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Menière disease

ORPHA45360
Synonym(s) -
Prevalence >1 / 1000
Inheritance Unknown
Age of onset Adult
ICD-10
  • H81.0
OMIM
UMLS
  • C0025281
MeSH
  • D008575
MedDRA
  • 10027183

Summary

Menière disease is a chronic idiopathic inner ear disorder characterized by spontaneous and recurrent attacks of vertigo, aural fullness, fluctuating low-frequency hearing loss, and/or tinnitus.

The incidence of Menière disease varies greatly due to differences in diagnostic criteria between studies. The prevalence in the United States has been estimated at 1/500.

Disease onset usually occurs between the ages of 20-50, and is usually unilateral (bilateral in 25-40% of cases). Attacks of vertigo can occur suddenly without warning, be preceded by headache or, more commonly, aural fullness along with increased tinnitus and hearing loss. They can last between 15 minutes to several hours, are exacerbated by head movements and can be accompanied by nausea, vomiting, sweating, diarrhea, and in some cases, nystagmus. The clinical course varies between patients, with some experiencing recurrent, unrelenting attacks of vertigo whereas others having long periods of remissions over years before decreasing in frequency and eventually ceasing. Between attacks patients may remain asymptomatic or experience light-headedness and disequilibrium. Sudden falls (drop attacks) have also been reported. Sensorineural hearing loss (which may begin with acoustic distortion, loudness intolerance) is usually fluctuating and progressive, and affects mainly low-frequencies. Hearing loss (sometimes severe) and tinnitus may persist.

Menière disease may be due to external causes (traumatic rupture of the membranous labyrinth, ischemia, viral infections), allergies, or autoimmune disorders. The exact pathogenesis is unknown but thought to be due to either an overproduction or a failure of absorption of endolymph, resulting in the expansion of the endolymphatic space (endolymphatic hydrops).

Diagnosis is clinical after the exclusion of all other causes of vertigo, tinnitus, and hearing loss. According to the American Academy of Otolaryngology guidelines, a definite diagnosis of MD is given if a patient has: >2 spontaneous episodes of vertigo (lasting 20 min or more each), at least one occasion of documented hearing loss, tinnitus or aural fullness in the affected ear, and the exclusion of other causes (MRI results are normal). Audiometric testing can evaluate a patient's hearing level and sensitivity to certain frequencies. Vestibular tests (i.e. electronystagmography, electrocochleography, and rotational chair test) are also necessary.

Differential diagnoses include vestibular schwannoma, Cogan syndrome, familial otosclerosis (see these terms), acute vestibular labyrinthitis or neuronitis, presbyacousis, and noise-induced hearing loss. Other causes of tinnitus such as metabolic disorders (i.e. zinc and vitamin deficiencies), neurological problems (head trauma, meningitis), dental disorders (temporomandibular joint anomaly; see this term), and certain drugs (i.e. anti-inflammatory medications, aminoglycosides) must also be excluded.

Most cases appear to be sporadic but familial clustering of the disease has also been reported.

There is no cure for Menière disease and treatment is symptomatic. Antivertigo drugs and antiemetics may provide temporary relief of symptoms. Oral or intratympanic injections of steroids have also been effective in some cases. For more severe invalidating vertigo, intratympanic low dose injections of gentamicin as well as surgical procedures (i.e. selective vestibular neurectomy, labyrinthectomy) are considered. The Meniett device, increasing local middle ear pressure, has been proposed to relieve vertigo in some.

Menière disease is not a life-threatening disease but it can have a significant effect on quality of life. With proper treatment quality of life can be significantly improved.

Expert reviewer(s)

  • Dr Didier BOUCCARA
  • Dr Evelyne FERRARY

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Detailed information

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  • FR (2012,pdf)
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