Oculomotor, auditory, and vestibular responses in myotonic dystrophy.
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 vestibulo-ocular 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.
Verhagen
W I
WI
Department of Neurology, Canisius-Wilhelmina Hospital Nijmegen, The Netherlands.
ter Bruggen
J P
JP
Huygen
P L
PL
D000293:Adolescent; D000328:Adult; D001307:Auditory Perception; D016057:Evoked Potentials, Auditory, Brain Stem; D005133:Eye Movements; D005260:Female; D006309:Hearing; D006311:Hearing Disorders / Q000503:physiopathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009223:Myotonic Dystrophy / Q000503:physiopathology*; D009759:Nystagmus, Pathologic / Q000503:physiopathology; D009801:Oculomotor Muscles / Q000503:physiopathology; D012021:Reflex, Abnormal; D014722:Vestibule, Labyrinth / Q000503:physiopathology*