You Are a Masterpiece

By Sophia Boccard

The day before Fourth of July, 2012, the second diagnosis came in. “It’s true, you have Usher syndrome,” announced the retinal specialist. Baffled and unwilling to accept the corroboration of the first retinal specialist’s initial diagnosis, I immediately responded with, “I’d like another opinion—is there anyone you can recommend?” In between both appointments, I had learned that Usher syndrome is the most common genetic cause of combined deafness and blindness.

  Credit: Evan McGlinn

Credit: Evan McGlinn

The hearing loss diagnosis was not a new discovery. It had been detected when I was 4; my parents were told I lost my hearing as a reaction to an antibiotic given to me during an emergency surgery. Since then, having moderate to severe bilateral hearing loss meant wearing hearing aids, attending speech therapy classes, and walking around with an assistive FM listening system in school.

Growing up with a hearing loss gave me a new perspective. I had to learn to defend myself against bullies who would occasionally yank my hearing aids and throw them in the trash. And yet, here I was refusing to accept this “other” condition and learning that I had a combined hearing and vision loss diagnosis. After 26 years of thinking otherwise, it was certainly a rude awakening to learn that the hearing loss was not caused by the antibiotic.

Just a few short weeks before, I had been sitting in my optometrist’s office for a routine eye exam to refill my prescription for contact lenses. The optometrist noticed some inconsistencies during my examinations and asked me to stay for more thorough exams. A standard 20-minute visit turned into a two-hour ordeal. After reviewing the results she somberly referred me to the retinal eye specialist who gave me the initial diagnosis.

This retinal eye specialist—who was the first person to utter, “You have Usher syndrome” to me—had the worst bedside manner. I refused to just take his word for it. Immediately after I left his office I cried—a lot—but then regained my composure and made a few calls to see a second retinal eye specialist doctor for a second opinion.

And so on that day in July, even after the second retinal specialist reiterated the first specialist’s diagnosis, I insisted on seeing a third specialist who would prove them both wrong.

I went to the National Eye Institute (NEI), part of the National Institutes of Health, in Bethesda, Maryland, for the third opinion. It was there at the NEI when everything came to a screeching halt and the final diagnosis was confirmed with a genetic test.

The test showed I was born with the mutated gene USH2A, a double recessive genetic disorder that requires both parents to have the exact same copy of the gene. Usher syndrome has three types—types 1, 2, and 3—each with many subtypes such as Usher 2A, Usher 3A, Usher 1F, etc. Combined there are approximately 400,000 people worldwide who have been diagnosed with Usher syndrome. But even with 400,000 people around the world with Usher, there still isn’t enough information easily accessible for individuals who receive this diagnosis.

  Sophia and her family.

Sophia and her family.

That day at the NEI launched the next phase of my life. In the biography of my life, the following four years, 2012 to 2016, could be titled “My Wasted Years.” This is when I became a recluse, felt sorry for myself, and cried. Every. Single. Day. I had no way of expressing my sadness or even articulating my feelings, as I didn’t even know what it was I was feeling and how to cope with it.

Those were the years I felt as if I went blind overnight (and let me be clear, I did not go blind overnight—nor will I go blind overnight). I felt like there was this black cloud following me everywhere, hovering over my head and bringing constant rain, making me feel like a drowned rat.

Depression became my reality and my identity. I told a few people about the diagnosis but couldn’t elaborate on the condition since I felt like my world was ending. My desire to live was negligible. How could I imagine life with no sight? What would the quality of my life be like? What would I do without my independence?

These were all questions that ran through my head as the life I imagined living slowly started disappearing from my mind into total darkness. For four long years I asked myself repeatedly, What is the point? What do I have to look forward to?

Then it hit me. Literally. I was walking through Times Square during rush hour, a scenario that can overwhelm most people, when I slammed into someone who screamed into my face, “Watch where you’re going, a**hole!” It was in that instant that an internal shift took over and I shouted back, “Get out of MY way, a**hole!”

It was then I accepted I needed to stop looking at myself as a victim of a mutated gene and to start owning it.

