Hostname: page-component-6766d58669-7cz98 Total loading time: 0 Render date: 2026-05-19T03:13:09.677Z Has data issue: false hasContentIssue false
  • English
  • Français

Marrow proliferation as a cause of hearing loss in beta-thalassaemia major

Published online by Cambridge University Press:  12 October 2007

D Thio ,
V Prasad ,
P Anslow  and
P Lennox
D Thio*
Affiliation:
Department of Otolaryngology, Musgrove Park Hospital, Taunton, UK
V Prasad
Affiliation:
Head and Neck Unit, The Royal Marsden NHS Foundation Trust, London, UK
P Anslow
Affiliation:
Department of Radiology Oxford Radcliffe Infirmary, Oxford, UK
P Lennox
Affiliation:
Department of Otolaryngology, Oxford Radcliffe Infirmary, Oxford, UK
*
Address for correspondence: Mr D Thio, Department of Otolaryngology. Musgrove Park Hospital, Taunton TA1 5DA, UK. E-mail: daniel.thio@yahoo.co.uk
Get access Rights & Permissions [Opens in a new window]

Abstract

Objective:

The aim of this report was to highlight the fact that hearing loss in thalassaemia patients can be related to marrow expansion affecting the ossicles, resulting in a conductive loss.

Case report:

A six-year-old boy with transfusion-dependent beta-thalassaemia developed a unilateral hearing loss shortly after commencing desferrioxamine therapy. Otoxicity was assumed, but the deficit was later found to be of a conductive nature, due to marrow proliferation within the ossicular chain as a consequence of the disease process – a phenomenon previously unreported in the literature.

Conclusion:

It is important to elucidate the precise nature of new onset hearing loss in patients receiving iron chelation therapy, in order to avoid unnecessary cessation of much needed medication, on the assumption of ototoxicity.

Keywords

ThalassaemiaHearing Loss, ConductiveDesferrioxamine

Information

Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 122 , Issue 11 , November 2008 , pp. 1253 - 1256
Copyright
Copyright © JLO (1984) Limited 2007

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.)

Article purchase

Temporarily unavailable

Footnotes

Presented at the European Society of Paediatric Otorhinolaryngology (ESPO), Rome, Italy, 25–30 June 2005.

References

1 Murphy, MF. Diseases of the blood. In: Kumar, P, Clark, M, eds. Clinical Medicine, 3rd edn. London: Baillière Tindall, 1994;313–17Google Scholar
2 Giardina, PJ, Grady, RW. Chelation therapy in β-thalassemia: the benefits and limitations of desferrioxamine. Semin Haematol 1995;32:304–12Google ScholarPubMed
3 De Virgiliis, S, Argiolu, F, Sanna, G, Cornacchia, G, Cossu, P, Cao, A et al. Auditory involvement in thalassemia major. Acta haematol 1979;61:209–15Google Scholar
4 Olivieri, NF, Buncic, JR, Chew, E, Gallant, T, Harrison, RV, Keenan, N et al. Visual and auditory neurotoxicity in patients receiving subcutaneous deferoxamine infusions. N Engl J Med 1986;314:869–73Google Scholar
5 Cases, A, Kelly, J, Sabater, J, Campistol, JM, Torras, A, Montoliu, J. Acute visual and auditory neurotoxicity in patients with end-stage renal disease receiving desferrioxamine. Clin Nephrol 1988;29:176–8Google ScholarPubMed
6 Gallant, T, Boyden, MH, Gallant, LA, Carley, H, Freedman, MH. Serial studies of auditory neurotoxicity in patients receiving deferoxamine therapy. Am J Med 1987;83:1085–90CrossRefGoogle ScholarPubMed
7 Kontzoglou, G, Koussi, A, Tsatra, J, Noussios, G, Vital, V, Sagarakis, G et al. Sensorineural hearing loss in children with thalassemia major in Nothern Greece. Int J Pediatr Otorhinolaryngol 1996;35:223–30Google Scholar
8 Wonke, B, Hoffbrand, AV, Aldouri, M, Wickens, D, Flynn, D, Stearns, M et al. Reversal of desferrioxamine induced auditory neurotoxicity during treatment with Ca-DTPA. Arch Dis Child 1989;64:77–82Google Scholar
9 Karimi, M, Asadi-Pooya, AA, Khademi, B, Asadi-Pooya, K, Yarmohammadi, H. Evaluation of the incidence of sensorineural hearing loss in beta-thalassemia major patients under regular chelation therapy with desferrioxamine. Acta Haematol 2002;108:79–83Google Scholar
10 Onerci, M, Aslan, S, Gϋmrϋk, F, Aksoy, S, Belgin, E, Özçelik, T et al. Audiologic and impedancemetric findings within thalassaemic patients. Int J Pediatr Otorhinolaryngol 1994;28:167–72CrossRefGoogle ScholarPubMed
11 Sheikha, A, Kameswaran, M, Okafor, BC, al-Saigh, AA. Otological manifestations of thalassaemia intermedia: evidence of temporal bone involvement and report of a unique cholesteatoma-like lesion. J Laryngol Otol 1992;106:316–21Google Scholar