岩骨胆脂瘤临床特点及诊治分析

Clinical analysis of petrous bonecholesteatoma:characteristics,diagnosis and treatment

目的：探讨颞骨岩骨部胆脂瘤的临床表现特点、诊断和治疗。方法：对我科收治的随访资料完整的12例岩骨胆脂瘤（PBC）患者进行回顾性分析。结果：①7例主要表现为长期耳流脓史伴听力下降，后期有面瘫、重度听力下降、眩晕发作；与慢性中耳炎病史及耳科手术史密切相关。另外5例，其病程隐匿，临床表现早期不以流脓为主，后期可有此表现；初诊以面瘫、严重的感音神经性聋或混合性聋，甚至眩晕发作等症状为主；②11例有不同程度的面神经功能损失，多数为Ⅳ-Ⅵ级；破坏累及耳蜗10例，内听道9例，半规管11例；10例选择经迷路一耳蜗（耳囊）入路手术，其余2例迷路入路手术；1例术中借助内镜；4例术中行颞骨次全切除，术腔填塞、耳道咽鼓管封闭术；随访3例复发，行二次手术；其余9例无复发。6例行面神经吻合，面神经功能由HouseBrackmannⅤ～Ⅵ级恢复到Ⅲ～Ⅳ；3例面神经减压，由Ⅳ～Ⅴ恢复到Ⅱ～Ⅲ级。结论：岩骨胆脂瘤病史中具有引起重度耳聋、面瘫的特点，先天性者病程隐匿，危害更甚；治疗原则为彻底去除病变，尽量保留功能。选择手术径路需考虑病变范围和听力状况，硬脑膜缺损、脑脊液漏时可选用颞骨次全切除术，病变深在，切除困难时，可借助内镜扩大视野。

Objective：To explore the clinical characteristics,diagnosis and surgical management of petrous bone cholesteatoma. Method： The data of 12 patients who underwent surgery for petrous bone cholesteatoma（PBC） were retrospectively analyzed with respects to the clinical characteristics, diagnosis and management. Result：Seven cases were characterized by a long otitis media history, severe hearing loss, and facial paralysis. Incontrast, 5 ca- ses were characterized with the symptoms of facial paralysis, hearing loss and vertigo attack and the absence of early otorrhea history. Translabyrinth approach was chosen for 2 cases,while translabyrinth-cochlear approach for 10. Cochlea was involved in 10 cases, while internal auditory cannal in 9 and semicircular cannal in 11. Otoscope was used in 1 case. Four patients were treated by partial resection of petrous apex and ear canal closure with good results. After years of follow-up, three recurrence cases were operated for a second time. Simultaneous facial nerve anastomosis or decompression was performed. The function of facial nerve recovered fromⅤ～Ⅵ to Ⅲ～Ⅳ （House and Brackmann grading） in 6 anastomosis cases and from Ⅳ - Ⅴ to Ⅱ --Ⅲin 3 cases of decompression. Conclusion：Petrous bone cholesteatoma was characterized by severe hearing loss, and facial paralysis. Surgical ap- proaches are decided according to the extent of lesion and hearing status. Our study indicated that otoscope would help to ensure a radical removal of the pathology in cases with deep involvement and restricted vision. Partial re section of petrous bone and ear canal closure could be effective solution for challenging cerebrospinal fluid otorrhea with large dural defects and protecting vital neurovascular structures.