I took a step back and recognized that all this time I had erroneously pitied myself. I felt sorry for my own future and what I was going to lose, and I forgot what it meant to just live life. In preparing for a future with vision loss, I was preparing for an apocalypse that would never come—unless I let it. I needed to stop judging myself and learn to respect myself all over again.

Thus 2016 marked the year of learning to be in control of my own reality, a brand new chapter in this biography of my life. I reached out to the NEI to connect me with someone with Usher syndrome who was willing to exchange emails and stories. Soon after, the NEI introduced me to another patient with USH2A who, after initially being pen (well, email) pals, we became “Ushties” (Usher + besties). Later that year I went to an Usher Syndrome Conference, held by the Usher Syndrome Coalition, in Seattle, and continued meeting many incredible, inspirational people who today are still some of my closest friends.

With these newfound connections I began advocating for both hearing loss and vision impairment communities. Through a friend, I was introduced to Hearing Health Foundation (HHF) and joined their Young Professionals Board for two years before being invited to sit on HHF’s Board of Directors. I also currently sit on the board of the Usher Syndrome Society and am involved with Young Professionals Groups at both the Foundation Fighting Blindness and the Greater New York Chapter of the ALS Association, fighting the progressive neurodegenerative condition known as Lou Gehrig’s disease.

My fiancé and I have also decided to learn American Sign Language as a tool to communicate with new friends from the Usher community. Fundraising and awareness events have started to fill up my calendar. I look at each event as an opportunity to educate those who are unfamiliar with the importance of hearing and vision health and what it means to lose your hearing or your vision, either in part or completely.

For me, it’s the lack of awareness about how the diagnosis of either hearing or vision loss can have an effect on the individual’s own mental health as well as that of their loved ones. There is not enough support for the recently diagnosed. The public isn’t familiar with how to accommodate someone with hearing or vision loss, and since both conditions are not entirely transparent—it’s difficult for people to recognize that they are communicating with an individual with hearing or vision loss —it makes it that much harder to help.

I’m most proud to have the opportunity to teach willing learners that being deaf or blind is not something to pity but rather something to respect. I strive to demonstrate to others that even with our limitations we can still do everything we want, even if we need a little extra help getting there.

Not too long ago someone said to my fiancé, “Sophia is so lucky to have you. You are an incredible person for staying with her even through her diagnosis.” Wait a second, what? My first thought was that my fiancé is the lucky one! To be fair, neither one of us is any more “lucky” than the other. We both equally  deserve each other—and despite comments like these, mostly well-intended (or not), no one will succeed at making me feel less of a person.

While we can each feel insignificant about our own lives when there are so many success stories of extraordinary people pulling off impossible accomplishments despite their limitations, let’s remember that we are each the star of our own story. We are the masterpieces of our own reality.

My story doesn't have a neat ending or a twist, reassuring the reader with a fairy tale conclusion. I’m just here to remind you, my new friend, that I’m not broken. I’m not half of a person; I’m not someone to “fix.” Usher syndrome is a part of who I am, but it is not my identity. I am a human being, who like any and everyone else, is whole and perfect just as I am.

If you have a health condition, don’t let it consume you. Own it. You are the badass who can survive against all odds. People are lucky to have you in their lives. Remind yourself to feel appreciated, get out there, and please, embrace your newfound celebrity status!

HHF board member Sophia Boccard works in digital marketing and lives in New York City. She wrote about an Usher Syndrome Society event she helped organize. This article original appeared in the Spring 2018 issue of Hearing Health magazine. For references, see hhf.org/spring2018-references.

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Stenting to Relieve One Specific Cause of Pulsatile Tinnitus

By Jayne Wallace for the Weill Cornell Medicine Brain and Spine Center

The Centers for Disease Control and Prevention estimates that 15 percent of the U.S. population, or 48 million people, have some type of tinnitus, hearing a ringing or buzzing in the absence of an external sound source.

Pulsatile tinnitus, in contrast, usually has a sound source. In these cases, affecting fewer than 10 percent of tinnitus patients, sounds are caused by turbulence in the blood flow around the ear. And among these cases, intracranial hypertension comprises about 8 percent of cases. This is when narrowing in one of the large veins in the brain causes a disturbance in the blood flow, leading to the pulsatile tinnitus.

