To present the successful treatment of sensorineural hearing loss secondary to Langerhans' cell histiocytosis with low-dose radiotherapy, and also the disparity between radiological resolution of Langerhans' cell histiocytosis lesions and lack of sensorineural hearing loss improvement, accompanied by a review of the literature on otolaryngological manifestations and management of Langerhans' cell histiocytosis. Langerhans' cell histiocytosis is a multisystem disease which frequently causes osseous lesions in the temporal bones. Hearing loss is usually conductive but may be sensorineural with lesions of the petrous temporal bone. We present a case of sensorineural hearing loss secondary to Langerhans' cell histiocytosis affecting the labyrinth and internal auditory meatus, which resolved following radiotherapy. Contralateral sensorineural hearing loss in the same patient, previously treated with chemotherapy, did not resolve despite radiological resolution of the temporal bone lesions. We suggest that timely radiotherapy for treatment of sensorineural hearing loss secondary to Langerhans' cell histiocytosis is an appropriate treatment option.