Familial Progressive Vestibulocochlear Dysfunction Caused by a COCH Mutation (DFNA9)

Abstract

Our patient proved to suffer from an autosomal dominant vestibulocochlear disorder caused by a COCH gene mutation. The remarkable medical history has some features in common with Meniere disease; however, there are also different clinical and neurophysiological features. In the family, phenotypic variability is present.