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Enlarged Vestibular Aqueduct Syndrome

EVA Research Project

University Hospitals Offers Comprehensive Research on Enlarged Vestibular Aqueduct Syndrome (EVA)

Specialized physician-scientists at University Hospitals Rainbow Babies & Children’s Hospital are changing the current research landscape on enlarged vestibular aqueduct syndrome (EVA) by charting a new pathway for clinical research discovery. By collecting individual, detailed patient data and compiling a research patient registry, our researchers seek to better understand EVA, its different forms of expression, prognosis of possible progression of hearing loss, and risk factors for deterioration of hearing. Additionally, we hope to propose evidence-based protocols for physicians, patients and families to improve EVA classification, management and treatment.

Understanding EVA and its Symptoms

Hearing loss usually brings a patient to a physician for diagnosis and treatment. Although early diagnosis and treatment is important with EVA, vestibular symptoms are difficult to identify in the very young. Detected with a computed tomography (CT) scan or magnetic resonance imaging (MRI) scan, EVA is the most common inner ear malformation associated with sensorineural hearing loss. As yet, doctors do not completely understand if genetic and/or environmental conditions cause EVA. Research on the genetic basis of the hearing loss associated with EVA is ongoing at other institutions.

Limited Research Affects Quality of Life for Children

EVA is considered an orphan disease, meaning that it is a condition that generally affects fewer than 200,000 people nationwide. Because EVA is so rare, there is relatively little scientific research conducted as compared to other diseases. In fact, much of what is known about EVA comes from isolated clinical observations and limited findings – not from comprehensive scientific research. Because of limited research, there are no identified predictive factors for EVA-associated hearing loss. In fact, no treatments exist to prevent or reverse EVA-associated hearing loss, which affects quality of life for diagnosed children and their families.

Opportunities for Further Exploration

There is a growing interest in EVA and opportunities for further exploration. A significant increase in recent studies and authoritative literature has been presented, including EVA-related diagnostic imaging, issues with treatment techniques, and associated abnormalities.

One of the greatest new developments in EVA research, we are delighted to report that the World Health Organization is now proposing to add new EVA-relevant diagnosis codes to the 11th revision of the International Classification of Diseases (ICD). This is a watershed moment for EVA research, and could significantly speed up EVA patient identification for research purposes.

EVA has traditionally required manual health records review to ensure correct patient identification, but this is prohibitive to many researchers. Normally, we use diagnosis codes to find our study populations because these codes are used for insurance claims, and are used everywhere in healthcare.

Having a diagnosis code for EVA is crucial to doing more research, so we're really excited about this new diagnosis code system.

Ways to Help

There are many ways to get involved in our efforts as we continue to research EVA. From making a donation, to participating in fundraising events or volunteering, there are a variety of opportunities to help us as we make further progress in learning more about this rare disorder. Learn more about the ways you can help.