Brainstem auditory evoked potentials features in thalassemia major

  • Jimmy Passat Department of Child Health, University of Indonesia Medical School/Dr. Cipto Mangunkusumo Hospital, Jakarta
  • Bulan Ginting Munthe Department of Child Health, University of Indonesia Medical School/Dr. Cipto Mangunkusumo Hospital, Jakarta
  • Fauzi Mahfuzh Department of Child Health, University of Indonesia Medical School/Dr. Cipto Mangunkusumo Hospital, Jakarta
  • Taralan Tambunan Department of Child Health, University of Indonesia Medical School/Dr. Cipto Mangunkusumo Hospital, Jakarta
DOI: https://doi.org/10.14238/pi41.3.2001.166-70
Keywords: thalassemia, brainstem evoked potentials, children

Abstract

Patients with thalassemia major are at high risk for hearing impairment. The objective of the study is to determine the prevalence, grade and type of hearing impairment according to brainstem auditory evoked potentials (BAEP) investigation in thalassemia major. A descriptive cross sectional study was conducted between December 1999 until August 2000 in 72 thalassemic patients between 3 and 18 years of age. Only 65 patients were evaluated, because of time limitation. The results showed the prevalence of hearing impairment in thalassemia major was 29.2%. Most of them were moderate to severe unilateral sensorineural hearing impairments. Mild sensorienural hearing impairment occured in only 12.3%. Conductive  hearing impairment was only found in 1 patient. Hearing impairment was frequently found between 7 to 12 years of age (41.5%). Conclusion: the prevalence of hearing impairment in thalassem a major according to BAEP investigation is high and BAEP examination should be done regularly in all of thalassemic patients to investigate early detection and treatment of hearing impairment. 

