Egeli Erol, Ciçekci Günferi, Silan Fatma, Oztürk Ozcan, Harputluoğlu Uğur, Onur Aşkin, Egeli Ayten, Yildiz Aysel
Department of Otorhinolaryngology and Head Neck Surgery, Düzce Faculty of Medicine, University of Abant Izzet Baysal, Konuralp, Turkey.
Int J Pediatr Otorhinolaryngol. 2003 May;67(5):467-71. doi: 10.1016/s0165-5876(03)00002-8.
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.
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.
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.
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.
耳聋的病因可分为遗传性、后天性和不明原因。在以往的报告中，不明原因的发病率较高。本研究的目的是探讨耳聋的病因，并减少不明原因组的病例。
本研究对耶迪特佩聋校的162名学生进行。由包括四名耳科医生、一名遗传学家、一名牙医、一名眼科医生和一名听力学家在内的医生团队进行耳科、体格和心理检查。
确定遗传原因占41.35%，后天性占37.65%，不明原因占20.98%。遗传病因分为家族性和综合征性亚组。在162名儿童中有60名发现有家族性原因，在这60例中有22例父母为近亲结婚。有11例与综合征有关。在一些耳聋儿童中发现了94种轻微异常。热性惊厥（36%）被确定为后天性病例中最常见的病因。
与其他研究相比，近亲结婚在家族性病例中占36.6%，在学校总数中占38.8%。在以往的报告中，不明原因的比例很高。通过一个庞大的顾问团队，这些不明原因的病例可以被描述为综合征的一部分。对早期诊断标准和轻微异常的评估可以帮助我们为儿童期耳聋提供早期康复。
