颞骨先天性胆脂瘤——附6例分析

目的 探讨颞骨先天性脂胆瘤的发病情况和诊断及治疗方法。方法 对1973-2000年收治的6例临床资料进行回顾性分析。结果 男5例，女1例。其中双侧1例；年龄6-63岁。病变范围：局限于中耳（鼓窦，乳突）4便，侵犯岩尖1例；原发于颞骨颧突（中耳未受侵犯）1例；合并周围性面瘫1例，耳后瘘管1例。传导性聋4例（5例），混合性聋1例，所有病例均行手术治疗：开放性乳突根治术5例，面神经吻合术1例，耳前颞下窝进路胆脂瘤除术1例。半年-3年随访，均无胆脂瘤复发，面瘫部分恢复。结论 进行性听力减退，伴面瘫或局部肿块，且既往无化脓性中耳炎，鼓膜完整，颞骨影像学检查显示局部骨破坏或高密度软组织影为本病的诊断依据；手术清除病灶为本病治疗的唯一措施。根据病灶范围及听力损失情况可选择保留或提高听力的术式，同时应注意保存或修复面神经功能；术后长期严格随访和清理术腔是预防复发的关键措施。

Congenital cholesteatoma of the temporal bone:report of 6 cases

ObjectiveTo study the clinical manifestation, diagnosis and treatment of the congenital cholesteatoma of the tempo-ral bone.MethodsSix cases of the congenital cholesteatoma of temporal bone were analyzed retrospectively from 1973 to 2000.ResultsOf 6 cases, 5 were male, 1 was female. The age range was 6 to 63 years. The lesions were located in singer ear in 5, both earsin 1, and involved in middle ear in 4, pyramis in 1, jugale of the temporal bone in 1. The patients complicated with facioplegia were 1,postauricular fistula 1, conduction deafness 4, mixed deafness 1. The operations were performed for all patients, including open radicalmastoidectomy in 5, facial nerve anastomosis in 1, cholesteatome extraction in 1. During the follow up period of 6 months to 3 years,the cholesteatomas were no recurrence.ConclusionsThe progressive hypoacusis, facioplegia or local mass, and the normal ear-drum are the clinical features of the disease. Imaging examinations are very important. The surgery is the only treatment. Not only thehearing but also the facial nerve should be protected. It is very important to clean mastoid cavity and follow-up survey after operation.