Bilateral congenital cholesteatoma: report of an emblematic case.

OBJECTIVES

To describe a case of bilateral congenital cholesteatoma (CC) of the middle ear with a focus on diagnostic clues, treatment and a review of the pertinent literature.

PATIENT AND METHODS

An 8-year-old child was incidentally noted to have whitish bilateral retrotympanic masses with normal hearing and referred to our department in January 2005. Microscopic examination of the ears and CT scan of the temporal bones led to a presumptive diagnosis of bilateral CC. The lesion on the right side was surgically removed, followed by that on the left side after 6 months; a retroauricular transcanal approach was adopted in both ears.

RESULTS

Anatomic integrity of the middle ear was achieved with preservation of pre-operative hearing. No signs of recurrence were evident 20 months after the last surgery.

CONCLUSIONS

Bilateral CC is a rare finding but otologists must be aware of it. Surgery must be planned early in order to achieve radical removal of the pathology and the preservation of middle ear structures.