Noise Annoys

By Kathi Mestayer

There I was, pumping gas into my car outside a convenience store, lost in thought. Then, the harsh yammering started. “Come inside for pizza and chicken wings! You can pick up candy and evil-carb junk food! Diet soda for a caffeine fix! Whaddya waiting for?”

The moot mute button

It was hard to pinpoint the noise at first, since I have an asymmetrical hearing loss. But I quickly located the source… a speaker on the gas pump. It had a mutilated “MUTE” button on the right side, which looked like it had been pressed about a hundred thousand times. So I optimistically joined the ghosts of muters past, pressing hard, harder, again, one more time, trying to make it shut up. No dice.

My peace of mind had been hijacked! If I’d had the option of swiping my card again to make it stop, I would have!

I glanced around, stressed, helpless, and furious. I got into my car, opened the glove box hoping for an ice pick to drive into the heart of the speaker, and saw a tube of sunscreen. As I headed back to the pump, my brain worked out a plan:

“Squirt the sunscreen into the speaker!”

“But they can see you from inside the convenience store! You’ll get arrested!”

“Actually, that could be an interesting adventure. Imagine the headlines!

‘Woman arrested for trying to get silence a gas-station speaker.’”

I could blog about it!

So, I took that tube of sunscreen, aimed it into the middle of the speaker, and squeezed until it was empty. Haha! The speaker was still shouting at me, but I could picture the sunscreen working its way into the speaker, choking it into silence.

Everybody Hates Noise!

When I emailed Seth Horowitz, author of “The Universal Sense: How Hearing Shapes the Mind,” about my ice pick idea, he replied that it would have been a waste of a perfectly good ice pick.

I had to agree. In his book, Horowitz writes, “The targeted use [of noise], from louder ads to deafening store music, is an important and often misused sales and marketing tool.” It can backfire, creating strong negative impressions of the business, product, or space. In fact, the only time I ever visited that gas station again was to take the photo for this article. And I didn’t buy any gas.

And sudden, loud, unexpected noises are likely to cause a hardwired stress response in our brains. In a recent essay in The New York Times, “The Cost of Paying Attention,” Matthew B. Crawford asks himself, from the noisy chaos of an airport, “Why am I so angry?”

Volume Optional

Noise doesn’t have to be loud to be disconcerting. Last year, in my art class, two fellow students were chatting quietly at the next easel. I couldn’t make out what they were saying, but they were just close enough, and just audible enough, that my brain would not quit trying. My brain, which often gives me feedback in this kind of situation, said, “You sure you want me to give up? It might be something interesting! I know I’m going to get it in the next couple of words!”

I was just starting to feel the frustration of trying, and failing, to understand the conversation, as I turned off my hearing aids. My own personal “mute” button.

There’s More of Us Out There

Lots of smart people are recognizing public-space noise, and attention piracy, as a real problem. In “Ambient Commons: Attention in the Age of Embodied Information,” author Malcolm McCullough writes, “Silence remains necessary for individual and especially cultural sanity. This is why modern cities enforce noise ordinances. You have a right to free speech, but not to amplify it from the rooftops all night, as if the air were an inconsequential void.”

And in his book, “The Unwanted Sound of Everything We Want,” author Garret Keizer chimes in: “In the end, after all the physicists, musicologists, and social theorists have had their say, there are only two kinds of human noise in the world: the noise that says, ‘The world is mine,’ and the noise that says, ‘It’s my world too.’ We need to quiet the first and make more of the second.”

And those who don’t take note of those wise words could end up with sunscreen in their speakers.

This is adapted from Hearing Health magazine staff writer Kathi Mestayer’s work on BeaconReader.com. 

Using Words Carefully

By Jane Madell, Au.D.

We are all influenced by words. Some have easy, uncomplicated meanings (book, tree, house) and others carry a lot of emotional baggage (disabled, slow, delayed, etc.). When audiologists talk with families and children we are working with, we need to think carefully about how we describe children and test results. We do not want our words to interfere with our message.

Normal vs. Abnormal

What does it mean to say that a child’s behavior or test results are “abnormal”? Is there another way we can discuss this? Can we talk about what we expect to see and compare it to what we are seeing? We need to be sure we are talking about a specific test or behavior and not making a statement about the whole child. We need to be sure we are not saying, “Your child is abnormal.” We are saying, “Your child’s hearing is abnormal.” In audiology we might say, “Children with typical hearing have responses above this line on the audiogram. Your child’s hearing is below this line.” When describing speech perception testing, we can talk about what typical children can understand and what we may need to do to help this child understand speech better. A speech-language pathologist or listening and spoken language specialist might say, “Children with typical language development have a vocabulary of XX words at this age, your child has a vocabulary of XX words.”

