Introducing the Council of Medical Trustees

By Yishane Lee

David S. Haynes, M.D., of Vanderbilt University, has been HHF’s medical director for the past couple of years, advising HHF on medical issues and serving on the Board of Directors and Council of Scientific Trustees. Dr. Haynes recently created HHF’s Council of Medical Trustees (CMT) as an expert panel of more than a dozen otologic and neurotologic physicians and surgeons that HHF can turn to for input regarding medically related issues, and provide comprehensive and up-to-date information about various hearing and balance diseases and conditions. The CMT builds on the long legacy of HHF’s Centurions, a group of medical doctors that had held a similar role.

In the Winter 2014 issue of Hearing Health, we are pleased to highlight the first of many articles about hearing and balance conditions in the magazine by members of the CMT. Dr. Haynes described Ménière’s disease, its definition, diagnosis, symptoms, treatment, outlook, and current research areas.

Two centuries after it was first named, Ménière’s has been a challenge to accurately diagnose, since its symptoms of vertigo, hearing loss, and/or tinnitus can be seen in many other conditions. As Dr. Haynes writes, “Having an experienced doctor who understands the conditions that can present with similar symptoms is essential. Because of the challenges in accurately diagnosing Ménière’s, the diagnosis can sometimes occur by process of elimination.” Another challenge has been determining the cause, although treatments to manage the condition have met with varying degrees of success as well as become less invasive.

The article is in print as well as online.

Two 2013 HHF Emerging Research Grant scientists are also investigating Ménière’s disease.

Peihan Orestes, Ph.D., of the University of California, Los Angeles, is testing the effect of gentamicin use on the contralateral (least affected) ear to stabilize vestibular function in patients with Ménière’s disease, and whether the contralateral ear can be retrained to help normalize vestibular function.

Ian Swinburne, Ph.D., of Harvard Medical School, is studying how the endolymphatic duct and sac stabilize the inner ear’s fluid environment in an effort to identify ways to restore or elevate this function to mitigate or cure Ménière’s disease.

Please have a look at our online dictionary of hearing terms and let us know in the comments below if you have specific areas you’d like us to address.

What Do the Lunatic Fringe, a Cheap Date, Groucho Marx, and the Sonic Hedgehog Have in Common?

By Yishane Lee

At a presentation Hearing Restoration Project scientist Neil Segil, Ph.D., made to a small audience at the HHF offices earlier this year, I was surprised (and delighted) to learn that a gene had the name of Lunatic Fringe—which is not to be confused with the Manic Fringe or the Radical Fringe. Dr. Segil mentioned the gene because it can affect the Notch signaling pathway, a form of cell communication that creates mosaic patterns of different cell types, like the kind we see in the inner ear for hearing. I wondered, how did the gene get this name?

The privilege for people who discover things is that they get to name it—be it a star, mountain, or gene. And the scientists who worked on genetically mapping the fruit fly, which has been studied for over a century, have a sense of humor.

Besides the fringe genes, there are these fruit fly genes that have been found or created: the Tinman refers to a gene that makes it hard to develop a heart; the Groucho Marx refers to a gene that produces excess facial bristles; the Cheap Date gene means the fruit fly is extra-sensitive to alcohol; the Ken and Barbie fruit fly lacks genitalia; and fruit flies with the I’m Not Dead Yet (INDY) gene live longer than usual. (See a list of funny fruit fly gene names.)

The problem, though, is that we discover we actually share quite a lot of ancestry—and therefore, genes—with the fruit fly, including those that cause hereditary diseases, telling a patient that he has a genetic condition related to a mutation in the Lunatic Fringe gene is not going to, well, fly. (Two faulty copies of the Lunatic Fringe gene, which encodes the development of the limbs and other parts of the body, leads to spinal defect known as spondylocostal dysostosis.)

So while these catchy names and the conditions they refer to are easy to remember, a few years ago the Human Genome Organization (HUGO) gene naming committee was petitioned to change them. And, faster than you can say Sonic Hedgehog (another gene name that was changed), the Lunatic Fringe—at least officially—became the LNFG.

Read more about genes related to hearing loss, including the HRP’s use of next-generation DNA sequencing.

Tell us what your favorite gene name is here in the comments!

Ears: The New Fingerprints?

By Tara Guastella

All ears are the same, right? Wrong.

