Congenital sensorineural deafness associated with EEG abnormalities, epilepsy and high familial incidence

A clinical study was carried out on 31 deaf children attending a school for the deaf in Beer Sheva, Israel. 71 per cent of the pupils were found to have familial deafness. EEG studies showed a high rate of abnormal tracings associated with congenital sensorineural deafness. A significant number of EEGs were paroxysmal. The majority of cases occurred among children with hereditary deafness. Behaviour disturbances were common among the children with abnormal EEGs. One-third of the pupils had additional congenital defects, including three cases of retinitis pigmentosa. Four children (12 X 9 per cent) had epilepsy; two cases of petit mal were of special interest. Hereditary aspects of congenital nerve deafness and the implications of the EEG findings are discussed.