Congenital Cholesteatoma in Adults-Interesting Presentations and Management

To report a series of adult patients diagnosed with congenital cholesteatoma (CC) with respect to symptoms, different varieties of presentation, surgical findings and approach used, complications and the postoperative results. A retrospective chart review of adult cases of CC who were treated in the period from January 2014–2017 was carried out in a tertiary care center. Levenson’s criteria were used for diagnosis. Diagnosis was confirmed by imaging and intraoperatively. Postoperative results and complications were also analyzed. Six adult cases of CC were studied with a mean follow up of 10 months. Interesting presentations included otitis media with effusion, non-resolving facial nerve palsy, post aural discharge and meningitis. It included 3 cases of petrous apex cholesteatoma, 2 patients with cholesteatoma involving both the middle ear and mastoid and 1 patient with mastoid cholesteatoma. The operative procedures included canal wall up mastoidectomy (1 patient), atticotomy (1 patient), canal wall down mastoidectomy (1 patient), translabyrinthine and transotic excision of mass with blind sac closure (2 patients) and partial labyrinthectomy (1 patient). Complications encountered during surgery were cerebrospinal fluid leak and worsening of hearing in 2 patients and 1 patient respectively. CC can have variety of interesting presentations in adult population and they may or may not have the classical white mass behind the tympanic membrane. Appropriate individualized surgical planning and intervention gives good results.