Karmarkar S, Bhatia S, Khashaba A, Saleh E, Russo A, Sanna M
Gruppo Otologico, Piacenza, Italy.
Am J Otol. 1996 Mar;17(2):288-92.
A retrospective analysis is presented of 44 cases of congenital cholesteatoma of the middle ear. Twenty-one patients had cholesteatoma localized to the posterosuperior mesotympanum in complete contrast to the commonly reported anterosuperior location seen in only two cases. The remaining 21 patients had cholesteatoma involving either the entire mesotympanum or epitympanum or both. The posteriorly located congenital cholesteatomas might represent a completely different entity and originate from the trapped epithelial cell rests in the posterior mesotympanum during the development of temporal bone. All but one patient were treated by using a closed tympanoplasty. Eight patients underwent a single-stage surgery. A preplanned second-stage procedure was performed in 33 patients, and three are awaiting their second stage. Residual disease was seen in 19 (57%) patients undergoing second-stage surgery. No patient has had recurrent disease so far. Thirty-eight (85%) patients had a preoperative air-bone gap of > or = 30 dB. Of the 33 patients considered for hearing results, 16 (48%) had a postoperative gap within 10 db.
本文对44例中耳先天性胆脂瘤进行了回顾性分析。21例患者的胆脂瘤局限于中鼓室后上部分，这与通常报道的仅2例患者所见的前上部分位置形成了鲜明对比。其余21例患者的胆脂瘤累及整个中鼓室或上鼓室或两者。位于后方的先天性胆脂瘤可能代表一种完全不同的实体，起源于颞骨发育过程中被困于中鼓室后部的上皮细胞残余。除1例患者外，所有患者均采用闭合式鼓室成形术治疗。8例患者接受了一期手术。33例患者进行了预先计划的二期手术，3例正在等待二期手术。19例（57%）接受二期手术的患者出现了残留疾病。迄今为止，没有患者出现复发性疾病。38例（85%）患者术前气骨导差≥30dB。在33例评估听力结果的患者中，16例（48%）术后气骨导差在10dB以内。
