The Power of a Mother’s Advocacy

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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RNA Injection Restores Hearing in Guinea Pigs

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. 

Moving Toward a Future Free of Drug-Induced Hearing Loss

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

Highlights from Our 2017 Annual Report

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.

Women’s History Through the Lens of HHF

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.

The Best Lesson Yet

By Layla Rudy

I was diagnosed with bilateral sensorineural hearing loss at age 3, after a series of unsuccessful doctor visits. Two years later, I was going to the audiologist to get my hearing aids for the first time. I remember being frightened by a picture in the waiting room of the shiny tube that comes out of the ear molds attached to hearing aids. My little brother was crying in the stroller as we left the doctor’s office for brunch.

Layla, age 5, with one of her brothers, James

I went to speech therapy for the next four or five years; during school, along with two other kids, I was pulled out of class a few times a week. I remember thinking that I didn’t belong there, that I was “normal,” and that everything was fine with me. But the teachers and administration didn’t know what to do with me—they thought something was wrong with me, and they just threw me in the same boat as the kids with speech problems or ADHD.

I was the only kid in my elementary school with hearing loss. While it was great that my mom wanted to mainstream me, looking back, it was clear the school didn’t know how to handle a student like me.

The problem was ignorance more than a lack of resources—although that was an issue, as well. My teachers plopped me in a desk—not always in the front of the room—and, not understanding how important the FM system was for me to hear in their classrooms, didn’t wear it. I thought the teachers didn’t care about me, but it turns out they just never learned how to deal with students with hearing loss. I was too embarrassed to say anything, so the FM systems often went completely unused. For the same reasons, I was never assigned a note-taker. I was the outlier in a room full of typical children.

Identical issues continued in middle school and high school. I developed anxiety from a young age and, by adolescence, the effects were more severe. In retrospect, a lot of my anxiety stemmed from my worries that my peers were talking about me, or that I was not fully aware of my surroundings. To put it simply, I was anxious because I couldn’t hear.

My mom had always told me that college would be easier—I could get a note-taker and finally have the resources I never had. When I applied to Brooklyn College, I found the Center for Student Disabilities Services on their website. I was thrilled to find that they had a section devoted to hearing loss and deafness. Better yet, professors were given a guide to understand how each disability was to be handled and treated.

So, when I arrived at their offices, I filled out forms and submitted my hearing test results. They had told me to come back in a few days, at which point I would have everything I had wished for in elementary school.

Ironically, I never bothered to go back to their offices, and I never bothered with their accommodations.

I have spent my whole life going home and teaching myself what the teacher had taught us in class (that I could not hear)—taking my own meticulous notes that my classmates begged to borrow. I have always wanted a note-taker, an FM system that gets consistently used, and empathetic teachers. Now that I finally have access to all of this, I don’t need it.

I realize I’ve subconsciously trained myself not to rely on anyone else; it’s not that I don’t want to ask for help, or that I’m too embarrassed. I know that if I need it, I can ask, unlike when I was in elementary school. But my hearing loss, my disability, has enabled me to rely on myself, to be aware of my surroundings, to better communicate with others.

And to me, self-reliance is the best lesson yet.

New Jersey native Layla Rudy is a freshman at Brooklyn College planning on studying biology to eventually conduct auditory research. She is also a participant in HHF’s “Faces of Hearing Loss” campaign.

Receive updates on life-changing hearing research and resources by subscribing to HHF's free quarterly magazine and e-newsletter.

Moving Toward a Future Free of Drug-Induced Hearing Loss

By Erik Robinson

A new 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.

HHF Council of Scientific Trustees member Peter Steyger, Ph.D., and colleagues produced a special Frontiers in Cellular Neuroscience publication on the topic of ototoxicity.

The compilation was published online as a special research topic by the journal Frontiers in Cellular Neuroscience on March 5, 2018. It includes both original research and focused reviews. The Pharmaceutical Interventions for Hearing Loss Working Group organized the effort at the behest of the Department of Defense (DoD) Hearing Center of Excellence.

“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 coauthor Peter Steyger, Ph.D., a professor of otolaryngology/head and neck surgery in the Oregon Health & Science University (OHSU) School of Medicine, and a member of Hearing Health Foundation’s Council of Scientific Trustees.

“Ototoxicity is a threat to hearing at any age and hearing loss remains a significant side effect of chemotherapy. This review highlights how far we’ve come in understanding that threat and provides us with a roadmap for developing more effective ways to recognize and address the problem,” adds coauthor Jian Zuo, Ph.D., of the department of developmental neurobiology at St. Jude Children’s Research Hospital in Memphis, Tennessee.

