Syndromal approaches to the nosology of hereditary deafness

Seventy types of hereditary deafness can be distinguished using the following features of the syndrome: 1) mode of genetic transmission, 2) characteristics of the deafness, 3) age of onset, 4) sonic frequencies involved and 5) associated abnormalities. The gene, in single or double dose, causing the hearing loss may also cause abnormalities of the external ear, or of the integumentary, visual, nervous, skeletal, or urinary systems. In this report the characteristics of the hearing loss in each of the types of hereditary hearing loss are reviewed in eight tables.