How Can You Change the World, If You Can't Hear It?

By Zeid Malas

How many times have you nodded your head in response to someone whose words you couldn't really hear, no matter how many times they repeated themselves?

How many times have you felt complete isolation when you were among a group of people, whether it was hanging out with friends or a family dinner? Not because you were using your phone or social media, but because you couldn't really keep up with the countless conversations happening at once.

How many times did you do your best to avoid the phone, preferring to text because you were afraid you wouldn't hear the person calling or that you would miss important information being said? Do you know of the pain felt when someone you care about wants to talk to you through Skype, but you just can’t follow the conversation?

What about YouTube, music, lectures, and videos? Such as when you fail to understand what's being said because you can’t hear it and no captions are provided.

How many times have you hesitated or been afraid to ask someone to repeat himself because you felt bad for the times he needed you to listen and understand him? Or maybe because it would change how people thought of you?

I mean every word when I say "I feel you."

Age 11 after taking medicine

When I was 9 years old I woke up one morning only to realize that I had lost 99% of my hearing in both ears for no reason. It wasn't hereditary, it wasn't physiological—it just happened. What did I do? There wasn't really anything I could do... I just accepted it. I taught myself to read lips and body language in order to understand what someone would be trying to say. It was my primary way of communication and with it I was able to finish high school and reach college.

Sadly, my weakest point is electronic devices such as speakers, the TV, the phone… etc. I can hardly make any sense of what comes out of them which makes it very painful not being able to hear and understand someone when they call me. Especially when I'm an online gamer and my games sometimes require me to Skype with my group to coordinate our strategy, about which I end up being clueless.

I've dealt with hearing loss for 10 years now, until last May when I had surgery on my right ear to get a cochlear implant. I made a documentary showing my last days with hearing loss, the surgery, and finally my first moments/days after.

Even though this has been a challenge and complication in life, it made me different from the rest of my peers. It made me someone you remember, someone who my professors will mark as present without the need to shout my name, someone patient and full of empathy, someone who continually works hard to provide the best results because that’s the only way I know how to live.

At age 18

I know many think I've been suffering because of this, and that's what I thought for a while, too. Until I realized that—weirdly enough—it's actually a blessing that I lost my hearing, for it's what made me so different from most people my age. I'm not saying I'm better, I'm just saying I don't waste time relating music to my life, making sure everyone knows what I’ve had for breakfast each day, asking everyone to share and like my profile picture on Facebook, using countless hashtags on Instagram to get followers, retweeting every celebrity tweet on Twitter, or building a story every day on Snapchat.

Because of my situation, I've learned to observe, learn, and understand solely using bodily cues, and to adapt to any difficult situation or people I may encounter. I do my best to make the best out of any situation. I'm not saying I'm Sherlock Holmes... I'm simply saying I'm Zeid Malas!

I made my disability my greatest ability... What are you going to do with yours?

6 Easy Tips for Protecting Your Hearing This Summer

By Laura Friedman

The Better Hearing Institute (BHI) and Hearing Health Foundation (HHF) are encouraging people of all ages to protect their hearing this summer so they can treasure the sounds of the season for a lifetime. Packing earplugs along with the sunscreen for summer outings is just one of six easy tips.

While many noisy recreational activities are part of summer fun, it’s extremely important to take precautions to ensure that these activities don’t harm our hearing.
 
Prolonged exposure to loud outdoor concerts, lawn mowers, power tools, motorized recreational vehicles, target shooting, sporting events and fireworks can potentially damage our ears. In fact, the single bang of a firecracker at close range can cause permanent hearing loss in an instant, making it forever more difficult to hear the quieter sounds of summer.
 
According to the World Health Organization (WHO), 1.1 billion teenagers and young adults (12 to 35 year olds) are at risk of hearing loss due to exposure to damaging levels of sound at noisy entertainment venues and the unsafe use of personal audio devices.
 
“Hearing is the sense that connects us to each other,” says William Hal Martin, Ph.D., Professor of Otolaryngology, National University of Singapore, Program Director MSc of Audiology, Center for Hearing, Speech & Balance, and Co-Director of Dangerous Decibels. “Exposure to high level sounds cannot only destroy our ability to hear, it can cause tinnitus—ringing in the ears.”
 
