对土耳其大量失聪儿童的评估。

Ozturk Ozcan, Silan Fatma, Oghan Fatih, Egeli Erol, Belli Seyda, Tokmak Abdurrahman, Egeli Ayten, Harputluoglu Ugur, Onder Halil Ibrahim, Zafer Cansu

Department of Otorhinolaryngology and Head and Neck Surgery, University of Abant Izzet Baysal, Duzce Faculty of Medicine, Duzce, Turkey.

Int J Pediatr Otorhinolaryngol. 2005 Mar;69(3):367-73. doi: 10.1016/j.ijporl.2004.11.001. Epub 2004 Dec 21.

OBJECTIVE

Evaluation of the deafness etiology, ear examination findings and hearing levels of deaf children in a large series.

PATIENTS AND METHODS

We studied 840 deaf primary school children (486 male, 354 female, mean age 12.69+/-3.52, range 5-22). A questionaire investigating the prenatal, perinatal and postnatal etiological causes was prepared and pedigree analysis was performed. After ENT examination, odyologic tests were performed.

RESULTS

The etiological features of deafness were found for genetic causes as 429 cases (51.1%), 127 children (15.1%) for acquired group and 284 children (33.8%) for unknown group. We confirmed 136 (15.5%) syndromic deaf children and 19 of them (13.9%) were autosomal recessive (AR), 105 of them (77.2%) were autosomal dominant (AD), six (4.4%) of them were X-linked. Two hundred and ninety-three deaf (33.4%) children were in the familial nonsyndromic group. In this group, the inheritance of 255 (87%) were AR, 23 (7.8%) were AD and 15 (5.2%) were X-linked recessive. Febrile convulsion was identified as the most common etiology in 36 (4.3%) cases in the acquired group. Three hundred and twenty-two (67.7%) children had profound HL (above 91 dB), 111 (23.3%) had severe HL and 43 (9%) had moderately severe HL. Sensorineural HL was found in 439 (92.2%) and mixed type hearing loss was seen 37 (7.8%) of 476 cases. We found many major and minor abnormalities and ocular, ear and dental pathologies. The prevalence of ear diseases was found in 203 (24.2%) of children. Impacted wax was found in 80 (9.5%) of 840 children with otoscopic examination and was the most common pathology, retraction in 70 (8.3%) and perforation in 15 (1.8%) followed it.

CONCLUSION

Preventable ear disease are important health problems among school children for the deaf because these diseases can affect the real level and type of deafness, so determining early diagnostic criteria, ear diseases and minor abnormalities is important for early rehabilitation. Syndromes can be prevented in pregnancy, infections can be prevented in prenatal or postnatal period but unknown group cannot be prevented although the unknown etiology can be reduced by multidiciplinary approach.

目的

对大量耳聋儿童的耳聋病因、耳部检查结果及听力水平进行评估。

患者与方法

我们研究了840名聋哑小学生（男486名，女354名，平均年龄12.69±3.52岁，范围5 - 22岁）。编制了一份调查产前、围产期及产后病因的问卷并进行系谱分析。耳鼻喉检查后，进行了听力学测试。

结果

发现耳聋的病因中，遗传因素占429例（51.1%），后天性因素占127例（15.1%），不明因素占284例（33.8%）。我们确诊了136例（15.5%）综合征性耳聋儿童，其中19例（13.9%）为常染色体隐性遗传（AR），105例（77.2%）为常染色体显性遗传（AD）, 6例（4.4%）为X连锁遗传。293例（33.4%）耳聋儿童属于家族性非综合征组。在该组中，255例（87%）为AR遗传，23例（7.8%）为AD遗传，15例（5.2%）为X连锁隐性遗传。热性惊厥被确定为后天性因素组中36例（4.3%）最常见的病因。322例（67.7%）儿童为重度听力损失（91dB以上），111例（23.3%）为重度听力损失，43例（9%）为中度重度听力损失。476例中有439例（92.2%）为感音神经性听力损失，37例（7.8%）为混合性听力损失。我们发现了许多主要和次要的异常以及眼、耳和牙的病变。203例（24.2%）儿童患有耳部疾病。在840例接受耳镜检查的儿童中，80例（9.5%）发现耵聍栓塞，这是最常见的病变，其次是70例（8.3%）中耳陷和15例（1.8%）鼓膜穿孔。