Hidden hearing loss in children and adolescents with sickle cell anemia

Abstract Objective To evaluate the auditory system for hidden hearing loss (HHL) and its association with clinical variables and endothelial dysfunction (ED) in children and adolescents with sickle cell anemia (SCA). Methods Participants included 37 patients with stable SCA and 44 healthy controls (HC group) (aged 6–18 years) with hearing thresholds ≤ 20 dB (dB) were evaluated for pure tone audiometry, tympanometry, acoustic reflex, otoacoustic emission, and auditory evoked potentials. Laboratory analysis of the lipid profile, and C-reactive protein levels and endothelial function using ultrasonographic imaging of the brachial artery to assess flow-mediated dilation were performed. Results The SCA group presented with a higher rate of increased contralateral acoustic reflex thresholds, compared to those in the HC group at all frequencies and in both ears (p  Conclusion In conclusion, our findings suggest that damage in the auditory system in SCA patients can be present involving retrocochlear structures, causing functional deficits without deterioration of auditory sensitivity.