  Dural arteriovenous fistula, MRA showed only subtle alterations as a result of atypical flows in the right transverse sinus (arrow). Photo courtesy of Deutsches Ärzteblatt International.

Dural arteriovenous fistula, MRA showed only subtle alterations as a result of atypical flows in the right transverse sinus (arrow). Photo courtesy of Deutsches Ärzteblatt International.

“Traditionally there has been no good treatment for many of these patients who are told to learn to live with it,” says Athos Patsalides, M.D., an interventional neuroradiologist at New York City’s Weill Cornell Medicine Brain and Spine Center, where he also serves as an associate professor of radiology in neurological surgery.

Till now, available treatments—medication or more complicated surgery—were either ineffective or produced side effects and other problems just as bad or worse. “That’s why we started the clinical trials for venous sinus stenting, a minimally invasive procedure that is very effective in alleviating the narrowing in the vein,” says Patsalides, who pioneered the use of VSS to treat patients with idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri because the symptoms tend to mirror those of a brain tumor.

“Many IIH patients suffer from vision loss, headaches, and pulsatile tinnitus, and I saw a pattern with patients experiencing resolution of the pulsatile tinnitus immediately after VSS,” Patsalides says.

This led to the possibility of using VSS for selected patients with pulsatile tinnitus. After the Food and Drug Administration approved the clinical trial, it began in May 2016 and has an estimated completion date of January 2021.

“In the stenting procedure, with the patient under general anesthesia, we insert a tiny, soft catheter into a vein located in the upper part of the leg and thread it up to the affected vein in the brain,” Patsalides says.

A self-expanding stent is deployed into the narrowed segment of the vein, relieving the stenosis, restoring normal blood flow, and reducing or eliminating the pulsatile tinnitus. “Happily, the patient is typically discharged from the hospital within 24 to 48 hours,” he says.

To learn more, see weillcornellbrainandspine.org. Hearing Health Foundation notes that the trial is ongoing, and that the procedure is potentially able to address only one specific cause of pulsatile tinnitus and should not be taken as a solution for other forms of tinnitus, which often has no known cause.

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Intern Awareness: Reflections from HHF

By Vicky Chan, Lauren McGrath, and C. Adrean Mejia

This spring, Hearing Health Foundation (HHF) has been fortunate to welcome two outstanding, dedicated young professionals to its office: Vicky Chan, Copywriting Intern and C. Adrean Mejia, Social Media and Digital Communications Intern. In honor of Intern Awareness Month, celebrated each April, Vicky and Adrean reflected on their experiences as members of the small yet mighty HHF team under the supervision of Lauren McGrath, Marketing Manager.

Vicky, a recent graduate from Brooklyn College with a Bachelor’s Degree in English, believes that interning at HHF has equipped her with invaluable, real-world experience to grow as both a professional and an individual. So far, Vicky has utilized her skills by editing, researching, and conducting interviews for HHF’s blog and magazine. A former writing tutor, Vicky acknowledges that writing for an official publication like Hearing Health magazine is markedly different than completing a college professor’s assignment. Unlike academic essays, Vicky’s articles at HHF must fit the voice of an established organization. And to effectively craft interview-style stories, Vicky has learned to put herself in the subject’s position to determine how they want their story to be portrayed.

  Adrean Mejia (left) interned with HHF as a Social Media and Digital Communications Intern this semester. Vicky Chan is a current Copywriting Intern.

Adrean Mejia (left) interned with HHF as a Social Media and Digital Communications Intern this semester. Vicky Chan is a current Copywriting Intern.

“Everyone at HHF has been open to my questions or suggestions to create an inviting learning environment,” Vicky notes. She’s pleased that HHF has given her purpose and the responsibility to produce written content about hearing health awareness. “Each time I complete an article or see my name on the byline, I feel a sense of satisfaction and pride because it shows that I am working towards my career goal—one step at a time.” Vicky plans to pursue a career in the book publishing field.

Adrean, who will soon complete his Bachelor’s Degree in Corporate Communications from Baruch College, recently ended his internship feeling inspired by HHF’s mission and enlightened by what he’s learned. Adrean, speaking honestly about the experience, admits, “At the beginning, adapting to the nonprofit sector and understanding the concepts related to hearing health were challenges for me.”

