Menière’s disease – a genetic imbalance?

Abstract

Meniere’s disease is an uncommon condition characterized by disabling episodes of vestibular and auditory disturbance. The symptoms of vertigo, tinnitus and fluctuating deafness result from endolymphatic hydrops within the inner ear. Onset is usually in the fourth or fifth decade of life, and attacks (each lasting up to 24 hours) occur in clusters with intervening periods of remission. The vertigo is often prostrating and accompanied by nausea and vomiting. In the early stages of the disease, the characteristic low-frequency hearing-loss recovers between attacks, but later it becomes permanent and progressive, leading to severe hearing-loss at all frequencies. A low-salt diet and drug treatment can help reduce the number and severity of the attacks.Although the majority of patients with Meniere’s disease do not report a significant family history, a genetic basis has previously been suggested. Fransen et al. now describe a seven-generation Belgian family with autosomal-dominant progressive sensorineural hearing-loss, in which many affected individuals also suffered episodes of vertigo, tinnitus and aural fullness, consistent with Meniere’s disease1xHigh prevalence of symptoms of Meniere’s disease in three families with a mutation in the COCH gene. Fransen, E. et al. Hum. Mol. Genet. 1999; 8: 1425–1429Crossref | PubMed | Scopus (120)See all