The Pediatric Cortical Visual Impairment Society sponsored last week’s Perkins CVI Conference in Boston, a time for rich learning and community building. Many of our members attended, including about half of our Board of Directors. Here are 10 highlights from the event:
1. How prevalent is CVI?
Have you seen this prevalence data from CVI Now? The white paper, created in partnership with McKinsey & Company, provided the backdrop for the conference theme: Building Recognition. McKinsey used US medical claims data to build a predictive model showing how many likely cases of CVI exist. While the study has some limitations (for example, the data do not account for uninsured people), they believe there are 180,000 cases of CVI in the US and that fewer than 20% of children with CVI have a diagnosis. In other words, for every child with a CVI diagnosis, there are four more likely cases of CVI. McKinsey is working to have this study peer-reviewed.
2. Adults with CVI steal the show
“The most interesting part of the conference was listening to the CVIers talk about their lived experiences,” says Mary Zatta, PCVIS board member and Perkins Director of Professional Development. A panel of adults with CVI graciously shared what it’s like to live with a brain-based visual impairment, what has empowered them on their journey, and how they manage their symptoms.
- Nicola McDowell, PhD, Senior Lecturer, Institute of Education, Massey University, New Zealand describes CVI as having “too many apps open on an old computer and it freezes; the only thing to do is reboot.”
- Nai, Assistive Technology Trainer, Braille Instructor, American Sign Language Interpreter, DeafBlind Specialist, describes CVI as the feeling of always looking for things – which is still true for them when using a tactile modality. “I always thought I was filling the suitcase completely, but I realized I’m missing entire sections of the suitcase even by touch.”
- Dagbjört Andrésdóttir, Classical Singer and Blogger, describes CVI as the “Where’s Waldo? Syndrome.” She says it’s like “looking for something 24/7” and that she has CVI meltdowns and blackouts which usually end up in a migraine that requires 2-3 days of recovery.
- Tina Zhu Xi Caruso, Photographer and Art Student, describes CVI as “blobs of color. I try to figure out who is a blob of color. It’s unpredictable and sometimes I don’t recognize people at all.”
3. “The child sees the world from the inside out”
John Ravenscroft, PhD, Chair of Childhood Visual Impairment at The University of Edinburgh, and Marguerite Tibaudo, MEd, Education Director of the Deafblind Program at Perkins, discussed why understanding perception is important to understanding CVI. Whether or not you’re a philosophical thinker, it is interesting – and dare I say “mind-bendy” – to explore various theories of perception. The takeaway? Everyone sees the world differently, depending on life experiences.
In practice, this means taking a student-centric mindset when teaching a child. “Even those with the best intentions need to hear their learner's communication regarding their visual needs,” says PCVIS board member and Director of CViConnect, Stephanie Steffer.
4. Eye movements as a window into brain function
The conference organizers dedicated a morning to scientific research using eye tracking to elucidate CVI and visual processing. “It was an amazing, full-circle moment to hear John Ravenscroft discuss the importance of learning what is salient to an individual based on their experiences, and then to later hear Melinda Chang (Pediatric Neuro-ophthalmologist at Children’s Hospital Los Angeles and PCVIS board member) share how her research analyzed image saliency with a learner’s search and fixation patterns,” Stephanie adds. Dr. Chang presented several studies with takeaways for assessing visual acuity, ocular-motor function and higher-level processing in children with CVI.
Attendees also heard from:
- Jeremy Wolfe, PhD, Harvard Medical School, on what eye movements can teach us about thought, cognitive strategies and causes of error, for example, in expert radiologists who scan images for lung tumors.
- Glen Prusky, PhD, Burke Neurological Institute Weill Cornell Medicine, on how his team uses eye-tracking video games to objectively measure fixation, saccades, smooth pursuits, visual fields and form vision, and quantitatively describe visual function in kids with diverse brain injuries.
5. Virtual reality – it’s more than fun and games
Lotfi Merabet, OD, PhD, MPH, Harvard Medical School and Massachusetts Eye and Ear, uses eye tracking and virtual reality in his Laboratory for Visual Neuroplasticity. This research is building an understanding of higher-order visual processing deficits, which can be hard to characterize in a clinical setting. The lab gathers information about visual identification, perception, selective attention, working memory and visuomotor processing. For example, a participant may be asked to search an on-screen or virtual representation of an image for a specific object in a cluttered environment, like a mirror in a bathroom. Then he or she may be asked to search the same image for an incongruent object, like a skateboard in a bathroom. Dr. Merabet’s research shows that participants with CVI are less likely to find a visual target, their reaction time slows with increasingly difficult tasks, they are more likely to use saliency cues than semantic cues and they have a much smaller spotlight of attention. For example, if you took a flashlight with a wide beam into a dark room, you’d search the area more efficiently than if your flashlight had a narrow beam. People with CVI may be using a narrow beam to search the visual world.
