Audiological evaluation of sickle cell disease patients
DOI:
https://doi.org/10.23925/1984-4840.2020v22i1a5Keywords:
anemia, sickle cell, hearing loss, hemoglobinopathies, prevalence, adult.Abstract
Objectives: The objectives of this study were to evaluate the prevalence of hearing loss in patients diagnosed with sickle cell disease (SCD) followed-up at the Hematology Outpatient Clinic of Conjunto Hospitalar de Sorocaba, Brazil, and to correlate the findings with the type of hemoglobinopathy. Methods: The study included 19 patients diagnosed with SCD, 68% of whom were SS, 16% SC, and 16% S-β-thalassemia. The evaluation of each patient comprised a directed anamnesis, otorhinolaryngological physical examination, and audiological evaluation with pure tone audiometry (PTA) and immittanciometry. 37% of the patients had some alteration in PTA and the mean of the thresholds showed a tendency to be higher in the high frequencies. Results: We found one patient (5%) with mild to moderate hearing loss bilaterally and six patients (32%) with thresholds >25 dB at frequencies of 250, 6,000 or 8,000 Hz in one or both ears. We did not observe alterations in the Speech Recognition Index (SRI) in any patient and immittanciometry showed a Type A curve in 100% of the ears evaluated. The relation between the type of hemoglobinopathy and the presence or absence of hearing loss was not significant. Conclusion: As described in the literature, we observed the presence of sensorineural hearing loss in high frequencies in patients with SCD and therefore it is concluded that the audiological monitoring of these patients is extremely important.Downloads
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Araujo PIC. O autocuidado na doenca falciforme. Rev Bras Hematol Hemoter. 2007;29(3):239-46. https://doi.org/10.1590/S1516-84842007000300010.
Kato GJ, Piel FB, Reid CD, Gaston MH, Ohene-Frempong K, Krishnamurti L, et al. Sickle cell disease. Nat Rev Dis Primers. 2018;4:18010. https://doi.org/10.1038/nrdp.2018.10.
Berry RA. Sickle cell anemia: audiological findings. J Am Audiol Soc. 1975;1(2):61-3.
Donegan JO, Lobel JS, Gluckman JL. Otolaryngologic manifestations of sickle cell disease. Am J Otolaryngol. 1982;3(2):141–4. https://doi.org/10.1016/s0196-0709(82)80045-8
Gould HJ, Crawford MR, Smith WR, Beckford N, Gibson WR, Pettit L, et al. Hearing disorders in sickle cell disease: cochlear and retrocochlear findings. Ear Hear. 1991;12(5):352–4. https://doi.org/10.1097/00003446-199110000-00008
Wilimas JA, McHaney VA, Presbury G, Dahl J, Wang W. Auditory function in sickle cell anemia. Am J Pediatr Hematol Oncol. 1988;10(3):214-6. https://doi.org/10.1097/00043426-198823000-00006
Crawford MR, Gould HJ, Smith WR, Beckford N, Gibson WR, Bobo L. Prevalence of hearing loss in adults with sickle cell disease. Ear Hear. 1991;12(5):349-51. https://doi.org/10.1097/00003446-199110000-00007
Todd GB, Serjeant GR, Larson MR. Sensori-neural hearing loss in jamaicans with ss disease. Acta Otolaryngol. 1973;76(4):268-72. https://doi.org/10.3109/00016487309121507
Al-Dabbous IA, Al Jam’a AH, Obeja SK, Murugan ANR, Hammad HA. Sensorineural hearing loss in homozygous sickle cell disease in Qatif, Saudi Arabia. Ann Saudi Med. 1996;16(6):641-4. https://doi.org/10.5144/0256-4947.1996.641
Silman S, Silverman CA. Auditory diagnosis: principles and applications. Buckinghamshire: Singular; 1997. 412 p.
Lichtman MA, Kaushansky K, Kipps TJ, Prchal JT, Levi M, Williams WJ. Williams manual of hematology. New York: McGraw-Hill Medical; 2011. 757 p.
Elwany S, Kamel T. Sensorineural hearing loss in sickle cell crisis. Laryngoscope. 1988;98(4):386-9. https://doi.org/10.1288/00005537-198804000-00005
Urban GE. Reversible sensori-neural hearing loss associated with sickle cell crisis. Laryngoscope. 1973;83(5):633-8. https://doi.org/10.1288/00005537-197305000-00001
Abou-Elhamd KEA. Otorhinolaryngological manifestations of sickle cell disease. Int J Pediatr Otorhinolaryngol. 2012;76(1):1-4. https://doi.org/10.1016/j.ijporl.2011.10.004
da Silva LPA, Nova CV, Lucena R. Sickle cell anemia and hearing loss among children and youngsters: literature review. Braz J Otorhinolaryngol. 2012;78(1):126-31. https://doi.org/10.1590/s1808-86942012000100020
Mgbor N, Emodi I. Sensorineural hearing loss in Nigerian children with sickle cell disease. Int J Pediatr Otorhinolaryngol. 2004;68(11):1413–6. https://doi.org/10.1016/j.ijporl.2004.05.009
Piltcher O, Cigana L, Friedriech J, Ribeiro FAQ, Costa SS. Sensorineural hearing loss among sickle cell disease patients from southern Brazil. Am J Otolaryngol Head Neck Med Surg. 2000;21(2):75-9. https://doi.org/10.1016/s0196-0709(00)85001-2
De Castro Silva IM, Magalhães IQ, Toscano RA, Gandolfi L, Pratesi R. Auditory-evoked response analysis in Brazilian patients with sickle cell disease. Int J Audiol. 2010; 49(4):272-6. https://doi.org/10.3109/14992020903321742
MacDonald CB, Bauer PW, Cox LC, McMahon L. Otologic findings in a pediatric cohort with sickle cell disease. Int J Pediatr Otorhinolaryngol. 1999;47(1):23–8. https://doi.org/10.1016/s0165-5876(98)90152-5
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