Department of ENT and Head & Neck Surgery, TU Teaching Hospital, Maharajgunj Medical Campus, Institute of Medicine, Kathmandu, Nepal.
JNMA J Nepal Med Assoc. 2020 Dec 31;58(232):1080-1082. doi: 10.31729/jnma.5199.
Congenital external canal atresia is one of the congenital ear anomalies that can occur in patients. Similarly, congenital cholesteatoma is also another congenital disease that is often diagnosed in early adulthood. Both the above-mentioned diseases can occur independently but the presence of both these entities is a rare occurrence and needs a high degree of suspicion aided by a computed tomography scan to make the diagnosis. We are presenting a case of a sixteen-year-old patient who presented with unilateral ear anomaly, earache, facial palsy, and postaural swelling and was diagnosed as a right sided congenital aural atresia with congenital cholesteatoma. He was surgically managed with right-sided modified radical mastoidectomy with canaloplasty and closure of mastoid fistula under general anesthesia.
先天性外耳道闭锁是患者可能发生的先天性耳部畸形之一。同样，先天性胆脂瘤也是另一种常发生于成年早期的先天性疾病。上述两种疾病均可独立发生，但同时存在这两种情况极为罕见，需要高度怀疑，辅以前庭 CT 扫描以做出诊断。我们现介绍 1 例 16 岁患者，该患者表现为单侧耳部畸形、耳痛、面瘫和耳后肿胀，被诊断为右侧先天性耳闭锁伴先天性胆脂瘤。该患者在全麻下接受了右侧改良乳突根治术伴外耳道成形术和乳突瘘管闭合术。
