Langerhans' cell histiocytosis – A rare cause of sudden onset unilateral sensorineural hearing loss

• Published in: Journal of laryngology and otology 1999-12, Vol.113 (12), p.1098-1100
• Main Authors: Hore, I., Mitchell, R. B., Radcliffe, G., Quiney, R., Walker, T.
• Format: Article
• Language: English
• Subjects: Adult; Biological and medical sciences; Clinical Records; Ear, auditive nerve, cochleovestibular tract, facial nerve: diseases, semeiology; Female; Follow-Up Studies; Hearing loss; Hearing Loss, Sensorineural - etiology; Hematologic and hematopoietic diseases; Histiocytosis; Histiocytosis, Langerhans-Cell - complications; Histiocytosis, Langerhans-Cell - diagnosis; Humans; Langerhans' cell; Magnetic Resonance Imaging; Medical sciences; Non tumoral diseases; Other diseases. Hematologic involvement in other diseases; Otorhinolaryngology. Stomatology; sensorineural
• ISSN: 0022-2151; 1748-5460
• DOI: 10.1017/S0022215100157998

Langerhans' cell histiocytosis is a rare disorder of unknown aetiology in which pathological Langerhans' cells accumulate and destroy local tissue. We report a 38-year-old female who presented with a sudden onset of left sensormeural hearing loss. Magnetic resonance imaging (MRI) revealed a contrast-enhancing lesion in the left mastoid and a second lesion in the hypothalamus. Following left mastoid exploration and biopsy a definitive diagnosis of Langerhans' cell histiocytosis was made and the patient was treated with external beam radiotherapy. Subsequent right femur and right mastoid involvement were successfully treated with steroids and cytotoxic chemotherapy. At one year follow-up the patient had residual left-sided sensorineural hearing loss with normal hearing in the right ear. To our knowledge, Langerhans' cell histiocytosis has not been previously reported as a cause of unilateral sudden onset sensorineural hearing loss. It should be considered in the differential diagnosis of this condition.