References

1. Vulvo R, Modell B, Georganda E. What is Cooley’s anemia. New Jersey: The New Jersey Chapter Cooley’s Anemia Foundation, 1994;1-104.
2. Sub Bagian Hematologi. Petunjuk diagnosis dan tatalaksana kasus thalassemia. Jakarta: Sub Bagian Hematologi, Bagian Ilmu Kesehatan Anak FKUI/RSCM, 1997.
3. Logothetis J, Constantoulakis M, Economidou J,et al. Thalassemia major (homozygous beta thalassemia), a survey of 138 cases with emphasis on neurologic and muscular aspects. Neurology,1972;22: 298-303.
4. DeVirgilis S, Argiolu F, Sanna G, et al. Auditory involvement in thalassemia major. Acta Haematol 1979; 61:209-15.
5. Meyerhoff WL, Liston SL. Metabolic hearing loss. In: Zorab,editor. Otolaryngology; 3 rd edition. Philadelphia: W.B.Saunder Company,1991;1671-9.
6. Argiolu F, Diana G, Avignone A, Cao A. Hearing impairment during deferoxamine therapy for thalassemia major. J Pediatr 1991;118:826-7.
7. Lamabadusuriya SP. Multiple nerve palsies in thalassemia major. Arch Dis Child 1989;64:1060-1.
8. Gallant T, Boyden MH, Gallant LA, Carley H, Freedman M. Serial studies of auditory neurotoxicity in patients receiving deferoxamine therapy. Am J Med 1987;83:1085-90.
9. Bentur Y, Koren G, Tesoro A, et al. Comparison of deferoxamine pharmacokinetics between asymptomatic thalassemic children and those exhibiting severe neurotoxicity. Clin Pharmacol Ther 1990;47:478-82.
10. Cohen A, Martin M, Mizanin J, et al. Vision and hearing during deferoxamine therapy. J Pediatr 1990;117: 326-30.
11. Barrat PS, Toogood IRG. Hearing loss attributed to desferrioxamine in patients with beta thalassemia major. Med J Aust 1987; 147:177-9.
12. Styles LA, Vichinsky EP. Ototoxicity in hemoglobinopathy patients chelated with desferrioxamine. J Pediatr hematol oncol 1996;18(1): 42-5.
13. Hall JW. Handbook of auditory evoked responses. Boston: Allyn and Bacon, 1992.
14. Stockard JJ, Pope-Stockard JE, Sharbrough DW. Brainstem auditory evoked potentials in neurology: methodology, interpretation, and clinical application. In: Aminoff MJ, editor. Electrodiagnosis in clinical neurology; 3rd edition. New York: Churchill Livingstone,1992;537-61.
15. Aminoff MJ. Brainstem auditory evoked potentials. Annual meeting of American Academy of Neurology. New York City: American Academy of Neurology.
16. Celesia GG, Grigg MM. Auditory evoked potentials. In: Niedelmeyer E, daSilva FL, editors. Electroencephalography: basic principles, clinical applications and related fields; 2nd edition. Baltimore: Urban & Schwarzenberg Inc, 1987:797-812.
17. Wong V, Li A, Lee AC. Neurophysiologic study of beta thalassemia major patients. J Child Neurol 1993; 8:330-5.
18. Konzoglou G, Kousi A, Tsatra J, et al. Sensorineural hearing loss in children with thalassemia major in Northern Greece. Int J Pediatr Otorhinolaryngol 1996; 35:223-30.
19. Zafeiriou DI, Kousi AA, Tsantali CT, et al. Neurophysiologic evaluation of long-term desferrioxamine therapy in beta-thalassemia patients. Pediatr Neurol 1998; 18:420-4.
20. Wahidijat I. Thalassemia dan permasalahannya di Indonesia. Naskah lengkap Kongres Nasional Ilmu Kesehatan Anak ke XI. Jakarta: IDAI Pusat,1999;293-6.
21. Amabile G, Stefano E, Bianco I,et al. Electrophysiological (EEG, BAEP, VEP) study in patients with beta thalassemia major. Acta Neurol Belg 1987; 87:181-90.
22. Triantafyllou N, Fisfis M, Sideris G, et al. Neurophysiological and neuro-otological study of homozygous beta thalassemia under long term desferrioxamine (DFO) treatment. Acta Neurol Scand 1991; 83:306-8.
23. Muhidin. Pengaruh desferioksamin tunggal terhadap kadar besi serum penderita thalassemia. Tesis. Jakarta: Bagian Ilmu Kesehatan Anak FKUI, 1986.
24. Wahidiyat I. Transfusi darah pada thalassemia. In: Gatot D, Abdulsalam M, Windiastuti E, editors. Darah dan tumbuh kembang: aspek transfusi. Naskah Lengkap Pendidikan Kedokteran Berkelanjutan Ilmu Kesehatan Anak XLI. Jakarta 24-25 Juni 1998.
25. Picton TW, Taylor MJ, Durieux-Smith A. Brainstem auditory evoked potentials in pediatrics. In: Aminoff MJ, editor. Electrodiagnosis in clinical neurology; 3rd edition. New York: Churchill Livingstone,1992;537-61.
26. Chiappa KH. Brainstem auditory evoked potentials: interpretation.
In: Chiappa KH, editor. Evoked potentials in clinical medicine, 2nf edition. New York: Raven Press, 1990;223-305.
27. Epstein CM. Brainstem auditory evoked potentials. American Academy of Neurology, Annual Courses, meeting April 25-May 1, 1993, New York City.
Published
2001-06-30
How to Cite
1.
Passat J, Munthe B, Mahfuzh F, Tambunan T. Brainstem auditory evoked potentials features in thalassemia major. PI [Internet]. 30Jun.2001 [cited 23Nov.2024];41(5-6):166-0. Available from: https://paediatricaindonesiana.org/index.php/paediatrica-indonesiana/article/view/1100
Issue
Vol 41 No 5-6 (2001): May 2001
Section
Articles

Authors who publish with this journal agree to the following terms:

Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.

Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.

Received 2016-12-05
Accepted 2016-12-05
Published 2001-06-30