The Degree of Hearing Loss for Children with EVA May Be Predictable.
It may be possible to predict the severity of hearing loss for children diagnosed with enlarged vestibular aqueduct, according to a new study published in JAMA-Otolaryngology-Head & Neck Surgery. The hope is that these findings will help parents of young children with EVA, and their physicians, better understand what lies ahead for their child’s hearing and speech based on the severity of that child’s malformation.
Enlarged vestibular aqueduct is the most common inner ear malformation associated with sensorineural hearing loss, which is caused by issues in the cochlea or the neural pathways that carry sound to the brain. Research shows that the malformation does not necessarily cause hearing loss, but both EVA and hearing loss are caused by the same types of underlying defects. There is no treatment specifically for EVA, however children experiencing hearing loss who have EVA are candidates for hearing aids or cochlear implantation depending on the degree of hearing loss.
This retrospective chart review, authored by physicians and researchers within the University Hospitals Rainbow Babies & Children’s Enlarged Vestibular Aqueduct (EVA) Research Project, is reportedly one of the first such studies to find a direct connection between the increasing width of the vestibular aqueduct and the degree of hearing loss a child experiences over time.
“There is still much to learn about the true causes and impacts of EVA,” said Mustafa Ascha, MS, first author of the study and a researcher within the UH Rainbow Department of Otolaryngology.
“Since EVA is considered an orphan disease, affecting fewer than 200,000 people per year, focused research on it has been limited,” said senior author Todd D. Otteson, MD, MPH, chief of pediatric otolaryngology at UH Rainbow. “This study may not change the treatment protocols already in place for children diagnosed with EVA in the short term, but it will enable physicians and families to appropriately plan a care trajectory that maximizes the child’s quality of life.”
The UH Rainbow Enlarged Vestibular Aqueduct Research Project is a donor-supported program that aims to change the current research landscape for EVA syndrome by charting a new pathway for clinical research discovery. The EVA Research Project is currently recruiting participants for a national EVA patient registry to collect patient experiences. The voluntary patient database will provide future researchers with the information necessary to better evaluate and make recommendations for children with EVA.
Source: University Hospitals Cleveland Medical Center
Image credit: University Hospitals, Rainbow Babies & Children’s Hospital
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