Ears are actually unique to each and every person, so much so that they are comparable in uniqueness to the fingerprint. Research has even suggested that ears may be a more effective identification tool than a fingerprint through the use of a new “image ray transform” technology. This technology shines beams of light on the tubular features of the outer ear, creating an image from the way light reflects off the ear’s curves.

What makes the ear so unique? One’s ears are fully formed at birth and age gracefully over time, making them an ideal body part to confirm identity. Fingerprints can change due to the development of calluses from repeated labor which can make them less reliable.

In almost every crime scene TV drama, you’re likely to see characters dusting for fingerprints. When we will see them dusting for earprints? Well, I guess it’s a lot less likely that your average criminal is pressing an ear against an object while committing a crime. Yet in 1998, the first murder conviction on the basis of ear identification occurred in England. The convicted suspect pressed his ear up against a newly washed window in the house (where the murder took place) to listen for movement.

Airports also regularly use biometric facial recognition programs in their security programs. The addition of earprints to this type of security could also prove a valuable way to identify travelers as well as any potential threats.

Earprints come with limitations when it comes to identification, however. Ears can be altered in shape through plastic surgery or from an accident. Wearing earrings or eyeglasses or having hair pushed behind the ear can also alter the shape of an earprint.

While an earprint will likely will not substitute the fingerprint in terms of identification capability, it could be a valuable addition for solving mysteries, saving lives, and likely many other uses.

Do Chickens Hold the Key to a Cure for Hearing Loss?

Chickens have the amazing ability to restore their own hearing, and this trait is inspiring a nonprofit organization in their search for a cure for hearing loss in humans.

The Hearing Health Foundation’s “Chirp the News” video features the group’s new mascot: a baby chick. Locked within the ears of this chick is the potential to restore hearing and cure tinnitus, or ringing in the ears. According to the National Institute on Deafness and Other Communication Disorders, about 36 million adults in the U.S. have some form of hearing loss, and 25 million are affected by tinnitus.

“As someone who lives with hearing loss every day, I am personally thrilled with the prospects for a cure,” said Shari Eberts, chairman of the Hearing Health Foundation’s Board of Directors, in an email to Healthline. “Life with hearing loss can be frustrating. Sometimes you miss the joke when everyone else is laughing, and sometimes you miss important information because you don't hear it. Supportive family and friends can make living with hearing loss easier, but a genuine cure would be life changing.” 

The Chicken’s Magic Ears

The secret to the chicken’s auditory magic is that supporting cells in its inner ear can replace hair cells that have been damaged by loud noises or other causes.

And chickens aren’t the only animals that can restore their own damaged hearing. All vertebrates other than mammals can do the same. And preliminary research has shown that mice can regain some of their hearing using supporting cells that turn into hair cells—in the lab, at least. 

Researchers supported by the Hearing Health Foundation hope to find a way to coax the supporting cells in the inner ears of people to transform into functional hair cells. Their goal is to have a cure within a decade.

The 10-Year Road to a Cure

For people with hearing loss, waiting a decade for a cure can seem like a lifetime. But in the world of research, this is a very short time to travel from initial scientific discoveries all the way to successful clinical trials in humans.

To speed the research along, the Hearing Health Foundation is supporting a collaboration known as the Hearing Restoration Project (HRP) that involves researchers from more than ten institutions, including Harvard Medical School.

To find a successful cure for hearing loss, researchers have plenty o work ahead of them—including identifying how supporting cells in the chicken's ear turn into hair cells, as well as finding potential compounds or drugs that can make this happen in people.

Eberts is optimistic that the project will hit its mark, and so is Ed Rubel, a professor of hearing science at the University of Washington and a member of the project team.

“With sufficient funding,” he says, “the consortium can discover effective pathways and hopefully some lead compounds to promote hair cell regeneration in the mammalian inner ear in the 10-year time frame.”

Many Eggs in Many Baskets

In his lab at the University of Washington, Rubel is working on one piece of the puzzle that may one day lead to hair cell regeneration in people. 

“The project on my own has to do with developing a new mouse model to test the pathways and, eventually, the drugs that come out of the HRP,” he said.

The mice developed in his laboratory will be shared among members of the consortium, so they can avoid having to develop their own mice. This kind of sharing is an important aspect of the collaboration, something that Eberts expects will save time and money.

For Rubel, working with the HRP has other benefits.

“The wonderful thing about the consortium,” he says, “is that it includes only people who really want to play in that kind of sandbox—that want to share information, share early-stage information, share the other things that they’re doing in their laboratories, and work together.”

