This study is based on the data concerinig 1060 childern with hearing loss, except for those of external origin, examined at our pedo-audiological clinic between 1970 and 1991. Among the 1060cases, genetic hearing loss with associated abnormalities was diagnosed in 25 cases (2.4%).
As to these 25 cases:
1) Almost all these cases showed bilateral and sensorineural hearing loss.
2) The degree of the hearing level showed moderate and severe loss.
3) As to the genetic causes of hearing loss, follwoing the classification by Konigsmark, there were more cases with the autosomal dominant transmission than in the other types, i. e. the autosomal dominant hearing loss was diagnosed in 14 cases out of the 25 (56%), the autosomal recessive type was in 8 cases (32%), the sex-linked type was in one case only.
Based on these findings, we conclude that it is very important to examine detailed family history including audiological examinations and also check the general status for diagnosis concerning genetic hearing loss.
