Usher's syndrome: electrophysiological tests of the visual and auditory systems.
The symptoms of Usher's syndrome - congenital hearing impairment and tapetoretinal dystrophy - are difficult to detect in young children. The electroretinogram (ERG), visual evoked potential (VEP), auditory nerve and brain-stem responses as well as the cochlear microphonic potentials were used to evaluate the defects of the visual and auditory systems in 20 patients, 3-45 years of age. This study demonstrates the usefulness, reliability and convenience of the electrophysiological tests for early diagnosis and functional evaluation of Usher's syndrome.
D000293:Adolescent; D000328:Adult; D001933:Brain Stem / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D003051:Cochlea / Q000503:physiopathology; D003638:Deafness / Q000151:congenital* / Q000175:diagnosis / Q000503:physiopathology; D004594:Electrophysiology; D004596:Electroretinography; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012160:Retina / Q000503:physiopathology; D012174:Retinitis Pigmentosa / Q000175:diagnosis / Q000235:genetics* / Q000503:physiopathology; D013577:Syndrome; D013997:Time Factors; D000159:Vestibulocochlear Nerve / Q000503:physiopathology; D014793:Visual Cortex / Q000503:physiopathology