Fortunately, the role turned out to be enjoyable for Adrean thanks to the accommodating, communicative staff. He is grateful for the experience, which has opened up his eyes to new horizons and enabled him to contribute to an inspiring mission. Now ready to begin his postcollegiate career, Adrean’s long-term professional goal is to achieve a high position in the entertainment and beauty industry to showcase his vision as an artist.

HHF is grateful to Vicky, Adrean, and the dozens of other interns who have offered their time and talents to further hearing loss research and awareness. The foundation looks forward to meeting its Summer 2018 interns, who will pursue projects related to awareness, advocacy, video production, communications, digital media, fundraising, and donor management. To learn about Fall 2018 opportunities, contact us at careers@hhf.org or visit www.hhf.org/join-our-team in August.

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Making Entertainment Relatable and Accessible for More

By C. Adrean Mejia

Films, plays, and television series have long served as platforms to create awareness of important topics that have otherwise been kept out of the spotlight. Hearing loss is one example of such a topic.

theater.jpg

As an organization that seeks to inform the public about the prevalence, prevention, and treatment of hearing loss, Hearing Health Foundation (HHF) applauds the growing prioritization of this issue in entertainment. We are pleased to know that the number of films featuring characters with hearing loss—played by actors with hearing loss—has risen with the years, generating greater public awareness of the third most common health condition in the United States. Complementing this trend of an increased presence of hearing loss on screen is the introduction of recent legislation to make entertainment more accessible to viewers with hearing loss.

Actors and characters with hearing loss expand society’s understanding of the condition. Hearing loss empowers abilities, emotions, and experiences unlike those of people with typical hearing. Some recent works with characters with hearing loss include the following:

The Silent Child tells the story of a profoundly deaf four-year-old girl who is about to attend a mainstream school with minimal support—until a social worker teaches her American Sign Language (ASL). The film communicates the disappointing statistic that over 78% of deaf children attend mainstream school without accommodations. A final comment that states that the creators “hope this film contributes in the fight for sign language to be recognized in every school across the globe.”

Children of a Lesser God, a play written in 1979, made its Broadway debut last April. The piece focuses on the professional and romantic relationship between a deaf janitor and a typical hearing teacher and shows the contrasting worlds off sound and silence. To Sarah, the janitor, deafness is an identity, not a defect.  

This Close is a TV series by two deaf writers and actors that narrates the true story of their lives. The show provides a close look of the everyday day life of two best friends, emphasizing their challenges and frustrations while highlighting the positive and beautiful things that their hearing loss brings to their existence.

HHF commends these and the artists behind similar works for the awareness their creations have generated. Likewise, the organization is proud to witness the introduction of new laws and procedures to make entertainment more accessible to the hearing loss community.

  Credit: Naugatuck Patch

Credit: Naugatuck Patch

The American with Disabilities Act (ADA) first broke barriers by advocating for the requirement that all video program distributors close caption their TV shows. But it wasn’t until recently, with the help of technology, that these rules have expanded. On November 2016, the Final Rule on the ADA Title III was signed, requiring all American movie theaters to provide accessibility for captions. Large cinemas now offer assistive listening, closed captions, and descriptive audio.

Broadway, too, has made tremendous improvements. In 2016, the Theater Development Fund (TDF) and The Broadway League, launched www.theatreaccess.nyc, a website with information about tickets prices, dates and accommodations for theatergoers with disabilities. In addition, TDF now provides accessibility programs with open captioning and/or ASL at select Broadway performances.

Entertainment has made progress in becoming more inclusive for people with hearing loss since the implementation of these programs, but additional work is needed. Though mandating captioning at movie theaters represents great progress, other entertainment settings, including sports arenas and concert halls, must follow suit.

To optimize the listening experience for audience members with hearing loss, more must adopt the use of hearing loops, which transmit sound from a PA system to hearing aids and cochlear implants. In December 2017, the state of Minnesota passed a bill requiring hearing loops in public meeting spaces, taking after similar New York City legislation from earlier in 2017.

