先天性胆脂瘤致幼儿耳蜗外侧移位及内耳道侵蚀。

Colyn White, Ryan Lauzardo, Si Chen
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摘要

本报告的主要目的是描述一个独特的临床病例先天性胆脂瘤完全外侧耳蜗移位和侵略性侵蚀的内耳道和外侧半规管在一个非常小的孩子。本报告涉及一名10个月大的女性,她最初在2.5个月大时出现左侧间歇性带血耳漏,临床听力学测试显示左耳感觉神经性听力损失。最初的计算机断层扫描(CT)显示一个破坏性的左颞骨病变取代了左耳囊和前庭导水管,并伴有侵蚀和累及内耳结构,包括耳蜗、半规管和硬脑膜后窝的侵蚀。左经颞入路切除胆脂瘤并关闭耳道。术后4个月,患者在颈动脉和咽鼓管附近的鼓室下部复发胆脂瘤,需要翻修左经颞门静脉入路至后窝。这个病例是一个非常小的孩子耳蜗完全外侧移位和内耳道和外侧半规管侵袭性侵蚀的独特病例。它有助于提醒耳鼻喉科医生先天性胆脂瘤的沉默但浸润性。患者年龄很小,1个月大时出现耳漏,这让人怀疑这种病变是否在子宫内就已经形成了。这为先天性胆脂瘤中发育性上皮细胞的理论提供了支持。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Lateral Displacement of Cochlea and Erosion of Internal Auditory Canal by a Congenital Cholesteatoma in a Young Child.

The main objective of this report is to describe a unique clinical case of a congenital cholesteatoma with complete lateral displacement of the cochlea and aggressive erosion of the internal auditory canal and lateral semicircular canals in a very young child. This report involves a 10-month-old female who initially presented with left intermittently bloody otorrhea at 2.5 months of age and audiology testing in the clinic demonstrated sensorineural hearing loss in the left ear. Initial computerized tomography (CT) revealed a destructive left temporal bone lesion displacing the left otic capsule and vestibular aqueduct with erosion and involvement of the inner ear structures including erosion into the cochlea and semicircular canals, and posterior fossa dura. A left transtemporal approach for cholesteatoma removal and ear canal closure was done. 4 months postoperatively, the patient required revision of the left transtemporal approach to the posterior fossa for recurrent cholesteatoma in the hypotympanum adjacent to the carotid artery and eustachian tube. This case is unique in the complete lateral displacement of the cochlea and aggressive erosion of the internal auditory canal and lateral semicircular canals in a very young child. It serves to remind otolaryngologists of the silent yet infiltrative nature of congenital cholesteatoma. The very young age of the patient and the presence of otorrhea at 1 month of age raise the question of whether this lesion could have been developing in utero. It lends support to the theory of developmental epithelial rests in congenital cholesteatoma.

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