Understanding Hereditary Nerve Deafness

Abstract

THE PROBLEM of hereditary nerve deafness is indeed a complex and challenging one. With the otologist having very little to offer medically or surgically, except in the rare case of a conductive malformation combined with the sensorineural loss, treatment today is limited largely to diagnosis and rehabilitation. Because some forms of hereditary deafness may develop insidiously and not become clinically apparent until later in life, because hereditary nerve deafness is sometimes associated with other abnormalities which may overshadow it, and because hereditary factors may be concealed, it is difficult to accurately estimate the frequency of this disease. It appears that hereditary deafness is actually a fairly common disease entity, possibly occurring somewhere between 1 in 2,000 and 1 in 6,000 births. Of all cases of congenital deafness, including deafness secondary to rubella, birth injury, and erythroblastosis, about one third to one half seem to be due to hereditary factors. At