An Analysis of Risk Factors for Sensorineural Hearing Loss in Children With Sickle Cell Disease

The prevalence of sensorineural hearing loss (SNHL) is higher among children with sickle cell disease (SCD). This study aims to describe potential hematologic and other risk factors for SNHL in this population.

Retrospective case series.

Tertiary hospital.

Using ICD codes for hearing loss and CPT codes for audiometry, we analyzed demographic, hematologic, treatment, and audiometric data in SCD patients at Children's National Medical Center between 2000 and 2018. SNHL was defined as a 4-frequency bone conduction (BC) pure-tone average (PTA) ≥15 decibels. Univariate and multivariate linear regression analyses were used to study the association between changes in pure-tone average and study variables. A random intercept mixed-effects regression analysis was utilized to examine the PTA trend in patients with 3 or more audiograms.

We identified 201 SCD patients under 18 years old with complete audiograms. Eighty-seven percent were homozygous for hemoglobin SS, of which 21.7% had unilateral and 13.4% had bilateral sensorineural hearing loss. Tobramycin (coef: 19.76, 95% CI: 10.73-28.78, P <0.001) and hydroxyurea (coef: 3.75, 95% CI: 0.27-7.12, P =0.017) were significantly associated with an increased bone conduction pure-tone average (BC PTA), while the incidence of iron overload was associated with a decreased BC PTA (Coef=-7.96, 95% CI: -14.91, -1.01, P =0.012). The pure-tone average trend analysis showed a decline in bone conduction over time (-0.2, 95% CI: -0.3 to -0.07, P =0.001). However, the trend analysis predicted an increase in BC PTA for those who used hydroxyurea for >5 years (0.4, 95% CI: 0.1-0.8, P =0.020) and those with a history of avascular necrosis (0.6, 95% CI: 0.3-1.0, P <0.001).

Children with SCD may be at high risk for SNHL. No single morbidity was associated with an increase in bone conduction PTA. Hydroxyurea use was associated with increased bone conduction PTA, while iron overload was associated with decreased bone conduction PTA. The etiology of SNHL in SCD is likely multifactorial. Routine audiometric studies are recommended for children with SCD, especially before and after starting new treatments.