HHF looks forward to a day where no one must live with hearing loss. As long as hearing remains out of reach for tens of million Americans, fair accommodations are the most ethical choice.

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Hearing Loss Lives with Me

By Sonya Daniel

Sonya Faces of HL.jpeg

I was born with bilateral sensorineural hearing loss. I didn’t know the official term for it until 2008. When I was a kid in elementary school I passed every hearing test that the mothers in the PTA administered. I was a pretty clever little girl. I learned that every test has a visible “tell” and knew how to guess “right” on all of them. I never wanted to fail any test. I learned to read lips, and assumed everyone heard that annoying ringing constantly. That, of course isn’t true.

The tinnitus became too overwhelming to deal with everyday. I hadn’t had my ears tested since I was little, so I didn’t know what to expect. It was much worse than I had ever imagined it would be. And now it had a name. I left the audiologist knowing at some point I’d be completely deaf. But, no one knows when that might be. I was a mother to three young boys. I wondered how much longer I’d hear, “Mommy, I love you.” Or If they’d hold out long enough to hear their grown-up man voices. How much longer until I couldn’t hear music?

Music is my passion. In fact, it’s my chosen profession. I never remember wanting to do anything but be a musician in some capacity. My dad played the guitar. My mother said when I was little I would sit in front of him and touch his guitar and I would stand in front of the stereo and touch the speakers. I suppose I was trying to “hear” the music. I knew I’d go to college and major in music as a vocalist. I knew I wanted to share my love for music and teach others.

College was a very difficult and stressful time. There was a course called “Sight Singing and Ear Training” required to complete my Bachelor’s in Music. I mean, come on! Ear training? I struggled. Professors struggled to teach me. Some never gave up because it was apparent I wasn’t going anywhere.

I did get to teach music to every level. I can’t do that anymore, but I still do music everyday. Sometimes in life you have to know that there are things that your body just won’t let you do. I’d like to be a 6’0” tall, blonde supermodel, too. My body said “no” to that and I think I’m ok.      
Living with tinnitus and hearing loss can be overwhelming and difficult. I’m not as afraid of living this way as I used to be. Everyone has a thing. This is just mine. I like to say I don’t live with hearing loss; it lives with me.

My journey has brought me to the cochlear implant. I’m a candidate in the preliminary stages of that process. Technology changes so fast it’s hard to keep up. My current devices have stronger receiver tubes and ear molds.

That’s just my journey with my ears. My life isn’t defined by or consumed with my ears, although it’s felt that way at times. I’m constantly learning and growing. I’m getting stronger with each high and low I face. But, isn’t that just life?

Sonya Daniel is a musician/teacher, writer, and voiceover artist. She is a participant in HHF’s “Faces of Hearing Loss” campaign.

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Clear Speech: It’s Not Just About Conversation

By Kathi Mestayer

In the Spring 2018 issue of Hearing Health, we talk about ways to help our conversational partners speak more clearly, so we can understand them better.

But what about public broadcast speech? It comes to us via phone, radio, television, and computer screen, as well as those echo-filled train stations, bus terminals, and airports. There’s room for improvement everywhere.

  This digital oscilloscope representation of speech, with pauses, shows that gaps as short as a few milliseconds are used to separate words and syllables. According to Frank Musiek, Ph.D., CCC-A, a professor of speech, language and hearing sciences at the University of Arizona, people with some kinds of hearing difficulties require longer than normal gap intervals in order to perceive them.    Credit: Frank Musiek

This digital oscilloscope representation of speech, with pauses, shows that gaps as short as a few milliseconds are used to separate words and syllables. According to Frank Musiek, Ph.D., CCC-A, a professor of speech, language and hearing sciences at the University of Arizona, people with some kinds of hearing difficulties require longer than normal gap intervals in order to perceive them.
Credit: Frank Musiek

In some cases, like Amtrak’s 30th Street Station in Philadelphia [LISTEN], clear speech is a real challenge. The beautiful space has towering cathedral ceilings, and is wildly reverberant, like a huge echo chamber. Even typical-hearing people can’t understand a word that comes over the PA system. Trust me; I’ve asked several times.

