Prevalence and risk factors of hearing loss and otological symptoms among Sickle Cell Disease patients in Saudi Arabia: a single center experience

Haila Alabssi; Maiadah  Alfares; Noura  AL Mulhim; Rabia  Latif; Nazish  Rafique; Nouf  Almulhim; Maram  Alismail; Samar  Algheryafi; Danah  Bokhari; Remah   Alzayyat; Amal  AlShaikh Sulaiman; Abrar  Alwaheed

doi:10.23750/abm.v95i2.15345

Prevalence and risk factors of hearing loss and otological symptoms among Sickle Cell Disease patients in Saudi Arabia: a single center experience

Authors

Haila Alabssi College of Medicine-Imam Abdulrahman Bin Faisal University https://orcid.org/0000-0001-7576-2315
Maiadah Alfares Department of physiology, College of Medicine-Imam Abdulrahman Bin Faisal University, Eastern Province, Dammam, Saudi Arabia, P.O. Box 1982 https://orcid.org/0000-0001-9681-8242
Noura AL Mulhim Department of physiology, College of Medicine-Imam Abdulrahman Bin Faisal University, Eastern Province, Dammam, Saudi Arabia, P.O. Box 1982 https://orcid.org/0000-0003-4652-0696
Rabia Latif Department of physiology, College of Medicine-Imam Abdulrahman Bin Faisal University, Eastern Province, Dammam, Saudi Arabia, P.O. Box 1982 https://orcid.org/0000-0001-6489-5809
Nazish Rafique Department of physiology, College of Medicine-Imam Abdulrahman Bin Faisal University, Eastern Province, Dammam, Saudi Arabia, P.O. Box 1982 https://orcid.org/0000-0002-1565-415X
Nouf Almulhim Imam Abdulrahman Bin Faisal University college of medicine https://orcid.org/0000-0002-1494-1931
Maram Alismail Imam Abdulrahman Bin Faisal University college of medicine https://orcid.org/0000-0002-6425-868X
Samar Algheryafi College of Medicine-Imam Abdulrahman Bin Faisal University https://orcid.org/0009-0000-3423-9157
Danah Bokhari Imam Abdulrahman Bin Faisal University college of medicine https://orcid.org/0009-0007-9166-4337
Remah Alzayyat Imam Abdulrahman Bin Faisal University college of medicine https://orcid.org/0000-0002-4145-7571
Amal AlShaikh Sulaiman Department of Otolaryngology Head and Neck Surgery -College of Medicine-Imam Abdulrahman Bin Faisal University, P.O. Box 1982, King Fahd Hospital of the University, Eastern Province, Khobar, Saudi Arabia https://orcid.org/0000-0002-9123-2821
Abrar J Alwaheed Department of Internal Medicine-College of Medicine-Imam Abdulrahman Bin Faisal University, P.O. Box 1982, King Fahd Hospital of the University, Eastern Province, Khobar, Saudi Arabia https://orcid.org/0000-0001-7740-4730

DOI: https://doi.org/10.23750/abm.v95i2.15345

Keywords:

Sickle cell disease, hearing, sensorineural, prevalence

Abstract

Background and aim: Sickle Cell Disease (SCD) is a genetic hematological disorder associated with various complications, including sensorineural hearing loss (SNHL). However, the prevalence and risk factors of SNHL among SCD patients remain unclear. The aim of the study is to determine the prevalence and risk factors of sensorineural hearing loss along with otological manifestations in SCD patients; to compare the findings between mild-moderate versus severe SCD patients. Research design and methods: A cross-sectional study was conducted between December 2022 and March 2023 at King Fahd University Hospital in Al Khobar. The study included fifty-four SCD patients (18-45 years old) who were receiving follow-up care. Participants underwent comprehensive audiological assessments; The Otology Questionnaire Amsterdam and clinical parameters were used to assess hearing in the patients. Results: Twelve patients (22.2%) had hearing loss on pure tone audiometry. Hearing loss risk factors in SCD patients were not statistically significant in our sample (P > 0.05). The Otology Questionnaire Amsterdam impact domain score and most of the complaints apart from ear itching and loss of taste showed a statistically significant correlation with SCD severity (P < 0.05). Conclusions: Sensorineural hearing loss is not uncommon in patients with sickle cell disease. Our study did not demonstrate any significant risk factors for hearing loss. However, the severity of various otological symptoms correlated significantly with SCD disease severity. Screening for ear-related complaints is therefore encouraged during patient encounters.

