Diagnosis and patterns of hearing loss in children with severe developmental delay
Stephen Trudeau1, Samantha Anne2, Todd Otteson3
1Case Western Reserve University School of Medicine, Cleveland, OH, United States of America.
Insights
Children with cognitive delays often have hearing loss, especially sensorineural hearing loss. Prompt sedated Auditory Brainstem Response (sABR) testing is crucial for timely diagnosis and treatment in these vulnerable children.
Area of Science:
- Pediatric Audiology
- Neurodevelopmental Disorders
- Hearing Science
Background:
- Behavioral audiometry (BA) is challenging for children with cognitive delays.
- Sedated Auditory Brainstem Response (sABR) testing is often necessary for accurate hearing assessment.
- Children with Down Syndrome (DS), Autism Spectrum Disorder (ASD), Global Developmental Delay (GDD), and Cerebral Palsy (CP) frequently require sABR.
Purpose of the Study:
- To evaluate diagnostic and hearing patterns in children with DS, ASD, GDD, and CP.
- To assess the effectiveness of sABR in children unable to complete reliable BA.
- To determine the incidence and type of hearing loss in these populations.
Main Methods:
- Retrospective chart review of children aged 0.5-18 years (2014-2019).
- Inclusion criteria: diagnosis of DS, ASD, GDD, or CP with unsuccessful BA testing.
- Data collected: testing patterns and audiometric data from sABR.
Main Results:
- Hearing loss (HL) diagnosed in 10% (GDD) to 46% (DS) of patients.
- Sensorineural HL accounted for up to 75% of cases in CP patients.
- Significant delays (8.6-21.8 months) were observed between initial BA and sABR.
Conclusions:
- High incidence of HL, particularly sensorineural HL, in children with cognitive delays.
- Delayed diagnosis and treatment due to multiple unsuccessful BA attempts.
- Recommend prompt sABR for children with severe cognitive delay hindering reliable BA.
Introduction & Objective:
Children with cognitive delay often experience challenges with obtaining hearing thresholds through behavioral audiometry (BA). This necessitates sedated Auditory Brainstem Response (sABR) testing. This study aimed to evaluate diagnostic and hearing patterns in children with Down Syndrome (DS), Autism Spectrum Disorder (ASD), Global Developmental delay (GDD), and Cerebral Palsy (CP) who were unable to complete reliable BA testing due to severe cognitive delay.
Methods:
Retrospective chart review on a cohort of children aged 0.5-18 years with a diagnosis of DS, ASD, GDD, or CP who underwent sABR due to unsuccessful BA testing. This was performed at a tertiary care institution from 2014 to 2019. Testing patterns and audiometric data were collected.
Results:
Across 15 DS, 39 ASD, 10 GDD, and 11 CP patients, the average time from first nondiagnostic BA to sABR ranged from 8.6 months (in GDD) to 21.8 months (in DS). The average number of BAs performed before sABR ranged from 1.6 (in ASD and GDD) to 2.7 (in DS). Hearing loss (HL) was diagnosed in 10%, 13%, 36% and 46% of patients with GDD, ASD, CP and DS respectively. Up to 75% of the HL was sensorineural (in CP patients).
Conclusion:
In children with significant cognitive delays, a high incidence of HL (especially SNHL) was identified, therefore high suspicion for HL should be held in these patients. Multiple unsuccessful BAs contribute to prolonged time to diagnosis and treatment, thus prompt sABR should be performed in patients whose severe cognitive delay inhibits reliable testing with BA.
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