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Prevalence of Hearing Loss in Adult Patients with Sickle Cell Disease: A Retrospective Cohort Study
Introduction: Sickle cell disease (SCD) is an inherited hematological disorder characterized by the presence of sickle-shaped red blood cells, leading to various systemic complications due to vaso-occlusion and hemolysis. ENT manifestations, particularly hearing loss, are less frequently highlighted but can significantly impact patients' quality of life and overall health outcomes.
Methods: A retrospective study was conducted within the TriNetX database. Queries targeted patients aged 18 or older with or without a diagnosis of sickle cell disease (SCD) who had a documented outpatient visit between May 1, 2022, and December 31, 2022. Cohorts were propensity score-matched by age, sex, ethnicity, and race. Selected ENT outcomes were extracted using relevant ICD-10 codes to compare the occurrence between the SCD and non-SCD cohorts.
Results: Among 8,934,438 adults identified, 13,870 were diagnosed with SCD. After propensity score matching, 13,870 patients were retained in each cohort, with a mean age of 40.9 years. The majority in each cohort were Black or African American (N=9,966, 71.9%) and a small proportion were Hispanic or Latino (N=613, 4.4%). Hearing loss was more likely in the SCD cohort (6.0% vs. 2.6%; OR=2.4, 95% CI=2.1-2.7, p<.001). When stratified by hearing loss type, only sensorineural hearing loss was found to be higher (3.2% vs. 1.3%; OR=2.5, 95% CI=2.1-3.0, p<.001), while conductive hearing loss incidence did not differ from the non-SCD cohort (p=.238). Other conditions found to be more likely in the SCD cohort were obstructive sleep apnea (11.9% vs. 6.8%; OR=1.8, 95% CI=1.7-2.0, p<.001), allergic rhinitis (5.9% vs. 3.6%; OR=1.7, 95% CI=1.5-1.9, p<.001), and acute or chronic sinusitis (5.2% vs. 4.5%; OR=1.2, 95% CI=1.1-1.3, p=.004).
Conclusions: SCD patients included in the TriNetX database have a higher risk of sensorineural hearing loss compared to those without SCD. This finding underscores the need for routine hearing assessments in SCD patients to mitigate this risk and improve overall patient care.