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Disease Profile

Ménière’s disease

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

#N/A

ICD-10

#N/A

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Meniere's disease; Meniere disease

Categories

Ear, Nose, and Throat Diseases

Summary

Ménière's disease is an abnormality of the inner ear. Signs and symptoms may include disabling vertigo or severe dizziness lasting from minutes to hours; tinnitus or a roaring sound in the ears; fluctuating hearing loss; and the sensation of pressure or pain in the affected ear. A small percentage of people have drop attacks. The disorder usually affects only one ear, but some people develop symptoms in both ears many years after their initial diagnosis. Although the exact cause of Ménière's disease is unknown, the symptoms are thought to be associated with a change in fluid volume within a portion of the inner ear known as the labyrinth. Treatment may include medications or surgery depending on the severity of the condition.[1][2]

Symptoms

The symptoms of Ménière's disease typically occur suddenly and may be experienced daily, or as infrequently as once a year. Vertigo, often the most debilitating symptom of Ménière's disease, typically involves a whirling dizziness that forces the affected person to lie down. Vertigo attacks can lead to severe nausea, vomiting, and sweating, and often come with little or no warning.[1]

Some people with Ménière's disease have attacks that start with tinnitus (ear noises), a loss of hearing, or a full feeling or pressure in the affected ear. It is important to remember that all of these symptoms are unpredictable. Typically, the attack is characterized by a combination of vertigo, tinnitus, and hearing loss lasting several hours. People experience these discomforts at varying frequencies, durations, and intensities. Some may feel slight vertigo a few times a year. Others may be occasionally disturbed by intense, uncontrollable tinnitus while sleeping. Affected people may also notice hearing loss or feel unsteady for prolonged periods. Other occasional symptoms of Ménière's disease may include headaches, abdominal discomfort, and diarrhea. A person's hearing tends to recover between attacks but over time may become worse.[1][3] Meniere's disease usually starts in one ear but it may extend to involve both ears over time.[3]

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Medical Terms Other Names
Learn More:
HPO ID
Percent of people who have these symptoms is not available through HPO
Autosomal dominant inheritance
0000006
Hearing impairment
Deafness
Hearing defect

[ more ]

0000365
Incomplete penetrance
0003829
Tinnitus
Ringing in ears
Ringing in the ears

[ more ]

0000360
Vertigo
Dizzy spell
0002321

Cause

The underlying cause of Ménière's disease is unknown, although it probably results from a combination of environmental and genetic factors.[2][3][4] Possible causes that have been studied include viral infections; trauma to the middle ear; middle ear infection (otitis media); head injury; a genetic predisposition; syphilis; allergies; abnormal immune system response; migraines; and noise pollution.[2][4][3]

The symptoms of Ménière's disease are thought to relate to changes in fluid volume in the inner ear, which contains structures necessary for normal hearing and balance. Changes in fluid volume may disrupt signals sent from the inner ear to the brain, or may lead to tears or ruptures of the structures that affect hearing and balance.[4][5]

More detailed information about the causes of symptoms associated with Ménière's disease is available on NIDCD's Web site.

Diagnosis

A diagnosis of Ménière's disease is often suspected based on the presence of characteristic signs and symptoms. These may include:[1]

  • Two or more episodes of vertigo lasting at least 20 min each
  • Tinnitus
  • Temporary hearing loss
  • A feeling of fullness in the ear

Proper diagnosis of Ménière's disease entails several procedures, including a medical history interview; a physical examination; hearing and balance tests; and medical imaging with magnetic resonance imaging (MRI). Accurate measurement and characterization of hearing loss are of critical importance in the diagnosis of Ménière's disease.[1]

Through the use of several types of hearing tests, physicians can characterize hearing loss as being sensory (arising from the inner ear) or neural (arising from the hearing nerve). Recording the auditory brain stem response, which measures electrical activity in the hearing nerve and brain stem, is useful in differentiating between these two types of hearing loss. Electrocochleography, recording the electrical activity of the inner ear in response to sound, helps confirm the diagnosis.[1]

To test the vestibular or balance system, physicians irrigate the ears with warm and cool water or air. This procedure, known as caloric testing, results in nystagmus, rapid eye movements that can help a physician analyze a balance disorder.[1]

Since tumor growth can produce symptoms similar to Ménière's disease, an MRI is a useful test to determine whether a tumor is causing the patient's vertigo and hearing loss.[1]

 

Treatment

At the present time there is no cure for Ménière's disease, but lifestyle modifications and some treatments can help affected people cope with the symptoms.[3] The symptoms of the disease are often controlled successfully by reducing the body’s retention of fluids through dietary changes (such as a low-salt or salt-free diet and no caffeine or alcohol).[1] Medications such as antihistamines, anticholinergics, and diuretics may lower endolymphatic pressure by reducing the amount of endolymphatic fluid.[2] Eliminating tobacco use and reducing stress levels may also help lessen the severity of symptoms.[1]

Symptoms such as dizziness, vertigo, and associated nausea and vomiting may respond to certain medications.[2] The U.S. Food and Drug Administration (FDA) recently approved a device for Ménière’s disease that fits into the outer ear and delivers intermittent air pressure pulses to the middle ear (pressure pulse treatment). The air pressure pulses appear to act on endolymph fluid to prevent dizziness.[1]

Different surgical procedures are an option for people with persistent, debilitating vertigo. Labyrinthectomy (removal of the inner ear sense organ) can effectively control vertigo, but sacrifices hearing and is reserved for patients with nonfunctional hearing in the affected ear. Vestibular neurectomy, selectively severing a nerve from the affected inner ear organ, usually controls the vertigo while preserving hearing but carries surgical risks. Recently, the administration of the ototoxic antibiotic gentamycin directly into the middle ear space has gained popularity worldwide for the control of vertigo associated with Ménière's disease.[1]

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

    Organizations Providing General Support

      Learn more

      These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

      Where to Start

      • The American Academy of Family Physicians has an information page on Meniere's disease. To access this information page, click on American Academy of Family Physicians.
      • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
      • Genetics Home Reference (GHR) contains information on Ménière's disease. This website is maintained by the National Library of Medicine.
      • The Merck Manuals Online Medical Library provides information on this condition for patients and caregivers.
      • The National Institute on Deafness and Other Communication Disorders (NIDCD) conducts and supports biomedical and behavioral research and research training in the normal and disordered processes of hearing, balance, smell, taste, voice, speech, and language. Click on the link to view information on this topic.
      • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

        In-Depth Information

        • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
        • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
        • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
        • PubMed is a searchable database of medical literature and lists journal articles that discuss Ménière's disease. Click on the link to view a sample search on this topic.

          References

          1. Ménière's Disease. National Institute on Deafness and Other Communication Disorders (NIDCD). June 2016; https://www.nidcd.nih.gov/health/menieres-disease.
          2. Ménière's disease. MedlinePlus. August 2015; https://www.nlm.nih.gov/medlineplus/ency/article/000702.htm.
          3. Timothy C. Hain. Meniere's Disease. American Hearing Research Foundation. October 2012; https://www.american-hearing.org/disorders/menieres-disease/.
          4. Ménière disease. Genetics Home Reference. June 2010; https://ghr.nlm.nih.gov/condition/meniere-disease.
          5. Berlinger NT. Meniere's disease: new concepts, new treatments. Minn Med. November 2011; 94(11):33-36.
          6. John C. Li. Meniere Disease (Idiopathic Endolymphatic Hydrops). Medscape Reference. November 2016; https://emedicine.medscape.com/article/1159069-overview.

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