|
|
|
|
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 8kHz),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 hyporefelxia with increased gain,and three had hyporeflexia with short term constants.This study conforms that in myotonic dystrophy,sensory system involvement can be found on both a peripheral and a central delay. |
|