Hearing Thresholds in Children and Adolescents with Sickle Cell Disease: A Comparative Study with Normal Hemoglobin Genotype in North-West Nigeria

Abstract

Context

Sickle cell disease (SCD) results in abnormal hemoglobin, leading to complications such as vaso-occlusive crises, chronic anemia, and organ damage. One significant yet often underreported complication is sensorineural hearing loss (SNHL). SNHL in SCD frequently begins with subtle elevation in hearing thresholds and can progress to permanent hearing impairment. This study compares the hearing thresholds of children and adolescents with SCD to those with normal hemoglobin genotype (HbAA).

Methods and Materials

A hospital-based, cross-sectional, case-control study was conducted involving 125 children and adolescents (ages 5–16 years) with SCD in steady state, compared with 125 age-matched healthy controls with HbAA genotype.

• Subjects were recruited from the Haematology Outpatient Clinic at Ahmadu Bello University Teaching Hospital (ABUTH).

• Controls were selected from the General Outpatient Clinic of the same hospital.
  Pure tone audiometry was used to determine hearing thresholds across standard frequencies.

Results

• The mean hearing thresholds in both ears were significantly higher among SCD subjects (20.0 ± 2.5 dB) compared to HbAA controls (14.2 ± 3.4 dB) (t = 12.918; p < 0.001).

• Adolescents with SCD demonstrated elevated thresholds at both low and high frequencies, with thresholds increasing with age.

• No significant gender difference was observed:

  • Males: 21.1 dB

  • Females: 21.2 dB

Conclusion

Children and adolescents with SCD have significantly elevated hearing thresholds compared to their peers with normal HbAA genotype. This hearing impairment worsens with age and affects both genders equally. These findings highlight the need for routine hearing assessments and targeted interventions for individuals with SCD to detect hearing loss early and prevent long-term complications.