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Familial vestibulopathy

A new dominantly inherited syndrome

Departments of Neurology and Surgery (Head and Neck), University of California, Los Angeles, School of Medicine, Los Angeles, CA.

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.

Address correspondence and reprint requests to Dr. Robert W. Baloh, Department of Neurology, Reed Neurological Research Center, UCLA School of Medicine, Los Angeles, CA 90024-1769.

Supported by NIH grants NINCD DC 01404 and NIA AG 09693.

Received May 10, 1993. Accepted for publication in final form June 18, 1993.

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