上海市先天性外畸形的临床分析.pdfVIP

上海市先天性外耳畸形的临床分析 摘 要 目的：了解先天性外耳畸形患儿的临床听力学特点、CT 表现，并进 行言语评估，为今后诊治先天性外耳畸形患儿提供依据。 方法：对自2005 年1 月到2008 年12 月至上海儿童医学中心就诊的 出生于上海市的先天性外耳畸形儿童73 例进行随访。将患儿分为耳廓畸 形加外耳道完全闭锁(闭锁组)、耳廓畸形加外耳道狭窄(狭窄组)及单纯 耳廓畸形，外耳道及鼓膜正常(单纯耳廓畸形组)。并将耳廓畸形按Marx 分类分为三度。对患者进行听性脑干反应测试（ABR）、骨导ABR、颞骨 高分辨 CT 检查，Gesell 发育量表进行言语能测试，综合评估其听觉能 力，并进行结果分析。 结果：1. 73 例中单耳病变62 例，双耳病变11 例，单侧病变占84.9%。 男性48 例，女性25 例，男性占65.7% 。84 耳中左侧30 耳，右侧54 耳， 右侧占64.2% 。2. 本次随访73 名儿童中发现颌面畸形7 例，脊柱畸形1 例，先天性心脏病4 例，并指(趾)畸形1 例，先天性腭裂2 例。3. 闭锁 组39 耳,中度及以上听力损失33 耳, 占84.6%。狭窄组31 耳，中度及以 上听力损失 12 耳，占38.7% 。单纯耳廓畸形组14 耳，中度及以上听力 损失5 耳，占35.7% 。 结论：本研究中先天性外耳畸形流行病学（性别偏向，单侧偏向） 与国外流行病学接近。本研究中先天性外耳畸形的伴发畸形比例明显低 于国内外参考文献。外耳畸形合并中耳畸形的比例较高，但还是有少部 分儿童合并内耳畸形，对这部分儿童的言语发育还需引起重视。用Gesell 发育量表进行语言评估，显示单纯外、中耳畸形的患儿言语发育略差于 正常儿童。 关键词：先天性，外耳，听觉评估 THE CLINICAL ANALYSIS OF THE CONGENITAL MALFORMATIONS OF THE EXTERNAL EAR IN SHANGHAI ABSTRACT Objective: To explore the audiological characteristics and the HRCT features of children with congenital malformations of the external ear ．To evaluate the degree of language development quotient ．And to provide guide for the further work. Patients and Methods: Seventy-three patients with congenital malformations of the external ear during 2005 to 2008 were involved in this study ．The children were divided into three groups according to the appearance of the external ear ．The first group consisted of 36 patients with auricle malformation and atresia of the external auditory meatus ．The second group consisted of 25 patients with abnormal external auditory meatus (no atresia)and auricle malformation ．The other 12 patients only with auricle malformation were included in the third group ． Resul