Deafness, Unilateral

Clinical Features

Smith (1939) described a sibship of 8 children, 4 of whom had total deafness in one or the other ear. The tympanic membranes were normal. Labyrinthine testing was normal. There was no history of consanguinity, mumps, or syphilis. The mother, her father, and her sister also had unilateral deafness while another sister became deaf and lost speech after measles. This latter sister married a deaf and dumb man. One of their 3 children, a girl, had unilateral deafness. She had 2 children, one of whom has unilateral deafness. Thus there were 9 persons with total unilateral deafness in 4 generations. Four were deaf in the right ear and 4 in the left, while the side was unknown in 1 case. Everberg (1960) studied 122 children with total unilateral deafness and normal hearing in the other ear. More than 1 case of unilateral deafness in the same family was found in 12 of the 122 families of these children.