Describing strengths and weaknesses

It is more useful to describe a child’s strength and weaknesses than to describe a child as having a disability (unless you are trying to get a school district to agree to services in an Individualized Education Program (IEP) meeting). Labeling a child as “disabled” does nothing to plan remediation. It is much more useful to make a list of strengths and areas of weakness. The areas of weakness, carefully defined, can result in a treatment plan. If testing identifies a hearing loss, it provides an opportunity to discuss possibilities for remediation such as selecting technology or determining therapy options. If a speech-language evaluation indicates specific areas of weakness (e.g, vocabulary, auditory memory, etc.), the report should discuss how these areas of weakness will affect language and literacy, and the therapy plan should specifically list areas to be worked on to improve skills.

When discussing test results with a child we need to remember that this is likely going to be distressing and we need remind the child that everyone has areas of strength and weakness, and we need to be sure to remind her what her strengths are.

Giving criticism or suggestions

Kids with hearing loss and other disabilities that affect access to academic information find it difficult to deal with the constant need for extra help, etc. They spend hours a week in therapy of one kind or another where they are working on areas of weakness and things that are difficult. They may be receiving preview and review services in school to help them keep up. The very fact that they need to go to see the teacher of the deaf for these services is an indication to the child that he is different than the other kids in his classroom. We need to be sure we are providing positive feedback along with areas needing improvement. If we need to give criticism, sandwich it between positive statements. While we need to give kids honest information, we need to be careful how we explain it. There is no value in telling kids that their work is “bad” or “poor,” or that “if they worked harder they would be able to do better.” We need to encourage them to work hard, but we will accomplish it more easily by being positive.

Teaching self-advocacy

An important part of success is helping kids advocate for themselves. It is very tempting as parents and professionals to advocate for our children. But we are not always there, so we need to help kids learn to advocate for themselves. They need to understand that they have a disability, and while this is not a statement about who they are as a person, it is a statement about what is needed to help them hear or learn. Talk about how to, recognize when you have missed something, and how to ask for help. If you do not know what page the teacher said to go to, raise your hand and ask for repetition, or look at your neighbor’s book. If you do not hear the answers of other kids to a teacher’s questions, ask the teacher to repeat. If you miss a friend’s comment, ask what was said. Practicing how to ask is very useful with parents or therapists modeling how to ask.

Introducing kids to others with the same disability

Kids need to know that they are not alone. When working with a child with hearing loss, it is useful to introduce them to other kids and to adults who have hearing loss. If there are celebrities who have hearing loss, it will make kids feel terrific to know about them. It helps them to understand that hearing loss is not going to interfere with being who they want to be. When it became public that Derrick Coleman has a hearing loss, it gave a boost to a lot of kids with hearing loss. I personally loved the commercial that Duracell put out with him saying something like, “They told me I couldn’t do this, but I was deaf so I didn’t listen.” What a powerful statement for kids!! Meeting physicians, audiologists, or teachers with hearing loss opens doors for kids. Audiologists, speech-language pathologists, listening and spoken language specialists, teachers of the deaf, and families can seek out adults with hearing loss for kids and parents to meet. It will help everyone feel stronger.

This post originally appeared on Hearing Health @ Hearing Health & Technology Matters’ blog on June 16, 2015. The author, Jane Madell, Au.D., is an audiologist, speech-language pathologist, and auditory verbal therapist with 40-plus years of experience in the greater New York City area.  

New Beginnings

By Margaret

Like many of you, after a fun-filled, active summer, my children are looking forward to their first day of school. With my first child, now entering the 1st grade, things were pretty seamless when she started big girl school.

This year my second child is starting Pre-K and while I have the same excitement (and anxiousness), I also have an extra set of challenges to prepare for; Emmy has hearing loss and will be in a mainstream classroom.

I compiled the below list for how to handle the beginning of the school year for parents of children with hearing loss. Some are my ideas and others were passed on from other parents. I hope by sharing this list I can help anyone else who has a child with hearing that is starting school.

