ANTICIPATION IN MENIERES-DISEASE

The aetiology of Meniere's disease (MD) remains obscure, but is likely to be multifactorial, one of the factors being a genetic predispositi on. Forty-one families with more than one living member with MD were a scertained and affected and normal relations examined. Blood was colle cted and DNA extracted and stored. In these families the mode of inher itance is autosomal dominant, the penetrance of the mutation being abo ut 60 per cent. Some of the family members exhibit a partial syndrome, vestibular symptoms predominating. Sporadic and familial cases exhibi t the same clinical features. The striking finding is the phenomenon o f anticipation, whereby with successive generations there is an earlie r age of onset and a tendency to more severe manifestation. The infere nce, considering that the cells which regulate endolymph are of neuroe ctodermal origin, is that, like other neurodegenerative disorders whic h show anticipation, MD manifestation is likely to be related to trinu cleotide expansion within a gene.