In that space, a large visual display in the center of the hall and the lines of people moving toward the boarding areas get the message across: It’s time to get on the train. I wonder why they even bother with the announcements, except that they signal that something is going on, so people will check the display.

Radio is very different, at least in my kitchen. There are no echoes, so I can enjoy listening to talk radio while I make my coffee in the morning. The other day, the broadcast about one of the station’s nonprofit supporters was described as: “…supporting creative people and defective institutions…”

Huh? That couldn’t be right. It took a few seconds for me to realize what had actually been said: “supporting creative people and effective institutions.” Inter-word pauses are one of the key characteristics of clear speech. A slightly longer pause between the words “and” and “effective” would, in this case, have done the trick.

In the meantime, I chuckle every time that segment airs (which is often), and wonder if anyone else thinks about the defective institutions!

Staff writer Kathi Mestayer serves on advisory boards for the Virginia Department for the Deaf and Hard of Hearing and the Greater Richmond, Virginia, chapter of the Hearing Loss Association of America.

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Novel Drug-Delivery Method to the Inner Ear

By Gary Polakovic, USC News

Researchers have developed a new approach to be able to repair cells deep inside the ear. The study, conducted by scientists at University of Southern California (USC) and Harvard University, demonstrates a novel way for a future drug to zero in on damaged nerves and cells inside the ear.

  Credit: Matthew Pla Savino/USC News

Credit: Matthew Pla Savino/USC News

“What’s new here is we figured out how to deliver a drug into the inner ear so it actually stays put and does what it’s supposed to do, and that’s novel,” says Charles E. McKenna, Ph.D., a corresponding author for the study and chemistry professor at the USC Dornsife College of Letters, Arts, and Sciences.

“Inside this part of the ear, there’s fluid constantly flowing that would sweep dissolved drugs away, but our new approach addresses that problem. This is a first for hearing loss and the ear,” McKenna adds. “It’s also important because it may be adaptable for other drugs that need to be applied within the inner ear.”

The paper was published April 4 in the journal Bioconjugate Chemistry. The authors include lead researcher Judith S. Kempfle, Ph.D., a 2011 and 2012 Emerging Research Grants scientist, as well as Hearing Restoration Project member Albert Edge, Ph.D., both at Harvard Medical School and The Eaton-Peabody Laboratories in Boston.

There are caveats. The research was conducted on animal tissues in a petri dish. It has not yet been tested in living animals or humans. Yet the researchers are hopeful given the similarities of cells and mechanisms involved. McKenna says since the technique works in the laboratory, the findings provide “strong preliminary evidence” it could work in living creatures. They are already planning the next phase involving animals and hearing loss.

The study breaks new ground because researchers developed a novel drug-delivery method. Specifically, it targets the cochlea, a snail-like structure in the inner ear where sensitive cells convey sound to the brain. Hearing loss occurs due to aging or exposure to noise at unsafe levels. Over time, hair-like sensory cells and bundles of neurons that transmit their vibrations break down, as do ribbon-like synapses, which connect the cells.

The researchers designed a molecule combining 7,8-dihydroxyflavone, which mimics a protein critical for development and function of the nervous system, and bisphosphonate, a type of drug that sticks to bones. This pairing delivered the breakthrough solution, the researchers say, as neurons responded to the molecule and regenerated synapses in mouse ear tissue. This led to the repair of the hair cells and neurons, which are essential to hearing.

“We’re not saying it’s a cure for hearing loss,” McKenna says. “It’s a proof of principle for a new approach that’s extremely promising. It’s an important step that offers a lot of hope.” Hearing loss affects two thirds of people over 70 years and 17 percent of all adults in the United States, and it is expected to nearly double in 40 years.

This is adapted from "Hearing Loss Study at USC, Harvard Shows Hope for Millions" on the USC News website. The authors of the April 4, 2018, Bioconjugate Chemistry study, “Bisphosphonate-Linked TrkB Agonist: Cochlea-Targeted Delivery of a Neurotrophic Agent as a Strategy for the Treatment of Hearing Loss,” include lead researcher Judith S. Kempfle, as well as Christine Hamadani, Nicholas Koen, Albert S. Edge, and David H. Jung of Harvard Medical School and The Eaton-Peabody Laboratories/Massachusetts Eye and Ear in Boston. Kempfle is also affiliated with the University of Tübingen Medical Center. Corresponding author Charles E. McKenna, as well as Kim Nguyen and Boris A. Kashemirov, are at USC Dornsife.

