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The authors report 5 cases of congenital cholesteatoma of the temporal bone where the diagnosis was made earlier in each successive case. The first diagnosis was made on the basis of a total paralysis of the facial nerve, the last was made on the basis of a transitory paralysis of the facial nerve associated in the majority of cases with deafness or reduced hearing. Early removal preserves fonction of the VII and VIIIth nerve and is the justification for a systematic X-ray in the Stenver's incidences to be carried out in any facial weakness even if it is transitory. The surgical approach of choice is the middle fossa approach. There is no drainage necessary because these cholesteatomas are not infected.
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