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Ménière's Disease

Ménière's disease

Definition

Ménière's disease is a disorder characterized by recurrent vertigo, sensory hearing loss, tinnitus, and a feeling of fullness in the ear. It is named for the French physician, Prosper Ménière, who first described the illness in 1861. Ménière's disease is also known as idiopathic endolymphatic hydrops; "idiopathic" refers to the unknown or spontaneous origin of the disorder, while "endolymphatic hydrops" refers to the increased fluid pressure in the inner ear that causes the symptoms of Ménière's disease.

Description

Patients with Ménière's disease have periodic attacks characterized by four major symptoms:

  • Vertigo. This is a spinning or whirling sensation that affects the patient's sense of balance; it is sometimes violent. The vertigo is often accompanied by nausea and vomiting.
  • Fluctuating loss of hearing.
  • Tinnitus. This is a sensation of ringing, buzzing, or roaring noises in the ear. The most common type of tinnitus associated with Ménière's is a low-pitched roaring.
  • A sensation of fullness, pressure, or discomfort in the ear.

Some patients also experience headaches , diarrhea, and pain in the abdomen during an attack.

Attacks usually come on suddenly and last from two or three to 24 hours, although some patients experience an aching sensation in the affected ear just before an attack. The attacks typically subside gradually. In most cases, only one ear is affected; however, 1015% of patients with Ménière's disease are affected in both ears. After a severe attack, the patient often feels exhausted and sleeps for several hours.

The spacing and intensity of Ménière's attacks vary from patient to patient. Some people have several acute episodes relatively close together, while others may have one or two milder attacks per year or even several years apart. In some patients, attacks occur at regular intervals, while in others, the attacks are completely random. In some patients, acute attacks are triggered by psychological stress, menstrual cycles, or certain foods. Patients usually feel normal between episodes; however, they may find that their hearing and sense of balance get slightly worse after each attack.

Demographics

The National Institute on Deafness and Other Communication Disorders (NIDCD) estimates that, as of 2003, there are about 620,000 persons in the United States diagnosed with Ménière's disease. Another expert gives a figure of 1,000 cases per 100,000 people. About 46,000 new cases are diagnosed each year; some neurologists, however, think that the disorder is underdiagnosed.

Ménière's disease has been diagnosed in patients of all ages, although the average age at onset is 3540 years of age. The age of patients in several controlled studies of the disorder ranged from 49 to 67 years.

Although Ménière's disease has not been linked to a specific gene or genes, it does appear to run in families. About 55% of patients diagnosed with Ménière's have significant family histories of the disorder. Women are slightly more likely than men to develop Ménière's; various studies report female-to-male ratios between 1.1:1 and 3:2.

There is no evidence as of 2003 that Ménière's disease occurs more frequently in some racial or ethnic groups than in others.

Causes and symptoms

The underlying causes of Ménière's disease are poorly understood as of late 2003. Some geneticists proposed in 2002 that Ménière's disease might be caused by a mutation in the COCH gene, which is the only human gene known to be associated with inherited hearing loss related to inner ear dysfunction. In 2003, however, two groups of researchers in Japan and the United Kingdom reported that mutations in the COCH gene are not responsible for Ménière's. Other theories about the underlying causes of Ménière's disease that are being investigated include virus infections and environmental noise pollution.

One area of research that shows promise is the possible relationship between Ménière's disease and migraine headache. Dr. Ménière himself suggested the possibility of a link, but early studies yielded conflicting results. A rigorous German study published in late 2002 reported that the lifetime prevalence of migraine was 56% in patients diagnosed with Ménière's disease as compared to 25% for controls. The researchers noted that further work is necessary to determine the exact nature of the relationship between the two disorders.

The immediate cause of acute attacks is fluctuating pressure in a fluid inside the inner ear known as endolymph. The endolymph is separated from another fluid called perilymph by thin membranes containing nerves that govern hearing and balance. When the endolymph pressure increases, there is a sudden change in the rate of nerve cells firing, which leads to vertigo and a sense of fullness or discomfort inside the ear. In addition, increased endolymph pressure irritates another structure in the inner ear known as the organ of Corti, which lies inside a shell-shaped structure called the cochlea. The organ of Corti detects pressure impulses, which it converts to electrical impulses that travel along the auditory nerve to the brain. The organ of Corti contains four rows of hair cells that govern a person's perception of the pitch and loudness of a sound. Increased pressure from the endolymph affects the hair cells, causing loss of hearing (particularly the ability to hear low-pitched sounds) and tinnitus.

Diagnosis

Diagnosis of Ménière's disease is a complex process requiring a number of different procedures:

