[Congenital cholesteatoma in children]

Objectives: To report on two cases of congenital cholesteatoma in patients aged 1 month. To review the clinical and radiological features of congenital cholesteatoma and to clarify the contribution of the CT scan in the diagnosis and the preoperative workup.

Patients and methods: The authors report two observations of congenital cholesteatoma. These children were investigated using high-resolution CT. The treatment was surgical in both cases.

Results: The children were 1 month old, presenting with facial paralysis. In the otoscopic exam, the tympanic membrane was normal in both cases, with a whitish retrotympanic soft mass. The diagnosis of congenital cholesteatoma was made based on the CT data.

Conclusion: Congenital cholesteatoma is rare. Otoscopic examination must be undertaken during the physical exam of a child at a young age to establish an early diagnosis. CT scan allows positive diagnosis and a precise preoperative workup.