As a person with hearing loss, Eberts supports the push to highlight the project's potential.

“Even though we are in the early stages of the research, we think it is very important that the public learn about our efforts,” she said. “We want them to know that there is hope for a cure and that there are researchers who consider curing hearing loss and tinnitus to be their life’s most important work.”

This article was repurposed with permission from Healthline.

Are You Wireless Enabled? Part Two

By Paul Harrison, Guest Author

This is the second in a two part series on wireless technology and hearing aids. Check out part one for more.

Wireless technology in hearing aids means that they are able to connect both with each other and with a number of different devices using a signal that is similar to the Bluetooth in mobile phones. When the hearing aids work together it is known as binaural technology. With this feature, they can communicate with each other and work together to improve your hearing. There are some binaural features that will analyze your environment, detect which hearing aid is receiving the clearer signal and then transmit this superior signal to the other side. This ensures that you are always getting the best sound available, whatever your environment may be. Binaural microphone applications work in the same way to ensure you hear sounds from all directions clearly but can give priority to speech over background noises.

Each manufacturer has their own range of accessories which can only be used with their own hearing aids. Some require a device called a streamer to be used in addition to the other accessories. This streamer is used to relay the signal from each device into the hearing aids and is often the point of control as well. Some manufacturers use a different or more powerful signal which can send the sounds directly without the need for this additional device.

Music Player

Obviously, you cannot wear hearing aids and headphones at the same time. With personal music players, unless you are listening at home through speakers, headphones are absolutely necessary. Some people are still unable to hear clearly with just headphones and still require the extra help from their hearing aids. There are now devices that essentially turn your hearing aids into the headphones for your music player. The signal is sent in the same way as the other devices and allows you to listen in comfort and you can easily adjust the volume to suit your needs.

Voices

Yes, technically you don’t connect to someone’s voice, but there are accessories available that help you to hear them more clearly. Remote Microphone devices have been designed to pick up the sound of another person’s voice and send it directly to your hearing aids. In crowded environments, people who wear hearing aids often find having a conversation difficult due to the level of background noise. The microphone accessories actively suppress this unwanted noise and send clear and audible speech straight to you. These devices are worn by the other speaker in a one to one conversation and allow you to hear everything they say clearly without having to be uncomfortably close to them. In a group situation such as a meeting, these devices can be placed on the table and they will pick up the individual voices so you don’t miss out on any of the discussion.

Remote Controls

Most hearing aid manufacturers offer some form of remote control for their hearing aids nowadays. Some are basic devices that simply allow you to adjust volume or programs, others are more advanced and give you control over things like clarity and comfort settings. Some of these devices are simple push button control and others have display screens with more in depth menu options. There are even some that have been designed to be as discreet as possible by looking exactly like other objects such as pens or being small enough to fit on a set of keys. These accessories are especially useful for people who may find that the small buttons on the hearing aids themselves are too fiddly to use.

According to YourHearing Wireless, technology is advancing all the time, making it easier for hearing aid users to enjoy things that they previously found difficult or frustrating. Each manufacturer has a different range of accessories so you would need to check to see what is available for your particular hearing aids. There are so many options available today it is possible for everyone to get themselves and their hearing aids wireless enabled.

Author Bio:  Paul Harrison has been in the Hearing aid industry for 20 years and in that time has worked at both manufacturer and retailer level before managing his own online hearing aid business www.yourhearing.co.uk which is a national network of local hearing aid audiologists who offer the main hearing aid brands at less than the high street but with the same quality aftercare and warranty.

You Can Hear, Thanks to the Darwin Fish

By Tara Guastella

Photo by lapradei/Flickr

I never thought that fish would be an evolutionary ancestor to humans. Monkeys and chimpanzees, yes—but fish?

It turns out that a certain fish, known as Polypterus and related species, have tiny holes in the top of their head called spiracles. A team of researchers in California recently showed how a small valve opens a bony lid over these spiracles that allows air to be sucked in and pumped out when the fish surface.

Strangely enough those same holes allowing the fish to breathe have modified themselves over time to become Eustachian tubes. Enabling us to hear, these tubes are small passageways on either side of the head that connect the upper part of the throat to the middle ear. They supply fresh air, drain fluid, and keep air pressure between the nose and the ear at a steady level.

