Congenital cholesteatomas in children: An embryologic correlation

The clinical findings in 37 children with congenital cholesteatoma of the middle ear, 17 of which have not been previously reported, are presented. Clinical findings and surgical observations are correlated with recent developmental studies. It is hypothesized that congenital cholesteatoma may originate from an epidermoid formation, which has been identified in the anterior superior lateral tympanic cavity adjacent to the anterior annulus during fetal development, and which normally is present early in development, involuting by 33 weeks’ gestation. It is proposed that the epidermoid formation may not always involute, and could serve as an embryologic anlage of congenital cholesteatomas.