“People of all ages are at risk of hearing loss from high level sounds, but it easily can be prevented by simple steps,” Martin continues. “It is important to recognize when your ears are in danger and to safeguard them so you can enjoy listening to friends, music, and sounds you love for the rest of your life.”
 
How Noise Affects Our Hearing
 
We hear sound when delicate hair cells in our inner ear vibrate, creating nerve signals that the brain understands as sound. But just as we can overload an electrical circuit, we also can overload these vibrating hair cells. Loud noise damages these delicate hair cells, resulting in sensorineural hearing loss and often tinnitus (ringing in the ears). The cells that are the first to be damaged or die are those that vibrate most quickly—those that allow us to hear higher-frequency sounds clearly, like the sounds of birds singing and children speaking.
Sound volume is measured in decibels, with the softest sound a normal hearing human can hear measuring at 0 dBA. Any sounds above 85 dBA for 8 or more hours are considered unsafe. Most firecrackers produce sounds starting at 125 dB peak SPL, presenting the risk of irreversible ear damage.
 
Repeated exposure to loud noise, over an extended period of time, presents serious risks to hearing health as well. If you have to shout over the noise to be heard by someone within arm’s length, the noise is probably in the dangerous range. Here are the warning signs:

• You have pain in your ears after leaving a noisy area.

• You hear ringing or buzzing (tinnitus) in your ears immediately after exposure to noise.

• You suddenly have difficulty understanding speech after exposure to noise; you can hear people talking but can’t understand them.

6 Easy Tips for Protecting Your Hearing This Summer

1. Walk away and plug your ears. If a loud noise takes you by surprise, quickly plug your ears with your fingers and walk away. Increasing the distance between you and the source of the sound will help reduce the intensity (or decibels) at which the sound is reaching your ears.

2. Use earplugs. When you know you’ll be around loud sounds, use earplugs. Disposable earplugs, made of foam or silicone, are often available at local pharmacies. They’re practical because you can still hear music and conversation when they’re in your ears. But when they fit snuggly, they’re effective in adequately blocking out dangerously loud sounds.

3. Leave the fireworks to the professionals. Be smart when you celebrate 4thof July festivities. Leave the fireworks to the professionals. And when watching the show, stay a safe distance away—where you can enjoy the colors and lights but not expose yourself and your family to loud noises. To protect your hearing, make sure you’re wearing earplugs and that they’re securely in place before the show begins. Also be sure to keep them in for the entire show.

4. Limit your time in noisy environments. Do all you can to limit the length of time you spend in a noisy environment. When you do participate in noisy activities, alternate them with periods of quiet. And remember to use ear protection.

5. Turn it down. When listening to smartphones and other electronics, keep them at a low volume. Importantly, limit your use of headphones and ear buds. Remember, it’s not just the volume that matters. It’s also the duration of time spent listening.

6. Visit your local hearing healthcare professional for custom-fitted ear protection and a hearing test. A hearing healthcare professional can provide a hearing test to determine your baseline hearing level and determine if you have any hearing loss that should be addressed. Hearing care professionals also can provide custom ear protection to ensure a proper fit.

The content for this blog post originated in a press release issued by The Better Hearing Institute on June 26, 2015. 

Mind Your Ears

By Marc A. Gallo

Many of us go about life enjoying the symphony of sounds from birds singing in the trees to your favorite band rockin’ out at a local music venue. These as well as other sounds give us great pleasure. But extensive exposure to sounds that might not seem very loud may in fact result in long term damage to your hearing.

Let Me Give You a Few Examples…

You work and live in the city. To get from your home to your job or to hook up with friends requires taking the subway. To get back home afterwards means you have to take the subway again. If you’re exposed to subway noise in excess of 15 minutes a day you will suffer permanent hearing loss over time.

Here’s another example…you just bought the new iPhone 6 from Apple. Killer phone…and you’re psyched to enjoy your music on iTunes. The new phone conveniently comes with earbuds. You pop ’em in and crank it up! It sounds loud but it’s not hurting you. Or is it? Believe it or not, the iPhone at its maximum volume exceeds 100 db. Listening to your music anywhere near 100 db for more than 30 minutes a day is causing permanent hearing damage.

