function on once side since the other side may eventually be affected. This situation could produce a total loss of vestibular function that can create a whole new set of problems with impaired vision and imbalance whenever one moves. The benefits of the destructive procedure on the incapacitating nature of the attacks of vertigo and imbalance must be weighed carefully against the risk of a complete loss of balance function.
Physical therapy also may help Ménières patients who have lost some of their balance function or are recovering from a procedure in which labyrinthine function had to be destroyed. As with all conditions that produce episodic and unpredictable attacks of vertigo, the anxiety and depression associated with the debilitating and unpredictable nature of Ménières must be recognized and treated.
While Ménières disease can sometimes be incapacitating for the patient, and frustrating for the doctor, recent research has lead to important advances in helping patients bring their lives back to normal.
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To request an appointment or refer a patient, please contact the Vestibular Disorder Staff at 410-955-3319.
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Adult Neurology: 410-955-9441
Pediatric Neurology: 410-955-4259
Adult Neurosurgery: 410-955-6406
Pediatric Neurosurgery: 410-955-7337
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