1. Speak Up: Before the school year begins, schedule meetings with teachers and administrators to discuss accommodations, such as classroom seating arraignments, note-taker services, in-class participation (i.e. repeating an answer of a classmate seated behind your child that s/he may not hear), and handling emergency situations, such as fire drills. If your child has an FM unit, this is a great time to teach teachers how to use it. It's just as important for them to know how to turn it on, as well as turn it off when they take bathroom breaks!

2. Teacher-Student Communications: It's important that your child feels comfortable and encouraged to communicate with their teachers when they feel they missed something said. Include your child in these meetings to help build that relationship, and as they get older, your child likely will have the best advice (and stories) to share.

3. Reinforce Good Practices, Politely: Kindly ask teachers to face your child when addressing the classroom. When writing on the chalkboard and talking at the same time, the teacher's voice is bouncing off the board instead of going forward, making it harder for your child to hear as well as lip-read.

4. Buddies, Not Bullies: Bullying should never be tolerated, however it sometimes comes up. Talk with teachers and administrators about how to mitigate tough situations and protect your child, without having your child feeling victimized and ostracized for having hearing loss.

5. Practice Makes Perfect: Develop a scripted answer for your child (and their siblings) when asked about his/her hearing aids and hearing loss. Not only does it put the parents at ease, but helps your child confidently self-advocate.

6. Show and Tell:  If your child mentions s/he received a lot of questions during the first week of school, ask their teacher to take five minutes in the beginning of class for your child to "show and tell" their hearing aids. Not only does it promote tolerance, but it teaches your child public speaking skills. 

If you have additional tips and suggestions for managing the start of school, please add share them in the comment box below or by e-mailing us at info@hhf.org.

Selecting the Right Earmuff

By Colin MacKenzie and Gary Klee

When combining hearing protection with safety glasses, face masks, etc., ensure performance is not adversely affected.

Wearing hearing protection can mean the difference between enjoying the sounds of everyday life and a lifetime of disability. Every day, we are exposed to potentially hazardous environmental noise. It is, therefore, critical that workers who must be present where the noise level is high and constant select either a muff or plug protector to reduce the noise level to an acceptable level. We will now discuss the factors you should consider when selecting the correct earmuff for the job.

Step 1: Determining Your Noise Level

Is the noise level and frequency content known (dB and Hz)?

If not, see the list of common noise sources on the next page. On some machines and power tools, you can find the dB level in the user manual or stated on a label on the machine.

If yes, follow this example: It is recommended that the calculated level under the earmuff should be under 85 dB (A). Therefore, subtract 85 dB from the noise level dB to find out the minimum protection level needed. The key is to provide enough attenuation but not enough to overprotect the wearer. Understand your whole-shift noise exposures and select the earmuff on the basis of that exposure, and do not use the highest measured noise level as your guide.

Example:

If you use a chainsaw, and the dB level is 110 dB (A). The recommended level under the ear cup should be below 85 dB (A). Therefore, you need an earmuff with an attenuation of at least 25 dB (110–85 =25).

When to consider selecting an earmuff with a lower protection level:

• Is the exposure time shorter than four hours? The earmuff protection level is based on noise exposure over an eight- hour working day. If the exposure is shorter, you should consider selecting a lower protection level.

• Do you need to hear important information from colleagues, warning signals etc.? Consider selecting a lower protection level or use an electronic level dependent earmuff.

• Are you suffering from hearing loss? If you have a hearing impairment, you may already have difficulty understanding speech in noisy environments. If you select the highest attenuation for hearing protectors, you may find it even more difficult to communicate or hear warning alarms.

• When to consider selecting an earmuff with a higher protection level:

• Does the noise consist mainly of low-frequency noise? If the noise is mainly low frequency, you should consider selecting a higher protection level because low-frequency noise is more difficult to block out.

• Do you need to wear safety glasses, face masks etc.? When combining hearing protection with safety glasses, face masks, etc., ensure performance is not adversely affected. If you are uncertain, seek additional advice and guidance or select an earmuff with a slightly higher protection level.

• Are there any other noise sources nearby? If there are other noise sources nearby, you should consider selecting a higher protection level.

Step 2: Choose the Correct Earmuff Style

• Are there any requirements to wear a hard hat at your workplace? If so, select a cap-mounted ear muff. Make sure that the hard hat you choose has universal slots that can be used in combination with the selected ear muff.

• Do you need to wear a bump cap or a hat for sun protection? Neckband ear muffs can be worn around the back of the neck, so users can wear them with bump caps, full-brim hard hats, or hats without attachment slots.

