The People Behind the Science

By Yishane Lee

Eight years ago we introduced a column called “Meet the Researcher.” Placed on the last page of the magazine (prime editorial real estate!), the MTR column was designed as a way to give our Emerging Research Grants (ERG) scientists a place to talk about their ERG project in more detail—and in lay terms for our readers—including its genesis, planned execution, and future goals.

Credit: Jane G. Photography

“Meet the Researcher” also is an opportunity for us to glimpse the person behind the science, with the researchers sharing how they became interested in their field and whether they have any personal connection to hearing conditions. Perhaps not surprisingly, many researchers do become interested in hearing and balance science as a result of their own experience with hearing loss. For instance, 2010 ERG scientist Judith Kempfle, M.D., told us she received an artificial eardrum at age 13, after many ear infections that her brother also got when they were kids growing up in Germany. With her ERG funded by the Royal Arch Masons General Grand Chapter International, Kempfle has gone on to work on many papers with Hearing Restoration Project member Albert Edge, Ph.D. (including a recent one about the effort to deliver drugs directly to the inner ear).

Ed Bartlett, Ph.D., Purdue University

Also a Royal Arch Masons grantee, 2011 ERG scientist Ed Bartlett, Ph.D., who published research on the lasting effects of blast shock waves on auditory processing, remembers asking his teacher whether we actually hear thoughts or if it something else. “So, I guess I was destined for auditory neuroscience,” says Bartlett, who also earned ERG funding in 2003, 2004, and 2009.

2011 and 2012 ERG scientist Regie Santos-Cortez, M.D., Ph.D., who earned the Collette Ramsey Baker Award named after HHF’s founder, spoke about the challenges of getting access to genetic information for her study that eventually pinpointed a gene mutation linked to a predisposition for ear infections. 2012 ERG scientist Bradley J. Walters, Ph.D., says he started out studying evolutionary biology, switched to studying regenerating damaged brain tissue, and then switched to hearing research. “I realized a lot of the ideas I had been working on in the brain could be applied to the ear,” he says. A 2017 paper he coauthored described successfully using gene therapy to regenerate hair cells in adult mice.

Alan Kan, Ph.D., University of Wisconsin, Madison

An early love of logic puzzles for 2013 ERG scientist Alan Kan, Ph.D., a Royal Arch Masons grantee, turned into studying audio engineering and his 2018 paper looking at how to improve speech understanding among people who use bilateral cochlear implants. Fellow 2013 Royal Arch Masons recipient Ross Maddox, Ph.D., remembers varying how he cupped his hands over his ears to get different sounds, leading to an interest in auditory processes and, eventually, research on how auditory and visual input is synthesized to understand sound.

After 26 years as a clinical audiologist, Royal Arch Masons 2014 ERG scientist Samira Anderson, Ph.D., switched to research. “Part of my motivation came from working with patients who struggled with their hearing aids,” she says. “I was frustrated that I was unable to predict who would benefit from hearing aids based on the results of audiological evaluations.” She produced three papers on the topic, bringing us closer to improving fit for and increasing the use of hearing aids.

Likewise, fellow Royal Arch Masons grantee Srikanta Mishra, Ph.D., produced two papers, one in 2017 and one in 2018, on children’s hearing that stemmed from his 2014 ERG grant—work that also led to a prestigious National Institutes on Deafness and Other Communication Disorders grant. And we liked the backstory for 2014 ERG scientist Brad Buran, Ph.D., so much that we put him on the cover of our magazine. Buran, who wears cochlear implants, multitasks during happy hour with his colleagues. “In an environment where it’s hard to hear,” he says, “within an hour they have all the information they need to use Cued Speech,” which uses visual representations of phonemes.

Beula Magimairaj, Ph.D., University of Central Arkansas

In 2015, we expanded our coverage of ERG recipients, so that every grantee is profiled in a “Meet the Researcher” column, all available online. Three papers resulted from the Royal Arch Masons grant received by 2015 ERG scientist Beula Magimairaj, Ph.D., and her research into children’s speech perception in noise and auditory processing (the third paper is in press). Funded by Hyperacusis Research Ltd., 2015 ERG scientist Kelly Radziwon, Ph.D., has managed to create a reliable animal model for loudness hyperacusis (essentially, inducing loudness intolerance in a rat and making sure it reacts to gradually increasing sound intensities) as well as finding a potential link between neuroinflammation and hyperacusis. 2015 and 2016 ERG scientist Wafaa Kaf, Ph.D.—who has 18 other family members (and counting!) who work in science—has been investigating Ménière’s disease, publishing on improving its diagnosis as it can be mistaken for other conditions, and the use of electrocochleography (ECochG) for diagnosing and monitoring the hearing and balance disorder.

Elizabeth McCullagh, Ph.D., University of Colorado

A karaoke fan who admits he “cannot resist Celine Dion,” Royal Arch Masons 2016 ERG scientist Richard Felix II, Ph.D. published on the greater-than-expected role of lower-level brain regions on speech processing. Fellow Royal Arch Masons grantee, 2016 ERG scientist Elizabeth McCullagh, Ph.D., makes her own cheese and beer in between uncovering new clues to sound localization problems in the genetic condition known as Fragile X syndrome, which can lead to autism.

