Hostname: page-component-586b7cd67f-l7hp2 Total loading time: 0 Render date: 2024-11-23T00:28:03.488Z Has data issue: false hasContentIssue false
  • English
  • Français

Otological manifestations of thalassaemia intermedia: evidence of temporal bone involvement and report of a unique cholesteatoma-like lesion

Published online by Cambridge University Press:  29 June 2007

Anwar Sheikha ,
Mohan Kameswaran ,
B. C. Okafor  and
Abdul-Aziz Al-Saigh
Anwar Sheikha*
Affiliation:
Department of Clinical and Laboratory HaematologyKing Saud University Medical College and Asir Central Hospital. Abha PO Box 641, Kingdom of Saudi Arabia.
Mohan Kameswaran
Affiliation:
Department of OtolaryngologyKing Saud University Medical College and Asir Central Hospital. Abha PO Box 641, Kingdom of Saudi Arabia.
B. C. Okafor
Affiliation:
Department of OtolaryngologyKing Saud University Medical College and Asir Central Hospital. Abha PO Box 641, Kingdom of Saudi Arabia.
Abdul-Aziz Al-Saigh
Affiliation:
Department of General Surgery, King Saud University Medical College and Asir Central Hospital. Abha PO Box 641, Kingdom of Saudi Arabia.
*
Dr Anwar Sheikha, College of Medicine, King Saud University, Abha, PO Box 641, Kingdom of Saudi Arabia.
Get access Rights & Permissions [Opens in a new window]

Abstract

Thalassaemia intermedia should be considered in any chronically anaemic patient presenting fromthe Middle East with hearing impairment. We report here three Saudi siblings with thalassaemia intermedia and features of severe bone marrow expansion, particularly invading the temporal bone. They were seen first for their otological problems before they had access to proper haematological evaluation. One member was admitted for surgical exploration of a cholesteatoma, which was then found to be marrow expansion of the temporal bone. Screening of the family revealed twomore anaemic siblings with thalassaemia intermedia. Audiological examination of all the family members showed that only the two affected members had a high frequency sen-sori-neural hearing loss.

Bone marrow expansion into the temporal bone is a rare feature of thalassaemia intermedia. Cholesteatoma-like lesion has not been previously described. It has to be considered in all cases of symptomatic thalassaemia intermedia manifesting with cavitation and lytic lesions in the mastoid system. The likelihood that sensorineu-ral hearing loss may complicate the thalassaemias israised and the possible mechanism for such involvement discussed. The proper management for different otological manifestations of the thalassaemias is suggested. These cases would suggest amore extensive involvement of the temporal bone in the thalassaemias than has been previously recognized. Further large scale studies are required to illuminate the subject.

Keywords

Thalassaemia, intermediaTemporal boneDeafness
Type
Main Articles
Information
The Journal of Laryngology & Otology , Volume 106 , Issue 4 , April 1992 , pp. 316 - 321
Copyright
Copyright © JLO (1984) Limited 1992