Step 3: Other Requirements

• Is the noise intermittent, or do you move in and out of noisy areas? Consider selecting an electronic level-dependent earmuff. The level-dependent earmuff protects against impulsive or intermittent hazardous noise while allowing situational awareness.

• Are you working with monotonous or stationary work tasks without the need to hear warning signals, etc.? Select an earmuff with a built-in AM/FM radio. Employees who wear radio earmuffs are more productive and motivated on the job.

• Are you working with monotonous work tasks and need to hear warning signals, etc.? Select an earmuff with both AM/FM radio and a level-dependent hearing function.

Common Noise Sources

Below are examples of different noise sources with their approximate sound pressure level in dB(A). These examples should only be used as guidance, as large variations may occur. The distance and surroundings also will affect the noise level.

Colin MacKenzie is President, Sales & Marketing at Hellberg Safety. Gary Klee is Product Manager, Above-the-Neck, at Protective Industrial Products Inc. (PIP), which is the exclusive supplier of Hellberg hearing protection in North America.

Photo Credit: Protective Industrial Products Inc.

12 Amazing Tips For Traveling With A Hearing Loss

By Janice Schacter Lintz

Having a hearing loss shouldn’t stop you from traveling. The following tips will ensure a smoother trip:

1. Sign up for travel alerts via email or text to avoid missing a flight. Gate/Flight Attendants, right or wrong, tend to forget to notify people of changes.
   

2. Have your hearing loss and any accommodation you need noted in your passenger/hotel profile in case of an emergency.
   

3. Specify in writing the specific HEARING access you need when booking a room. Otherwise, you are likely to receive a wheelchair accessible room. Hotels should offer a simple remote to activate the television’s closed captions, wake/shake/visual alerts and a hearing aid compatible telephone or TTY depending on your needs.
   

4. Research the places you plan to visit to determine the access available. Advise them ahead of time in writing, the accommodation you need. Send a letter if you do not receive the requested access. Access will only change when people complain.
   

5. Take extra batteries and back-ups of your hearing aids/processors. Running out of batteries can ruin a trip. The same is true if your hearing aid/processor breaks.
   

6. Bring a paper and pen to communicate in a noisy setting.
   

7. Pack a portable dehumidifier to dry out hearing aids/processors if traveling to a humid location. Sweat and humidity may affect their performance.
   

8. Transport all your supplies in a case in your carry-on bag to ensure everything remains intact. Store the bag in your in-room safe to avoid potential theft or loss. Check your homeowner’s policy to confirm coverage when traveling domestically and internationally.
   

9. Mention your hearing loss in advance to the TSA or customs personnel to avoid misunderstanding. Removing hearing aids/cochlear implant processors during TSA screening is unnecessary. Have a copy of the rules with you to avoid issues.
   

10. Load an iPad with movies since very few airlines offer closed captioning for in-flight programing. The good news is airlines such as Virgin are beginning to offer closed captions on some flights.
    

11. Take the hotel’s business card and written directions to ensure you arrive at the proper destination.
    

12. Safeguard your hearing aids/processors while swimming with AquaVault’s portable safe. It solves the issue of where to store your hearing aids when you remove them to swim. The lightweight safe attaches to the back of your lounge chair and easily fits in your luggage. A thief would have to remove he entire lounge chair to steal the safe.

Most importantly, have fun!

This piece was originally featured on JohnnyJet.com, a travel blog and resource committed to easier, better and cheaper travel.

The author, Janice Schacter Lintz, is the CEO of Hearing Access & Innovations, which works to improve accessibility for people with hearing loss. 

Orchestrating Hair Cell Regeneration

By the Stowers Institute for Medical Research

The older we get, the less likely we are to hear well, as our inner ear sensory hair cells succumb to age or injury. Intriguingly, humans are one-upped by fish here. Similar hair cells in a fish sensory system that dots their bodies and forms the lateral line, by which they discern water movement, are readily regenerated if damage or death occurs.

A neuromast sensory structure (green) of the zebrafish lateral line, which helps the fish detect water movement, is shown among surrounding cells (cell nuclei in red).

Credit: Piotrowski Lab, Stowers Institute for Medical Research

A new study in the July 16 online and August 10 print issue of Developmental Cell, from Stowers Institute for Medical Research Associate Investigator Tatjana Piotrowski, Ph.D., zeros in on an important component of this secret weapon in fish: the support cells that surround centrally-located hair cells in each garlic-shaped sensory organ, or neuromast. “We’ve known for some time that fish hair cells regenerate from support cells,” Piotrowski explains, “but it hasn’t been clear if all support cells are capable of this feat, or if subpopulations exist, each with different fates.”

