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Updated: Jun 21, 2026
Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss
Published on: January 25, 2016
An Analysis of Risk Factors for Sensorineural Hearing Loss in Children With Sickle Cell Disease
Emily R Youner1, Thomas L Haupt2, Muhammad El-Shatanofy1
1Division of Otolaryngology-Head and Neck Surgery, The George Washington University School of Medicine & Health Sciences.
Insights
Sensorineural hearing loss (SNHL) is common in children with sickle cell disease (SCD). While hydroxyurea and tobramycin increased hearing loss, iron overload decreased it, suggesting a multifactorial cause.
Area of Science:
- Pediatric Hematology
- Otolaryngology
- Audiology
Background:
- Sensorineural hearing loss (SNHL) is a recognized complication in children with sickle cell disease (SCD).
- Understanding risk factors is crucial for early detection and intervention in this vulnerable population.
Purpose of the Study:
- To investigate hematologic and treatment-related risk factors associated with SNHL in pediatric SCD patients.
- To analyze audiometric trends and identify predictors of hearing deterioration over time.
Main Methods:
- Retrospective case series analysis of 201 SCD patients under 18 years old.
- Utilized ICD and CPT codes for data extraction, including demographic, hematologic, and audiometric parameters.
- Employed linear regression and mixed-effects models to assess associations between variables and bone conduction pure-tone average (BC PTA).
Main Results:
- 21.7% unilateral and 13.4% bilateral SNHL identified in SCD patients.
- Tobramycin and hydroxyurea use were associated with increased BC PTA, while iron overload showed a decrease.
- Long-term hydroxyurea use (>5 years) and avascular necrosis history predicted increased BC PTA over time.
Conclusions:
- Children with SCD are at significant risk for SNHL, likely due to multifactorial causes.
- Routine audiometric screening is recommended for pediatric SCD patients, particularly before and during new treatment regimens.
- Further research into the complex interplay of factors affecting hearing in SCD is warranted.
Objectives:
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.
Study Design:
Retrospective case series.
Setting:
Tertiary hospital.
Methods:
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.
Results:
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).
Conclusion:
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.
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