CAJ | 학술논문

目的：分析讨论先天性前庭窗缺如或闭锁的影像学诊断技术及个性化手术方法。方法采用高分辨率CT(High resolution CT, HRCT)、后期多平面重组（Multi-plane reformation, MPR)技术对2010.7-2014.8临床上表现为自幼传导性耳聋、外耳道鼓膜正常、鼓室压图为“A”型曲线、盖莱试验阴性的患者进行筛选诊断，并进行外科听力重建手术。结果术前HRCT及MPR影像诊断为先天性前庭窗畸形6例（耳）、先天性镫骨底板固定2例（耳），经术中探查证实为前庭窗、镫骨及面神经畸形，其中6例以TORP或Piston进行个性化听力重建。结论 HRCT冠状位及MPR可较好的显示前庭窗、镫骨及面神经的畸形，尤其是对固定的镫骨底板及前庭窗闭锁骨板的区分；对于前庭窗闭锁且被面神经完全覆盖的病例，鼓岬开窗不失为一种有效的方法，目前国内外文献尚未见此技术的报告。
목적：분석토론선천성전정창결여혹폐쇄적영상학진단기술급개성화수술방법。방법채용고분변솔CT(High resolution CT, HRCT)、후기다평면중조（Multi-plane reformation, MPR)기술대2010.7-2014.8림상상표현위자유전도성이롱、외이도고막정상、고실압도위“A”형곡선、개래시험음성적환자진행사선진단，병진행외과은력중건수술。결과술전HRCT급MPR영상진단위선천성전정창기형6례（이）、선천성등골저판고정2례（이），경술중탐사증실위전정창、등골급면신경기형，기중6례이TORP혹Piston진행개성화은력중건。결론 HRCT관상위급MPR가교호적현시전정창、등골급면신경적기형，우기시대고정적등골저판급전정창폐쇄골판적구분；대우전정창폐쇄차피면신경완전복개적병례，고갑개창불실위일충유효적방법，목전국내외문헌상미견차기술적보고。
Objective To analyse and discuss the radiological diagnosis and surgical technology of the congenital atresia of the oval window and related structure. Methods From July 2010 through August 2014, the high resolution CT (HRCT) and the multi-planar reformation (MPR) were used to detect the patients with conductive hearing loss, normal external ear canal, in?tact tympanic membrane, type A tympanogram and negative Gelle’s test. The patients with malformation of oval window and re?alated structure underwent surgical exploration. Result Six patients (ears) were detected congenital absence or atresia of the oval window with malformed facial nerve (class 4B) and two patients (ears) were detected footplate fixation (class 2). In the surgi?cal exploration of these ears, the malformed oval window, stapes and facial never was approved. The hearing reconstruction was made in six ears. The vestibular and promontory drilled-out technique was used to reconstruct the ossicular chain. Conclusion The coronal HRCT CT imaging and the MPR can provide us practical and definite information of abnormal oval window, stapes and facial nerve, especially in the discrimination of OW atresia plate and the fixed stapedial footplate. The promontory drill-out technique, fenestration in the bottom of the scala tympani of the basal turn, provides us a new method in the hearing reconstruc?tion when the area of OW was fully covered by malformed facial nerve. This technique was first reported in the literature.