Hearing Health Blog
To better identify and treat these central auditory processing disorders that appear despite normal ear function, 2016 Emerging Research Grants (ERG) scientist Richard A. Felix II, Ph.D., and colleagues have been investigating how the brain processes complex sounds such as speech.
By Lauren McGrath
Assistant Teacher Ms. Tiana Brown with two of her preschool students at Clarke.
The clock moves toward 9:00 AM as two teachers oversee the listening check with their preschool students, ages four to five, to verify that their hearing devices are operating properly. A critical test for children with hearing loss, the check is step one each day for colleagues Ms. Kathryn Smith, Teacher of the Deaf, and Ms. Tiana Brown, Assistant Teacher at Clarke Schools for Hearing and Speech in New York.
Assured that all devices allow optimal access to sound, Ms. Kathryn and Ms. Tiana are ready to begin a busy day in the classroom. Beyond following a typical preschool curriculum with pre-reading, pre-academics, math, science, art, music, and language, the two teachers lead social and emotional development and self-help instruction. Throughout the day, Ms. Kathryn and Ms. Tiana track students’ progress toward goals they've defined as part of each child’s professional team. Each team is comprised of a unique set of professionals, based on individual students' strengths and needs.
Both Ms. Kathryn and Ms. Tiana have long been passionate about working with children. Ms. Tiana takes pride in being an advocate who can provide emotional support to kids and Ms. Kathryn feels fortunate to spend her career working with young people who are full of wonder and excitement.
Ms. Kathryn Smith, Teacher of the Deaf, smiles with a student.
Ms. Kathryn holds a Bachelor's in Communication Disorders with a minor in Deaf Studies from SUNY New Paltz and a Master’s in Deaf Education from Hunter College. Ms. Tiana completed her Bachelor’s in Communication Disorders at St. John’s University. After developing interests in aural rehabilitation in school, working with children who are deaf or hard-of-hearing—where they can contribute to the success of many children with unique perspectives and experiences—was a natural career choice for both Ms. Kathryn and Ms. Tiana.
The progress that Clarke students make, despite not having the same abilities as their typical-hearing peers, impresses the teachers. Though the children have an “added challenge at the starting line,” they experience tremendous growth as a result of their efforts made both independently and in collaboration with their families and professionals, says Ms. Kathryn. She recalls a few of her classroom’s latest accomplishments. One child is celebrating her newfound ability to put her FM system on all by herself. Another student who recently received a cochlear implant is regularly responsive to the sound of his name in the noisy classroom.
Ms. Tiana reflects on positive experiences outside the classroom, such as daily trips to the park, which she particularly enjoys. “As soon as we step outside, a whole new world opens up for them. They tell me about the sounds they hear and the sights they observe—and I know they’re not missing out on a single piece of life.” She feels most rewarded at work when a student expresses gratitude for help she provided.
At 2:30 PM, the Clarke students make their way out of school and home to their families. As staff, Ms. Kathryn and Ms. Tiana also build relationships with the school’s families who, like the students, greatly admire the teachers and look to them for guidance. Ms. Kathryn reminds parents and families not to lose sight of their child in the diagnosis. “Your child has a hearing loss, but it is not all of them. Your hopes and dreams for your child can still be achieved; they may just take a different route.”
By Yishane Lee
The U.S. Food and Drug Administration (FDA) has approved a novel drug to protect against ototoxicity (harmfulness to hearing) due to the use of aminoglycoside antibiotics to treat severe infections. The FDA approval paves the way for a Phase I clinical trial to test whether the drug, found to be significantly protective in animals, is safe for humans.
Mature lateral line hair cells from larval zebrafish (shown with the neuromast sensory organ enlarged) serve as a platform for studying drugs and genes that modulate hair cell susceptibility to ototoxic agents.
