Three patients who presented with episodic vertigo followed by gait imbalance and oscillopsia had profound bilateral vestibular loss despite normal hearing. All had a parent with similar findings. The patients, their affected parent, and multiple other family members had a history of migraine headaches, although several of the latter had normal vestibular function. Acetazolamide stopped or markedly decreased the frequency of vertigo attacks in the three patients treated but had little effect on the chronic vestibular loss. This is the first report of a dominantly inherited bilateral vestibulopathy associated with normal hearing.