颞骨先天性胆脂瘤11例分析

A Study of 11 Cases of Conganital Cholesteatoma in Temporal Bone

目的探讨颞骨先天性胆脂瘤的病变部位、范围、临床症状、诊断及手术方式。方法回顾性分析1998年1月～2009年1月诊治的11例颞骨先天性胆脂瘤患者的临床资料。结果 11例先天性中耳胆脂瘤患者的年龄为9～38岁,中位数为15岁,男6例,女5例,术前平均听阈为39dBHL,气-骨导差为20～55dB。病变仅位于鼓室7例,累及乳突4例,其中1例患者外耳道后壁受累破坏。手术方式:3例行鼓室成形术,7例行闭合式乳突根治加鼓室成形术,1例行开放式乳突根治加鼓室成形术。术后6个月平均听阈为28dBHL,气骨导差在20dB以内;术后1年复查CT没有发现胆脂瘤残留或复发。结论先天性胆脂瘤通常起源于鼓膜前上象限,可侵犯中耳、乳突和听小骨,造成传导性听力损失;病变侵犯听小骨时应手术治疗,术后可获得较好的听力重建效果。

Objective To investigate the location, range, clinical symptoms and operative technique of congenital cholesteatoma of temporal bone. Methods A retrospective review of clinical and surgical records of 11 patients with congenital cholesteatoma enrolled at Otorhinolaryngology Head and Neck Surgery of the Second People＇s Hospital of Hefei from January 1998 to January 2009. Results The mean age of the 11 cases of congenital cholesteatoma patients was 15 years （9 to 38 years）, of which 6 cases were male and 5 female. All patients had pure tone audiometry and high--resolution temporal bone CT scan. The mean preoperative PTA was 39 dB, the mean AI3G was 45 dB. By surgical procedures, 3 cases were tympanoplasty, 7 cases a canal wall up tympanomastoidectomy, and one case canal wall down tympanomastoidectomy. All patients were followed up for at least one and a half years. After six months the mean PTA was 28 dB HL, air bone gap was within 20 dB. ACT review found no resid- ual or recurrent cholesteatoma. Conclusion The congenital cholesteatoma usually originates in the anterior superior quadrant, but may variably occupy the middle ear and mastoid, thus resulting in ossicular destruction and conductive hearing loss. when congenital cholesteatoma violations ossicles operated should be in time to control the progress of congenital cholesteatorna and obtain better hearing reconstruction.