Clinical manifestations and local inflammatory-destructive changes in congenital cholesteatoma of the mastoid process

Congenital cholesteatoma of the mastoid process is less common than its other temporal bone locations. OBJECTIVE: To summarize current data on the clinical manifestations and diagnosis of congenital cholesteatoma of the mastoid process. MATERIAL AND METHODS: A bibliographic search was done in the eLibrary, PudMed, and ScienceDirect databases using the keywords: "mastoid process" and "cholesteatoma". Articles were selected based on their relevance to congenital cholesteatoma of the mastoid process. Only reports describing cholesteatoma localized exclusively to the mastoid process without involvement of the tympanic cavity or antrum, confirmed by radiological examination data and intraoperative findings, were considered. A total of 28 publications were selected, which described 37 cases of congenital cholesteatoma of the mastoid process. RESULTS: Based on the severity of clinical symptoms and destructive changes, the following stages of congenital mastoid cholesteatoma development were identified: Stage I - the cholesteatoma is asymptomatic and constitutes an incidental finding; Stage II - nonspecific symptoms appear: swelling behind the ear, pain in the retroauricular region and neck, and intermittent ear discharge (through a fistula in the posterior wall of the external auditory canal while the eardrum remains intact). Radiological and intraoperative findings reveal destruction of the mastoid cortex, the posterior wall of the external auditory canal, and the mastoid apex without exposure of the dura mater of the posterior cranial fossa; stage III - headache and dizziness also manifest. There is destruction of the inner mastoid wall with exposure of the dura mater of the posterior cranial fossa, compression of the cerebellar hemisphere and/or endolymphatic sac, destruction of the posterior semicircular canal wall, involvement of the sigmoid sinus and the jugular bulb in the process. CONCLUSION: It is important to note that congenital mastoid cholesteatoma is asymptomatic for a long period. Unlike middle ear cholesteatoma, it does not impair hearing and is invisible on otoscopy. Consequently, it is diagnosed only in adulthood, following the development of mastoid destruction and the involvement of surrounding structures.