Syndromic etiology in children at schools for the deaf in Turkey

• Published in: International journal of pediatric otorhinolaryngology Vol. 68; no. 11; pp. 1399 - 1406
• Main Authors: Silan, Fatma, Demirci, Levent, Egeli, Ayten, Egeli, Erol, Onder, Halil Ibrahim, Ozturk, Ozcan, Unal, Zehra Seda
• Format: Journal Article
• Language: English
• Published: Ireland Elsevier Ireland Ltd 01.11.2004
• Subjects: Abnormalities, Multiple - epidemiology; Adolescent; Adult; Audiometry; Child; Consanguinity; Deafness; Disabled Children - education; Education of Hearing Disabled; Etiology; Female; Genetic; Hearing Loss, Bilateral - epidemiology; Hearing Loss, Bilateral - etiology; Hearing Loss, Sensorineural - epidemiology; Hearing Loss, Sensorineural - etiology; Humans; Male; Schools; Syndrome; Turkey - epidemiology; Turkey; Genetic; Deafness; Etiology; Syndrome
• ISSN: 0165-5876; 1872-8464
• DOI: 10.1016/j.ijporl.2004.05.007

The aim of this study was to determine the syndromic etiology of bilateral severe sensorineural hearing disorders in children and current etiological causes to reduce the cases in the unknown group. This study was conducted on 550 students of five schools for the deaf in Istanbul and Zonguldak, Turkey. Otologic, audiologic, dysmorphologic, ophtalmologic and dental examinations were performed in all children. Familial and medical histories were obtained. The etiology of hearing loss was genetic in 346 (62.90%), acquired in 107 (19.45%) and unknown in 97 (%17.63) cases. A total of 619 malformations were defined in 550 children and 99 of them belonged to a syndrome. We identified 33 different syndromes for these 99 syndromic children. Syndromic etiology was found in 18.0% of the total and 28.61% of the subjects with genetic etiology. Most common syndrome was Waardenburg syndrome which occurred in 33 children. The incidence of hereditary hearing impairment is very high in developing countries compared to developed countries. Prevention is essential to reduce the incidence, multidiciplinary approach and genetic counselling are necessarry in this regard.