Hearing in early amphibians developed from the spiracles adapting to become the tympanic membrane (also known as the eardrum), a thin cone-shaped membrane that separates the external ear from the middle ear. The tympanic membrane is similar to skin and transmits sound to the brain through the stapes, the tiniest bone in the body.

Interestingly, the stapes evolved from a long bone, known as the hyomandibular bone, that braced the lower jaws of sharks and other early jawed fish. This bone eventually shrank in size to form the stapes.

The other two inner ear bones alongside the stapes—the malleus and the incus—also formed from bones that braced the articulation of the upper and lower jaws. These bones decreased in size and became restricted to the inner ear. So, these three little bones that enable us to hear derived from the larger jaw bones of ancient fish.

If not for the evolutionary experiments of these prehistoric fish breathing air through the top of their heads, humans may never have evolved a keen sense of hearing.

Now that you know how our hearing evolved, learn more about how hearing works and how the human ear processes sound. Watch our captioned How Hearing Works video.

How Can a Chicken Cure Hearing Loss & Tinnitus? Find out in our new PSA!

By Tara Guastella

I’m so excited to share that we have recently launched a new Public Service Announcement (PSA): Chirp the News! The PSA introduces HHF’s new mascot, a baby chicken.

Why a baby chicken?

The key to restoring hearing in humans is to regenerate cells deep within the inner ear. In fact, most types of hearing loss in humans results from damage to these cells, called hair cells. While humans cannot regenerate hair cells in the inner ear after they are damaged, chickens can. In fact, most animals other than mammals can regenerate these delicate cells, restoring their hearing spontaneously after damage.

This knowledge is the basis for our Hearing Restoration Project (HRP). The HRP is a consortium of over a dozen top scientists from across the world working collaboratively to take what we know happens in chickens and translate that to people. Our HRP scientists estimate clinical trials testing a genuine, biologic cure for hearing loss can occur within the next decade. Since over 90 percent of tinnitus cases occur with an underlying hearing loss, a cure for hearing loss is also likely the key to a cure for tinnitus.

Most scientific research is conducted in isolation: one researcher or one lab trying to solve a major health issue. Our unique HRP consortium model is breaking the mold by encouraging our scientists to work as a team sharing data, tools, resources, and ideas. By working collaboratively, the timeline to a cure is expedited: five times as fast with the power of collaboration.

We won’t be able to get to a cure for hearing loss and tinnitus without your help—please donate today and be a part of the cure.

Congratulations to the First Deaf NFL Player to Play in the Super Bowl: Derrick Coleman!

By Tara Guastella

Hearing loss is sometimes considered an invisible disability. You usually can’t see hearing loss but the impact it has can be life-altering. Many even try to hide their hearing loss for fear of embarrassment. Others embrace it and do everything in their power to not let it hamper them. Seattle Seahawks fullback Derrick Coleman—who played in the Super Bowl Sunday—embraces it.

I was so moved when I first watched the Duracell commercial (below) that featured Coleman. Over the years at HHF we’ve been in touch with so many actors, sports stars, politicians, and other celebrities that are afraid of “coming out” about their hearing loss. But Coleman freely and openly talks about his hearing loss in a positive way.

Coleman, at the age of 23, broke the mold and has had an enormous impact on each and every person that experiences or knows someone who experiences hearing loss. He has become a role model for those young and old alike. He told the New York Times, “If you really want something, you find a way to make it happen.”

Coleman made it happen during last night’s game. And over the past few weeks he has made it happen for some of his biggest fans: children and young adults with hearing loss. He personally responded to an inspirational letter sent to him via Twitter from Riley Kovalcik, a 9-year-old girl who wears two hearing aids. A couple of days later Coleman surprised Riley and her twin sister with tickets to last night’s big game. Adham Talaat of Bridgewater, N.J., who is training for May’s NFL draft, believes that Coleman will pave the way for other players who are deaf. The senior at Gallaudet University in Washington, D.C., says he never had a deaf role model until he learned of Coleman.

I am so proud of Coleman and the Seahawks for their victory against the Denver Broncos last night (and this is coming from a diehard NY Giants fan!).

Do Your Super Bowl Sunday Plans Include Ear Plugs?

By Yishane Lee

Professional football games this season have put the spotlight on a disturbing trend: the purposely loud stadium, as a strategy to flummox the opposing team.

