Hearing loss among adults with sickle cell disease in an endemic region: a prospective case-control study

BACKGROUND: Sickle cell disease (SCD) is a common disease in the Eastern province of Saudi Arabia. One of the underreported complications of sickle cell disease is sensorineural hearing loss (SNHL). OBJECTIVES: The aim of this study was to estimate the prevalence of SNHL in patients with sickle cell disease by comparison with a control group from hematological diseases, and to consider the possible options that might minimize the occurrence of SNHL. DESIGN: Prospective case-control study. SETTING: Outpatient clinics in the Eastern province over a 9-month duration from July 2014 to March 2015. PATIENTS AND METHODS: Cases were selected consecutively and controls were matched for age and sex. Means for pure tone audiometry measurements and hearing thresholds were compared. MAIN OUTCOME MEASURE(S): SNHL in patients with sickle cell disease. RESULTS: Nine patients (22.5%) had hearing loss using pure tone audiometry. The mean of pure tone averages in the patient group was 21.1 on the right side and 21.5 on the left side, while the control group average was 15.4 on the right side and 15.2 on the left side. Comparison of the means of the hearing thresholds on each side for both groups showed statistically significant differences in all frequencies. CONCLUSIONS: Hearing loss is a common complication in patients with SCD. Annual hearing assessment is highly recommended to discover this complication as early as possible. LIMITATIONS: Relatively small sample size. The study was in a single outpatient clinic. Recall bias in the number of hospital admissions in the last ten years.