Sensorineural hearing loss in children with sickle cell anemia and its association with endothelial dysfunction.

OBJECTIVES

To investigate the prevalence of sensorineural hearing loss (SNHL) in children and adolescents with sickle cell anemia (SCA) and its association with endothelial dysfunction (ED).

METHODS

Fifty-two participants with stable SCA and 44 apparently healthy (AA genotype) participants aged 6-18 years were evaluated for pure tone audiometry and endothelial function using ultrasonographic imaging of the brachial artery to assess flow-mediated dilation (FMD). Laboratory analysis of the lipid profile and C-reactive protein levels was performed.

RESULTS

In the SCA group, 15 (28.8%) patients presented with SNHL. The FMD values were reduced in the SCA with SNHL group compared with the SCA without SNHL and healthy groups. Logistic regression analysis showed that FMD was associated with SNHL independent of the lipid profile and SCA characteristics (odds ratio [95% confidence interval] = 0.614 [0.440-0.858]; p = 0.004).

DISCUSSION: SNHL is a common complication in SCA; furthermore, this study identified a significant association between ED and SNHL. Damage to the vascular endothelium because of inflammation in SCA reduced blood flow in the inner ear. Thus, this circulatory disorder culminates in vaso-occlusive process and induces auditory disorders, such as SNHL.