Atresia Of External Auditory Canal And Conductive Deafness

Clinical Features

Hefter and Ganz (1969) described a woman and 3 of her 4 children with atresia of the external auditory canal and conductive deafness. The bony stenosis of the external meatus was so marked that the eardrums were not visible. The mastoid processes were found to be poorly pneumatized on radiography. At surgery the middle ear structures were found to be in various stages of hypoplasia or aplasia.

Robinow and Jahrsdoerfer (1979) observed an extensively affected kindred with 12 affected members over 5 generations. There were several instances of male-to-male transmission. Stenosis rather than atresia of the auditory canal was present in some. There were no associated anomalies.

Inheritance

The transmission pattern of the disorder in the families reported by Hefter and Ganz (1969) and Robinow and Jahrsdoerfer (1979) was consistent with autosomal dominant inheritance.