Hong Joon Pyo, Kim Min-Beom
Department of Otorhinolaryngology-Head and Neck Surgery, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, Korea.
J Audiol Otol. 2023 Jan;27(1):45-50. doi: 10.7874/jao.2022.00031. Epub 2022 Apr 12.
Congenital middle ear cholesteatoma (CMEC) accounts for 2%-5% of all cases of middle ear cholesteatoma. CMEC is rare and diagnostically challenging; therefore, only a few cases are reported in the literature, and the pathophysiology of this condition remains largely unclear. Currently, epidermoid formation, amniotic fluid contamination, lack of the tympanic ring, and squamous metaplasia are among the mechanisms implicated in the pathogenesis of CMEC; however, no single theory satisfactorily explains the pathophysiology of this disorder. We report a case of CMEC in a young male patient, who showed a symmetric, binaural whitish mass posterior to the anterosuperior quadrant of the tympanic membrane. A few reports have described bilateral CMEC; however, no study has reported symmetrical CMEC as observed in this case. Bilateral tympanoplasty concomitant with cholesteatoma removal was performed, and histopathological evaluation of the resected specimen showed closed-type cholesteatoma. In this report, we describe a rare case of symmetric CMEC, which supports the epidermoid formation theory associated with CMEC, together with a literature review.
先天性中耳胆脂瘤（CMEC）占所有中耳胆脂瘤病例的2% - 5%。CMEC较为罕见，诊断具有挑战性；因此，文献中报道的病例较少，且这种疾病的病理生理学在很大程度上仍不清楚。目前，表皮样形成、羊水污染、鼓膜环缺失和鳞状化生是CMEC发病机制中涉及的一些机制；然而，没有单一理论能令人满意地解释这种疾病的病理生理学。我们报告一例年轻男性患者的CMEC病例，该患者鼓膜前上象限后方可见对称的双耳白色肿物。有少数报告描述过双侧CMEC；然而，尚无研究报道过如此例所见的对称性CMEC。我们进行了双侧鼓室成形术并切除胆脂瘤，对切除标本的组织病理学评估显示为闭合型胆脂瘤。在本报告中，我们描述了一例罕见的对称性CMEC病例，该病例支持与CMEC相关的表皮样形成理论，并进行了文献综述。
