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, 10–15% 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 35–40 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 60–90 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 3–6 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 60–90% 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 95–98% 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 used—steroids 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): 85–100.

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): 497–516.

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.

Silverstein, H., and L. E. Jackson. "Vestibular Nerve Section." Otolaryngologic Clinics of North America 35 (June 2002): 655–673.

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): 185–187, 191–194.

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): 744–748.

Weisleder, P., and T. D. Fife. "Dizziness and Headache: A Common Association in Children and Adolescents." Journal of Child Neurology 16 (October 2001): 727–730.

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 Otolaryngology—Head 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