发生于岩部的先天性胆脂瘤很少见。报告1例,患者男性,23岁。因左耳自幼听力减退,左侧面肌无力4年就诊。既往无眩晕及急性中耳炎史。检查两侧鼓膜正常,左耳中度混合性聋,双耳对侧镫骨肌反射未引出,但两侧声顺图正常。CT显示,左侧岩部有软组织阴影,肿块毗邻骨结构有广泛破坏。MRI显示,左岩部及乳突 Congenital cholesteatoma occurring in the rock department is rare. Report of 1 case, male patient, 23 years old. Left ear hearing loss due to childhood, left facial muscle weakness 4 years treatment. No previous history of vertigo and acute otitis media. Check the normal tympanic on both sides of the left ear moderate mixed deafness, bilateral contralateral stapedius reflex did not lead to, but both sides of the tone map normal. CT showed that the left part of the rock has soft tissue shadow, mass adjacent to the bone structure has extensive damage. MRI shows the left rock and papillae