摘要目的 探讨先天性外耳道狭窄合并胆脂瘤的临床特征,提高其诊治率.方法 回顾性分析2003年3月至2006年6月间收治的伴有窦道形成的先天性外耳道狭窄合并胆脂瘤患者的临床资料,共10例(10耳).结果 10耳均有耳后皮肤破溃流脓史,外耳道口直径约2 mm,颞骨CT显示10耳均有外耳道区域骨质吸收缺损,致骨性耳道增宽,增宽的外耳道内被软组织密度影填充.术中见10耳之外耳道内均充满胆脂瘤,且伴有耳道后上壁及下壁骨质压迫吸收.10耳均行外耳道成形术,其中8耳同时行听力重建术.术后病理报告均为胆脂瘤组织,未见腮裂囊肿及瘘管组织.10耳术后外耳道均通畅,行听力重建术的8耳,听力改善(听力级)20～35 dB.随访1～3年,未见胆脂瘤复发.结论 先天性外耳道狭窄伴发胆脂瘤者,其外耳道骨壁极易受累及,从而出现外耳道骨壁缺损及耳后或颈部窦道形成,颞骨CT检查可发现外耳道骨壁缺损及胆脂瘤密度影.

abstractsObjective To investigate the clinical features, differential diagnosis and management of congenital stenosis of external auditory canal (CSEAC) with cholesteatoma. Methods The clinical information for 10 cases of CSEAC with cholesteatoma was retrospectively reviewed. Results The patients' ages ranged from 4.75 to 22 years (average 12 years). The diameter of the external auditory canal (EAC) was <2 mm. All 10 ears had a history of postaural fistulae or sinuses. Bone erosion of EAC was distinctly shown in high-resolution computed tomography (HRCT) of all cases, as well as soft tissue masses, which led to enlargement of the bony canals. All patients underwent canaloplasty;eight ears received hearing reconstructions at the same time. Cholesteatoma in EACs was confirmed during the operations, accompanied by compression and destruction of the post-superior and/or inferior bony wall. Postoperative pathologic examinations proved the diagnosis of cholesteatoma, and excluded any tissue of bronchial cleft cyst or fistula. After a follow-up 1 to 3 years, no recurrent cholesteatoma was found in any of the 10 cases. All reconstructed EACs were clean and smooth. The hearing levels in the eight ears that received hearing reconstructions improved 20-35 dBHL. Conclusions In CSEAC with eholesteatoma, the bony wall of EAC is most commonly involved. This involvement will lead to bone erosion of the EAC and may subsequently lead to the formation of postaural or cervical sinuses. HRCT of temporal bone can show characteristic signs of soft tissue mass in EAC, with adjacent bone erosion.