Leong H K, Portmann M
Institut G. Portmann, Bordeaux, France.
Rev Laryngol Otol Rhinol (Bord). 1992;113(1):41-5.
Five cases of congenital cholesteatoma of the middle ear and mastoid as defined by Derlacki's criteria were encountered over a 14 month period. They make up 5% of all cases of cholesteatoma managed over the same period of time. Three were young children and all presented with unilateral hearing loss. One had associated multiple congenital abnormalities of the external and middle ear. Only in one patient was the diagnosis made on initial otoscopic examination and the remainder diagnosed on CTscan, myringotomy and tympanotomy. All were operated on; three by the intact canal wall technique, one by the canal down technique with mastoid cavity obliteration and one by atticotomy with lateral attic wall reconstruction. One patient required a second stage operation for excision of an extension of disease deep to the superior semicircular canal via the middle cranial fossa approach in order to preserve cochlear function. These five cases illustrate the diagnostic pitfalls of this condition in which silent danger lurks behind an intact tympanic membrane.
在14个月的时间里，遇到了5例符合德拉克基标准的中耳和乳突先天性胆脂瘤病例。它们占同期所有胆脂瘤病例的5%。其中3例为幼儿，均表现为单侧听力损失。1例伴有外耳和中耳的多种先天性异常。仅1例患者在初次耳镜检查时确诊，其余患者通过CT扫描、鼓膜切开术和鼓室切开术确诊。所有患者均接受了手术；3例采用完整外耳道壁技术，1例采用外耳道下技术并闭塞乳突腔，1例采用上鼓室切开术并重建上鼓室外侧壁。1例患者需要二期手术，通过中颅窝入路切除上半规管深部的病变延伸部分，以保留耳蜗功能。这5例病例说明了这种疾病的诊断陷阱，即无声的危险潜藏在完整鼓膜之后。