  • Patient history, including family history. A primary care physician will ask the patient to describe the symptoms experienced during the attacks, their severity, the dates of recent attacks, and possible triggers.
  • Physical examination. Patients often come to the doctor's office with signs of recent vomiting; they may be pale and sweaty, with a fast pulse and higher than normal blood pressure. There may be no unusual findings during the physical examination, however, if the patient is between episodes. If the doctor suspects Ménière's disease on the basis of the patient's personal or family history, he or she will examine the patient's eyes for nystagmus, or rapid and involuntary movements of the eyeball. At this point, a primary care physician may refer the patient to an audiologist or other specialist for further testing.
  • Hearing tests. There are several different types of hearing tests used to diagnose Ménière's. The Rinne and Weber tests use a tuning fork to detect hearing loss. In Rinne's test, the examiner holds the stem of a vibrating tuning fork first against the mastoid bone and then out-side the ear canal. A person with normal hearing or Ménière's disease will hear the sound as louder when it is held near the outer ear; a person with conductive hearing loss will hear the tone as louder when the fork is touching the bone. In Weber's test, the vibrating tuning fork is held on the midline of the forehead and the patient is asked to indicate the ear in which the sound seems louder. A person with conductive hearing loss on one side will hear the sound louder in the affected ear, while a person with Ménière's disease will hear the sound louder in the unaffected ear. Other hearing tests measure the person's ability to hear sounds of different pitches and volumes. These may be repeated in order to detect periodic variations in the patient's hearing.
  • Balance tests. The most common balance tests used to diagnose Ménière's disease are the Romberg test, in which the patient is asked to stand upright and steady with eyes closed; the Fukuda test, in which the patient is asked to march in place with eyes closed; and the DixHallpike test, in which the doctor moves the patient from a sitting position to lying down while holding the patient's head tilted at a 45-degree angle. Patients with Ménière's disease tend to lose their balance or move from side to side during the first two tests. The DixHallpike test is done to rule out benign paroxysmal positional vertigo (BPPV), a condition caused by small crystals of calcium carbonate that have collected within a part of the inner ear called the utricle. Some patients with Ménière's disease may have a positive score on the Dix-Hallpike test, indicating that they also have BPPV.
  • Blood tests. These are ordered to rule out metabolic disorders, autoimmune disorders, anemia, leukemia, or infectious diseases (Lyme disease and neurosyphilis).
  • Transtympanic electrocochleography (ECoG). This test involves the placement of a recording electrode close to the cochlea of the patient's ear; it is done to detect distortion of the membranes in the inner ear. ECoG is most accurate when performed during an attack of Ménière's.
  • Electronystagmography (ENG). This test is done to evaluate the functioning of the patient's vestibular and oculomotor (eye movement) systems. It takes about 6090 minutes to complete and includes stimulating the inner ear with air or water of different temperatures as well as measuring and recording the patient's eye movements in response to lights and similar stimuli. ENG can cause dizziness and nausea; patients are told to discontinue all medications for two weeks before the test and to take the test on an empty stomach.
  • Imaging studies. MRIs and CT scans are done to detect abnormalities in the shape or structure of the cochlea and other parts of the inner ear, to rule out tumors, and to detect signs of multiple sclerosis.

Treatment team

A family care practitioner may suspect the diagnosis of Ménière's disease on the basis of the patient's history and physical examination, but the tests required to rule out other diseases or disorders may require specialists in endocrinology, neurology, cardiology, otolaryngology, and internal medicine. Diagnostic hearing tests may be administered by an audiologist. Surgical treatment of Ménière's is usually performed by an otolaryngologist or otologist. A nutritionist or dietitian should be consulted to plan a low-salt diet for the patient.

Patients whose attacks are triggered by emotional stress may be helped by therapists who teach biofeedback, meditation, or other techniques of stress reduction.

Treatment

Medical treatment

Medical management of Ménière's disease involves prophylaxis (prevention of acute attacks) as well as direct treatment of symptoms. Prophylactic treatment begins with diet and nutrition . A low-salt diet is recommended for almost all patients with Ménière's, as reducing salt intake helps to lower the body's overall fluid volume. Lowered fluid volume in turn reduces the amount of fluid in the inner ear. Patients should avoid foods with high sodium content, including pizza, smoked or pickled fish, and other preserved foods. Other foods that commonly trigger acute attacks include chocolate; beverages containing caffeine or alcohol, particularly beer and red wine; and foods with high carbohydrate or high cholesterol content. Since nicotine also triggers Ménière's attacks, patients are advised to stop smoking. The doctor may also prescribe a diuretic, usually Dyazide or Diamox, to lower the fluid pressure in the inner ear. Diuretic medications help to prevent acute attacks but will not stop an attack once it has begun.

Medications that are given to treat the symptoms of an attack include drugs that help to control vertigo by numbing the brain's response to nerve impulses from the inner ear. These include such benzodiazepine tranquilizers as diazepam (Valium) or alprazolam (Xanax), and such antinausea drugs as prochlorperazine (Compazine). The doctor may also prescribe steroid medications to reduce inflammation in the inner ear.

Surgical treatment

Surgery is usually considered if the patient has not responded to 36 months of medical treatment and is healthy enough to undergo general anesthesia. There are four surgical procedures that are commonly done to treat Ménière's disease:

  • Endolymphatic sac decompression or shunt. In this procedure, the surgeon inserts a small tube or valve to drain excess endolymph fluid into a space near the mastoid bone and/or removes some of the bone surrounding the endolymphatic sac in order to reduce pressure on it. The success rate is about 6090% for controlling vertigo, but the procedure often improves the patient's hearing.
  • Vestibular nerve sectioning. This procedure is typically done in patients who still have fairly good hearing in the affected ear. The surgeon enters the internal canal of the ear and separates the nerve bundles governing hearing from the nerve bundles that govern the sense of balance, in order to control the patient's vertigo without sacrificing hearing.
  • Labyrinthectomy. Labyrinthectomies are performed only in patients whose hearing has already been damaged or destroyed by the disease. The surgeon removes the entire labyrinth of the inner ear. Both vestibular nerve sectioning and labyrinthectomy have a 9598% success rate in controlling vertigo, but the patient's hearing may be impaired.
  • Transtympanic medication perfusion. This procedure involves delivering medications into the middle ear through an incision in the eardrum. Once in the middle ear, the drugs are absorbed into the inner ear. Two types of drugs are usedsteroids and aminoglycoside antibiotics (most commonly gentamicin). Medication perfusion is reported to have a 90% success rate.

