A Unified Classification of Middle Ear and Petrous Bone Congenital Cholesteatomas

BACKGROUND: The aim of this study was to classify congenital cholesteatoma along an entire spectrum of involvement ranging from the middle ear to petrous apex.

METHODS: A total of 131 patients (85 adults and 46 children) underwent operations for congenital cholesteatoma over the duration of 27 years.

RESULTS: For most cases, middle ear mucosa was normal, the first ossicle eroded by the mass was the stapes, and the mastoid air cell system was well-pneumatized on intraoperative and radiographic views. Totally 34% of patients presented with facial nerve weakness and 45% of these cholesteatomas arose from the supralabyrinthine area (32.8%) and from the petrous apex (12.2%).

CONCLUSION: In this unified classification system, the otologist sees congenital cholesteatoma as a continuum, with facial nerve involvement and anacusis as part of the picture. This system of congenital cholesteatoma accommodates the supralabyrinthine and petrous bone locations of the disease.

Cite this article as: Honnurappa V, Mahajan N, Kumar Vijayendra V, Redleaf M. A unified classification of middle ear and petrous bone congenital cholesteatomas. J Int Adv Otol. 2022;18(4):315-319.