WHILE account is taken of the many new papers on the auditory system in animals, this report is concerned particularly with clinical studies on sensorineural deafness.
Cassidy et al1 studied hereditary kidney disease associated with deafness (Alport's syndrome) in 476 members of seven families, and found that more than one half of the men and one third of the women had hearing losses. Early deafness and severe renal disease yielded a bad prognosis, particularly in the men. Late progressive deafness with kidney disease and normal life span was also seen. A symptomatic, mild, high-tone loss in the women with this syndrome was a significant finding. In addition to red cells and protein in the urine, there was inability to conserve water in the face of dehydration.
A syndrome of severe nerve deafness associated with an abnormal electrocardiogram, syncopal attacks, and sudden death in childhood is described in nine