The research was supported by the American Academy of Otolaryngology–Head and Neck Surgery Herbert Silverstein Otology and Neurotology Research Award, an American Otological Society Research Grant, and a grant from the National Institute of Deafness and other Communicative Disorders (R01 DC007174).

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Cochlear to Support Hearing Research By Reaching One Million Ears

Cochlear Million Ear Logo.jpg

Today marks the start of Better Hearing & Speech Month (BHSM), a campaign to advance public knowledge of communication disorders. To celebrate, international hearing implant manufacturer Cochlear is launching the #MillionEar Challenge with the goal of informing one million people about the importance of hearing health and research.

Proceeds from the campaign will benefit Hearing Health Foundation (HHF)’s longstanding Emerging Research Grants (ERG) program. Cochlear has pledged to donate to ERG when the #MillionEar Challenge is met.

“Awareness is at the heart of Hearing Health Foundation's efforts to prevent, treat, and cure hearing loss," said Nadine Dehgan, HHF’s Chief Executive Officer. "I am deeply grateful Cochlear is committed to raising awareness of hearing loss, which will inspire more to pursue hearing tests and life-changing treatments."

  HHF staff thanks Cochlear in their own #MillionEar Challenge shirts.

HHF staff thanks Cochlear in their own #MillionEar Challenge shirts.

Cochlear’s generous gift will allow HHF to continue funding up-and-coming scientists who investigate various hearing and balance conditions. Such funding has historically led to the development of many new treatments including cochlear implants which, today, benefit more than 300,000 people worldwide.

You can support the #MillionEar campaign with the purchase of a t-shirt, available in child and adult sizes. Read the full press from Cochlear release here.

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Is It Overstimulation?

By Eric Sherman

My younger son Cole has been wearing cochlear implants (CI) since 2005. He was barely a toddler, between 18 and 24 months old, when he rejected them.

The initial response from our audiologist was, “We just mapped your son, just do your best to keep the processor on his head.” Unique to every CI wearer, mapping adjusts the sound input to the electrodes on the array implanted into the cochlea. It is meant to optimize the CI user’s access to sound.

But after several weeks, and our audio-verbal therapist told us there was something wrong and referred us to another pediatric audiologist, Joan Hewitt, Au.D.

  Eric Sherman and his son, Cole

Eric Sherman and his son, Cole

We learned that refusing to wear CI processors is generally a symptom of a problem that a child can’t necessarily express. Their behavior becomes the only way to communicate the issue.

“Our brains crave hearing,” Hewitt says. “Children should want to have their CIs on all the time. If a child resists putting the CIs on in the morning, cries or winces when they are put on, or fails to replace the headpiece when it falls off, there is a strong possibility that the CIs are providing too much stimulation. Some children appear shy or withdrawn because the stimulation is so great that interacting is painful. Others respond to overstimulation by being loud and aggressive.” 

Hewitt says research discussed at the Cochlear Implant Symposium in Chicago in 2011 (or CI2011, run by the then-newly created American Cochlear Implant Alliance) addressed the issue of overstimulation. A study that was presented, titled "Overstimulation in Children with Cochlear Implants," listed symptoms that indicated children were overstimulated by their cochlear implants: reluctance or refusal to wear the device, overly loud voices, poor articulation, short attention span or agitated behavior, and no improvement in symptoms despite appropriate therapy.  

When the researchers reduced the stimulation levels, they found very rapid improvement in voice quality and vocal loudness and gradual improvement in articulation. Finally, they found “surprising effects on the children's behavior”—the parents reported a marked improvement in attention and reduction in agitation.

In “Cochlear Implants—Considerations in Programming for the Pediatric Population,” in AudiologyOnline, Jennifer Mertes, Au.D., CCC-A, and Jill Chinnici, CCC-A, write: “Children are not little adults. They are indeed, unique, and to address their CI needs, they require an experienced clinician. Most children are unable to provide accurate feedback while the audiologist programs their cochlear implant and therefore, the clinician must take many things into account.”

