[Genetic sensorineural hearing loss in childhood].
OBJECTIVE
This study aimed to determine the severity, age of presentation, and genetic etiology in hearing handicapped children.


METHODS
Three groups of children were studied: children who received treatment for hearing loss at the Otolaryngology Department of Ege University Hospital between 1993 and 1999 (1645 children) and throughout 2000 (212 children), and 443 students of Izmir Tülay Aktaş Deaf School during 1998 and 1999. All the children underwent otolaryngologic examination and a detailed history taking, audiometric examinations were undertaken in the second and third groups, as well. In children suspected of having a genetic etiology, the pedigrees were drawn and the mode of inheritance was determined.


RESULTS
In the first group, awareness of the families to hearing loss and the presentation were at ages two and four, respectively. The most common accompanying anomaly was Down syndrome. Consanguinity was found in 27%, 8%, and 33%, respectively. Moderate to severe hearing loss was detected in 44% of the second group, and in 28% of the third group. Of the pedigrees obtained from 123 families, 86 were nonsyndromic and 27 were syndromic.


CONCLUSIONS
Genetic inheritance plays a substantial role in sensorineural hearing loss in children, with consanguinity being the major culprit. Detection of hearing losses at younger ages will improve the success of hearing rehabilitation programs.
Turan
Onur
O
Ege Universitesi Tip Fakültesi Kulak Burun Boğaz Hastaliklari Anabilim Dali, Izmir, Turkey.
Apaydin
Fazil
F
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D001299:Audiometry; D002648:Child; D002675:Child, Preschool; D003241:Consanguinity; D012048:Correction of Hearing Impairment; D004314:Down Syndrome / Q000150:complications; D004519:Education, Special; D005260:Female; D020022:Genetic Predisposition to Disease; D006319:Hearing Loss, Sensorineural / Q000453:epidemiology* / Q000235:genetics* / Q000534:rehabilitation; D006801:Humans; D007223:Infant; D008297:Male; D010375:Pedigree; D012307:Risk Factors; D014421:Turkey / Q000453:epidemiology