The drug, ORC-13661, was developed by University of Washington professors Edwin Rubel, Ph.D., and David Raible, Ph.D., who are members of Hearing Health Foundation’s Scientific Advisory Board and Hearing Restoration Project, respectively, and Fred Hutchinson Cancer Research Center scientist Julian Simons, Ph.D. “While this program was not directly funded by HHF, both David and I have definitely been supported by HHF for a long time,” Rubel says. “This is a drug to prevent hearing loss that we've developed over the past 15-plus years.”
Rubel points out the drug’s two main features: “It is a brand new drug with a composition of matter patent, not one that is used for other medical purposes and being repurposed; and it is the first drug that was developed, from the get-go, to protect hair cells from ototoxic injury.”
After screening libraries of potential chemicals to see which stopped hair cell death in zebrafish lateral line system, Rubel, Raible, and team identified the best candidate and then boosted its effectiveness by tweaking its chemical structure; results were published in the Journal of Medicinal Chemistry in January 2018.
Rubel adds, “Toxicity studies in zebrafish, rats, and dogs required by the FDA show superior safety and nearly 100 percent hearing protection at all frequencies.” If the Phase I trial shows the drug is safe for humans, the next step is to test its efficacy among patients using aminoglycosides.
By Carol Stoll
Stimulus frequency otoacoustic emissions (SFOAEs) are sounds generated by the inner ear in response to a pure-tone stimulus. Hearing tests that measure SFOAEs are noninvasive and effective for those who are unable to participate, such as infants and young children. They also give valuable insight into cochlear function and can be used to diagnose specific types and causes of hearing loss. Though interpreting SFOAEs is simpler than other types of emissions, it is difficult to extract the SFOAEs from the same-frequency stimulus and from background noise caused by patient movement and microphone slippage in the ear canal.
2014 Emerging Research Grants (ERG) recipient Srikanta Mishra, Ph.D., and colleagues have addressed SFOAE analysis issues by developing an efficient data-driven analysis procedure. Their new method considers and rejects irrelevant background noise such as breathing, yawning, and subtle movements of the subject and/or microphone cable. The researchers used their new analysis procedure to characterize the standard features of SFOAEs in typical-hearing young adults and published their results in Hearing Research.
Mishra and team chose 50 typical-hearing young adults to participate in their study. Instead of using a discrete-tone procedure that measures SFOAEs one frequency at a time, they used a more efficient method: a single sweep-tone stimulus that seamlessly changes frequencies from 500 to 4,000 Hz, and vice versa, over 16 and 24 seconds. The sweep tones were interspersed with suppressor tones that reduce the response to the previous tone. The tester manually paused and restarted the sweep recording when they detected background noises from the subject’s movements.
The SFOAEs generated were analyzed using a mathematical model called a least square fit (LSF) and a series of algorithms based on statistical analysis of the data. This model objectively minimized the potential error from extraneous noises. Conventional SFOAE features such as level, noise floor, and signal-to-noise ratio (SNR) were described for the typical-hearing subjects.
Overall, the results of this study demonstrate the effectiveness of the automated noise rejection procedure of sweep-tone–evoked SFOAEs in adults. The features of SFOAEs characterized in this study from a large group of typical-hearing young adults should be useful for developing tests for cochlear function that can be useful in the clinic and laboratory.
Srikanta Mishra, Ph.D, was a 2014 Emerging Research Grants scientist and a General Grand Chapter Royal Arch Masons International award recipient. For more, see “Sweep-tone evoked stimulus frequency otoacoustic emissions in humans: Development of a noise-rejection algorithm and normative features” in Hearing Research.
We need your help supporting innovative hearing and balance science through our Emerging Research Grants program. Please make a contribution today.
By Vicky Chan
Like most moms, Brandy has always been a champion for her three sons, Anthony, 12, Andersyn, 10, and Ayden, 7. Her sons are unlike most sons; each has bilateral sensorineural hearing loss and enlarged vestibular aqueduct syndrome.