While mammals also have support cells, they unfortunately do not respond to hair cell death in the same way. So understanding how zebrafish support cells respond to hair cell loss may provide insight into how mammalian support cells might be coaxed into regenerating hair cells as well. Zebrafish are particularly amenable to studies of regeneration because transparent embryos and larvae render developmental processes visible and experimentally accessible.

Piotrowski and her team treated zebrafish larvae with the antibiotic neomycin, which kills hair cells, then monitored support cell proliferation in regenerating neuromasts for three days using time-lapse movies. “These single cell lineage analyses were tremendously time-consuming but very informative,” Piotrowski notes. The study’s lead author, Andrés Romero-Carvajal, Ph.D., previously a predoctoral researcher at the Stowers Institute, carefully kept track of every individual support cell’s location and behavior across different time-lapse frames.

The researchers determined that approximately half of the dividing support cells differentiated into hair cells, while the rest self-renewed. Self-renewal is an equally important fate, Piotrowski points out, because it ensures maintenance of a reserve force that, if necessary, can spring into regenerative action. The researchers also observed that lineage fate of support cells hinged on where they were located in the neuromast, as self-renewing cells were found clustered at opposite poles while differentiating cells were distributed in a random, circular pattern close to the center. 

Such distinct support cell locations were “strongly indicative of differences in gene expression”, Piotrowski says, so the team turned its attention to exploring some of the genes and signaling pathways involved. A study of gene expression patterns showed that members of the Notch and Wnt pathways were expressed in different parts of the neuromast, specifically the Notch members in the center and the Wnt members at the poles. To determine if and how these two pathways regulate each other, the researchers used an inhibitor to turn off Notch signaling in neuromasts. This halt in Notch activity mimics the halt known to occur immediately after neomycin-induced hair cell death. After inhibitor treatment, they saw transient upregulation of Wnt ligands in the neuromast center, along with support cell proliferation. The majority of the proliferating cells became hair cells.

“We found that Notch directly suppresses differentiation (of support cells into hair cells), and indirectly inhibits proliferation by keeping Wnt in check,” Piotrowski explains. “Previously, others thought perhaps it was Wnt that had to be downregulated, to initiate regeneration. However, our data support the loss of Notch signaling as a more likely trigger.” Essentially, the process of restoring injured or dead hair cells in neuromasts is jump-started by the transient suppression of Notch, while its eventual reactivation restores the balance, ensuring that not all support cells answer the call to regenerate through proliferation and differentiation.

Piotrowski’s research is partially supported by the Hearing Health Foundation through its Hearing Restoration Project (HRP), which emphasizes collaborations across multiple institutions to develop new therapies for hearing loss. By continuing to illuminate the intricacies of hair cell regeneration in zebrafish, she and her team are providing other HRP scientists with candidate genes and molecular pathways to probe in other models such as chicken and mice, with the goal of providing insight that could someday make human inner ear hair cells readily replaceable.

The study was also funded by the Stowers Institute and the National Institute on Deafness and Other Communication Disorders of the National Institutes of Health (award RC1DC010631). The content is solely the responsibility of the authors and does not necessarily represent the official views of the NIH.

Other Institute contributors include Joaquín Navajas Acedo; Linjia Jiang, Ph.D.; Agnė Kozlovskaja-Gumbrienė; Richard Alexander; and Hua Li, Ph.D.

Lay summary of findings

Hair cells in sensory structures called neuromasts, which form the sensory system fish use to orient themselves in water, are similar to mammalian inner ear hair cells responsible for our sense of hearing. Unlike the latter, however, they are constantly replaced after damage or death. In the current issue of Developmental Cell, Stowers Associate Investigator Tatjana Piotrowski, Ph.D., and members of her lab closely examine, in zebrafish, the support cells from which hair cells regenerate. By tracking individual support cells during neuromast regeneration, first author Andrés Romero-Carvajal, Ph.D., shows that approximately half become hair cells, while the rest self-renew as support cells. These lineage decisions are coordinated by interactions between the Notch and Wnt signaling pathways and are location-specific, as differentiation into hair cells occurs toward the center of neuromasts and self-renewal occurs at opposite poles of the structures. Piotrowski hopes her lab’s findings in zebrafish may be extrapolated to mammals someday, to help provide basic insight needed to progress towards the ultimate goal of regenerating human inner ear hair cells.

