[Menière's disease : evidence and controversies]

Menière's disease is recognized as the idiopathic form of recurrent inner ear disease with the trias of hearing loss, tinnitus and vertigo with aural fullness and Menière's syndrome as the non-idiopathic form. Subentities with unknown pathogenesis are Lermoyez' s syndrome and Tumarkin crises. A common pathogenetic factor is the disturbance of endolymphatic and perilymphatic osmotic and hydrostatic pressure due to defined etiologies or to idiopathic attacks. Etiologies of Menière's syndrome can be pathologic middle ear pressure, anomalies of the vestibular and cochlear aqueducts, round window topography, patency of the ductus peruniens and the utriculo-endolymphatic valve. Indications for treatment are assessed according to the AAO-HNS guidelines and the neurotological function tests and dizziness inventories. Betahistine is recommended as first choice medical treatment as on-label or high dosage administration. In case of medical treatment failure and if hearing is worth saving, endolymphatic shunt surgery (ELS) is the first choice. If deafferentiation of the labyrinth is needed vestibular neurectomy (VE) should be performed. Local gentamicine administration has good long-term results but macula function can often recover and hearing is often deteriorated. Future aspects for the treatment are new experimental results with gene transfer to vestibular hair cells.