In people, hearing cells don’t regenerate so the loss is irreversible. That’s why it is crucial to understand the mechanisms that affect hearing and how to prevent loss of hearing, Steyger says. The introductory editorial, “Moving Toward a Future Free of Ototoxicity,” highlights the latest scientific research exploring how certain pharmaceuticals damage the inner ear while others can protect it. It also highlights the need for better monitoring and detection of hearing loss over time, especially among patients being treated with antibiotics.

Steyger, who lost hearing as a child after being treated with antibiotics for meningitis when 14 months old, noted that hearing loss affects a surprisingly large proportion of the population—rising from an estimated 1 in 500 newborns to as many as half of all people age 75 or older. The research encapsulated in the new e-book includes 23 scientific articles from 93 authors and represents the state of the science in both prevention and treatment of ototoxicity hearing loss. (The e-book is available to all, free of charge.)

“This compilation will help to propel our knowledge forward and underscore the need to better understand the dangers of ototoxicity. The DoD Hearing Center of Excellence is honored to host and mobilize this important effort,” says coauthor Carlos Esquivel, M.D., a neurotologist and chief medical officer in the Clinical Care, Rehabilitation, and Restoration Branch of the DoD Hearing Center of Excellence at Joint Base San Antonio in Texas.

In addition to Steyger, Zuo, and Esquivel, the publication’s editors include Lisa Cunningham, Ph.D., of the National Institute on Deafness and Other Communication Disorders, and Kelly Watts, Au.D., of the DoD Hearing Center of Excellence.

This originally appeared in OHSU News and was republished with permission. 

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.

Ugly Emotions

By Lisa Peten

Some time ago, I was having a text conversation with a friend who also has bilateral sensorineural hearing loss. When I relayed to her that I am thankful in many ways for the changes that have occurred in having this condition, she was outraged that I would say such a thing. This prompted her to rant 1,001 ways in which her hearing loss is essentially ruining her life.

Despite being 20 years my senior, we share similar emotions in dealing with our late-deafened and hard-of-hearing experiences. Since we are usually so very aligned in our thoughts and emotions, I imagine my appreciation for this condition caused her outrage and to be even more perplexed about my gratitude for this condition. In any event, we hashed through this discussion and came to an “agree to disagree” conclusion which posed a crossroads in our friendship. We closed this discussion with authentic, raw emotion, conjuring up dispassionate experiences which urged persuasion to change the other’s mind. In the end, we were both thankful for the honest sharing and I learned a thing or two more about compassion and empathy.

I learned that “ugly” emotions (i.e. rage, envy, and hate) about being deaf/hard-of-hearing have not been given the time, space, or energy to address them. They are sometimes buried falsely with the perception that these types of emotions are unacceptable. Emotions like helplessness, frustration, and sadness seem to easily find ready support, but the ugly emotions present an elephant in the room never to be acknowledged.

After years of being unsuccessful in burying “ugly” emotions, I found three techniques to love yourself through these moments:

Share Immediately

For a long time I told no one except my family about my hearing challenges. It was too vulnerable an act for me to bare. Nowadays, sharing this detail immediately has had a positive effect on my experiences at restaurants, retail establishments, and even medical visits beyond the ENT and audiologists. Lessons learned along the way has taught me to release the emotions quickly, to avoid an emotional explosion later.

Dispense Energy

When emotions of any kind, especially the ugly, surface, dispensing activity provides another early release. In the past, I would practically run from humiliating situations when hearing incorrectly. Now, I can easily own up to the error and explain my hearing loss. While the embarrassment is still present, it does release my ugly emotions and allow the moment to pass without festering.

Forgive with Compassion

I continue to struggle with others’ reactions to my hearing loss. Eleven years after my diagnosis, I still feel sullen when I communicate with people who I have known before my hearing loss. Often times, after they learn of my hearing loss, they speak louder and unconsciously use body language to essentially ignore me, especially during group conversations. I know this is ignorance or discomfort on their part, but it still hurts. I have learned to fire away compassion for all of us in order to ease these emotionally ugly moments.

I hope the point of contention of my hearing loss someday subsides. However, in the meantime, I will own all emotions associated with my hearing loss, and will remember to extend greater compassion and empathy to those of us who need it when ugly-filled emotions become present.

Lisa Peten is a self-help hearing coach, mind-body empowerment speaker, and health advocate blogger, on a mission to empower health-hungry communities to become more proactive in obtaining nourishing foods and seeking holistic health alternatives. She is the founder of Sound Health and Hearing, a health coaching boutique consultancy. She is also a participant in HHF's Faces of Hearing Loss campaign.

Receive updates on life-changing hearing research and resources by subscribing to HHF's free quarterly magazine and e-newsletter.