Three patients who presented with episodic vertigo followed by gait im
balance and oscillopsia had profound bilateral vestibular lass despite
normal hearing. All had a parent with similar findings. The patients,
their affected parent, and multiple other family members had a histor
y of migraine headaches, although several of the latter had normal ves
tibular function. Acetazolamide stopped or markedly decreased the freq
uency of vertigo attacks in the three patients treated but had little
effect on the chronic vestibular loss. This is the first report of a d
ominantly inherited bilateral vestibulopathy associated with normal he
aring.