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Ménière disease

Orpha number ORPHA45360
Synonym(s) -
Prevalence 1-5 / 10 000
Inheritance Multigenic/multifactorial
Age of onset All ages
ICD-10
  • H81.0
ICD-O -
OMIM
UMLS
  • C0025281
MeSH
  • D008575
MedDRA
  • 10027183
SNOMED CT
  • 13445001

Summary

Menière's disease is characterized by attacks of vertigo associated with sensorineural hearing loss and tinnitus. It is a rare disorder with a prevalence that varies from 0.2 in 2,000 (Sweden) to 1.5 in 2,000 (USA), depending on the study and inclusion criteria. The constant pathological finding is a progressive distension of the endolymphatic space of the inner ear, known as endolymphatic hydrops. In most cases, attacks of vertigo and tinnitus of variable duration evolve for several years before regressing, whereas deafness remains severe (~60 dB). In some cases (5 to 25 %), the disorder becomes bilateral with an extremely disabling vertigo. Etiology of Menière's disease remains unknown. Diagnosis is suspected on the basis of the triad of symptoms of vertigo with nausea or vomiting, fluctuating hearing loss and tinnitus, often with a feeling of aural fullness. Magnetic resonance imaging (MRI) results are normal and no specific audiometric test enables confirmation of the diagnosis. However, the disease can be excluded when the underlying causes of endolymphatic hydrops are known. Management of Menière's disease consists of anti-vertigo medications during vertigo attacks associated with a long-term treatment aimed at reducing the frequency and intensity of attacks. Depending on the clinical picture, different classes of therapeutic drugs can be used (betahistidine, diuretics, vasodilators, corticoids, anxiolytics etc.). In case of severe invalidating vertigo, local treatment (pressure regulation by transtympanic tube placement, Meniett®, or chemical destruction of the vestibular hair cells by local aminoglycoside injection) or surgery (decompression of the endolymphatic sac or vestibular neurectomy) should be discussed, depending on the severity of the symptoms and the results of the vestibular tests.

Expert reviewer(s)

  • Dr Evelyne FERRARY

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Detailed information

Article for general public
  • FR (2012,pdf)
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