About the Stowers Institute for Medical Research

The Stowers Institute for Medical Research is a non-profit, basic biomedical research organization dedicated to improving human health by studying the fundamental processes of life. Jim Stowers, founder of American Century Investments, and his wife, Virginia, opened the Institute in 2000. Since then, the Institute has spent over one billion dollars in pursuit of its mission.

Currently, the Institute is home to almost 550 researchers and support personnel; over 20 independent research programs; and more than a dozen technology-development and core facilities.

The above post is reprinted, with permission, from materials provided by Stowers Institute for Medical Research.

Your financial support will help to ensure we can continue this vital research and find a cure in our lifetime! Please help us accelerate the pace of hearing and balance research and donate today. Your HELP is OUR hope!

If you have any questions about this research or our progress toward a cure for hearing loss and tinnitus, please contact Hearing Health Foundation at info@hhf.org.

URGENT: Demand Hearing Loss to be Acknowledged as a Disability

Recently, Hearing Health Foundation learned that the Centers for Disease Control and Prevention (CDC) conducted a study on the prevalence of disability in the U.S. The study examined vision loss, cognition, mobility, self-care and independent living, but failed to mention hearing loss, the third most common public health concern after diabetes and heart disease.

Hearing Health Foundation is outraged by this gross oversight and finds the exclusion of people living with hearing loss from the report to be a troubling concern. Failing to acknowledge hearing loss diminishes the fact that having a hearing loss is a concern worthy of attention and treatment, as well its impact on a person's quality of life, ability to work, and full participation in society.

Hearing Health Foundation is not sitting back quietly, and neither should you! We will be sending representatives at the White House and CDC a letter asking them to take swift and meaningful steps to correct this gross error, acknowledge hearing loss as a disability, and amend the report accordingly. 

If you would like to take action with HHF, please sign our petition on Change.org. You can also download this letter, sign and return it to us by e-mail or mail (Take Action, c/o Hearing Health Foundation, 363 7th Ave, NY, NY, 10001). We will be sending all letters on September 1st. 

If you have any questions or would like to share your own letter with us, please email us at info@hearinghealthfoundation.org.

Thank you,

Claire Schultz 

Chief Executive Officer 

Hearing Health Foundation

Sign up for our monthly Hearing Health e-newsletter to receive the latest research updates from the lab, hear from those directly impacted by hearing loss and learn about ways for you to help make hearing loss a thing of the past. 

2015 Emerging Research Grants Approved!

By Laura Friedman

Hearing Health Foundation is excited to announce that the 2015 Emerging Research Grants (ERG) have been approved by our Board of Directors, after a rigorous scientific review process. The areas that we are funding for the 2015 cycle are:

• Central Auditory Processing Disorder (CAPD): Four grants were awarded for innovative research that will increase our understanding of the causes, diagnosis, and treatment of central auditory processing disorder, an umbrella term for a variety of disorders that affect the way the brain processes auditory information. All four of our CAPD grantees are General Grand Chapter Royal Arch Masons International award recipients.
  

• Hyperacusis: Two grants were awarded that is focused on innovative research (e.g., animal models, brain imaging, biomarkers, electrophysiology) that will increase our understanding of the mechanisms, causes, diagnosis, and treatments of hyperacusis and severe forms of loudness intolerance. Research that explores distinctions between hyperacusis and tinnitus is of special interest. Both of our Hyperacusis grants were funded by Hyperacuis Research.
  

• Ménière’s Disease: Two grants were awarded for innovative research that will increase our understanding of the inner ear and balance disorder Ménière’s disease. One of the grants is funded by The Estate of Howard F. Schum and the other is funded by William Randolph Hearst Foundation through their William Randolph Hearst Endowed Otologic Fellowship.
  

• Tinnitus: Two grants were awarded for innovative research that will increase our understanding of the mechanisms, causes, diagnosis, and treatment of tinnitus. One of the grants is funded by the Les Paul Foundation and the other grantee is the recipient of The Todd M. Bader Research Grant of The Barbara Epstein Foundation, Inc.

To learn more about our 2015 ERG grantees and their research proposals and goals, please visit: http://hearinghealthfoundation.org/2015_researchers

Hearing Health Foundation is also currently planning for our 2016 ERG grant cycle. If you're interested in naming a research grant in any discipline within the hearing and balance space, please contact development@hhf.org.