I Have Tinnitus and It’s Only Getting Worse

I’m a musician, performer and loud-mouthed Irish-Italian…a dangerous mix. Having rehearsed and performed in bands and studios over all these years has caused some degree of hearing loss but worse yet, tinnitus. It’s the dreaded ringing of the ears.

When I go to bed at night, I hear ringing. When I wake up, I hear ringing. When I meditate, I hear ringing. I will never experience silence again. Don’t end up like me or many celebrities like Pete Townsend, Danny Elfman, Will.I.Am and many others. We neglected our ears and now we’re paying the price.

But There’s Hope!

Start protecting your ears today! Turn down the volume. It doesn’t need to be SO loud to be enjoyed. Wear ear plugs when you go to sporting events and concerts. These venues normally exceed 110 db when everyone screams after a touchdown or an encore!

Protect your ears even when you’re doing normal everyday tasks like mowing the lawn, vacuuming your carpet and blowing your leaves. Excessive sound pressure levels, albeit briefly, still have a deleterious affect on your hearing in the long term.

Believe me, you want to enjoy the sound of silence. I wish I could.

This post originally appeared on Mind The Gap's blog on March 27, 2015. The author, Marc A. Gallo. has recorded, performed and published music for Great Egg Music since 1990 and is currently President of Mind The Gap, a provider of on hold music and messaging services. 

5 Tips for Summer Hearing Aid Care

By Courtney Campbell, Au.D.

The summer months bring so many wonderful things—warm weather, sunshine, outdoor activities, and vacations—but for people who wear hearing aids, summer also brings extra maintenance.

Moisture is the enemy of hearing devices, so one of the biggest challenges during the warmer months is the accumulation of moisture in them. This moisture can be caused by humidity in the air, perspiration, or accidental splashes of water at the beach or pool or from a sprinkler.

Here are five ways to keep your devices dry and safe:

1. Use hearing aid covers for behind-the-ear styles. There are several different types on the market. Hearing aid “sweatbands” are made from an all-natural fabric that repels moisture, dust, and dirt. There are also hearing aid sheaths that are made of a water-resistant, spandex-nylon material that also keep out dirt, sweat, and moisture. Both options protect the hearing aid from outside moisture while letting sound come in naturally.
   

2. Dry instruments overnight. Recommended for use year-round, desiccant jars and electric hearing aid dryers are special containers that either use desiccant beads or electric drying technology to suck excess moisture out of the hearing aids. They double as overnight storage and should be used nightly.
   

3. Leave the battery doors open. To avoid corrosion in the battery compartment, leave the battery doors on your instruments open or take the battery out when you aren’t wearing the hearing aids.
   

4. Use clips to keep hearing aids secure. When participating in an activity with lots of movement, use specially designed, lightweight lanyards that attach your BTE or in-the-ear (ITE) hearing aids to your clothing or glasses. If your devices do fall out of your ears, they won’t get too far!
   

5. Disinfect your hearing aids. Warmth and moisture are breeding grounds for bacteria. Always be sure to disinfect your hearing aids before inserting them in your ears. There are many disinfectant wipes specially made for this purpose.

And finally, if you are near water and do not need your hearing aids for safety reasons, always take them out and put them in a safe place. But if the hearing aids do get wet or even submerged, remove them from the water immediately, dry them off, take out the battery, and place them in a desiccant jar or electric hearing aid dryer.

Which styles of hearing aids are best suited to active lifestyles? Find out here.

Courtney Campbell, Au.D., is an audiologist at A&A Hearing Group in Chevy Chase, Maryland, and has been wearing hearing aids for over a decade. 

Gene Discoveries May Lead to Regeneration of Cells Needed for Hearing

By Jeffrey Norris

The researchers identified patterns of gene expression that may determine whether the ear’s inner pillar cells can give rise to new hair cells, which are key to hearing.

School of Medicine scientists have discovered biological mechanisms that appear to play a role in the regeneration of cells in the inner ear.