Author Biography

Noura AL Mulhim, Department of physiology, College of Medicine-Imam Abdulrahman Bin Faisal University, Eastern Province, Dammam, Saudi Arabia, P.O. Box 1982

MBBS, MSc, PhD

References

Rees DC, Williams TN, Gladwin MT. Sickle-cell disease. The Lancet. 2010;376(9757):2018–31. doi:10.1016/s0140-6736(10)61029-x

Jastaniah W. Epidemiology of Sickle Cell Disease in Saudi Arabia. Annals of Saudi Medicine. 2011;31(3):289–93. doi:10.4103/0256-4947.81540

Mangla A, Ehsan M, Agarwal N, Maruvada S. Sickle Cell Anemia. Brenner’s Encyclopedia of Genetics: Second Edition [Internet]. 2022 Nov 30 ;429–31. Available from: https://www.ncbi.nlm.nih.gov/books/NBK482164/

Inusa B, Hsu L, Kohli N, et al. Sickle cell disease—genetics, pathophysiology, clinical presentation and treatment. International Journal of Neonatal Screening. 2019;5(2):20. doi:10.3390/ijns5020020

Weatherall DJ, Clegg JB, editors. The Thalassaemia Syndromes. Oxford, UK: Blackwell Science Ltd; 2001.

Pecker LH, Schaefer BA, Luchtman-Jones L. Knowledge insufficient: the management of haemoglobin SC disease. Br J Haematol. 2017 Feb;176(4):515–26.

Zahnert T. The Differential Diagnosis of Hearing Loss. Dtsch Arztebl Int. 2011 Jun 24;

Kral MC, Brown RT, Hynd GW. Neuropsychological aspects of pediatric sickle cell disease. Neuropsychol Rev. 2001 Dec;11(4):179–96.

Kiser ZM, Clark KA, Sumner JL, Vercellotti GM, Nelson MD. Association between Sensorineural Hearing Loss and Homozygous Sickle Cell Anemia: A Meta-Analysis. Blood. 2019 Nov 13;134(Supplement_1):3453–3453.

A Solomon, NA Shofoluwe, IB Mohammed, et al. Risk Factors for Sensorineural Hearing Loss Among Children and Adolescents with Sickle Cell Disease in. 2021 April doi:10.13140/RG.2.2.13747.96801.

Kraak JT, van Dam TF, van Leeuwen LM, Kramer SE, Merkus P. The Otology Questionnaire amsterdam: A Generic patient‐reported outcome measure about the severity and impact of ear complaints. validation, reliability and responsiveness. Clinical Otolaryngology. 2020;45(4):506–16. doi:10.1111/coa.13545

Recommended procedure for tympanometry: British Society of Audiology. British Journal of Audiology. 1992;26(4):255–7. doi:10.3109/03005369209076644

Olusanya BO, Davis AC, Hoffman HJ. Hearing loss grades and the International classification of functioning, disability and health. Bull World Health Organ. 2019 Oct 1;97(10):725–8.

Siegel J. Otoacoustic Emissions. The Senses: A Comprehensive Reference. 2008;3:237–61.

Abdala C, Visser-Dumont L. Distortion Product Otoacoustic Emissions: A Tool for Hearing Assessment and Scientific Study. Volta Rev. 2001;103(4):281–302.

threshold A, audiometry P, tests hearing, loss noise. The Effect of Various Patterns of Personal Listening Devices on Hearing Among University Students in Saudi Arabia. Acta Informatica Medica. 2022;30(3):225.

