Oculomotor, Auditory, and Vestibular Responses in Myotonic Dystrophy

• Published in: Archives of neurology (Chicago) Vol. 49; no. 9; pp. 954 - 960
• Main Authors: Verhagen, Wim I. M, ter Bruggen, Jan Pieter, Huygen, Patrick L. M
• Format: Journal Article
• Language: English
• Published: Chicago, IL American Medical Association 01.09.1992
• Subjects: Adolescent; Adult; Auditory Perception; Biological and medical sciences; Diseases of striated muscles. Neuromuscular diseases; Evoked Potentials, Auditory, Brain Stem; Eye Movements; Female; Hearing; Hearing Disorders - physiopathology; Humans; Male; Medical sciences; Middle Aged; Myotonic Dystrophy - physiopathology; Neurology; Nystagmus, Pathologic - physiopathology; Oculomotor Muscles - physiopathology; Reflex, Abnormal; Space life sciences; Vestibule, Labyrinth - physiopathology; Human; Electrooculography; Vestibular function; Neuromuscular diseases; Nervous system diseases; Audiometry; Myotonic dystrophy; Brain stem; Auditory evoked potential; Exploration; Saccadic eye movement
• ISSN: 0003-9942; 1538-3687
• DOI: 10.1001/archneur.1992.00530330078020

• 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.