Over a lifetime, these cells often are damaged or die due to oxidative stress, excessive noise exposure or toxic drugs. The accumulated loss can significantly compromise hearing. Nearly one in four people ages 65-74, and half who are 75 or older, are candidates for hearing aids because of disabling hearing loss.

The discoveries could lead to new ways of evaluating, in animal models, experimental drug treatments intended to prevent hearing loss or restore hearing, and might even lead to methods for regenerating vital cells that have been lost, said Stefan Heller, PhD, professor of otolaryngology.

A paper describing the findings, as well as new methods to quickly link changes in cell function during development to molecular changes within cells, was published June 9 in Cell Reports. Heller is the senior author of the paper. Postdoctoral scholars Jöerg Waldhaus, PhD, and Robert Durruthy-Durruthy, PhD, share the lead authorship.

Discoveries by Stefan Heller and his colleagues could lead to new ways of evaluating, in animal models, experimental drug treatments intended to prevent hearing loss or restore hearing. - Steve Fisch

Sound waves striking the eardrum cause vibrations that are transmitted through tiny bones in the middle ear to fluid within the snail-shell-shaped cochlea of the inner ear. Specialized cochlear cells in a region called the organ of Corti use hairlike sensors to detect the vibrations in cochlear fluid and then trigger nerve signals that are sent to the brain.

“Compared to other senses, we know very little about how hearing works,” Heller said. “The cells are rare. We have to crack open a bone to get to them. They perish quickly, so we must work fast.” There are 120 million retinal cells in a mouse eye, Heller said, but only 3,200 hair cells in a mouse ear.

By using new techniques to rapidly and deeply probe individual cells, Heller’s team has begun to close the knowledge gap.

Molecular mysteries

Many of the biophysical properties of hair cells are understood. Different hair cells along the cochlear spiral are tuned to respond to distinct ranges of sound frequency based on differences in their electrical properties. Frequency is encoded by the place and the properties of the cells’ locations in the cochlea. This understanding has led to the development of cochlear implants to restore hearing in deaf people.

However, little is known about the molecular biology that determines how hair cells develop at specific locations and how different electrical properties arise among hair cells specialized to detect different frequencies. This makes it difficult for scientists to envision strategies to regenerate the specialized cells or to prevent their death, particularly in the high-frequency region of the cochlea, where cells are more susceptible to injury.

Once hair cells die in a mature mammal, they are not replaced. But scientists have recently determined that a supporting cell type, called the inner pillar cell, has the potential to regenerate hair cells in newborn mice.

In its new study of 2-day-old mice, Heller’s lab team measured the activity of 192 genes. The researchers determined which genes were turned on, or “expressed,” in each of 808 hair cells and supporting cells from either the apex or base of the organ of Corti. They quantified this gene expression by measuring the amount of RNA produced from each gene.

The researchers identified patterns of gene expression that may determine whether inner pillar cells can give rise to new hair cells. Similarly, they discovered gradual changes in the expression of specific genes across cells that span the organ of Corti from its base to its apex that may be crucial for the establishment and maintenance of a population of hair cells that responds to a range of sound frequencies.

Crunching the data

Using powerful number-crunching software to analyze the large amount of genetic data, Heller’s lab team accurately identified the two known types of hair cells and the seven known types of supporting cells and created a computer-generated map of their locations within the organ of Corti. They did this using only the genetic data, but then used other previously known DNA sequences to independently verify the accuracy of the cell identification and mapping.

The strategy the researchers used to predict the spatial location of cells within the organ of Corti from gene-expression data also should prove useful to biologists who study other types of cells in different organs, Heller said.

Rapid advances in single-cell gene-expression analysis are likely to supplant a standard technique called in-situ hybridization, according to Heller. The standard technique relies on labeled genetic probes to target individual genes one by one in order to identify specific cell types. The new approach of measuring hundreds of genes in parallel and reconstructing the organs in the computer appears to be more accurate and powerful.

“Molecular gradients play a key role in developmental biology, but in the past researchers depended on identifying gradients in one molecule at a time,” Heller said. “With these new techniques, we are identifying cells that, for example, have molecular characteristics of stem cells, by analyzing the expression of many genes all at once, and we know precisely where they are located.”