Complementary and alternative (CAM) treatments

Acupuncture is an alternative treatment that has been shown to help patients with Ménière's disease. The World Health Organization (WHO) lists Ménière's disease as one of 104 conditions that can be treated effectively with acupuncture. In addition, such stress management techniques as autogenic training, visualization, deep breathing, and muscle stretching are helpful to many patients in lowering the frequency of acute attacks.

Recovery and rehabilitation

Patients with Ménière's are referred to rehabilitation therapy if they have not benefited from dietary changes or medication. In vestibular rehabilitation therapy, the therapist first assesses the patient's general muscular strength and coordination, gait and balance, and the triggers as well as the severity and frequency of the vertigo. Rehabilitation itself involves both balance retraining exercises and habituation exercises, which are designed to weaken the brain's response to specific positions or movements that trigger vertigo.

Clinical trials

As of 2003, no clinical trials for Ménière's disease were listed in the National Institutes of Health (NIH) database.

Prognosis

Ménière's disease is not fatal; however, there is no cure for it. Medical treatment between attacks and/or surgery are intended to lower the patient's risk of further hearing loss. Although patients with milder forms of the disorder may be able to control their symptoms through dietary changes alone, the long-term results of Ménière's disease typically include progressive loss of hearing, increasing vertigo, or permanent tinnitus.

Special concerns

Although Ménière's disease is not fatal by itself, it can lead to injuries caused by falls or motor vehicle accidents (if the patient has a severe attack while driving). Although moderate exercise is beneficial, patients diagnosed with Ménière's should avoid occupations or sports that require a good sense of balance (e.g., house painting, construction work, or other jobs that require working on ladders; bicycle or horseback riding; mountain climbing; some forms of yoga, etc.) In addition, patients should check their house or apartment for loose rugs, inadequate lighting, unsafe stairs, or other features that could lead to slipping and falling in the event of a sudden attack. A small minority of patients are prevented by severe vertigo from working at any form of regular employment and must file disability claims.

Resources

BOOKS

Haybach, P. J. Ménière's Disease: What You Need to Know. Portland, OR: Vestibular Disorders Association, 2000.

"Ménière's Disease." Section 7, Chapter 85 in The Merck Manual of Diagnosis and Therapy. Edited by Mark H. Beers, MD, and Robert Berkow, MD. Whitehouse Station, NJ: Merck Research Laboratories, 1999.

Pelletier, Kenneth R., MD. The Best Alternative Medicine, Part II, "CAM Therapies for Specific Conditions: Ménière's Disease." New York: Simon & Schuster, 2002.

PERIODICALS

Hain, T. C., and M. Uddin. "Pharmacological Treatment of Vertigo." CNS Drugs 17 (2003): 85100.

Li, John, MD, and Nicholas Lorenzo, MD. "Endolymphatic Hydrops." eMedicine, January 18, 2002. <www.emedicine.com/neuro/topic412.htm>.

Li, John, MD. "Inner Ear, Ménière Disease, Surgical Treatment." eMedicine, July 17, 2001. <www.emedicine.com/ent/topic233.htm>.

Morrison, A. W., and K. J. Johnson. "Genetics (Molecular Biology) and Ménière Disease." Otolaryngologic Clinics of North America 35 (June 2002): 497516.

Radtke, A., T. Lempert, M. A. Gresty, et al. "Migraine and Ménière's Disease: Is There a Link?" Neurology 59 (December 10, 2002): 17001704.

Silverstein, H., and L. E. Jackson. "Vestibular Nerve Section." Otolaryngologic Clinics of North America 35 (June 2002): 655673.

Silverstein, H., W. B. Lewis, L. E. Jackson, et al. "Changing Trends in the Surgical Treatment of Ménière's Disease: Results of a 10-Year Survey." Ear, Nose, and Throat Journal 82 (March 2003): 185187, 191194.

Usami, S., K. Takahashi, I. Yuge, et al. "Mutations in the COCH Gene are a Frequent Cause of Autosomal Dominant Progressive Cochleo-Vestibular Dysfunction, But Not of Ménière's Disease." European Journal of Human Genetics 11 (October 2003): 744748.

Weisleder, P., and T. D. Fife. "Dizziness and Headache: A Common Association in Children and Adolescents." Journal of Child Neurology 16 (October 2001): 727730.

OTHER

National Institute on Deafness and Other Communication Disorders (NIDCD) Health Information. Ménière's Disease. NIH Publication No. 98-3404. Bethesda, MD: NIDCD, 2001.

ORGANIZATIONS

American Academy of OtolaryngologyHead and Neck Surgery. One Prince Street, Alexandria, VA 22314. (703) 836-4444; TTY: (703) 519-1585. webmaster@entnet.org. <http://www.entnet.org>.

Ear Foundation. 1817 Patterson Street, Nashville, TN 37203. (615) 284-7807 or (800) 545-HEAR; Fax: (615) 284-7935. earfound@earfoundation.org. <http://www.theearfound.org>.

National Institute on Deafness and Other Communication Disorders (NIDCD), National Institutes of Health, 31 Center Drive, MSC 2320, Bethesda, MD 20892-2320. nidcdinfo@nidcd.nih.gov. <http://www.nidcd.nih.gov>.