6. A CVI screening tool conceived by a person with CVI
Nicola McDowell presented the Austin Assessment, an iPad-based screening tool for CVI. Aside from building a valid tool to screen for CVI, Nicola’s goal was to create a program that is easy for the assessor to use – even without vision training – and fun for the child so that they “do not feel like they are being assessed.” The screener is a shape-matching game in two rounds, the first being multi-colored and the second single-colored. It measures processing time as complexity increases, accuracy as task demand increases, the average time to make a match, and eye movements during the task. Many conference-goers had the chance to contribute to her research by playing the game as part of the control group.
7. Protactile demonstration
“My favorite part was the protactile demonstration from Nai,” says CVI parent and PCVIS board member Kathryne Hart. Proctactile is a communication method used by deafblind people that relies on touch, rather than visual methods like sign language. “They gave us so many ideas for teaching and learning O&M, math and ELA.” For example, Nai explained how someone might provide directions by tracing them step-by-step on the back or how to explain number line concepts using different points on the body.
8. A CVI registry maintained by the National Institutes of Health (NIH)
NIH representatives attended the conference to learn more about CVI and share their contributions to the cause. When the National Eye Institute (part of the NIH) issued a call for input on its strategic plan, 163 of the 252 responses they received were about CVI. This remarkable showing from our CVI community captured attention and, as a result, CVI is officially on the radar.
“The most exciting thing is the NEI developing a database - that's a win!” – Mary Zatta
The NIH is building a CVI registry to capture data on kids with CVI. Scientists will be able to apply for access to the database and contact interested participants to help answer their research questions. Kristina Hardy, National Institute of Neurological Disorders and Stroke at NIH, described some of the benefits of building a CVI registry. In the short term, this will help the medical community refine diagnostic batteries, improve the rate of diagnosis, identify meaningful outcomes, and document the scope and prevalence of co-occurring conditions. In the long term, this data can lead to understanding the neural basis of CVI, identifying biomarkers, examining clinical effectiveness, cost-effectiveness, or comparative effectiveness, and developing and evaluating neuroplasticity interventions. This collective work may have collateral possibilities like increased awareness, data to support policy changes, pathways to insurance coverage for services and a precision medicine approach.
9. A working definition of CVI
An interdisciplinary group facilitated by NIH has spent the past six months creating a working definition of CVI. The definition has five parts, which may be refined over time as the registry collects more data.
- 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.
- The visual dysfunction in CVI may manifest as lower- and/or higher-order afferent visual deficits, leading to characteristic behaviors in affected individuals.
- While CVI may be comorbid with other neurodevelopmental disorders, CVI is not primarily a disorder of language, learning, or social communication.
- The underlying neurologic insult of the developing brain may go unrecognized or undiagnosed until later in life.
10. Celebrating the CVI community
Dr. Richard “Skip” Legge, founder of PCVIS and the American Conference on Pediatric Cortical Visual Impairment, concluded the meeting with a walk down memory lane. As an ophthalmologist, he didn’t always recognize or understand CVI. Once he dedicated the energy to exploring CVI and its impact on children, he knew he had to bring together as many bright minds as possible to build understanding, generate public awareness and inspire advocacy for people with CVI.
A lot has changed since then. “As someone who has attended a lot of these CVI conferences over the past 15 years,” Stephanie remarks, “the growth in attendance is refreshing. It’s always nice to see familiar faces we might only connect with in person at these events, but it is a good feeling to see we are adding so many new faces to this community.”
Kathryne explains how meaningful this sort of gathering is to her. “Being with the community of everyone who gets it. Being able to meet the people we have connected with and followed on social media. Seeing familiar faces and meeting new ones. Linking up with like minds on legislative and educational policy. Finding support for what we would like to see brought into existence.”
Bringing CVI stakeholders together will always be crucial to the momentum of our cause. If you missed the Perkins CVI Conference, don’t despair. You’ll have another chance to gather with the CVI community in October, at the Innovations in CVI Conference and the preceding PCVIS annual meeting.
Wednesday, Oct. 2, 2024, 5 - 7:30 pm
Sheraton Pittsburgh Hotel at Station Square
Indicate your plans to attend the annual meeting when you register for the Innovations in CVI Conference.