Prevalence of Hearing Loss in Adult Patients With Sickle Cell Disease: A Retrospective Cohort Study

Sickle cell disease is an inherited hematological disorder characterized by sickle-shaped red blood cells that cause vaso-occlusion and hemolysis. While systemic complications are well described, otolaryngologic manifestations such as hearing loss remain underrecognized despite their impact on quality of life.

A retrospective study used the TriNetX database to analyze adults (> 18 years) with or without sickle cell disease who had outpatient visits between May 1, 2022, and December 31, 2022. Propensity score matching by demographics was applied. Otolaryngological outcomes were extracted using relevant ICD-10 codes to compare occurrence between sickle cell disease and non-sickle cell disease cohorts.

Among 8,934,438 adults identified, 13,870 had sickle cell disease. After matching, each cohort retained 13,870 patients (mean age = 40.9 years). Hearing loss was more likely in the sickle cell disease cohort (6.0% vs. 2.6%; OR = 2.4, 95% CI = 2.1-2.7, p < 0.001). When stratified by hearing loss type, only sensorineural hearing loss was significantly higher (3.2% vs. 1.3%; OR = 2.5, 95% CI = 2.1-3.0, p < 0.001), while conductive hearing loss incidence did not differ (p = 0.238). Other conditions more frequent in sickle cell disease included obstructive sleep apnea (11.9% vs. 6.8%), allergic rhinitis (5.9% vs. 3.6%), and sinusitis (5.2% vs. 4.5%) (all p < 0.01).

This large, propensity score-matched analysis demonstrated a significant association between sickle cell disease and otolaryngologic complications, particularly sensorineural hearing loss. While there are limitations in how hearing is reported in large database studies, these findings underscore the need for routine hearing assessments to mitigate this risk and improve overall patient care for sickle cell disease patients.