Vestibular Disorders Association (VEDA). P. O. Box 4467, Portland, OR 97208-4467. (503) 229-7706. (800) 837-8428. veda@vestibular.org. <http://www.vestibular.org>.

Rebecca J. Frey, PhD

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Ménière's Disease

Ménière's disease

Definition

Ménière's disease is a condition characterized by recurrent vertigo (dizziness ), hearing loss , and tinnitus (a roaring, buzzing, or ringing sound in the ears).

Description

Ménière's disease was named for the French physician Prosper Ménière, who first described the illness in 1861. It is an abnormality within the inner ear. A fluid called endolymph moves in the membranous labyrinth or semicircular canals within the bony labyrinth inside the inner ear. When the head or body moves, the endolymph moves, causing nerve receptors in the membranous labyrinth to send signals to the brain about the body's motion. A change in the volume of the endolymph fluid, or swelling or rupture of the membranous labyrinth is thought to result in Ménière's disease symptoms.

Causes & symptoms

Causes

The cause of Ménière's disease is unknown as of 2002; however, scientists are studying several possible causes, including noise pollution, viral infections , or alterations in the patterns of blood flow in the structures of the inner ear. Since Ménière's disease sometimes runs in families, researchers are also looking into genetic factors as possible causes of the disorder.

One area of research that shows promise is the possible relationship between Ménière's disease and migraine headache . Dr. Ménière himself suggested the possibility of a link, but early studies yielded conflicting results. A rigorous German study published in late 2002 reported that the lifetime prevalence of migraine was 56% in patients diagnosed with Ménière's disease as compared to 25% for controls. The researchers noted that further work is necessary to determine the exact nature of the relationship between the two disorders.

A study published in late 2002 reported that there is a significant increase in the number of CD4 cells in the blood of patients having an acute attack of Ménière's disease. CD4 cells are a subtype of T cells, which are produced in the thymus gland and regulate the immune system's response to infected or malignant cells. Further research is needed to clarify the role of these cells in Ménière's disease.

Another possible factor in the development of Ménière's disease is the loss of myelin from the cells surrounding the vestibular nerve fibers. Myelin is a whitish fatty material in the cell membrane of the Schwann cells that form a sheath around certain nerve cells. It acts like an electrical insulator. A team of researchers at the University of Virginia reported in 2002 that the vestibular nerve cells in patients with unilateral Ménière's disease are demyelinated; that is, they have lost their protective "insulation." The researchers are investigating the possibility that a viral disease or disorder of the immune system is responsible for the demyelination of the vestibular nerve cells.

Symptoms

The symptoms of Ménière's disease are associated with a change in fluid volume within the labyrinth of the inner ear. Symptoms include severe dizziness or vertigo, tinnitus, hearing loss, and the sensation of pain or pressure in the affected ear. Symptoms appear suddenly, last up to several hours, and can occur as often as daily to as infrequently as once a year. A typical attack includes vertigo, tinnitus, and hearing loss; however, some individuals with Ménière's disease may experience a single symptom, like an occasional bout of slight dizziness or periodic, intense ringing in the ear. Attacks of severe vertigo can force the sufferer to have to sit or lie down, and may be accompanied by headache, nausea, vomiting , or diarrhea . Hearing tends to recover between attacks, but becomes progressively worse over time.

Ménière's disease usually starts between the ages of 20 and 50 years; however, it is not uncommon for elderly people to develop the disease without a previous history of symptoms. Ménière's disease affects men and women in equal numbers. In most patients only one ear is affected but in about 15% both ears are involved.

Diagnosis

An estimated three to five million people in the United States have Ménière's disease, and almost 100,000 new cases are diagnosed each year. Diagnosis is based on medical history, physical examination, hearing and balance tests, and medical imaging with magnetic resonance imaging (MRI).

In patients with Ménière's disease, audiometric tests (hearing tests) usually indicate a sensory type of hearing loss in the affected ear. Speech discrimination, or the ability to distinguish between words that sound alike, is often diminished. In about 50% of patients, the balance function is reduced in the affected ear. An electronystagnograph (ENG) may be used to evaluate balance. Since the eyes and ears work together through the nervous system to coordinate balance, measurement of eye movements can be used to test the balance system. For this test, the patient is seated in a darkened room and recording electrodes, similar to those used with a heart monitor, are placed near the eyes. Warm and cool water or air are gently introduced into each ear canal and eye movements are recorded.

Another test that may be used is an electrocochleograph (EcoG), which can measure increased inner ear fluid pressure.

Treatment

Because there is no cure for Ménière's disease, most treatments are aimed at reducing its symptoms, especially tinnitus. General measures to mask the tinnitus include playing a radio or tape of white noise (low, constant sound). Exercising to improve blood circulation

and reducing the intake of salt, alcohol, aspirin, caffeine , and nicotine may relieve Ménière's disease symptoms.

Ayurveda

Ayurvedic practitioners believe that tinnitus is a vata disorder. (Vata is one of three doshas, or body/mental types.) The patient can drink a tea prepared from 1 tsp of a mixture of comfrey , cinnamon, and chamomile two to three times a day. Yogaraj guggulu in warm water can be taken two or three times a day. Gentle massage of the mastoid bone (behind the ear) with warm sesame oil may help relieve tinnitus. Placing three drops of garlic oil into the affected ear at night may also be effective.