Brandy’s journey as a parent-advocate had a difficult start. She was completely unfamiliar with hearing loss in children before she became a mother, and accessing proper treatment for the trio was a challenge. Brandy juggled numerous audiologist appointments that were a five-hour round-trip drive from home. And, for her oldest child, Anthony, a hearing loss diagnosis came two years delayed.
Clockwise from left: Ayden, Andersyn, Anthony, and Brandy.
Anthony had typical speech development and passed all his first- and second-year wellness and hearing checks by his pediatrician. When he was 2, Anthony fell and hit his head. Brandy suspected the trauma had caused either hearing loss or a cognitive disorder, but the doctors assured her Anthony suffered no permanent damage and took no action for him.
Brandy’s instincts were correct. When her second child, Andersyn, was diagnosed with hearing loss at birth a few months after Anthony’s head injury, she insisted Anthony receive a detailed hearing evaluation. Born in 2005, Anthony never received a newborn screening despite the passage of the Newborn and Infant Hearing Screening and Intervention Act of 1999, which mandated the practice.
The legislation quickly improved the rate of newborn hearing screening. In 2005, 94.2% of babies in the U.S. were screened, but some states lagged behind. In Tennessee, where all three of Brandy’s sons were born, only 66.9% of newborns were tested—the lowest in the nation. Unfortunately, Anthony was among the 30.1% of Tennessee’s babies not screened. However, by Andersyn’s birth in 2007, the state’s rate increased to 91%. It was only due to Brandy’s perseverance that Anthony was ultimately given a comprehensive exam, diagnosed with severe bilateral hearing loss, and fitted for hearing aids.
Brandy’s message is that newborn screening is vital. “If your child has hearing loss, it is best to start intervention as soon as possible and have your child fitted for hearing aids or cochlear implants if they need them.”
With his hearing aids, Anthony was fascinated by all the new sounds he could hear—including the squishy sound of Brandy’s flip-flops as the pair walked through a parking lot. At that moment, Brandy realized it was likely that Anthony, like Andersyn, was born with hearing loss, but it only became detectable to her after his head injury.
Andersyn was given a newborn hearing test so Brandy knew immediately that he had severe bilateral hearing loss. Later on, one audiologist suggested he wasn’t benefiting from his hearing aids, but Brandy knew differently; with Andersyn’s hearing aids turned up, a sound as subtle as crinkling paper near his ears would startle him. Andersyn now does exceptionally well with hearing aids, as does Brandy’s third and youngest child, Ayden, who was also born with severe hearing loss in both ears. The boys’ doctors have cited a genetic connection of unknown cause.
Today, hearing loss is an ordinary part of life for her three boys, thanks to Brandy’s tireless advocacy. With help from FM systems and speech therapy, Anthony, Andersyn, and Ayden all receive a mainstream education. They enjoy baseball, basketball, hunting, swimming, riding four wheelers, and fishing. HHF’s CEO, Nadine Dehgan, exclaims, “All three boys are incredibly fortunate to have Brandy, a devoted mother who has prioritized their hearing health.”
Anthony, Andersyn, and Ayden are participants in HHF's "Faces of Hearing Loss" campaign.
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This April, Hearing Health Foundation (HHF) draws your attention to Auditory Processing Disorder (APD), a condition that causes impairments in sound localization—the ability to identify sound sources—and has been closely linked to autism.
The CEO of Oklahoma’s Hough Ear Institute and a 26-year U.S. Army veteran, Richard Kopke, M.D. (1996 ERG), coauthored a March 2018 paper in Molecular Therapy about the use of small interfering RNAs (siRNAs) to block the activity of the Notch signaling pathway gene Hes1. Hes1 has been shown to itself block supporting cells from converting into hair cells. The team found that a sustained release of siRNAs in mouse cochleae through nanoparticles after deafening resulted in the recovery of some hearing ability, measured using auditory brainstem responses. Compared with the control mice, the injected mice showed less overall hair cell loss and early signs of immature hair cell development, which the authors say may signal hair cell regeneration. —Y.L.
