Familial hereditary progressive XXXX among Saudi population.

@entity1 @entity2803 @entity1274 @entity3252 @entity393 @entity15206
Clinical and audiological studies of 234 Saudi @entity1 from the central area of Saudi Arabia with progressive @entity1274 were carried out in Riyadh. One hundred and sixty-four came from 38 families, i.e. 70% of the total and 30% were sporadic cases. Consanguinity was found in 80.8%. Their @entity393 was characterized as being bilateral sensorineural, starting at 1 kHz frequency and gradually sloping the pure tone audiogram to severe and profound @entity393 at 8 kHz. Special tests for recruitment indicate hair cell lesions. Caloric tests were normal in 56 @entity1 tested. No abnormal internal ears were found in a C.T. scan done for 84 cases. Serology for @entity15206 and non- @entity15206 agents were done for (124) selected cases. Syphilis was negative, however, positive immunoglobulins for @entity2803 , herpes simplex type 1 and 2, and @entity3252 were seen in 52 cases. All @entity1 came from one area called Qassim where old customs and the tradition of consanguineous marriage were still practiced. Some of these families are now under molecular and cytogenetic study.
