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Wim I. M. Verhagen, PhD; Jan Pieter ter Bruggen; Patrick L. M. Huygen, PhD

Arch Neurol. 1992;49(9):954-960.

Abstract
• In 13 patients with myotonic dystrophy, oculomotor, auditory, and vestibular tests were performed. All 13 patients showed one or more abnormalities. There was a significant increase in the penetrance of the separate abnormalities with age. Saccadic slowing was found in 10 patients, in a severe form in three. Seven patients had a sensorineural high-tone hearing loss (30 to 85 dB at 8 kHz), which was in excess of that expected for their age, that could be attributed to myotonic dystrophy. Brain-stem auditory evoked potentials showed a significant interwave delay of the I-V interval (0.35 to 0.7 milliseconds). An abnormal vestibuloocular reflex was found in six patients; three had vestibular hyperreflexia with increased gain, and three had hyporeflexia with short time constants. This study confirms that in myotonic dystrophy, sensory system involvement can be found on both a peripheral and a central level.

Author Affiliations
From the Departments of Neurology, Canisius-Wilhelmina Hospital, Nijmegen (Dr Verhagen), and De Wever Hospital, Heerlen (Dr ter Bruggen), and the Department of Otolaryngology, University Hospital Nijmegen (Dr Huygen), the Netherlands.

Footnotes
Accepted for publication April 27, 1992.

Reprint requests to Department of Neurology, Canisius-Wilhelmina Hospital Nijmegen, PO Box 9015, 6500 GS Nijmegen, the Netherlands (Dr Verhagen).

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