A special publication orchestrated by five of the nation’s leading hearing experts compiles the latest research into hearing loss caused by drugs and solvents—how it occurs, how to treat it, and how to prevent it.
A free e-book comprising 23 scientific articles from 93 authors, “Cellular Mechanisms of Ototoxicity” was published by Frontiers in Cellular Neuroscience in March 2018. “We’re trying to elevate ways for the human population to avoid losing this important sensation for experiencing and communicating with the world around us,” says co-editor Peter Steyger, Ph.D. (1995–96 ERG), a professor of otolaryngology–head and neck surgery in the Oregon Health & Science University (OHSU) School of Medicine. A member of HHF’s Council of Scientific Trustees, Steyger lost hearing at age 14 months after being treated with antibiotics for meningitis.
“Ototoxicity is a threat to hearing at any age, and hearing loss remains a significant side effect of chemotherapy,” says co-editor Jian Zuo, Ph.D., of St. Jude Children’s Research Hospital in Memphis, Tennessee. Additional editors included experts from the Department of Defense Hearing Center of Excellence and the National Institute on Deafness and Other Communication Disorders. —OHSU News
By Gina Russo
Hearing Health Foundation (HHF) is pleased to share that our latest Annual Report is now available for your review. The report provides in-depth coverage of our activities, events, and achievements for fiscal year 2017 (October 1, 2016 - September 30, 2017). Highlights from the report include the following:
Promising investigative work in hearing and balance. Thanks to the generosity of many individuals and organizations, HHF funded nine Hearing Restoration Project (HRP) projects and 14 Emerging Research Grant (ERG) recipients last year.
Using three animal models, HRP scientists continued their collaborative quest (pp. 14-18) to permanently cure hearing loss and tinnitus through hair cell restoration. Says HRP consortium member David Raible, Ph.D., “Collaboration is the key to innovation. Although there is a romantic picture of a scientist slaving away in isolation, toiling toward ‘eureka’ moments, science works best with communication and sharing ideas.”
Meanwhile, our ERG recipients (pp. 19-22) were awarded grants to fund their up-and-coming work in the areas of Ménière's Disease, tinnitus, central auditory processing disorder (CAPD), hearing health, Usher syndrome, hearing loss in children, and stria vascularis.
Introduction of a research program, Ménière's Disease Grants (MDG). HHF funded two former ERG recipients, Drs. Gail Ishiyama and Ian Swinburne, who will help to advance our understanding of the inner ear and balance disorder Ménière's Disease. These grants (p. 23) were generously supported by a family committed to finding better treatments and cures for this disabling condition.
Preview of our exciting Hearing Loss Survey findings. HHF, in partnership with the Hearing Loss Association of America (HLAA), surveyed 2,300 people to understand how to better serve the needs of the hearing loss community. Affordability and accessibility of hearing healthcare was an underlying trend in the responses (pp. 11-12), and participants overwhelmingly support the government playing a role in access to hearing loss treatment. A full survey manuscript and executive summary will be available in April 2018.
Thank you to all our fundraisers and donors. We were fortunate to receive large-scale support from Cantor Fitzgerald Charity Day, marathon runner Catherine McLoughlin, and the De Francesco family. You can read about each on pp. 8-10. Additionally, you will find a complete list of our FY’17 donors on pp. 26-36, who all helped make a difference in hearing loss research.
Continued outstanding financial transparency. HHF retained top marks from all charity watchdogs (BBB Wise Giving Alliance, Charity Navigator, CharityWatch, and Guidestar) and was recognized, for the second consecutive year, as a “Best Charity for Your Donation” by Consumer Reports. Examine our Report’s Financials on pp. 37-39 for more information about how we efficiently steward donor dollars.
