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    Etiology of deafness at the Yeditepe School for the deaf in Istanbul
    (Elsevier Sci Ireland Ltd, 2003) Egeli, Erol; Çiçekci, Günferi; Sılan, Fatma; Öztürk, Özcan; Harputluoğlu, Uğur; Onur, Aşkın; Egeli, Ayten
    Objective: The etiology of deafness can be classified as genetic, acquired and unknown. An unknown etiology was a high incidence in previous reports. The aim of this study is to explore the etiology of deafness and to reduce the cases in the unknown group. Methods: This study was conducted on 162 students at the Yeditepe School for the deaf. Otologic, physical and psychological. examinations were performed by a team of doctors including four otologists, a geneticist, a dentist, an ophthalmologist and an audiologist. Results: A genetic cause was identified in 41.35%, acquired 37.65% and unknown 20.98%. Genetic etiologies were stratified as familial and syndromic subgroups. A familial cause was found in 60 of 162 children and in 22 of those 60 cases, the parents had a consanguineous marriage. There was 11 cases associated with a syndrome. Ninety four minor abnormalities have been established in some deaf children. Febrile convulsion (36%) was identified as the most common etiology in acquired cases. Conclusion: In contrast with other studies the consanguineous marriage was present in 36.6% of the familial cases and 38.8% in total of the school. An unknown etiology was reported in high rates in previous reports. These unknown cases could be described as a part of a syndrome by a crowded team of consultants. Evaluation of early diagnostic criterias and minor abnormalities can help us to provide early rehabilitation of deafness in childhood.
  • Küçük Resim
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    Syndromic etiology in children at schools for the deaf in Turkey
    (Elsevier Sci Ireland Ltd, 2004) Sılan, Fatma; Demirci, Levent; Egeli, Ayten; Egeli, Erol; Önder, Halil İbrahim; Öztürk, Özcan; Ünal, Zehra Seda
    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, ophtalmologic 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, multidiciplinary approach and genetic counselling are necessarry in this regard.