摘要
目的探讨先天性中外耳畸形临床流行病学特征。方法对2006.4–2017.11来我院住院治疗(371例,448耳)及2016.10-2017.11月来我院门诊治疗(非住院)的(180例,202耳)先天性中外耳畸形患者共(551例,650耳)进行临床流行病学分析。结果男性403例(73.1%)>女性148例(26.9%);右侧275例(49.9%)﹥左侧177例(32.1%)﹥双侧99例(18.0%)。小耳畸形Ⅲ度最多,占83.0%(219/264耳,有照片者),小耳畸形程度越重纯音听力越差。外耳道闭锁90.6%(492/543耳,有记录或照片),狭窄7.6%(41/543耳),听骨畸形100%(88/88例,鼓室手术者)。其中375例有多发畸形和/或合并症,比例从高到低依次为:半面短小67.2%(252/375例)、副耳14.0%(68/485耳)、耳前瘘管10.7%(52/485耳)、面瘫7.5%(28/375例)、耳屏畸形7.4%(36/485耳)。远位脏器畸形:心脏畸形5.0%(2/40例,有心脏超声)、肾脏畸形2.8%(1/36例,有肾脏超声)、手指/足趾畸形1.3%(5/375例)。合并症比例从高到低依次为:外耳道胆脂瘤16.2%(32/198例,手术者)、耳后脓肿3.0%(6/198例)、中耳胆脂瘤2.5%(5/198例)。多发畸形发生率为87.7%(483/551例)。综合征Treacher-Collins综合征4%(15/375例);Goldenhar综合征2.4%(9/375例)。结论先天性中外耳畸形患者男性多于女性,右侧多于左侧多于双侧,小耳畸形Ⅲ度最多,程度越重纯音听力越差。98.2%外耳道闭锁或狭窄,100%听骨畸形。耳面颈部多发畸形依次为半面短小、副耳、耳前瘘管、面瘫、耳屏畸形,常见合并症有外耳道、耳后脓肿、中耳胆脂瘤。可有心脏、肾脏、四肢远位畸形或综合征。
Objective To report clinical epidemiologic characteristics of congenital deformation of middle and outer ears(CDMOE).Methods A total of 551 patients(650 ears)with CDMOE,including 371 patients(448 ears)hospitalized between 2006.4 and 2017.11 and 180 patients seen at the outpatient clinic from 2016.10 to 2017.11,were included.Results Of the patients,403(73.1%)were male and 148(26.9%)were females.Right side was involved in 75 cases(49.9%),left side in 177 cases(32.1%)and both sides in 99 cases(18%).Microtia was rated as grade III in 219 of 264 cases(83%),with its severity parallel to pure tone auditory thresholds.The incidence was 90.6%(492/543)for atresia of external auditory meatus and 7.6%(41/543)for meatal stenosis.Ossicular deformation was identified in all 88 cases undergoing exploratory tympanotomy.Multiple deformation and/or comorbidities were seen in 375 cases,including HFM(252/375,67.2%),accessory ear(68/485,14%),preauricular fistula(52/485,10.7%),facial paralysis(28/375,7.5%)and tragus deformation in(36/485,7.4%).Distant organ deformation involved heart(2/40,5.0%),kidney(1/36,2.8%,by ultrasound)and fingers/toes(5/375,1.3%).Complications included external auditory canal cholesteatoma(32/198,16.2%),middle ear cholesteatoma(10/198,5.1%)and retroauricular abscess(6/375,1.6%).Syndromic conditions included Treacher-Collins syndrome(15/375,4%)and Goldenhar syndrome(9/375,2.4%).Conclusions CDMOE is more common in males than in females,with the right side involved more often than the left side.Grade III microtia is most common with parallel pure tone hearing impairment.Meatal atresia or stenosis occur in 98.2%of patients with congenital external auditory malformation with all having ossicular deformity.Comorbid ear deformities(in the order of decreasing incidence)may include short half face,accessory ear,preauricular fistula,facial paralysis and deformity of the tragus.Common complications include external auditory canal,middle ear cholesteatoma and posterior ear abscess.Common complications can include cholesteatoma of outer or middle ear and retroauricular abscess.Other deformities involving the heart,kidney or extremities and syndromic conditions may also be seen.
作者
王青森
汤丽川
邹艺辉
WANG Qingsen;TANG Lichuan;ZOU Yihui(Department of Otolaryngology-Head,Neck Surgery,Chinese PLA General Hospital,Beijing 100853,China)
出处
《中华耳科学杂志》
CSCD
北大核心
2018年第1期26-29,共4页
Chinese Journal of Otology
基金
解放军总医院临床扶持基金(2016FC-CXYY-2009)
解放军总医院专利转化基金(42411D19)~~
关键词
先天性
中外耳畸形
临床流行病学
Congenital Deformation
Middle and Outer Ears
Clinical Epidemiology
