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Updated: Jul 30, 2026
Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss
Published on: January 25, 2016
Otologic findings in a pediatric cohort with sickle cell disease
C B MacDonald1, P W Bauer, L C Cox
1Department of Otolaryngology--Head and Neck Surgery, Boston Medical Center, MA 02118-2393, USA.
Insights
Sensorineural hearing loss (SNHL) is less common in young children with sickle cell disease (SCD) than previously reported. This study found a 3.5% prevalence of SNHL in pediatric patients, suggesting age-dependent hearing loss in SCD.
Area of Science:
- Pediatric Audiology
- Hematology
- Genetics
Background:
- Sickle cell disease (SCD) is linked to sensorineural hearing loss (SNHL) in older individuals.
- Limited research exists on SNHL prevalence in young children with SCD.
Purpose of the Study:
- To determine the prevalence of SNHL in a cohort of pediatric patients with SCD.
- To investigate the association between SCD and hearing loss in younger children.
Main Methods:
- Audiometric evaluations were performed on 84 pediatric patients with SCD.
- A total of 154 audiograms were analyzed from patients with a mean age of 7.8 years.
Main Results:
- Three out of 84 patients (3.5%) were diagnosed with mild SNHL.
- Middle ear effusion accounted for the majority of abnormal audiograms (19 subjects).
- No patients exhibited hearing loss severe enough to require amplification.
Conclusions:
- The observed low prevalence of SNHL in this pediatric cohort differs from previous findings in older populations.
- Age may be a significant factor in the prevalence of SNHL among individuals with sickle cell disease.
Objective:
Sensorineural hearing loss (SNHL) has been associated with sickle cell disease (SCD) in older children and adults. Few studies have investigated this association in younger children.
Design:
In a cohort of pediatric patients followed at our sickle cell clinic, with a mean age of 7.8 years, 154 audiograms were completed on 84 patients.
Results:
This produced 22 subjects with abnormal audiograms. Middle ear effusion was the etiology of 19 subjects' audiometric findings. Three children were found to have mild SNHL. None of the subjects had hearing loss of severity which would warrant amplification. The prevalence rate for SNHL in our cohort of pediatric patients was 3.5%.
Conclusion:
This differs from past reports, and may reflect an age dependent prevalence of SNHL in this disease.
