By Hyperacusis Research

At the 2024 Association for Research in Otolaryngology (ARO) MidWinter Meeting, held in early February in Anaheim, California, our partner Hyperacusis Research hosted a dinner where several researchers presented their latest findings.

“The annual ARO meeting is the world’s largest gathering of hearing experts, where 1,800 attendees can see over 200 panel presentations and discuss nearly 1,000 scientific experiments,” says Hyperacusis Research president Michael Maholchic. 

“Our [late] founder, Bryan Pollard, who introduced hyperacusis with pain as a new diagnosis, made it a point for Hyperacusis Research to be a visible presence every year. We continue that tradition,” Maholchic continues. 

“In addition to the 11 posters and one panel session dedicated to hyperacusis, we organized an adjacent dinner session. Researchers heard two patients describe the daily struggles of living with noise-induced pain, a panel presented current research, and a lively discussion followed among the 40 attendees. At the end, I asked those present to come forward, keep in touch, and strive to help us."

At the dinner Hyperacusis Research board member Ken Devore shared the story of his noise-induced hyperacusis, acquired after too much music exposure. Ken has suffered from pain hyperacusis for most of his adult life and, like many patients, finds that noise above his tolerance level causes setbacks which increase his sensitivity and pain. Seeking very quiet environments has been necessary to recover from setbacks and prevent make his hyperacusis from worsening. 

These researchers presented overviews of their work.

• Wei Sun, Ph.D., is an associate professor in the department of communication disorders and sciences at the University at Buffalo and a 2024 Emerging Research Grants scientist generously funded by Hyperacusis Research. He discussed the neural mechanisms of the FOXG1 mutation—a gene known to cause hyperacusis—to see how genetically determined hyperacusis can illuminate mechanisms of the more common types of acquired hyperacusis. 

• Megan Beers Wood, Ph.D., is a postdoctoral research fellow at Johns Hopkins University and a 2022 and 2023 ERG scientist generously funded by Hyperacusis Research. She explained her work on how to detect and quantify pain caused by sound and the role of the protein CGRP-α in inflammation and pain perception. 

• Benjamin Auerbach, Ph.D., an assistant professor at the University of Illinois at Urbana-Champaign, discussed the importance of developing research models of the range of hyperacusis experience, and the measurement of rodents’ avoidance behavior to unpleasant sounds. 

• Catherine Weisz, Ph.D., of the National Institute on Deafness and Other Communication Disorders, spoke about new and sophisticated techniques to research the circuitry of olivocochlear efferent neurons, which are important to healthy sound processing. 

• Jinsheng Zhang, Ph.D., a professor of communication sciences and disorders at Wayne State University, spoke about infrared light therapy on treating tinnitus in rats—with potential insights for hyperacusis. (He is for now omitted in the video above as he and his co-authors have not yet published what he presented.)

• Peter Steyger, Ph.D., a professor of biomedical sciences and director of the Translational Hearing Center at Creighton University, emphasized the need to define the research mission and attract high-quality proposals and funding. An ERG scientist in the 1990s, Steyger is also a longtime former member of HHF’s Council of Scientific Trustees, which oversees the ERG grants.

A Closer Look

Hyperacusis Research recently discussed Dr. Sun’s research grant with Iver Juster, M.D., the chair of its Scientific Advisory Board. Dr. Juster offered these comments and observations, based on this initial question:

“How can inborn hyperacusis shed light on acquired hyperacusis?” 

For simplicity, I’ll define inborn hyperacusis (IH) as entirely or very nearly entirely genetically determined, and acquired hyperacusis (AH) as hyperacusis following one or more injuries (which may be sound, drugs, toxins, etc.). Think of IH as something that’s in principle amenable to directly manipulating a gene or its downstream products.

• Susceptibility to AH: It’s likely that susceptibility to AH is at least partly influenced by genetics. This is true about most chronic conditions (like diabetes or high blood pressure). This susceptibility might explain why some people with no history of auditory problems acquire hyperacusis at a single concert and others don’t, even when they listen to very loud music. This was suggested by presentations on a variant of the GJB2 gene at ARO in 2023.

• Importance of basic science: The more we understand the entire biology of sound processing and its resulting perceptual experiences, the likelier we are to get to a cure. We don’t know enough yet to know how much knowledge we actually need to get there most efficiently. We need to better understand the different ways the brain responds to auditory input from the periphery and en route, and how the brain and lower central nervous system offer efferent messages toward the periphery. Genetics are involved in how well all this works.

• The search for genetic causes: A gene variant that causes the experience of hyperacusis at the brain level would teach us a lot about how the brain processes sound. What exactly is the connection between the gene and the protein it codes for? Why exactly would a miscoding cause sound to be processed in a way that was abnormally loud and painful?

• Pinpointing GABA: Can we learn something about how things go awry in people with IH that would appropriately reduce the central sensitivity that seems to occur with AH? Dr. Sun is planning to test the drug vigabatrin (which works on the neurotransmitter, GABA), on the mouse model; and some AH patients anecdotally claim to benefit from less toxic drugs that at least partly work through the GABA system like gabapentin, pregabalin, benzodiazepines, or butalbital.

• Central gain theory: If hyperacusis—particularly pain hyperacusis—is maintained as a chronic condition by the central nervous system (central gain), I am guessing that genetics plays a role in determining who gets how much central gain. And if it turns out it doesn’t, that’s important information, too.

• Role of epigenetics: It’s also possible that epigenetics (modification of whether or how efficiently genes express) plays a role, too. To understand how genes turn on and off—and under what circumstances and to be able to predict the effects of that—we need to understand the genes that ultimately inform the auditory experience.

Hearing Health Foundation sincerely thanks Hyperacusis Research for their ongoing generous support of hyperacusis investigations through our Emerging Research Grants program. 

Read more of Hyperacusis Research’s February Newsletter here.

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