Homeopathy

Homeopathic remedies are chosen based on each patients specific set of symptoms. Salicylic acidum is indicated for patients who experience a roaring sound, deafness, and giddiness. Bryonia is recommended for patients with headache, a buzzing or roaring sound in the ear, and dizziness that is worsened by motion. Cocculus is indicated for those who experience dizziness and nausea. Conium is chosen for the patient who experiences light sensitivity and dizziness that is worsened by lying down. Carbonium sulphuratum is recommended for patients

who experience a roaring with a tingling sensation and clogged ears. Kali iodatum is chosen for patients who have long-term ringing in the ears and no other symptoms. Theridion is indicated for patients who experience sensitivity to noise and dizziness with nausea and vomiting that is worsened by the slightest motion.

Other remedies

Other alternative medicine disciplines which have treatments to help relieve symptoms of Ménière's disease are:

  • Acupuncture . The acupuncture ear points neurogate, kidney, sympathetic, occiput, heart, and adrenal may relieve dizziness associated with Ménière's disease. Chronic cases may be treated at the body points on the spleen, triple warmer, and kidney meridians. The World Health Organization (WHO) lists Ménière's disease as one of 104 conditions that can be treated effectively with acupuncture.
  • Aromatherapy. The essential oils of geranium, lavender , and sandalwood may be added to bath water. Lavender or German chamomile oils may be used as massage oils.
  • Body adjustments. Chiropractors or osteopaths may adjust the head, jaw, and neck to relieve movement restrictions that could affect the inner ear. Craniosacral therapists may gently move bones of the skull to relieve pressure on the head.
  • Herbals. Ginkgo (Ginkgo biloba ) improves circulation which may improve tinnitus and Ménière's disease. Ginkgo is a powerful antioxidant and blood thinner. Ginkgo relieves tinnitus in about half of the patients who use it. Fenugreek (Trigonella foenum-graecum ) tea (steeped in cold water) stops cricket noises and ringing in the ears. Chamomile (Matricaria recutita ) promotes relaxation and may help the patient to sleep.
  • Reflexology. Working the cervical spine, ear, and neck points on the hands and feet and the points on the bottoms and sides of the big toes may relieve tinnitus.
  • Relaxation techniques. Biofeedback, yoga , massage, and other stress-reduction techniques can promote relaxation and divert the patient's attention away from tinnitus. Stress can worsen tinnitus and bring on an attack of Ménière's disease so relaxation techniques can be beneficial.
  • Supplements. Magnesium deficiency may cause tinnitus. Magnesium supplementation may relieve the tinnitus associated with Ménière's disease and protect the ears from damage resulting from loud sounds. Vitamin B12 supplementation has improved tinnitus in patients deficient in this vitamin. Other supplements recommended for the treatment of Ménière's disease include vitamins C, B1, B2, and B6 and zinc.
  • TENS. Transcutaneous electrical nerve stimulation reduced tinnitus in 60% of the Ménière's disease patients in a study of tinnitus sufferers. Patients received six to 10 treatments biweekly. A few of the study patients reported temporary or permanent worsening of tinnitus, however, the cause of the tinnitus in these patients was not specified.

Allopathic treatment

There is no cure for Ménière's disease, but medication, surgery, and dietary and behavioral changes can help control or improve the symptoms.

A special hearing aid is available which makes a soft noise to mask the ringing and other noises associated with Ménière's disease. This device does not interfere with hearing or speech.

Medications

Symptoms of Ménière's disease may be treated with a variety of oral medicine or through injections. Antihistamines, like diphenhydramine, meclizine, and cyclizine can be prescribed to sedate the vestibular system. A barbiturate medication like pentobarbital may be used to completely sedate the patient and relieve the vertigo. Anticholinergic drugs, like atropine or scopolamine, can help minimize nausea and vomiting. Diazepam has been found to be particularly effective for relief of vertigo and nausea in Ménière's disease. There have been some reports of successful control of vertigo after antibiotics (gentamicin or streptomycin) or a steroid medication (dexamethasone) are injected directly into the inner ear. Some researchers have found that gentamicin is effective in relieving tinnitus as well as vertigo.

A newer medication that appears to be effective in treating the vertigo associated with Ménière's disease is flunarizine, which is sold under the trade name Sibelium. Flunarizine is a calcium channel blocker and anticonvulsant that is presently used to treat Parkinson's disease , migraine headache, and other circulatory disorders that affect the brain.

Surgical procedures

Surgical procedures may be recommended if the vertigo attacks are frequent, severe, or disabling and cannot be controlled by other treatments. The most common surgical treatment is insertion of a small tube or shunt to drain some of the fluid from the canal. This treatment usually preserves hearing and controls vertigo in about one-half to two-thirds of cases, but it is not a permanent cure in all patients.

The vestibular nerve leads from the inner ear to the brain and is responsible for conducting nerve impulses related to balance. A vestibular neurectomy is a procedure where this nerve is cut so the distorted impulses causing dizziness no longer reach the brain. This procedure permanently cures the majority of patients and hearing is preserved in most cases. There is a slight risk that hearing or facial muscle control will be affected.

A labyrinthectomy is a surgical procedure in which the balance and hearing mechanism in the inner ear are destroyed on one side. This procedure is considered when the patient has poor hearing in the affected ear. Labyrinthectomy results in the highest rates of control of vertigo attacks, however, it also causes complete deafness in the affected ear.

Expected results

Ménière's disease is a complex and unpredictable condition for which there is no cure. The vertigo associated with the disease can generally be managed or eliminated with medications and surgery. Hearing tends to become worse over time, and some of the surgical procedures recommended, in fact, cause deafness.

