CONGENITAL SENSORINEURAL DEAFNESS ASSOCIATED WITH EEG ABNORMALITIES, EPILEPSY AND HIGH FAMILIAL INCIDENCE

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Abstract
A clinical study was carried out on 31 deaf children attending a school for the deaf in Beer Sheva, Israel. Of the pupils, 71% had familial deafness. EEG studies showed a high rate of abnormal tracings associated with congenital sensorineural deafness. A significant number of EEG were paroxysmal. The majority of cases occurred among children with hereditary deafness. Behaviour disturbances were common among the children with abnormal EEG. Of the pupils, 1/3 had additional congenital defects, including 3 cases of retinitis pigmentosa. Four children (12.9%) had epilepsy; 2 cases of petit mal were of special interest. Hereditary aspects of congenital nerve deafness and the implications of the EEG findings are discussed.