Dandachli Mhd Ammar, Dandachli Mhd Nabil
Department of Otolaryngology - Head and Neck Surgery, Damascus University, Syria.
Department of Otolaryngology - Head and Neck Surgery, Damascus University, Syria.
Int J Surg Case Rep. 2024 Dec;125:110360. doi: 10.1016/j.ijscr.2024.110360. Epub 2024 Sep 27.
Congenital cholesteatoma is a rare entity that arises from epithelial cell rests in the middle ear, parts of temporal bone or even intracranially. However, it can go unrecognized for several years making the consideration of such diagnosis a bit tricky among otologists.
We are reporting a case of a young female adult who presented with unilateral hearing loss for one year and intermittent episodes of facial twitching in addition to periods of recurrent unilateral facial palsy that happened about three times in the past two years.
Otoscopic examination revealed a slight whitish mass behind the superior half of an intact tympanic membrane. High-resolution computed tomography (HRCT) and magnetic resonance imaging (MRI) proposed a differential diagnosis of congenital cholesteatoma and facial schwannoma. Canal wall up (CWU) mastoidectomy with exploration of anterior epitympanum and partial facial nerve decompression was made with complete removal of cholesteatoma mass and ossicular reconstruction.
A recurrent or non-resolving facial asymmetry in an otherwise healthy adult should raise the suspicion among otolaryngologists to investigate the possibility of congenital cholesteatoma.
先天性胆脂瘤是一种罕见的疾病，起源于中耳、颞骨部分甚至颅内的上皮细胞残余。然而，它可能多年未被发现，这使得耳科医生在考虑此类诊断时有些棘手。
我们报告一例年轻成年女性病例，该患者出现单侧听力损失一年，伴有间歇性面部抽搐，此外在过去两年中还发生过约三次复发性单侧面神经麻痹。
耳镜检查发现完整鼓膜上半部分后方有一个轻微的白色肿物。高分辨率计算机断层扫描（HRCT）和磁共振成像（MRI）提出先天性胆脂瘤和面神经鞘瘤的鉴别诊断。进行了开放式乳突根治术（CWU），探查上鼓室前部并进行部分面神经减压，完整切除胆脂瘤肿物并进行听骨链重建。
在其他方面健康的成年人中，反复出现或无法缓解的面部不对称应引起耳鼻喉科医生的怀疑，以调查先天性胆脂瘤的可能性。
