Prevalence and factors associated with hearing loss in pediatric beta-thalassemia patients receiving blood transfusion and iron-chelation therapy
Keywords:
hearing loss, iron-chelation therapy, deferasirox, deferiprone, beta-thalassemia, audiometryAbstract
Introduction: Thalassemia is one of the most common inherited blood disorders worldwide. Hearing loss is more frequently observed in pediatric thalassemia patients receiving blood transfusions and iron chelation therapy compared to normal children. Studies on risk factors for hearing loss have not yet reached definitive conclusions. Identifying the factors associated with hearing loss in this patient group will help guide the development of appropriate treatment plans for pediatric beta-thalassemia patients undergoing iron chelation therapy in the future.
Objectives: To investigate the prevalence and factors associated with hearing loss in pediatric thalassemia patients who receiving blood transfusions and iron-chelation therapy.
Methods: This research was a retrospective cross-sectional analytical study conducted on patients receiving treatment at Nakornping hospital. Data were collected from medical records between January 1, 2021 and December 31, 2022. Inclusion criteria included patients diagnosed with beta-thalassemia (ICD10: D56.1) under 15 years of age, receiving iron-chelation therapy, and undergoing annual examinations, including blood ferritin levels and audiometry.
Results : Of the 96 patients, 24 experienced hearing loss. Patients with Beta thalassemia/Hemoglobin E exhibited a trend towards increased hearing loss compared to those with Beta thalassemia Major ([AOR 4.57, 95% CI 0.97-21.60, p=0.055). Additionally, deferasirox use was associated with a higher risk of hearing loss compared to deferiprone (AOR 3.38, 95% CI 1.02-11.18, p=0.046). However, receiver operating characteristic (ROC) curve analysis revealed a modest accuracy of 69.21% (95% CI: 59.53-78.90), indicating poor predictive performance. Consequently, despite statistically significant findings, the current predictive model may not be sufficiently robust for clinical implementation.
Conclusion: This study suggests potential recommendations for monitoring hearing impairments in children receiving deferasirox, particularly those with Beta thalassemia/Hemoglobin E who require regular blood transfusions. However, the study was limited by its small sample size and lack of longitudinal data. Future studies with larger sample sizes and longitudinal data are necessary to confirm the findings of this study.
References
Weatherall DJ, Clegg JB. Inherited haemoglobin disorders: an increasing global health problem. Bull World Health Organ. 2001;79(8):704-12.
Capellini MD, Cohen A, Porter J, Taher A, Viprakasit V. Guidelines for the management of transfusion dependent thalassaemia (TDT). 3rd ed. Nicosia, Cyprus: Thalassaemia International Federation; 2014.
Kontoghiorghes GJ, Kolnagou A, Peng CT, Shah SV, Aessopos A. Safety issues of iron chelation therapy in patients with normal range iron stores including thalassaemia, neurodegenerative, renal and infectious diseases. Expert Opin Drug Saf. 2010;9(2):201-6. doi:10.1517/14740330903535845.
Levine JE, Cohen A, MacQueen M, Martin M, Giardina PJ. Sensorimotor neurotoxicity associated with high-dose deferoxamine treatment. J Pediatr Hematol Oncol. 1997;19(2):139-41.doi:10.1097/00043426-199703000-00008.
Ambrosetti U, Dondè E, Piatti G, Cappellini MD. Audiological Evaluation in Adult Beta-Thalassemia Major Patients under Regular Chelation Treatment. Pharmacol Res. 2000;42(5):485-7. doi: 10.1006/phrs.2000.0722.
Osma U, Kurtoglu E, Eyigor H, Yilmaz MD, Aygener N. Sensorineural hearing loss in β-thalassemia patients treated with iron chelation. Ear Nose Throat J. 2015;94(12):481-5. doi:10.1177/014556131509401206
Shenoy S. Hematopoietic stem cell transplantation for sickle cell disease: current practice and emerging trends. Hematology Am Soc Hematol Educ Program. 2011;2011:273-9. doi: 10.1182/asheducation-2011.1.273.
Mansilla-Soto J, Rivière I, Sadelain M. Genetic strategies for the treatment of sickle cell anaemia. Br J Haematol.2011;154(6):715-27.doi:10.1111/j.1365-2141.2011.08773.x.
Tanphaichitr A, Kusuwan T, Limviriyakul S, Atipas S, Pooliam J, Sangpraypan T, et al. Incidence of ototoxicity in pediatric patients with transfusion-dependent thalassemia who are less well-chelated by mono and combined therapy of iron chelating agents. Hemoglobin. 2014;38(5):345-50. doi: 10.3109/03630269.2014.940462.
De Virgiliis S, Argiolu F, Sanna G, Cornacchia G, Cossu P, Cao A, et al. Auditory involvement in thalassemia major. Acta Haematol.1979;61(4):209-15. doi: 10.1159/000207658.
Kong MH, Goh BS, Hamidah A, Zarina AL. The prevalence of sensorineural hearing loss in β-thalassaemia patients treated with desferrioxamine. Med J Malaysia. 2014;69(1):9-12.
Budak B, Muluk NB, Gumruk F, Budak G. Pure tone and high frequency audiometries in beta thalassemia major. KBB ve BBC Dergisi.2008;16(1):10-5.
Chao YH, Wu KH, Lin CY, Tsai MH, Peng CT, Wu HP, et al. Audiologic and vestibular assessment in patients with β-thalassemia major receiving long-term transfusion therapy. Pediatr Blood Cancer. 2013;60(12):1963-6. doi: 10.1002/pbc.24699.
Sonbolestan M, Mokhtarinezhad F, Omrani M. An evaluation of sensorineural hearing loss in thalassaemic patients treated with desferrioxamine and its risk factors. Journal of Research in Medical Sciences. 2005;10(4):210-6.
Porter JB, Jaswon MS, Huehns ER, East CA, Hazell JW. Desferrioxamine ototoxicity: evaluation of risk factors in thalassaemic patients and guidelines for safe dosage. Br J Haematol. 1989;73(3):403-9. doi: 10.1111/j.1365-2141.1989.tb07761.x.
Bentur Y, Koren G, Tesoro A, Carley H, Olivieri N, Freedman MH. Comparison of deferoxamine pharmacokinetics between asymptomatic thalassemic children and those exhibiting severe neurotoxicity. Clin Pharmacol Ther. 1990;47(4):478-82. doi:10.1038/clpt.1990.60.
Tartaglione I, Carfora R, Brotto D, Barillari MR, Costa G, Perrotta S, et al. Hearing Loss in Beta-Thalassemia: Systematic Review. J Clin Med. 2021;11(1):102.doi: 10.3390/jcm11010102.
Manara R, Ponticorvo S, Perrotta S, Barillari MR, Costa G, Brotto D, et al. Auditory cortex hypoperfusion: a metabolic hallmark in Beta Thalassemia. Orphanet J Rare Dis. 2021;16(1):349. doi: 10.1186/s13023-021-01969-0.
Khan MA, Khan MA, Seedat AM, Khan M, Khuwaja SF, Kumar R, et al. Sensorineural Hearing Loss and Its Relationship with Duration of Chelation Among Major β-Thalassemia Patients. Cureus. 2019;11(8):e5465. doi: 10.7759/cureus.5465.
Derin S, Azık FM, Topal Y, Topal H, Karakuş V, Çetinkaya PU, et al. The incidence of ototoxicity in patients using iron chelators. J Int Adv Otol. 2017;13(1):136-9. doi: 10.5152/iao.2016.1852.
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