Prevention

Because the cause of Ménière's disease is not definitely known as of 2002, there are no proven strategies for its prevention. Stress reduction and relaxation may prevent attacks of Ménière's disease. Wearing earplugs while exposed to loud sounds will help to prevent hearing damage and worsening of tinnitus.

Resources

BOOKS

"Ménière's Disease." The Alternate Advisor: The Complete Guide to Natural Therapies and Alternative Treatments. Edited by Robert. Richmond, VA: Time-Life Books, 1997.

The Merck Manual of Diagnosis and Therapy. 17th ed., edited by Mark H. Beers and Robert Berkow. Whitehouse Station, NJ: Merck Research Laboratories, 1999.

Pelletier, Kenneth R., MD. The Best Alternative Medicine, Part II, "CAM Therapies for Specific Conditions: Ménière's Disease." New York: Simon & Schuster, 2002.

PERIODICALS

Ballester, M., P. Liard, D. Vibert, and R. Hausler. "Ménière's Disease in the Elderly." Otology and Neurotology 23 (January 2002): 7378.

Corvera, J., G. Corvera-Behar, V. Lapilover, and A. Ysunza. "Objective Evaluation of the Effect of Flunarizine on Vestibular Neuritis." Otology and Neurotology 23 (November 2002): 933937.

Driscoll, C. L., et al. "Low-Dose Gentamicin and the Treatment of Ménière's Disease: Preliminary Results." Laryngoscope 107 (January 1997): 8389.

Friberg, U., and H. Rask-Andersen. "Vascular Occlusion in the Endolymphatic Sac in Ménière's Disease." Annals of Otology, Rhinology, and Laryngology 111 (March 2002): 237245.

Fung, K., Y. Xie, S. F. Hall, et al. "Genetic Basis of Familial Ménière's Disease." Journal of Otolaryngology 31 (February 2002): 14.

Ghosh, S., A. K. Gupta, and S. S. Mann. "Can Electro-cochleography in Ménière's Disease Be Noninvasive?" Journal of Otolaryngology 31 (December 2002): 371375.

Mamikoglu, B., R. J. Wiet, T. Hain, and I. J. Check. "Increased CD4+ T cells During Acute Attack of Ménière's Disease." Acta Otolaryngologica 122 (December 2002): 857860.

Radtke, A., T. Lempert, M. A. Gresty, et al. "Migraine and Ménière's Disease: Is There a Link?" Neurology 59 (December 10, 2002): 17001704.

Saeed, Shakeel R. "Diagnosis and Treatment of Ménière's Disease." British Medical Journal 316 (January 1998): 368.

Spencer, R. F., A. Sismanis, J. K. Kilpatrick, and W. T. Shaia. "Demyelination of Vestibular Nerve Axons in Unilateral Ménière's Disease." Ear, Nose and Throat Journal 81 (November 2002): 785789.

Steenerson, Ronald L., and Gaye W. Cronin. "Treatment of Tinnitus with Electrical Stimulation. Otolaryngology-Head and Neck Surgery 121 (November 1999): 511513.

Yetiser, S., and M. Kertmen. "Intratympanic Gentamicin in Ménière's Disease: The Impact on Tinnitus." International Journal of Audiology 41 (September 2002): 363370.

ORGANIZATIONS

American Academy of Otolaryngology-Head and Neck Surgery. One Prince Street, Alexandria, VA 22314. (703) 836-4444. <http://www.entnet.org>.

The Ménière's Network. 1817 Patterson Street, Nashville, TN 37203. (800) 545-4327. <http://www.earfoundation.org>.

Vestibular Disorders Association. P.O. Box 4467, Portland, OR 97208-4467. (800) 837-8428. <http://www.vestibular.org>.

Belinda Rowland

Rebecca J. Frey, PhD

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Ménière's Disease

Ménière's Disease

Definition

Ménière's disease is a condition characterized by recurrent vertigo (dizziness), hearing loss, and tinnitus (a roaring, buzzing or ringing sound in the ears).

Description

Ménière's disease was named for the French physician Prosper Ménière, who first described the illness in 1861. It is an abnormality within the inner ear. A fluid called endolymph moves in the membranous labyrinth or semicircular canals within the bony labyrinth inside the inner ear. When the head or body moves, the endolymph moves, causing nerve receptors in the membranous labyrinth to send signals to the brain about the body's motion. A change in the volume of the endolymph fluid, or swelling or rupture of the membranous labyrinth, is thought to result in Ménière's disease symptoms.

Causes and symptoms

Causes

The cause of Ménière's disease is unknown as of 2002; however, scientists are studying several possible causes, including noise pollution, viral infections, or alterations in the patterns of blood flow in the structures of the inner ear. Since Ménière's disease sometimes runs in families, researchers are also looking into genetic factors as possible causes of the disorder.

One area of research that shows promise is the possible relationship between Ménière's disease and migraine headache. Dr. Ménière himself suggested the possibility of a link, but early studies yielded conflicting results. A rigorous German study published in late 2002 reported that the lifetime prevalence of migraine was 56% in patients diagnosed with Ménière's disease as compared to 25% for controls. The researchers noted that further work is necessary to determine the exact nature of the relationship between the two disorders.

A study published in late 2002 reported that there is a significant increase in the number of CD4 cells in the blood of patients having an acute attack of Ménière's disease. CD4 cells are a subtype of T cells, which are produced in the thymus gland and regulate the immune system's response to infected or malignant cells. Further research is needed to clarify the role of these cells in Ménière's disease.