Leading the charge are the Seattle Seahawks, who made it to the Super Bowl XLVIII. Regardless of whether or not they beat the Denver Broncos this weekend, the Seahawks fans will already be remembered—for better or worse—as the noisiest in history. They broke the Guinness record twice this season, most recently with a rating of 137.6 decibels (dB) in December.

Seattle’s own Derrick Coleman, who doesn’t experience the din of the crowds, confronts his hearing loss head on in this Duracell commercial. Coleman has become an inspiration for all those with hearing loss.

At 130 dB, the human ear is subject to immediate and permanent hearing loss. By comparison, a jackhammer is 110 dB, an ambulance siren is 120 dB, and a jet taking off is 140 dB.

My ears hurt just thinking about this insanity. People routinely bring their children to games, subjecting them to lasting hearing damage. All of this is in the name of not only team spirit but, perhaps more importantly, messing up communication for the opposing team and causing them to make mistakes. According to a January 17 article in the Wall Street Journal, “Seahawk opponents committed 174 false start penalties since [Seattle’s CenturyLink Field] opened in 2002, the most in the National Football League.” During the NFC Championship game on January 19, San Francisco 49ers players were even fitted with custom earplugs to drown out the noise and protect their ears.

Some of the organizers of these extremely loud crowd roars—the fan association leaders—seem completely unperturbed by the risk of permanent hearing loss. Earlier this season, the Seattle Seahawks were in a noise-off with the Kansas City Chiefs, whose fans are led by Ty Rowton. (Alarmingly, he says he brings his kids to games, and they do not use ear plugs.) “If we can help our team win, that's what matters most to us. We don't think about hearing loss,” Rowton told Soundcheck, a public radio program, in December.

The author of a front-page New York Times article last November about the phenomenon, Joyce Cohen (who has hyperacusis), was also interviewed for the show, and said, “I think truly, ignorance rules the day. People have no idea, there's been no education about this.” While a Seattle hearing healthcare center donated 30,000 earplugs to fans, they were too big to fit children. A sixth-grader told Cohen that he endured a steady roar “so loud that the insides of you rattle.”

The noise from Seahawks fans is loud enough to trigger a seismograph, used to measure earthquakes. In 2011 the fans’ jumping, stomping, and screaming created enough sound energy equal to a magnitude 1 or 2 earthquake, according to the Seattle Times.

If you’re lucky enough to score tickets to this Sunday’s big game, please don’t forget a pair of earplugs. I’m all for rooting for your team, but not at the expense of your hearing. Frankly, this makes the painted, shirtless fans braving subzero temps seem like geniuses by comparison.

One Woman's Grand Passion for Music

By Yishane Lee

Nancy M. Williams joined the HHF board in March of 2012 and has been an active member since. She coped with the loss as a child, accepted it as an adult, and now has become an advocate for hearing research—all experiences she writes about in the Winter issue of Hearing Health magazine.

Williams has had an interesting career, going from two decades of marketing and management consulting (after earning degrees from Stanford and Harvard Business School), to growing an online music community through her online magazine Grand Piano Passion. She won the 2009 Lamar York Nonfiction Prize for a heart-wrenching essay she wrote about returning to piano playing after a 25-year hiatus, and which has spurred her writing as well as playing.

In her article for Hearing Health, titled “A Grand Passion,” Williams writes:

In kindergarten, after I sang “Three Blind Mice” too loudly on the big rag rug in our classroom, I was diagnosed with a high-frequency hearing loss. My parents, worried about the social stigma, refused the recommended hearing aid, a decision that boomeranged when I reached middle school. “You can’t hear secrets,” complained a girl with green eyeliner at lunchtime. “Don’t sit with us anymore.”  

I was devastated. My parents broke down and had me fitted with an aid, a behind-the-ear model, bulky by today’s standards.

My parents had acted with the best of intentions in a society that tolerated hearing loss even less than ours does today. Yet the incident in the lunchroom stayed with me for a long time. To compensate, I rarely admitted to anyone that I had a hearing loss.

Playing the piano again, and writing about it, and joining HHF led Williams to finally be fully open about her hearing loss. To do it required overcoming decades of shame, which she writes about openly both in our piece and in her magazine.

Williams has also been very involved with helping other musicians with hearing loss. She’s a huge asset to our organization, offering strategic advice and tips both as a businessperson and a consumer who uses hearing aids. We hope that you enjoy her story in the Winter issue, part of a special package about music, musicians, and hearing loss and tinnitus.