NIH initiative to advance CVI

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Cerebral/cortical visual impairment (CVI) is the leading cause of childhood blindness and low vision, but because of a lack of awareness by clinicians and standard diagnostic definitions, CVI is often underdiagnosed and misdiagnosed. The NIH and the CVI community is on a path to change that.

The National Eye Institute at National Institutes for Health (NIH) is partnering with two other NIH institutes, the National Institute of Neurological Disorders and Stroke (NINDS) and the Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), to address the gaps in research and care for individuals with CVI. These gaps include a lack of a standard definition of CVI evidence-based diagnostic tools and guidelines, and unclear management and rehabilitation strategies.

The goals of NIH’s CVI initiative are to

foster awareness,
increase the CVI research pool,
establish consensus on CVI and the diagnostic process,
and build a CVI registry.

This is our hub to capture the progress of NIH’s CVI Initiative, and share the big moments happening for our CVI community.

CVI Working Definition

NIH published a working definition of CVI to support diagnosis, and so clinicians can use this definition to better understand, recognize, and refer children suspected to have CVI. The American Association of Pediatrics also published guidance for pediatricians on the diagnosis and care of children with CVI.

Less than 20% of kids with CVI have a diagnosis of CVI and we’re one step closer to making sure no kid with CVI is missed.

CVI is a neurodevelopmental disorder characterized by deficits of visual function and functional vision that are caused by neurologic damage to visual pathways and processing areas in the brain.
Chang, M. & Merabet, L. (2024). “Special Commentary: Cerebral/Cortical Visual Impairment Working Definition.” Ophthalmology, 131, 12, 1359 – 1365.

Five elements to help clinicians identify CVI

CVI encompasses a spectrum of visual impairments caused by an underlying brain abnormality that affects the development of visual processing pathways and is characterized by deficits in visual function and functional vision;
the visual dysfunction in CVI is greater than expected by any comorbid ocular conditions alone;
the visual dysfunction in CVI may manifest as lower-order or higher-order afferent visual deficits, or both, leading to characteristic behaviors in affected individuals;
although CVI may be comorbid with other neurodevelopmental disorders, CVI is not primarily a disorder of language, learning, or social communication; and
the underlying neurologic insult of the developing brain may go unrecognized or undiagnosed until later in life.

Our hope is this new definition will help healthcare providers, including ophthalmologists and optometrists, pediatricians, and neurologists, better understand the diverse CVI presentations and promote earlier diagnoses.
Gordon, S., Kerr, A., Wiggs, C., Chiang, M. (2024) “What Is Cerebral/Cortical Visual Impairment and Why Do We Need a New Definition?” Ophthalmology, 131, 12, 1357 – 1358.

Dive into the published articles:

NIH CVI Workshop

In November 2023, the NIH hosted a CVI workshop. Speakers included CVI researchers and medical providers, NEI data scientists, researchers from the cerebral palsy and autism communities, an adult with CVI and a CVI parent, and CVI educators.

It was an incredible day that brought the CVI community together toward building much-needed consensus around CVI, the diagnostic process, exploring what the CVI registry might look like, and how to support caregivers and people with CVI so they can access the resources, supports, and services they need find success and access their world.

Explore highlights and watch the recording.

The National Eye Institute (NEI) sets CVI as a research priority

The National Eye Institute (NEI) at the National Institutes for Health (NIH) released their Strategic Plan: Vision for the Future and CVI has a clear presence. This is a big win for the CVI community. NEI/NIH is an important driver of innovative research (and funding for research) to better understand the eye and the visual system. So when CVI is set as a research priority for the first time ever, this is something to celebrate!

CVI is front and center in the NEI Strategic Plan because of strong advocacy from the CVI community. NEI opened an online Request for Information (RFI) at the end of 2019 and the CVI community—medical professionals, educators and therapists, and families—came together to show our strong collective voice. As result, CVI was the single most mentioned issue (nearly two-thirds of the total 252 responses).

NEI’s new mission statement is centered around driving innovative research, fostering collaboration, recruiting a talented and diverse workforce, and educating providers, scientists, policymakers, and the public.

The National Institutes of Health (NIH) hosted a CVI workshop in November of 2023, to foster awareness, increase the CVI research pool, establish consensus on CVI and the diagnostic process, and build a CVI registry. You can watch a recording of the entire day’s presentations.

The condition [CVI] has historically been under recognized.
NEI Strategic Plan: Vision for the Future

What does NEI’s Strategic Plan say about CVI?

In the NEI Strategic Plan: Vision for the Future, you can find CVI in several places. The strategic plan has six areas of emphasis—CVI is discussed in Biology and Neuroscience of Vision, Data Science, and Quality of Life.

Pages 63-64: the report states the need to coordinate research on CVI that includes

bringing together scientific and stakeholder communities,
conducting research “to identify biomarkers to allow for more accurate diagnosis, risk prediction, and evaluation of treatment efficacy,”
creating an “evidence-based age appropriate test battery and CVI classification system,”
understanding “the neural basis of CVI,”
exploring “neuroplasticity-based therapies,”
researching and disseminating “effective clinician awareness strategies to increase timely recognition and diagnosis of CVI.”

Page 96: CVI is used as an example for the plan to “develop registries to track and better understand diseases, conditions, and interventions.”

Pages 100-101: the report highlights how current CVI research “unlocks complexities of brain-based visual impairment and neuroplasticity.”

Pages 103-105: the report discusses the research gaps in the Quality of Life area. CVI has a strong presence with the emphasis on research to explore individualized rehabilitation strategies, comorbidities and integrated care management, and connecting neuroscience to vision rehabilitation research. Some specifics include:

“Develop and evaluate evidence-based practices for rehabilitation of ocular- and brain-based visual impairments based on standardized outcome measures.”
“Enhance the ability to diagnose brain-based visual impairments quickly and accurately, assess the range of abilities present, and design strategies tailored to a given individual.”
“Study the application of advanced imaging techniques to examine the causes of brain-based visual impairment and how neural connections can be modulated to affect function.”

[W]hile vision research has focused on diagnosis and treatment of ocular causes of vision loss, the field is in the early stages of understanding brain-based vision loss from conditions such as CVI, TBI, or stroke. Different rehabilitation strategies are needed for brain-based, compared with ocular, causes of vision loss.
NEI Strategic Plan: Vision for the Future

Pages 108-109: you’ll find a two-page callout all about CVI that covers the causes of CVI, impacts on visual function, clinical and public health impact, and diagnosis, management, and rehabilitation. The report clearly states that CVI is the leading cause of visual impairment in children and that “more research is needed to accurately diagnose and optimally treat those affected.”

Pages 133-134: shows the results of the Request for Information and states that “two-thirds of the total comments focused on cerebral (cortical) visual impairment (CVI), which is now a leading cause of childhood blindness in the U.S.”

Check out the entire NEI Strategic Plan: Vision for the Future (2021-2025).