Another possible factor in the development of Ménière's disease is the loss of myelin from the cells surrounding the vestibular nerve fibers. Myelin is a whitish fatty material in the cell membrane of the Schwann cells that form a sheath around certain nerve cells. It acts like an electrical insulator. A team of researchers at the University of Virginia reported in 2002 that the vestibular nerve cells in patients with unilateral Ménière's disease are demyelinated; that is, they have lost their protective "insulation." The researchers are investigating the possibility that a viral disease or disorder of the immune system is responsible for the demyelination of the vestibular nerve cells.

Symptoms

The symptoms of Ménière's disease are associated with a change in fluid volume within the labyrinth of the inner ear. Symptoms include severe dizziness or vertigo, tinnitus, hearing loss, and the sensation of pain or pressure in the affected ear. Symptoms appear suddenly, last up to several hours, and can occur as often as daily to as infrequently as once a year. A typical attack includes vertigo, tinnitus, and hearing loss; however, some individuals with Ménière's disease may experience a single symptom, like an occasional bout of slight dizziness or periodic, intense ringing in the ear. Attacks of severe vertigo can force the sufferer to have to sit or lie down, and may be accompanied by headache, nausea, vomiting, or diarrhea. Hearing tends to recover between attacks, but becomes progressively worse over time.

Ménière's disease usually starts between the ages of 20 and 50 years; however, it is not uncommon for elderly people to develop the disease without a previous history of symptoms. Ménière's disease affects men and women in equal numbers. In most patients only one ear is affected but in about 15% both ears are involved.

Diagnosis

An estimated 3-5 million people in the United States have Ménière's disease, and almost 100,000 new cases are diagnosed each year. Diagnosis is based on medical history, physical examination, hearing and balance tests, and medical imaging with magnetic resonance imaging (MRI).

Several types of tests may be used to diagnose the disease and to evaluation the extent of hearing loss. In patients with Ménière's disease, audiometric tests (hearing tests) usually indicate a sensory type of hearing loss in the affected ear. Speech discrimination or the ability to distinguish between words that sound alike is often diminished. In about 50% of patients, the balance function is reduced in the affected ear. An electronystagnograph (ENG) may be used to evaluate balance. Since the eyes and ears work together through the nervous system to coordinate balance, measurement of eye movements can be used to test the balance system. For this test, the patient is seated in a darkened room and recording electrodes, similar to those used with a heart monitor, are placed near the eyes. Warm and cool water or air are gently introduced into the each ear canal and eye movements are recorded.

Another test that may be used is an electrocochleograph (EcoG), which can measure increased inner ear fluid pressure.

Treatment

There is no cure for Ménière's disease, but medication, surgery, and dietary and behavioral changes, can help control or improve the symptoms.

Medications

Symptoms of Ménière's disease may be treated with a variety of oral medicine or through injections. Antihistamines, like diphenhydramine, meclizine, and cyclizine can be prescribed to sedate the vestibular system. A barbiturate medication like pentobarbital may be used to completely sedate the patient and relieve the vertigo. Anticholinergic drugs, like atropine or scopolamine, can help minimize nausea and vomiting. Diazepam has been found to be particularly effective for relief of vertigo and nausea in Ménière's disease. There have been some reports of successful control of vertigo after antibiotics (gentamicin or streptomycin) or a steroid medication (dexamethasone) are injected directly into the inner ear. Some researchers have found that gentamicin is effective in relieving tinnitus as well as vertigo.

A newer medication that appears to be effective in treating the vertigo associated with Ménière's disease is flunarizine, which is sold under the trade name Sibelium. Flunarizine is a calcium channel blocker and anticonvulsant that is presently used to treat Parkinson's disease, migraine headache, and other circulatory disorders that affect the brain.

Surgical procedures

Surgical procedures may be recommended if the vertigo attacks are frequent, severe, or disabling and cannot be controlled by other treatments. The most common surgical treatment is insertion of a small tube or shunt to drain some of the fluid from the canal. This treatment usually preserves hearing and controls vertigo in about one-half to two-thirds of cases, but it is not a permanent cure in all patients.

The vestibular nerve leads from the inner ear to the brain and is responsible for conducting nerve impulses related to balance. A vestibular neurectomy is a procedure where this nerve is cut so the distorted impulses causing dizziness no longer reach the brain. This procedure permanently cures the majority of patients and hearing is preserved in most cases. There is a slight risk that hearing or facial muscle control will be affected.

A labyrinthectomy is a surgical procedure in which the balance and hearing mechanism in the inner ear are destroyed on one side. This procedure is considered when the patient has poor hearing in the affected ear. Labyrinthectomy results in the highest rates of control of vertigo attacks, however, it also causes complete deafness in the affected ear.

Alternative treatment

Changes in diet and behavior are sometimes recommended. Eliminating caffeine, alcohol, and salt may relieve the frequency and intensity of attacks in some people with Ménière's disease. Reducing stress levels and eliminating tobacco use may also help.

Acupuncture is an alternative treatment that has been shown to help patients with Ménière's disease. The World Health Organization (WHO) lists Ménière's disease as one of 104 conditions that can be treated effectively with acupuncture.

Prognosis

Ménière's disease is a complex and unpredictable condition for which there is no cure. The vertigo associated with the disease can generally be managed or eliminated with medications and surgery. Hearing tends to become worse over time, and some of the surgical procedures recommended, in fact, cause deafness.

Prevention

Since the cause of Ménière's disease is unknown as of 2002, there are no current strategies for its prevention. Research continues on the environmental and biological factors that may cause Ménière's disease or induce an attack, as well as on the physiological components of the fluid and labyrinth system involved in hearing and balance. Preventive strategies and more effective treatment should become evident once these mechanisms are better understood.