Should you have any questions about the report, please get in touch with us at info@hhf.org. To learn more about naming a grant and other giving opportunities, contact development@hhf.org. If you are able to help us fund research programs in 2018, we encourage you to make a contribution to HHF before September 30, 2018 to see your name included on the list of donors in next year’s Annual Report.
By C. Adrean Mejia
Before Women’s History Month concludes, Hearing Health Foundation (HHF) would like to highlight the accomplishments of women in science, technology, engineering, and mathematics (STEM), including those who have been instrumental to our own progress toward preventing, treating, and curing hearing loss and related conditions.
Historically, STEM has been majority male, but the growing inclusion of women in the industry is closing the gender gap. In fact, LinkedIn reports the percentage of women entering STEM roles in the last four decades is greater than that of any other professional sector. In 1978, the STEM workforce was only 10% female, while today about a third of this field is comprised of women.
Emerging Research Grants (ERG) recipient Dr. Wafaa Kaf administers a hearing screening. Credit: Missouri State University.
As individuals and as an organization that values inclusiveness, we all at HHF applaud the trend of growing opportunity for women in scientific professions, while remaining equally grateful to the male researchers and Board members who offer their commitment, support, and expertise. Our founder was a woman; 60 years ago, Mrs. Collette Ramsey Baker began a quest to find better treatments and cures for hearing and balance conditions which is championed by all today.
We would like to acknowledge the outstanding women on HHF’s Board of Directors, whose altruism and intelligence have furthered hearing research and HHF’s growth. Our Board Chair, Elizabeth Keithley, Ph.D., who has been an auditory researcher for more than 30 years, began her association with HHF as a grant reviewer. Dr. Keithley has conducted and published a number of studies related to the mechanisms of inflammation and aging on the inner ear.
From left: HHF Board Chair Elizabeth Keithley, Ph.D., and Board member Judy Dubno, Ph.D.
Board member Judy Dubno, Ph.D., professor at the Medical University of South Carolina, is considered one of the most important otolaryngology researchers in the nation. Her work has focused on auditory perception, hearing loss, and speech recognition. Dr. Dubno was also a contributor to the report that successfully urged the FDA to create a category of over-the-counter hearing aids to make hearing loss treatment more accessible to American adults.
Also serving on the Board is Ruth Anne Eatock, Ph.D., of the University of Chicago, who studies sensory signaling by hair cells and neurons in the inner ear. She was recently published in The Journal of Neuroscience for her investigation of inner ear sensory cells in rodents.
HHF is also thankful for the three female scientists who are part of our Hearing Restoration Project (HRP) consortium working to permanently cure hearing loss: Ronna Hertzano, M.D., Ph.D., Tatjana Piotrowski, Ph.D., and Jennifer S. Stone, Ph.D. Their labs at the University of Maryland, Stowers Institute for Medical Research, and the University of Washington, respectively, have uncovered valuable insights related to a biological cure for hearing loss.
Our Emerging Research Grants (ERG) program has empowered many brilliant, female researchers, including those recently published: Wafaa Kaf, Ph.D., researching new techniques to diagnose Ménière's disease; Michelle Hastings, Ph.D., investigating early genetic intervention for Usher syndrome; Elizabeth McCullagh, Ph.D., examining the connection between sound localization difficulties and Fragile X Syndrome; and Samira Anderson, Au.D., Ph.D., working to improve hearing aid fit to enhance usage.
Finally, we are fortunate to have Nadine Dehgan serving as our CEO. Ms. Dehgan plays a crucial role in our growth and programming efficiency, and her leadership experience and passion for how hearing science can better people’s lives has made her a strong fit to drive HHF forward.
HHF deeply values the work of all individuals who bring us closer to a world without hearing loss and tinnitus. For Women’s History Month, we’re honored to call special attention to the women who have been part of these life-changing efforts in the spirit of Mrs. Ramsey Baker, whose determination and selflessness still inspires us today.
