[Congenital cholesteatoma of the middle ear in children]

In a series of 16 middle ear cholesteatomas of a congenital type are reported in children; the youngest (18 months) presented with a bilateral case. Whereas a simple tympanoplasty could cure a localized pearl, typically anterosuperior in the mesotympanum, the stapes is fast eroded (7 cases) if progression goes on. Intact canal wall technique in 2 stages was the typical procedure. Good hearing results were generally achieved (except in one case of fixed footplate): 9 cases/14 with an ABG within 20 dB and an AC level within 30 dB.