Familial early-onset progressive vestibulopathy without hearing impairment

A family with early-onset vestibulopathy is presented. The 34-year-old father had experienced brief attacks of vertigo and currently suffers from unsteadiness and oscillopsia during head movements. His two young sons also experience brief attacks of spontaneous vertigo. In vestibular testing all three subjects showed reduced caloric responses. However, only the father showed reduced otolith function (as reflected by the vestibular-evoked myogenic potentials). Further, all three subjects had walked before the age of 1 year and none of them had had any auditory symptoms. It is suggested that they have familial early-onset progressive vestibulopathy affecting the canals before the otoliths but sparing cochlear function.