Rare Gene Cariant Associated with Middle Ear Infections

By Baylor College of Medicine

Many parents have heard the night-time cry of “my ear hurts.” For some children, this might happen frequently beginning in infancy and even persist into adulthood. An international consortium led by those at Baylor College of Medicine may have taken the first step on the road to understanding why only some people get frequent painful or chronic middle ear infections. The culprit may be rare genetic variants in a gene called A2ML1.

A report on their work appears online in the journal Nature Genetics.

In studies led by Dr. Regie Lyn P. Santos-Cortez, assistant professor of molecular and human genetics at Baylor, researchers looked for a genetic component to the disorder. Santos-Cortez is not only a genetics researcher, she was also trained as an otolaryngologist in her native Philippines, and she knows the toll such infections take.

After graduation, she went on a medical missionary trip to an indigenous Filipino population in one area of the country where most of the people were related.

There she created a family tree or pedigree that identified, among other things, who within the same community suffered from recurrent ear infections and who did not.

“The pedigree was huge,” she said. “It was several pages long and wide.”

Everyone had similar socioeconomic status, swam in the same sea water, were or had been mostly breastfed, ate the same food, and had the same exposure to cigarette smoke, which made an environmental factor an unlikely cause.

Luckily, next-generation sequencing that allowed her to determine the genetic sequence of several people in the population was available. Without that technological advance, she said, she did not think they could have identified the gene.

Within the indigenous community, she found that 80 percent of those who carry the variant in the A2ML1 gene developed otitis media. They also found the same gene variant in three otitis-prone children in a group in Galveston, Texas.

So far, they have identified this rare genetic cause for susceptibility to middle ear infections in 37 Filipinos, one Hispanic-American and two European-Americans. It is likely that the variant has been present in the population in the Philippines and in Galveston at least 150 years and may even be the result of a “founder” effect, which suggests one person from outside the population, more likely from Spain, brought the gene variant into the two populations.

Additionally, rare A2ML1 variants were identified in six otitis-prone children who were Hispanic- or European-American, and none of these variants occurred in thousands of individuals without otitis media.

She does not think this is the only gene involved in predisposing children to middle ear infections, but it could be an important one. The protein involved may play a role in the immune system that protects the ear. Perhaps the variant somehow derails the protection the protein should provide.

Another gene called alpha 2-macroglobulin or A2M, which encodes a protein that is found at high levels in very infected ears, is formed in such a way that it can trap proteases, enzymes that can kill infectious microbes but can also damage the mucosa of the middle ear if left unchecked.

Because the protein sequences of A2M and A2ML1 are highly identical, they may have similar or overlapping functions and one might compensate for the other when it is non-functional. An antibiotic drug called bacitracin is used in drop form to treat the problem in Europe. However, because bacitracin dampens the effect of A2M it may not be the best treatment for people who have genetic variants in A2ML1, she said.

“There are many other antibiotic drops on the market,” said Santos-Cortez.

The finding of the variant is a start, she said. She and her colleagues hope to look further into the mechanism by which A2ML1 defects cause otitis media susceptibility.

Others who took part in this work include Xin Wang, Anushree Acharya, Izoduwa Abbe, Biao Li, Gao T. Wang and Suzanne M. Leal, all of Baylor; Charlotte M. Chiong, Ma Rina T Reyes-Quintos, Ma Leah C. Tantoco, Marieflor Cristy Garcia, Erasmo Gonzalo D V Llanes, Patrick John Labra, Teresa Luisa I. Gloria-Cruz, Abner L. Chan, Eva Maria Cutiongco-de la Paz and Generoso T. Abes, all of the University of the Philippines Manila-National Institutes of Health; Arnaud P. Giese, Saima Riazuddin and Zubair M. Ahmed, University of Maryland at Baltimore; Joshua D Smith, Jay Shendure, Michael J. Bamshad and Deborah A. Nickerson, all of the University of Washington at Seattle; E. Kaitlynn Allen and Michele M. Sale of the University of Virginia in Charlottesville; Kathleen A. Daly of the University of Minnesota in Minneapolis; Janak A. Patel and Tasnee Chonmaitree of the University of Texas Medical Branch at Galveston.

Funding for this work came from the Hearing Health Foundation; Action On Hearing Loss and the National Organization for Hearing Research Foundation (to R.L.P.S.-C.); the University of the Philippines Manila–National Institutes of Health (to G.T.A. and R.L.P.S.-C.); and U.S. National Institutes of Health (Grants U54 HG006493 (to D.A.N.), R01 DK084350 (to M.M.S.), R01 DC003166 (to K.A.D.), R01 DC005841 (to T.C.), R01 DC011803 and R01 DC012564 (to S.R. and Z.M.A.), and R01 DC011651 and R01 DC003594 (to S.M.L.).