World Health Organization Disability Assessment Schedule II (WHODAS II). A Compendium of Tests, Scales and Questionnaires. 2020;539–46. doi:10.4324/9781003076391-150

Al Jabr I. Hearing loss among adults with sickle cell disease in an endemic region: A prospective case-control study. Annals of Saudi Medicine. 2016;36(2):135–8. doi:10.5144/0256-4947.2016.135

Okbi MH, Alkindi S, Al Abri RK, Mathew J, Nagwa AA, Pathare AV. Sensorineural hearing loss in sickle cell disease—a prospective study from Oman. The Laryngoscope. 2011;121(2):392–6. doi:10.1002/lary.21374

Todd GB, Serjeant GR, Larson MR. Sensori-Neural Hearing Loss In Jamaicans With Ss Disease. Acta Otolaryngol. 1973 Jan 8;76(1–6):268–72.

Piltcher O, Cigana L, Friedriech J, Ribeiro F de AQ, Selaimen da Costa S. Sensorineural hearing loss among sickle cell disease patients from southern Brazil. Am J Otolaryngol. 2000 Mar;21(2):75–9.

Towerman AS, Hayashi SS, Hayashi RJ, Hulbert ML. Prevalence and nature of hearing loss in a cohort of children with sickle cell disease. Pediatric Blood & Cancer. 2018;66(1). doi:10.1002/pbc.27457

Nelson MD, Bennett DM, Lehman ME, Okonji AI. Dizziness, falls, and hearing loss in adults living with sickle cell disease. American Journal of Audiology. 2022;31(4):1178–90. doi:10.1044/2022_aja-22-00059

Aderibigbe A, Ologe F, Oyejola B. Hearing thresholds in sickle cell anemia patients: emerging new trends? J Natl Med Assoc. 2005;

Friedman EM, Luban NLC, Herer GR, Williams I. Sickle Cell Anemia and Hearing. Annals of Otology, Rhinology & Laryngology. 1980;89(4):342–7.

Rissatto-Lago M. Hearing Damage Caused by Sickle Cell Disease. In: Sickle Cell Disease. IntechOpen; 2022.

Strum D, Kapoor E, Shim T, Kim S, Sabetrasekh P, Monfared A. Prevalence of Sensorineural Hearing Loss in Pediatric Patients with Sickle Cell Disease: A Meta‐analysis. Laryngoscope. 2021 May 22;131(5):1147–56.

Al-Dabbous IA, Al Jam’a AH, Obeja SK, Murugan AN, Hammad HA. Sensorineural hearing loss in homozygous sickle cell disease in Qatif, Saudi Arabia. Annals of Saudi Medicine. 1996;16(6):641–4. doi:10.5144/0256-4947.1996.641

Onakoya PA, Nwaorgu OGB, Shokunbi WA. Sensorineural hearing loss in adults with sickle cell anaemia. Afr J Med Med Sci. 2002 Mar;31(1):21–4.

Kantow S, Seangpraw K, Ong-Artborirak P, Tonchoy P, Auttama N, Bootsikeaw S, et al. Risk factors associated with fall awareness, falls, and quality of life among ethnic minority older adults in upper northern Thailand. Clinical Interventions in Aging. 2021;Volume 16:1777–88. doi:10.2147/cia.s328912

Hebbel RP. Ischemia-reperfusion injury in sickle cell anemia. Hematology/Oncology Clinics of North America. 2014;28(2):181–98. doi:10.1016/j.hoc.2013.11.005

Shah N, Beenhouwer D, Broder MS, Bronte-Hall L, De Castro LM, Gibbs SN, et al. Development of a severity classification system for sickle cell disease. ClinicoEconomics and Outcomes Research. 2020;Volume 12:625–33. doi:10.2147/ceor.s276121