Children with developmental disabilities — 1 in 6 children in the United States — are less likely to access a gold-standard hearing assessment in the first three months of hearing care, a recent paper in the Journal of Autism and Developmental Disorders reported.
Many developmental disabilities, such as cerebral palsy, Down syndrome, autism and intellectual disability co-occur with reduced hearing. This happens because there are overlapping causes and risk factors for the two conditions such as genetics, exposure to some viruses during pregnancy, prematurity or requiring care in the NICU. It is important to have a timely diagnosis of reduced hearing and access to appropriate interventions to maximize developmental outcomes.
However, children with developmental disabilities can experience a variety of barriers in accessing high-quality health care. In hearing health care, current clinical methods of measuring hearing — audiogram or auditory brainstem response (ABR) — are based on the assumption of typical development. That is, they might require that a child perform specific age-appropriate — but not developmentally appropriate — behavioral tasks to indicate that a sound has been heard. Or, if a child requires a physiologic measure like ABR, their medical status might contraindicate sedation that would aid in the test’s reliability. As a result, these gold-standard measures may not be accessible — defined as both the availability of a gold-standard assessment and obtaining the required data to determine hearing status. This, in turn, can delay diagnosis and receipt of appropriate interventions.
The recent study quantifies the relative risk of children with developmental disabilities not obtaining a gold-standard hearing assessment during their first three months of hearing care.
Using electronic health records from the Audiological and Genetic Database, hearing assessments were reviewed from 131,783 children (0-18 years) at three hospitals in the United States from 2008 to 2018. The study found that children with developmental disabilities were nearly four times more likely not to access a gold-standard hearing assessment than children in the comparison group.
For subgroups, the relative risk was:
- 13 times for children with multiple disabilities
- 11 times for children with intellectual disability
- 10 times for children with cerebral palsy
- Six times for children with Down syndrome
- Three times for children with autism
“There are likely multiple possible factors contributing to the disparities we observed. One factor we are particularly interested in is the misalignment among the developmental abilities, health profile of the child, and requirements of the hearing tests,” said first author Angela Bonino, PhD, assistant professor of Hearing and Speech Sciences.
“Children with developmental disabilities have delays in at least one domain of development (for example, communication, cognition, motor, emotional/social and sensory). Abilities can be diverse and/or complex across the different domains. So, it’s possible for children to have delays across all domains or only some domains. It’s also possible for children to have a delay in one domain and be advanced in another domain relative to similar age peers. Unfortunately, our behavioral testing methods are based on a model of typical development. Thus, these methods assume relatively similar abilities across and within developmental domains. If the developmental demands of the behavioral test method are not aligned with the child’s developmental abilities, this can result in no or little hearing data being collected.”
According to Bonino, the common assumption in the field is that if behavioral testing results are not obtained, hearing will be measured with an ABR test instead — but the study data do not support this.
“There are a variety of reasons this may be happening, but one consideration is that children with developmental disabilities often have co-occurring health conditions that pose extra risk when using sedation. But because the ABR requires that a child be quiet during testing, sedation is often required after about 6 months of age when natural sleep is less likely,” she said.
“This work is important for ensuring that all children, not just those who are typically developing, are afforded high-quality hearing care,” said Anne Marie Tharpe, PhD, chair of the Department of Hearing and Speech Sciences. “Approximately 30%-40% of children with permanent hearing loss have additional disabilities. Thus, having developmentally appropriate procedures to test their hearing is crucial to their care and outcomes.”
The study findings outline a need for establishing hearing assessment testing methods and hearing assessment guidelines for children with developmental disabilities to offset adverse consequences on development.
“When children seek hearing health care, they should be able to access a hearing evaluation that will accurately and quickly determine their hearing status regardless of their age, developmental abilities or medical profile,” Bonino said.
