Juvenile xanthogranuloma. Report of a case with audition impairment.

A nine-month-old boy with a large perauricular nodular mass extending into the external auditory canal was seen. The mass was present since birth and was responsible for conductive hearing loss. Light microscopy and immunohistochemical studies were consistent with juvenile xanthogranuloma. Initiation of local and systemic corticosteroid therapy was followed within two weeks by complete regression of the mass and full recovery of hearing. The clinical features, histologic findings, and differential diagnosis of this condition are discussed.