Syndromic etiology in children at schools for the deaf in Turkey.

OBJECTIVE

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.

METHODS

This study was conducted on 550 students of five schools for the deaf in Istanbul and Zonguldak, Turkey. Otologic, audiologic, dysmorphologic, ophthalmologic and dental examinations were performed in all children. Familial and medical histories were obtained.

RESULTS

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.

CONCLUSION

The incidence of hereditary hearing impairment is very high in developing countries compared to developed countries. Prevention is essential to reduce the incidence, multidisciplinary approach and genetic counselling are necessary in this regard.