Differential Presentation of Congenital Cholesteatoma in Twins with Atypical Location

Abstract

Abstract Objective We present an extremely rare report of congenital cholesteatoma (CC) in identical twins with atypical location. Methods Two consecutive cases of CC in identical twins from diagnosis to treatment and a literature review are presented. Results The twin children presented to our otology and lateral skull base clinic with different clinical picture. Both underwent thorough audiological workup and imaging and early surgical intervention. The 6-year follow-up of these two children has been uneventful with no recurrence of disease and hearing restoration. Conclusion Multiple theories exist to explain this rare pathology. Modified Levenson’s criteria define it as white mass behind intact tympanic membrane. Atypical location of CC with recurrent otitis media is not uncommon; hence, further study is required. To our best knowledge, this is the second reported occurrence in identical twins but first report with differential presentation and atypical location in both cases.