Hidden hearing loss in children and adolescents with sickle cell anemia.

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 < 0.05). There were significant differences in the brainstem auditory evoked potentials between the SCA and HC groups. In the SCA group, the waves III and V latencies were increased (p = 0.006 and 0.004 respectively), and the I-III and I-V interpeak intervals were longer (p = 0.015 and 0.018 respectively) than those in the HC group. There was no association between the audiological measures and clinical and metabolic variables and sickle cell anemia complications including endothelial function and therapy.

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.