KEY TERMS

Myelin A whitish fatty substance that acts like an electrical insulator around certain nerves in the peripheral nervous system. It is thought that the loss of the myelin surrounding the vestibular nerves may influence the development of Ménière's disease.

T cell A type of white blood cell produced in the thymus gland that regulates the immune system's response to diseased or malignant cells. It is possible that a subcategory of T cells known as CD4 cells plays a role in Ménière's disease.

Tinnitus A roaring, buzzing or ringing sound in the ears.

Transcutaneous electrical nerve stimulation (TENS) A treatment in which a mild electrical current is passed through electrodes on the skin to stimulate nerves and block pain signals.

Vertigo The medical term for dizziness or a spinning sensation.

Resources

BOOKS

Beers, Mark H., MD, and Robert Berkow, MD, editors. The Merck Manual of Diagnosis and Therapy. Whitehouse Station, NJ: Merck Research Laboratories, 2004.

Pelletier, Kenneth R., MD. The Best Alternative Medicine, Part II. "CAM Therapies for Specific Conditions: Ménière's Disease." New York: Simon & Schuster, 2002.

PERIODICALS

Ballester, M., P. Liard, D. Vibert, and R. Hausler. "Ménière's Disease in the Elderly." Otology and Neurotology 23 (January 2002): 73-78.

Corvera, J., G. Corvera-Behar, V. Lapilover, and A. Ysunza. "Objective Evaluation of the Effect of Flunarizine on Vestibular Neuritis." Otology and Neurotology 23 (November 2002): 933-937.

Friberg, U., and H. Rask-Andersen. "Vascular Occlusion in the Endolymphatic Sac in Ménière's Disease." Annals of Otology, Rhinology, and Laryngology 111 (March 2002): 237-245.

Fung, K., Y. Xie, S. F. Hall, et al. "Genetic Basis of Familial Ménière's Disease." Journal of Otolaryngology 31 (February 2002): 1-4.

Ghosh, S., A. K. Gupta, and S. S. Mann. "Can Electrocochleography in Ménière's Disease Be Noninvasive?" Journal of Otolaryngology 31 (December 2002): 371-375.

Mamikoglu, B., R. J. Wiet, T. Hain, and I. J. Check. "Increased CD4+ T cells During Acute Attack of Ménière's Disease." Acta Otolaryngologica 122 (December 2002): 857-860.

Radtke, A., T. Lempert, M. A. Gresty, et al. "Migraine and Ménière's Disease: Is There a Link?" Neurology 59 (December 10, 2002): 1700-1704.

Spencer, R. F., A. Sismanis, J. K. Kilpatrick, and W. T. Shaia. "Demyelination of Vestibular Nerve Axons in Unilateral Ménière's Disease." Ear, Nose and Throat Journal 81 (November 2002): 785-789.

Steenerson, Ronald L., and Gaye W. Cronin. "Treatment of Tinnitus with Electrical Stimulation." Otolaryngology-Head and Neck Surgery 121 (November 1999): 511-513.

Yetiser, S., and M. Kertmen. "Intratympanic Gentamicin in Ménière's Disease: The Impact on Tinnitus." International Journal of Audiology 41 (September 2002): 363-370.

ORGANIZATIONS

American Academy of Otolaryngology-Head and Neck Surgery, Inc. One Prince St., Alexandria VA 22314-3357. (703) 836-4444. http://www.entnet.org.

Ménière's Network. 2000 Church St., P.O. Box 111, Nashville, TN 37236. (800) 545-4327. http://www.healthy.net/pan/cso/cioi/mn.htm.

On-Balance, A Support Group for People with Ménière's Disease. http://www.midwestear.com/onbal.htm.

Vestibular Disorders Association. P.O. Box 4467, Portland, OR 97208-4467. (800) 837-8428.

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"Ménière's Disease." Gale Encyclopedia of Medicine, 3rd ed.. . Retrieved August 19, 2017 from Encyclopedia.com: http://www.encyclopedia.com/medicine/encyclopedias-almanacs-transcripts-and-maps/menieres-disease

Ménière's disease

Ménière's disease (mayn-yairz) n. a disease of the inner ear characterized by episodes of deafness, buzzing in the ears (tinnitus), and vertigo. Symptoms last for several hours and between attacks the affected ear may return to normal. It is thought to be caused by the build-up of fluid in the inner ear and is treated by drugs or surgery. Medical name: endolymphatic hydrops. [ P. Ménière (1799–1862), French physician]

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"Ménière's disease." A Dictionary of Nursing. . Encyclopedia.com. 19 Aug. 2017 <http://www.encyclopedia.com>.

"Ménière's disease." A Dictionary of Nursing. . Encyclopedia.com. (August 19, 2017). http://www.encyclopedia.com/caregiving/dictionaries-thesauruses-pictures-and-press-releases/menieres-disease

"Ménière's disease." A Dictionary of Nursing. . Retrieved August 19, 2017 from Encyclopedia.com: http://www.encyclopedia.com/caregiving/dictionaries-thesauruses-pictures-and-press-releases/menieres-disease

Menières disease

Menière's disease Chronic condition of the inner ear affecting hearing and balance. Symptoms are deafness, vertigo and ringing in the ears (tinnitus). Caused by excessive fluid in the inner ear, it occurs in middle age or later. It is generally treated with antihistamine drugs.

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