Hostname: page-component-78c5997874-mlc7c Total loading time: 0 Render date: 2024-11-18T01:22:09.008Z Has data issue: false hasContentIssue false
  • English
  • Français

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: [email protected]
Get access Rights & Permissions [Opens in a new window]

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

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Angeli, S. I., Luxford, W. M., Lo, W. M. (1996) Magnetic resonance imaging in the evaluation of Langerhans' cell histiocytosis of the temporal bone: Case report. Otolaryngology – Head and Neck Surgery 114: 120–124.CrossRefGoogle ScholarPubMed
Broadbent, V., Egeler, R. M., Nesbit, E. Jr, (1994) Langerhans' cell histiocytosis - clinical and epidemiological aspects. British Journal of Cancer 70: (Suppl. XXIII) S11–S16.Google Scholar
Brookhouser, P. E. (1996) Sensorineural hearing loss in children. Pediatric Clinics of North America 43: 1195–1216.CrossRefGoogle ScholarPubMed
Cohn, A. M., Sataloff, J., Lindsay, J. R. (1970) Histiocytosis X (Letter-Swiwe Disease) with involvement of the inner ear. Archives of Otolaryngology 91: 24–29.CrossRefGoogle Scholar
Chisoim, J. J. (1954) Otorhinologic aspects of Hand-SchullerChristian disease. Laryngoscope 64: 486–496.CrossRefGoogle Scholar
Chu, T., Jaffe, R. (1994) The normal Langerhans' cell and the LCH cell. British Journal of Cancer 70: (Suppl. XXIII) S4–S10.Google Scholar
Gadner, H., Heitger, A., Grois, N., Gatterson-Menz, I., Ladisch, S. (1994) A treatment strategy for disseminated Langerhans cell histiocytosis. Medical and Pediatric Oncology 23: 72–80.CrossRefGoogle ScholarPubMed
Irving, R. M., Broadbent, V., Jones, N. S. (1994) Langerhans' cells histiocytosis in childhood: management of head and neck manifestations. Laryngoscope 104: 64–70.CrossRefGoogle Scholar
Kannourakis, G., Abbas, A. (1994) Monoclonal antibody therapy in Langerhans' cell histiocytosis – feasible and reasonable. British Journal of Cancer 70: (Suppl. XXffl) S37–S40.Google Scholar
Kelly, K. M., Pritchard, J. (1994) Monoclonal antibody therapy in Langerhans– cell histiocytosis – feasible and reasonable? British Journal of Cancer 70: (Suppl. XXIII) S54–S55.Google Scholar
Ladisch, S., Gadner, H. (1994) Treatment of Langerhans' cell histiocytosis – evolution and current approaches. British Journal of Cancer 70: (Suppl. XXffl) S41–S46.Google Scholar
Lopez-Rois, G., Benitez, J. T. (1968) Histiocytosis; Histopathological study of the temporal bone. Annals of Otology, Rhinology and Laryngology 77: 1171–1180.CrossRefGoogle Scholar
Marion, M. S., Hinojosa, R., Libi, S. (1995) Histopathology update. American Journal of Otolaryngology 16: 194–195.CrossRefGoogle ScholarPubMed
McCaffrey, T. V., McDonald, T. J. (1979) Histiocytosis X of the ear and temporal bone: review of 22 cases. Laryngoscope 89: 1735–1742.CrossRefGoogle Scholar
McClain, K., Weiss, R. A. (1994) Viruses and Langerhans' cell histiocytosis: Is there a link? British Journal of Cancer 70: S34–S36.Google Scholar
McClelland, J., Broadbent, V., Yeomans, E., Malone, M., Pritchard, J. (1990) Langerhans' cell histiocytosis; the case for conservative treatment. Archives of Diseases in Childhood 65: 301–303.CrossRefGoogle Scholar
Qurashi, M. S., Blayney, A. W., Breatnach, F. (1993) Aural symptoms as primary presentation of Langerhans' cell histiocytosis. Clinical Otolaryngology 18: 317–323.CrossRefGoogle Scholar
Rosenwasser, H.. (1940) Lipoid granulomatosis (Hand-Schuller-Christian disease involving the middle ear and temporal bone. Archives of Otolaryngology 32: 1045–1053CrossRefGoogle Scholar
Schuknech, H. F., Penman, H. B.. (1948) Hand-SchullerChristian disease and eosinophilic granuloma of the skull. Annals of Otology, Rhinologyand Laryngology 57: 643–674.CrossRefGoogle Scholar
Sheppard, I. J., Milford, C. A. M., Anslow, P. (1996) MRI in the detection of acoustic neuromas — a suggested protocol for screening. Clinical Otolaryngology 21: 301–304.CrossRefGoogle ScholarPubMed
Smith, R. J. H., Evans, J. N. G. (1984) Head and neck manifestations of histiocytosis-X. Laryngoscope 94: 395–399.CrossRefGoogle ScholarPubMed
The Writing Group of the Histiocytosis Society (Chu, T., D'Angio, G. J., Favara, B., Ladisch, S., Nesbit, M., Pritchard, J.) (1987) Histiocytosis in children. Lancet 1: 208–209.Google Scholar
Tos, M.. (1966) A survey of Hand-Schuller-Christian's disease in otolaryngology. Acta Oto-Laryngologica 62: 217–228.CrossRefGoogle ScholarPubMed
Wifiman, C. L., Busque, L., Griffiths, B. B., Favara, B. E., McClain, K. L., Duncan, M. H., Gilliland, D. G. (1994) Langerhans cell histiocytosis – a clonal proliferative disease. New England Journal of Medicine 331: 154–160.Google Scholar
Yu, R., Chu, C. E., Burwela, L., Chu, A. C. (1994) Langerhans' cell histiocytosis: a clonal proliferation of Langerhans' cells. Lancet 343: 767–768.CrossRefGoogle ScholarPubMed