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NEUROLOGY 2004;63:2376-2379
© 2004 American Academy of Neurology

Suggestive linkage to chromosome 6q in families with bilateral vestibulopathy

J. C. Jen, MD, PhD, H. Wang, MS, H. Lee, BS, C. Sabatti, PhD, R. Trent, MB, PhD, FRACP, I. Hannigan, RN, K. Brantberg, MD, PhD, G. M. Halmagyi, MD, FRACP, S. F. Nelson, MD and R. W. Baloh, MD

From the Departments of Neurology (Drs. Jen and Baloh), Surgery (Head and Neck) (Dr. Baloh), and Human Genetics (Drs. Wang, Lee, Sabatti, and Nelson), UCLA School of Medicine, Los Angeles, CA; Department of Clinical Neurophysiology (Drs. Trent, Hannigan, and Halmagyi), University of Sydney, Australia; and Department of Audiology (Dr. Brantberg), Karolinska Hospital, Stockholm, Sweden.

Address correspondence and reprint requests to Dr. Joanna C. Jen, UCLA Dept. of Neurology, Box 951769, Los Angeles, CA 90095-1769; e-mail: jjen{at}ucla.edu

Background: Of the more than 40 genetically defined dominantly inherited hearing loss syndromes, only a few are associated with bilateral vestibulopathy. No genetic mutations have been identified in families with bilateral vestibulopathy and normal hearing.

Objective: To perform a genome-wide scan for linkage in four families with dominantly inherited bilateral vestibulopathy.

Methods: Patients in four families reported brief episodes of vertigo followed by imbalance and oscillopsia. Bilateral vestibulopathy was documented with quantitative rotational testing. Most patients with bilateral vestibulopathy also had migraine. A 10 cM genome-wide screen was conducted using 423 microsatellite markers to identify linkage with vestibulopathy.

Results: The authors identified a 24 cM region on chromosome 6q suggestive of linkage to vestibulopathy in these four families (maximum lod score of 2.9 at marker D6S1556). A small fifth family with a different phenotype was not linked to this region on chromosome 6q.

Conclusions: This is the first report of linkage in families with dominantly inherited vestibulopathy and normal hearing. Genetic heterogeneity is likely with inherited vestibulopathy.

Received July 2, 2004. Accepted in final form October 14, 2004.






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