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

Bate, C. M., Humphries, G. (1977) Alpha-beta thalassaemia. Lancet, i: 1031.CrossRefGoogle Scholar
Ben-Bassat, I., Hertz, M., Selzer, G., Ramot, B. (1977) Extramedullary haemopoiesis with multiple tumor-simulating mediastinal masses in a patient with Beta-thalassaemia intermedia. Israel Journal of Medical Sciences, 13: 1206.Google Scholar
Engelhard, D., Cividalli, G., Rachmilewitz, E. A. (1975) Splenectomy in homozygous beta thalassaemia: a retrospective study of 30 patients. British Journal of Haematology, 31: 391–403.CrossRefGoogle ScholarPubMed
Erlandson, M. E., Brilliant, R., Smith, C. M. (1964) Comparison of 66 patients with thalassaemia major and 13 patients with thalassaemia intermedia: including evaluation of growth, development, maturation and prognosis. Annals of the New York Academy of Sciences, 119: 727.CrossRefGoogle Scholar
Friedman, E. M, Herer, G. R., Luban, N. L. C, Williams, I. (1980) Sickle cell anaemia and hearing. Annals of Otology, Rhinology and Laryngology, 89: 342–347.CrossRefGoogle ScholarPubMed
Genovese, M. G., Neretto, G., Poti, A., Benincasa, A. (1979) A case of mediastinal extramedullary hemopoiesis (EMH) in a patient with intermediate thalassaemia. Minerva medicine, 70: 981.Google Scholar
Gratwick, G. M., Bullough, P. G., Bohme, W. H. O., Markenson, A. L., Peterson, C. M. (1978) Thalassaemia osteoarthropathy. Annals of Internal Medicine, 88: 494.CrossRefGoogle ScholarPubMed
Hazell, J. W. P., Modell, C. B. (1976) ENT complications in thalassaemia major. Journal of Laryngology and Otology, 9: 877.CrossRefGoogle Scholar
Issaragrisil, S., Piankijagum, A., Wasi, P. (1981) Spinal cord compression in thalassaemia. Report of 12 cases and recommendations for treatment. Archives of Internal Medicine, 141: 1033–1036.CrossRefGoogle ScholarPubMed
Koide, Y., Hando, R., Yoshikawa, Y. (1964) Distribution of some oxidizing enzymes in the cochlea. Ada Oto-Laryngologica, 58: 344–354.CrossRefGoogle ScholarPubMed
Logothetis, I., Economidou, I., Constantoulakis, M., Augustaki, O. (1971) Cephalofacial deformities in thalassaemia major (Cooley's anaemia). American Journal of Diseases of Children, 121: 300–306.CrossRefGoogle Scholar
Martin, T. E, Nathan, D. G. (1990) Treatment of Cooley's Anaemia. Blood, 76: 435–444.Google Scholar
Modell, B., Berdoukas, V. (1984) The Clinical approach to Thalassaemia. Thalassaemia intermedia, Gruneand Stratton Ltd: London, p. 242–255.Google Scholar
Morgenstein, K. M., Manace, E. D. (1969) Temporal bone histopathology in sickle cell disease. Laryngoscope, 79: 2172–2180.CrossRefGoogle ScholarPubMed
Nasab, A. H. (1979) Clinical and laboratory findings in the initial diagnosis of homozygous beta-thalassaemia in the Fars province, Iran. British Journal of Haematology, 43: 57.CrossRefGoogle ScholarPubMed
Pippard, M. J., Callender, S. T., Warner, G. T., Weatherall, D. J. (1979) Iron absorption and loading in beta-thalassaemia intermedia. Lancet, ii: 819.CrossRefGoogle Scholar
Rustamov, R. S., Dadasheva, T. S., Limborskaya, S. A. (1981) Varying forms of beta-thalassaemia among children of Azerbaidzhan. Problems of haematology and blood transfusion, 26, part 9, 3–12 (in Russian).Google Scholar
Sbyrakis, S., Karagiorga-Lagana, M., Voskaki, I., Efthimou, H., Karaklis, A. (1987) A simple index for initiating transfusion treatment in thalassaemia intermedia. British Journal of Haematology, 87: 479–484.CrossRefGoogle Scholar
Serjeant, G. R., Norman, W., Todd, G. B. (1975) The internal auditory canal and sensorineural hearing loss in homozygous sickle cell disease. Journal of Laryngology and Otology, 89: 453–455.CrossRefGoogle ScholarPubMed
Serjeant, G. R. (1985) Sickle cell disease. In The Nervous System, Oxford University Press: New York. p. 233–246.Google Scholar
Todd, G. B., Serjeant, G. R., Larson, M. R. (1973) Sensorineural hearing loss in Jamaicans with SS disease. Ada Otolaryngologica, 76: 268–272.CrossRefGoogle ScholarPubMed
Weatherall, D. J., Clegg, J. B. (1981) The thalassaemia syndromes. Thalassaemia intermedia. Blackwell Scientific Publications: Oxford, p. 645–682.Google Scholar