Kikuchi Masahiro, Yamamoto Etsuo, Shinohara Shogo, Shiomi Yousaku, Fujiwara Keizo, Shiomi Yoshiko, Watanabe Futoshi, Tanabe Makito
Department of Otolaryngology, Kobe City General Hospital, Kobe.
Nihon Jibiinkoka Gakkai Kaiho. 2003 Aug;106(8):797-807. doi: 10.3950/jibiinkoka.106.797.
We conducted a retrospective study to identify the clinical features and surgical observations of congenital cholesteatoma. Sixty patients were diagnosed and underwent surgery for congenital cholesteatoma between April 1987 and May 2002. All diagnoses were made on the basis of two operative findings: 1. the tympanic membrane manifested neither retraction, perforation, nor granulation. 2. the tympanic membrane was not continuous with the cholesteatoma. In this series, congenital cholesteatoma accounted for 7% of all cholesteatomas (853 ears). The patient age ranged from 2 to 48 years. The male to female ratio was 4:1. Seventeen patients had multiple cholesteatoma. Fifty-three patients exhibited closed-type cholesteatomas, while the remaining 7 patients had open-type cholesteatomas that had formed as a flat surface of the epidermis. Patients with open-type cholesteatomas presented with a much more pronounced conductive hearing loss and ossicular erosion or malformation. Twenty-two patients with relatively small cholesteatomas were analyzed to estimate the origin of their cholesteatomas. Of the 22 patients, 13 had anterior superior quadrant (ASQ-type) and 9 had posterior superior quadrant (PSQ-type) cholesteatomas. The mean age at the time of detection was older in the PSQ-type group than in the ASQ-type group and the frequency of ossicular erosion or malformation was more prominent in the PSQ-type group than in the ASQ-type group. The primary site of origin was thought to be the portion between the tympanic ostium of the auditory canal and the semicanal for tensor tympani in the ASQ-type group and near the incudostapedial joint in the PSQ-type group. A planned staged procedure was performed in 29 patients, 15 patients (52%) had residual lesions situated mostly on the oval window, the round window, an exposed facial nerve or an exposed lateral semicircular canal. The frequency of residual lesions in patients who presented with extended, multiple cholesteatoma and those with ossicular malformation was comparable to the frequency of patients who did not present with these features.
我们进行了一项回顾性研究，以确定先天性胆脂瘤的临床特征和手术观察结果。1987年4月至2002年5月期间，60例患者被诊断为先天性胆脂瘤并接受了手术。所有诊断均基于两项手术发现：1. 鼓膜既无内陷、穿孔，也无肉芽形成。2. 鼓膜与胆脂瘤不连续。在该系列中，先天性胆脂瘤占所有胆脂瘤（853耳）的7%。患者年龄在2至48岁之间。男女比例为4:1。17例患者有多发胆脂瘤。53例患者表现为闭合型胆脂瘤，其余7例患者有开放型胆脂瘤，其形成于表皮的平坦表面。开放型胆脂瘤患者的传导性听力损失和听骨侵蚀或畸形更为明显。对22例胆脂瘤相对较小的患者进行分析，以估计其胆脂瘤的起源。在这22例患者中，13例为前上象限（ASQ型）胆脂瘤，9例为后上象限（PSQ型）胆脂瘤。PSQ型组检测时的平均年龄比ASQ型组大，PSQ型组听骨侵蚀或畸形的发生率比ASQ型组更突出。ASQ型组的主要起源部位被认为是外耳道鼓室口与鼓膜张肌半管之间的部分，PSQ型组的主要起源部位被认为是砧镫关节附近。29例患者接受了计划性分期手术，15例患者（52%）有残留病变，主要位于卵圆窗、圆窗、暴露的面神经或暴露的外侧半规管。出现广泛多发胆脂瘤和听骨畸形的患者残留病变的发生率与未出现这些特征的患者相当。
