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Langerhans cell histiocytosis is a rare disorder, with resultant bilateral sensorineural hearing loss unreported in adults. A 42-year-old man presented with 4 months of right-sided tinnitus and hearing loss treated initially as otitis media with effusion. He re-presented 5 months later with progressive bilateral hearing loss--sensorineural (&gt;100 dB) on pure tone audiogram. CT showed bilateral petrous temporal bone and calvarial lesions. Biopsy confirmed diagnosis of Langerhans cell histiocytosis and chemotherapy was started. Though uncommon, Langerhans cell histiocytosis should be considered among the differentials of persistent otological symptoms, as its progressive nature can cause bilateral irreversible sensorineural hearing loss.

A case of bilateral sensorineural hearing loss from Langerhans cell histiocytosis