Vestibulocochlear Dysfunction, Progressive

Verhagen et al. (1988) described a family with vestibular areflexia and progressive hearing loss developing from the age of about 40 years onward and displaying a pattern of autosomal dominant inheritance. Verhagen and Huygen (1991) reported the same disorder in a member of the family previously thought to be unaffected at the age of 42 years. At the age of 47 years she showed progressive hearing loss and left-sided tinnitus as well as head movement-dependent oscillopsia and instability in the dark.