先天性中耳胆脂瘤

目的探讨先天性中耳胆脂瘤的位置、范围、临床症状、影像学特征及手术治疗。方法本文回顾性分析了解放军总医院耳鼻咽喉-头颈外科自1995年1月～2005年10月诊治的952例胆脂瘤患者的治疗结果,对其中10例先天性中耳胆脂瘤的起源、临床特征及手术前后的听力进行了评估。结果10例先天性中耳胆脂瘤患者的平均年龄为16岁(10～24岁),其中7例为男性,3例为女性,出现症状到临床确诊的平均时间为2年。术前平均纯音听阈为55dB,平均气-骨导差为45dB,有2例患者的鼓膜像上可以看到典型的先天性中耳胆脂瘤表现。所有病例均进行了高分辨率颞骨CT扫描。8例术前分别被诊断为耳硬化症或听骨链畸形。所有病例都在外耳道径路鼓室探查清除胆脂瘤后进行了一期鼓室成型术,除了1例因为病变广泛选择了完壁式乳突根治和鼓室成型术。本组所有病例的胆脂瘤均位于中-后鼓室,主要是在砧镫关节处。术后的平均气-骨导差小于20dB。所有病例术后至少随访1年半,2例病人因为术后听力下降进行了二期手术。本组病例中经手术探查或CT复查,没有发现胆脂瘤残留或复发。结论原发性中耳胆脂瘤临床罕见,常被延误诊断,其预后和残留、复发比例主要决定于病变范围。

Congenital cholesteatoma of the middle ear

Objective To investigate the location,extension,clinical symptom,image features,and operation treatment of the congenital cholesteatoma of middle ear. Methods This was a retrospective review of 952 cholesteatomas patients treated from January 1995 to December 2005 at Department of Otolaryngology-Head and Neck Surgery,General Hospital of PLA,Beijing. Among them,the origin,dinical features and pre-and-post operottive hearing of 10 congenital cholesteatoma of the middle ear cases were assessed. Results The mean age of 10 cases with congenital cholesteatoma of the middle ear was 16 years (ranged from 10 to 24 years old),6 (%) cases were older than 18 years. There were 7 men and 3 women,and the average delay of diagnosis was more than 2 year. The mean preoperative PTA was 55 dB,the mean ABG was 45 dB. Only in 2 patients,typical cholesteatomas were seen behind eardrum image. HRCT were completed in all patients. Most of the patients (8 cases,80%) were diagnosed as otosclerosis or malformation of the ossicular chain before operation. All the patients underwent a one-stage tympanoplasty after drum exploration by external auditory meatus and the removal of cholesteatoma except one,who underwent a CWU mastoidectomy for extensive cholesteatoma. The choleasteatomas localized to the posterior tympanum or mesotympanum in all the patients. The mean postoperative A-B gap was within 20 dB. All patients were followed-up at least for 1.5 years after operation secondary operation was performed in 2 patients because the hearing declined. No residual or recurrence of cholesteatoma was found. Conclusion Congenital cholesteatoma of middle ear (CCME) is a rare entity and usually delayed to diagnosis in clinical practice. The rate of residual lesions and the prognosis of CCME were mainly depended on the extensions of the lesions.