Case of a child with Langerhans cell histiocytosis presenting remained conductive hearing loss

Abstract

  A two-year-old girl was manifesting otorrhea, conjunctival chalazion and skin induration. She had not been diagnosed for a year, however, a biopsy from granuloma-like mass in the ear canal provided pathological definitive diagnosis as Langerhans cell histiocytosis (LCH). Further investigation revealed a pulmonary cyst in the right lung field and lytic lesion in the left temporal bone, suggesting that the type of the LCH was multiple system without risk organ. Chemotherapy according to the Japan LCH Study Group protocol led all of her LCH localization to total remission. CT scan of the temporal bone showed bone remineralization but the incus had disappeared. Thus, conductive hearing loss remained after the treatment. LCH is a rare and multifocal disease, more frequent in children and its prognosis worsens according to younger age. The initial clinical presentation could be only repetitive otitis with otorrhea. The present case suggested that a biopsy should be performed in the presence of a mass in the ear canal at the early stage, and long-term follow up with audiometry is necessary so as not to overlook hearing loss in children with LCH involving temporal bone.