Congenital Middle Ear Cholesteatoma: A Report of 3 Cases and a Literature Review.

Abstract

Background: Congenital cholesteatoma is defined as a white mass behind an intact eardrum without a history of otitis media or previous otologic procedures. Congenital cholesteatoma is a relatively rare disease that accounts for about 2% to 5% of all cholesteatomas. However, the actual incidence rate of congenital cholesteatoma may be underestimated. Conductive hearing loss is the most common presenting symptom. The current study aims to describe the clinical characteristics and management of patients with congenital cholesteatoma and promote awareness of the disease in unilateral or asymmetric conductive hearing loss patients. Methods: In this study, we report a case series of 3 patients including 1 child, 1 adolescent, and 1 young adult, managed in our department between June and August 2023, and present a summary of the literature. Results: Congenital cholesteatoma is primarily a pediatric disease, but it has also been reported in adults. Two cases presented with unilateral secretory otitis media, and 1 case presented with asymmetric unilateral conductive hearing loss. Two patients of Potsic stage III congenital middle ear cholesteatomas underwent transcanal endoscopic ear surgery, and 1 patient of Potsic stage IV underwent conventional microscopic approach canal wall-up mastoidectomy combined with endoscopy. Conclusions: In children or young adults with persistent unilateral or asymmetric conductive hearing loss, congenital middle ear cholesteatoma should be considered. Congenital cholesteatoma cannot be ruled out in children with unilateral secretory otitis media.