These include:

  1. The audiologists' past experiences with other patients

  2. Updated information regarding the child's progress (from parents, therapists, and teachers 

  3. Audiometric test measures

  4. Observations of the child during programming

  5. Objective measurements

  6. If age appropriate, the clinician will train the child to participate in programming

Many of the decisions made during programming appointments come from the clinician's knowledge and experience, rather than the child's behavioral responses. But your child’s reactions should also be taken into account.

If your child continues to refuse to wear their processors after a remapping, take into consideration your audiologist’s experience and mapping approach and seek a second opinion. When we met with Hewitt, she found our child’s map was overstimulating. Once she remapped using a different approach, our son had no problem wearing his CI processor again.

Los Angeles marketing executive Eric Sherman is the founder of Ci Wear, a patented shirt designed to secure and protect cochlear implant processors. April is National Autism Awareness Month. Read about how Sherman and Cole manage Cole’s hearing loss and autism spectrum disorder conditions in  “When It’s Not Just Hearing Loss” in the Fall 2016 issue of Hearing Health.

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Let’s Make Noise Safer

By Vicky Chan

April 25 is International Noise Awareness Day, an annual, vital reminder to take a stand against noise exposure and to spread awareness about the underestimated threat of noise-induced hearing loss (NIHL). Seemingly harmless rhythms, roars, and blasts heard daily from music, trains, and machinery are, in fact, among the top offenders of NIHL.

Noise-induced hearing loss (NIHL) progressively occurs after chronic exposure to loud sounds. The frequency and intensity of the sound level, measured in decibels (dB), increases the risk of NIHL. Gradual hearing loss can result from prolonged contact with noise levels of 85 dB or greater, such as heavy city traffic. Noises of 110 dB or more, like construction (110 dB), an ambulance (120dB), or the pop of firecrackers (140-165 dB) can damage one’s hearing in a minute’s time.

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NIHL is the only type of hearing loss that is completely preventable, yet billions of individuals endanger themselves daily. Over 1.1 billion young adults ages 12 to 35—an age group that frequently uses headphones to listen to music—are at risk. Already, an estimated 12.5% of young people ages of 6 to 19 have hearing loss as a result of using earbuds or headphones at a high volume. A device playing at maximum volume (105 dB) is dangerous, so exposure to sounds at 100 dB for more than 15 minutes is highly discouraged.

Most major cities around the world have transit systems that put commuters in contact with sounds at 110 dB. BBC News found that London’s transit systems can get as loud as 110 dB, which is louder than a nearby helicopter taking off. The sound levels of some stations exceed the threshold for which occupational hearing protection is legally required. New York City has one of the largest and oldest subway systems in the world where 91% of commuters exceed the recommended levels of noise exposure annually. In a study on Toronto’s subway system, 20% of intermittent bursts of impulse noises were greater than 114 dB.

People who work in certain fields are more vulnerable to NIHL than others. Professional musicians, for instance, are almost four times as likely to develop NIHL than the general public. Military personnel, who are in extremely close proximity to gunfire and blasts, are more likely to return home from combat with hearing loss and/or tinnitus than any other type of injury. And airport ground staff are surrounded by high-frequency aircraft noises at 140 dB. In all of these professions, the hazard of NIHL can be significantly mitigated with hearing protection.

NIHL is permanent. Increased exposure to excess noise destroys the sensory cells in the inner ears (hair cells), which decreases hearing capacity and leads to hearing loss. Once damaged, the sensory cells cannot be restored. To find a solution, Hearing Health Foundation’s (HHF) Hearing Restoration Project (HRP) conducts groundbreaking research on inner ear hair cell regeneration in hopes of discovering a life-changing cure.

Nearly three-quarters of those who are exposed to loud noises rarely or never use hearing protection. It is our dream that someday, NIHL will be reversible as a result of the HRP. Until then, to make noise safer, HHF advises protection by remembering to Block, Walk, and Turn. Block out noises by wearing earplugs or protective earmuffs. Walk away or limit exposure to high-levels of noises. Turn down the volume of electronic devices.

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