The above post is reprinted from materials provided by Baylor College of Medicine.

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We're currently planning for our 2016 grant cycle. If you're interested in naming a research grant in any discipline within the hearing and balance space, such as Usher Syndrome, hyperacusis, stria, or tinnitus, please contact development@hhf.org. 

Life-Saving Antibiotics Increase Risk of Hearing Loss

By Oregon Health & Science University

Approximately 80% of infants in neonatal intensive care units receive antibiotics known to be toxic to the ear. 

Seeking to stem the tide of permanent hearing loss from the use of life saving antibiotics, researchers at Oregon Health & Science University have found that patients stricken with dangerous bacterial infections are at greater risk of hearing loss than previously recognized. Inflammation from the bacterial infections substantially increased susceptibility to hearing impairment by increasing the uptake of aminoglycoside antibiotics into the inner ear, the researchers report. Their findings are published in online in the journal Science-Translational Medicine.

“Currently, it’s accepted that the price that some patients have to pay for surviving a life-threatening bacterial infection is the loss of their ability to hear. We must swiftly bring to clinics everywhere effective alternatives for treating life-threatening infections that do not sacrifice patients’ ability to hear,” said Peter S. Steyger, Ph.D.*, professor of otolaryngology, head and neck surgery, Oregon Hearing Research Center, Oregon Health & Science University School of Medicine. “Most instances in which patients are treated with aminoglycosides involve infants with life-threatening infections. The costs of this incalculable loss are borne by patients and society. When infants lose their hearing, they begin a long and arduous process to learn to listen and speak. This can interfere with their educational trajectory and psychosocial development, all of which can have a dramatic impact on their future employability, income and quality of life.”

Aminoglycosides, antimicrobials that are indispensable to treating life-threatening bacterial infections, are toxic to the ear. Relied on by physicians to treat meningitis, bacteremia and respiratory infections in cystic fibrosis, aminoglycosides kill the sensory cells in the inner ear that detect sound and motion.

Infants in neonatal intensive care units, or NICUs, are at particular risk. Each year, approximately 80 percent of 600,000 admissions into NICUs in the United States receive aminoglycosides. The rate of hearing loss in NICU graduates is 2 to 4 percent compared with 0.1 to 0.3 percent of full-term births from congenital causes of hearing loss.

When Steyger and colleagues gave healthy mice a low amount of aminoglycoside, the rodents experienced a small degree of hearing loss. If the mice had an inflammation that is typical of the infections treated with aminoglycosides in humans, the mice experienced a vastly greater degree of hearing loss.

The study lays the groundwork for improving the standard of care guidelines for patients receiving aminoglycosides. To shield patients’ hearing, the researchers called for the development of more targeted aminoglycosides and urged clinicians to choose more targeted, non-ototoxic antibiotics or anti-infective drugs to treat patients stricken with severe infections.

Due to their widespread availability and low cost, aminoglycosides are used frequently worldwide. Clinical use of aminoglycosides is limited due to the known risk of acute kidney poisoning and permanent hearing loss, yet are crucial life-savers in cases with potentially fatal infections.

Scientists who contributed to the OHSU study, “Endotoxemia-mediated inflammation potentiates aminoglycoside-induced ototoxicity,” include: Steyger; Ja-won Koo, M.D., Ph.D.; Lourdes Quintanilla-Dieck, M.D.; Meiyan Jiang, Ph.D.; Jianping Liu, M.D., Ph.D.; Zachary D. Urdang, B.S.; Jordan Allensworth, B.S.; Campbell P. Cross, B.A.; and Hongzhe Li, Ph.D.

This research was supported by: National Research Foundation of Korea grant 2011-0010166; Seoul National University Bundang Hospital 03-2011-007 (J.K.W.); National Institute of Deafness and Other Communication Disorders R01 DC004555, R01 DC12588 (P.S.S.), R03 DC011622 (H.L.), and P30 DC005983; and the Department of Otolaryngology at OHSU (L.Q.D.).

*Peter S. Steyger, Ph.D., is a prior Hearing Health Foundation board member and previous head of our Council of Scientific Trustees.

The above post is reprinted from materials provided by Oregon Health & Science University.