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

Published online by Cambridge University Press:  29 June 2007

I. Hore ,
R. B. Mitchell ,
G. Radcliffe ,
R. Quiney  and
T. Walker
I. Hore*
Affiliation:
Department of Otolaryngology, The Royal Free Hospital, London, UK.
R. B. Mitchell
Affiliation:
Department of Otolaryngology, The Royal Free Hospital, London, UK.
G. Radcliffe
Affiliation:
Department of Otolaryngology, The Royal Free Hospital, London, UK.
R. Quiney
Affiliation:
Department of Otolaryngology, The Royal Free Hospital, London, UK.
T. Walker
Affiliation:
Department of Histopathology, The Royal Free Hospital, London, UK.
*
Address for correspondence: Mr I. Hore, F.R.C.S., Department of Otolaryngology – Head and Neck Surgery, The Roayl Free Hospital, Pond Street, London NW3 2QG. Fax: 0171 830 2422 e-mail: ianhore@yahoo.co.uk
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Abstract

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.

Keywords

Hearing loss, sensorineuralHistiocytosis, Langerhans' cell
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 113 , Issue 12 , December 1999 , pp. 1098 - 1100
Copyright
Copyright © JLO (1984) Limited 1999

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