Brar Sabrina, Wolf Dennis M, Faoury Morad, Barwell Julian, Saggar Anand, Daya Hamid
Department of Otolaryngology, St George's University Hospital, London, UK.
Department of Genetics, St George's University Hospital, London, UK.
Eur Arch Otorhinolaryngol. 2023 Dec;280(12):5649-5654. doi: 10.1007/s00405-023-08239-8. Epub 2023 Sep 22.
Cholesteatoma is a rare middle ear pathology. It can be classified into acquired and congenital forms. Although benign, cholesteatomas can cause significant morbidity including hearing loss, infection, facial palsy and thrombosis. Congenital cholesteatomas are incredibly rare and bilateral disease has not commonly been published in the literature.
We describe the case of female identical (monozygotic, monochorionic, diamniotic) twins who both developed congenital cholesteatomas. In this report, we review the aetiology, treatment, embryology and pathology of cholesteatoma.
The patients have been followed up 15 years after their initial surgery with promising results - pure-tone audiometry and repeat scans have not illustrated any disease recurrence.
This paper presents one of the only cases of female monozygotic twins presenting with unilateral and bilateral cholesteatomas.
胆脂瘤是一种罕见的中耳病变。它可分为后天性和先天性两种类型。尽管胆脂瘤是良性的，但它可导致严重的发病情况，包括听力丧失、感染、面瘫和血栓形成。先天性胆脂瘤极其罕见，双侧发病的情况在文献中并不常见。
我们描述了一对同卵（单卵、单绒毛膜、双羊膜）双胞胎女性均患先天性胆脂瘤的病例。在本报告中，我们回顾了胆脂瘤的病因、治疗、胚胎学和病理学。
患者在首次手术后已随访15年，结果良好——纯音听力测定和复查扫描均未显示任何疾病复发。
本文介绍了仅有的几例同卵双胞胎女性单侧和双侧胆脂瘤病例之一。
