$Unique_ID{BRK03991} $Pretitle{} $Title{Meniere Disease} $Subject{Meniere Disease Endolymphatic Hydrops Labyrinthine Hydrops Labyrinthine Syndrome Lermoyez Syndrome } $Volume{} $Log{} Copyright (C) 1986, 1987, 1989 National Organization for Rare Disorders, Inc. 272: Meniere Disease ** IMPORTANT ** It is possible the main title of the article (Meniere Disease) is not the name you expected. Please check the SYNONYMS listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Endolymphatic Hydrops Labyrinthine Hydrops Labyrinthine Syndrome Lermoyez Syndrome General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. Meniere's Disease is a disorder characterized by recurrent prostrating dizziness (vertigo), possible hearing loss and ringing sounds (tinnitus). It is associated with dilation of the membranous labyrinth (endolymphatic hydrops) in the ear. Symptoms The attacks of dizziness (vertigo) in Meniere Disease appear suddenly and usually last a few hours. Vertigo consists of the sensation that the room or objects are rotating around the patient. The dizziness often subsides gradually. The attacks may be associated with nausea and vomiting. The patient may have a recurrent feeling of fullness or pressure in the affected ear, and hearing tends to fluctuate. Over the years hearing may progressively worsen. The unusual noises heard by the patient (tinnitus) may be constant or intermittent. The sounds may be more intense before, after or during an attack of vertigo. Usually, one ear is affected, but both ears are involved in 10% to 15% of patients with Meniere's Disease. (For more information on tinnitus, choose "tinnitus" as your search term in the Rare Disease Database.) In Lermoyez's variant of Meniere Disease, hearing loss and tinnitus may precede the first attack of vertigo by months or years, and the hearing may improve with onset of the vertigo. Sometimes Meniere Disease can occur without vertigo. In this type of the disorder, the endolymphatic distention is limited to the cochlea, the snailshell-like spiral tube in the inner ear. Causes The cause of Meniere Disease is not known. Possibly the membrane between the inner and middle ear has become more porous, causing a change in the osmotic pressure in the labyrinth. Local disturbance of the salt/water balance leading to edema of the fluid inside the labyrinth (endolymph), characterizes this disorder but it is not clear why this occurs. Other possible causes are disturbance of the autonomic regulation of the endolymphatic system; local allergy of the inner ear; and vascular disturbance of a layer of fibrous vascular tissue covering the outer wall of the cochlear duct (stria vascularis). Stress and emotional disturbances are often associated with an increase in frequency of the attacks. Affected Population Onset of Meniere Disease is most common during the fifth decade of life. The disorder occurs somewhat more frequently in males than in females. A recent study suggests that 0.4 percent of the population in the United States may be affected by Meniere's Disease. Therapies: Standard Symptomatic relief of the dizziness can sometimes be obtained with anticholinergic drugs such as atropine or scopolamine. These minimize gastrointestinal symptoms mediated by the tenth cranial nerve. Antihistamines such as diphenhydramine, meclizine or cyclizine can sedate the vestibular system. Barbiturates such as phenobarbital can be used for general sedation during severe attacks. Diazepam appears to be particularly effective in relieving the distress of severe dizziness by sedating the vestibular system. An operation to implant a shunt to drain off excess fluid thus relieving pressure on the inner ear can lend temporary relief of dizziness and hearing loss. In order to keep dizziness messages from going to the brain, surgeons may also cut the vestibular nerve, although this is a high-risk procedure and may result in cutting the cochlear nerve (which governs hearing) or the nerve which controls the facial muscles. To avoid this, the RVN procedure was developed in 1978. Surgeons remove a small section of bone from behind the outer ear and attach an electrode on the cochlear nerve. A small earphone producing steady clicks is placed in the outer ear. The clicks are picked up by the inner ear, transmitted through the cochlear nerve, and monitored on a computer screen hook-up. A pattern change on the computer would signal any disturbance to the cochlear nerve. The fibers of the vestibular nerve are severed layer by layer. The first few days after this procedure may be difficult, but the dizziness may be relieved with almost no hearing loss. Therapies: Investigational When recurring attacks of vertigo become more frequent and severe, and intensive medical therapy has failed to control them, the patient with Meniere's disease becomes a potential candidate for surgery to help his/her symptoms. Surgery for this disorder presently can be divided into two groups: Conservative and Destructive types. Conservative--used if residual hearing is good or aidable through a hearing aid. Three approaches are used within this category: 1) the endolymphatic shunt; 2) the middle cranial fossa vestibular neurectomy, and 3) the retrolabyrinthine vestibular neurectomy. Destructive--used if residual hearing is poor and cannot be helped with amplification. Three such operations are in use today: 1) the oval window labyrinthectomy; 2) the postauricular labyrinthectomy; and 3) the translabyrinthine vestibular neurectomy. All modern surgical treatment of Meniere's disease involves microsurgical techniques and, in some instances, laser technology. For further information on experimental surgery for Meniere Disease contact: Dr. Margareta Moller Presbyterian University Hospital, Room 948 230 Lothrup St. Pittsburgh, PA 15213 (412) 647-0444 Ear Research Foundation Dept. P, 1921 Floyd St. Sarasota, FL 34239 This disease entry is based upon medical information available through April 1989. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Meniere Disease, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 The E.A.R. Foundation ATTN: Meniere's Network 2000 Church Street Nashville, TN 37236 (615) 329-7807 (Voice & TDD) Meniere Crouzon Syndrome Support Network 2375 Valentine Dr., #9 Prescott, AZ 96303 Vestibular Disorders Association 1015 22nd Avenue, D-230 Portland, OR 97210-3079 (503) 229-7348 American Tinnitus Association P.O. Box 5 Portland, OR 97207 (502) 248-9985 NIH/National Institute of Deafness & Other Communication Disorders (NIDCD) 9000 Rockville Pike Bethesda, MD 20892 (301) 496-5751 References The Merck Manual of Diagnosis and Therapy: Berkow et al., eds.: Merck Sharp & Dohme (1982).