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NEUROLOGY 1991;41:1878
© 1991 American Academy of Neurology

Hearing loss in facioscapulohumeral muscular dystrophy

O. F. Brouwer, MD, G. W. Padberg, MD, PhD, C.J.M. Ruys, MSc, R. Brand, PhD, J. A.P.M. de, Laat, PhD and J. J. Grote, MD, PhD

From the Departments of Neurology (Drs. Brouwer and Padberg, and C.J.M. Ruys), Medical Statistics (Dr. Brand), and Otorhinolarymgology (Drs. de Laat and Grote), University of Leiden, The Netherlands.

The coincidence of facioscapulohumeral muscular dystrophy (FSHD) with sensorineural hearing loss and retinal abnormalities might imply genetic heterogeneity of FSHD. We performed screening audiometry in 56 patients with autosomal dominant FSHD and in 72 healthy family members, and found that the difference in hearing level between 4,000 Hz and 6,000 Hz was significantly greater in FSHD patients than in controls, independently both for the left and right ear. We conclude that this change of hearing function is part of the disease and may lead to severe hearing loss in some patients. The fact that it was present in all families is another argument against genetic heterogeneity.

Address correspondence and reprint requests to Dr. O.F. Brouwer, Department of Neurology, University Hospital Leiden, P.O. Box 9600, 2300 RC Leiden, The Netherlands.

Supported by a grant from the "Association Française contre les Myopathies."

Received April 2, 1991. Accepted for publication in final form May 21, 1991.




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