Rahul Mittal, Ph.D., University of Miami Miller School of Medicine

2016 ERG scientist Harrison Lin, Ph.D., funded by The Barbara Epstein Foundation Inc., credits his older brother, also an otolaryngologist, for developing in him a love for science. He coauthored a January 2018 JAMA Otolaryngology–Head & Neck Surgery paper that detailed the gap between hearing loss diagnoses and treatments. 2016 ERG scientist Rahul Mital, Ph.D., who says he’d write fiction if not doing research, published an overview of hair cell regeneration, and Julia Campbell, Au.D., Ph.D., whose 2016 grant was funded by the Les Paul Foundation, understands firsthand what is feels like to have tinnitus, a topic she recently published a paper that investigated mild tinnitus in young patients with typical hearing. Hyperacusis Research-funded 2016 ERG scientist Xiying Guan, Ph.D., whose parents grew up doing manual labor in China, published a paper evaluating a treatment for conductive hyperacusis.

Some of our 2017 ERG scientists are already publishing. Royal Arch Masons grantee Inyong Choi, Ph.D., produced research on hybrid cochlear implants, which make use of residual hearing to produce more natural hearing. Oscar Diaz-Horta, Ph.D., whose 2017 ERG grant was funded by the Children’s Hearing Institute, investigated hair cell bundle structure and orientation. Very regretfully, Diaz-Horta died unexpectedly just as this paper was published.

Ian Swinburne, Ph.D., Harvard Medical School

Ian Swinburne, Ph.D., one of our Ménière’s Disease Grants scientists during its inaugural year in 2017, published a paper detailing one possible cause of Ménière’s disease. Swinburne and team discovered a structure in the inner ear’s endolymphatic sac acts a pressure-sensitive relief valve. Its failure may account for problems with inner ear fluid pressure and volume that may lead to hearing and balance disorders, including Ménière’s. “One activity I loved as a child was waterworks: building canals and aqueducts out of sand or dirt and then pouring water through them just to watch it flow,” he says. “Now I recognize an echo of that play in my study of water pressure and flow within the ear.”

We very much look forward to published research from all of our ERG scientists, including our latest crop of 2018 ERG scientists, whose ranks include a former college mascot, a violinist, a horse rider (of a horse named Gandalf), a Tibetan neuroscientist (and cookbook writer), a cricket player, a nonprofit cook who has prepared meals for 50,000 people, a dancer (including in flash mobs), and a builder of airplane scale models. Our ERG scientists deliver surprises of all sorts, from their backgrounds and how they got to where they are to the ground-breaking science they are spearheading in the lab.

We need your help supporting innovative hearing and balance science through our Emerging Research Grants program. Please make a contribution today.

Understanding a Pressure Relief Valve in the Inner Ear

By Ian Swinburne, Ph.D.

The inner ear senses sound to order to hear as well as sensing head movements in order to balance. Sounds or body movements create waves in the fluid within the ear. Specialized cells called hair cells, because of their thin hairlike projections, are submerged within this fluid. Hair cells bend in response to these waves, with channels that open in response to the bending. The makeup of the ear’s internal fluid is critical because as it flows through these channels its contents encode the information that becomes a biochemical and then a neural signal. The endolymphatic sac of the inner ear is thought to have important roles in stabilizing this fluid that is necessary for sensing sound and balance.

This study helps unravel how a valve in the inner ear's endolymphatic sac acts to relieve fluid pressure, one key to understanding disorders affected by pressure abnormalities such as Ménière’s disease.

While imaging transparent zebrafish, my team and I found a pressure-sensitive relief valve in the endolymphatic sac that periodically opens to release excess fluid, thus preventing the tearing of tissue. In our paper published in the journal eLife June 19, 2018, we describe how the relief valve is composed of physical barriers that open in response to pressure. The barriers consist of cells adhering to one another and thin overlapping cell projections that are continuously remodeling and periodically separating in response to pressure.

The unexpected discovery of a physical relief valve in the ear emphasizes the need for further study into how organs control fluid pressure, volume, flow, and ion homeostasis (balance of ions) in development and disease. It suggests a new mechanism underlying several hearing and balance disorders characterized by pressure abnormalities, including Ménière’s disease.

Here is a time-lapse video of the endolymphatic sac, with the sac labeled “pressure relief valve” at 0:40.

2017 Ménière’s Disease Grants scientist Ian A. Swinburne, Ph.D., is conducting research at Harvard Medical School. He was also a 2013 Emerging Research Grants recipient.

Ménière's Disease Grantee Featured in Reader's Digest

Credit: Agnieszka Marcinska, Shutterstock

Ian Swinburne, Ph.D., a 2018 Ménière's Disease Grant (MDG) recipient, shared his expertise regarding vertigo with Reader's Digest in an article called "What Causes Vertigo? 15 Things Neurologists Wish You Knew" published in March 2018. 

"The spinning, dizzying loss of balance which earmarks vertigo can come without warning," the article opens. Various professionals provide information about its duration, how it feels, and different types.

HHF-funded Dr. Swinburne notes specifically that the inner ear and balance disorder Ménière's disease can cause vertigo. He explains that "[b]outs of vertigo likely arise in patients with Ménière's disease, because the inner ear's tissue tears from too much fluid pressure—causing the ear's internal environment to become abnormal.'" He is currently pursuing a research project to understand the inner ear stabilizes fluid composition, which he believes will help to identify ways to restore or elevate this function to mitigate or cure Ménière's disease.

View the full article from Reader's Digest, here.