The research was funded by the National Institutes of Health (grants DC006167 and DC012250), the Stanford Initiative to Cure Hearing Loss and Hearing Health Foundation’s Hearing Restoration Project.

Stanford’s Department of Otolaryngology-Head & Neck Surgery also supported the work.

Republished with permission from the Stanford School of Medicine's Office of Communication & Public Affairs.

Men's Health and Hearing Health are Linked

By Laura Friedman

Hearing health affects so many aspects of a man’s life that routine hearing tests should be part of a healthy lifestyle. Hearing Health Foundation and Better Hearing Institute (BHI) which are encouraging hearing tests during Men’s Health Month in June and Men’s Health Week (June 15-21). Addressing hearing loss can help men safeguard their wellbeing and quality of life. And new research shows that people with hearing loss who use hearing aids enjoy a better overall quality of life and are more likely to be optimistic, have a strong social network, tackle problems actively, and feel engaged in life. At the same time, an increasing number of studies are showing a link between hearing loss and other health conditions.

Men are more likely to suffer from hearing loss than women. But luckily, the vast majority of people with hearing loss can benefit from hearing aids. In fact, most people who currently wear hearing aids say it not only helps their overall ability to communicate effectively in most situations, but it also has a positive effect on their relationships. Most hearing aid users in the workforce even say it has helped their performance on the job.

Other research shows that addressing hearing loss can help protect your earnings. One study showed that the use of hearing aids reduced the risk of income loss dramatically—by 90-100% for those with milder hearing loss, and from 65 -77% for those whose hearing loss was severe to moderate.

What’s more, people with hearing difficulty who use hearing aids get more pleasure in doing things and are even more likely to exercise and meet up with friends to socialize!

Men who want to maintain a healthy, fulfilling lifestyle should know that new technological advances have revolutionized hearing aids in recent years. Today’s hearing aids can automatically adjust to all kinds of sound environments and filter out noise. Many are virtually invisible, sitting discreetly and comfortably inside the ear canal. Some are even waterproof, and others are rechargeable. Best of all, many are wireless, so you can stream sound from smartphones, home entertainment systems and other electronics directly into your hearing aid(s) at volumes just right for you.

5 Men’s Health Motivators for Getting a Hearing Test:

1. Your hearing may say something about your heart. Cardiovascular and hearing health are linked. Some experts say the inner ear is so sensitive to blood flow that it’s possible that abnormalities in the cardiovascular system could be noted here earlier than in other less sensitive parts of the body.
   

2. Hearing loss is about twice as common in people with diabetes. Studies show that people with diabetes are about twice as likely to have hearing loss. When broken down by age, one study showed that those 60 and younger are at greater risk.
   

3. Addressing hearing loss may benefit cognitive function. Research shows a link between hearing loss and dementia, leading experts to believe that interventions, like hearing aids, could potentially delay or prevent dementia. Research is ongoing.
   

4. Hearing loss is tied to sleep apnea. Research shows that sleep apnea is significantly associated with hearing loss at both high and low frequencies. Findings suggest that sleep apnea is a systemic disease and is associated with an increased risk of hearing loss, along with a number of diseases like diabetes, high blood pressure, heart disease, and stroke.
   

5. Hearing loss is tied to depression. Studies show that hearing loss is associated with an increased risk of depression in adults of all ages, but is most pronounced in 18 to 69 year olds. Research also shows that the use of hearing aids reduces depressive symptoms.

BHI and HHF are encouraging men of all ages to take a free, quick, and confidential online hearing check at BetterHearing.org to help determine if they need a comprehensive hearing test by a hearing healthcare professional.

The content for this blog post originated in a press release issued by The Better Hearing Institute on June 3, 2015. 

Les Paul, Wizard of Waukesha

By Sue Baker

Les Paul often said he learned everything he needed to know while growing up in Waukesha. I met Les Paul when I worked at the Waukesha County Museum, and wanted to put together an exhibit about him. We became good friends for what would be the last 10 years of his life (he died in 2009), and these are just some of the stories he told me.

Lester Polsfuss was born June 9, 1915, in Waukesha, Wisconsin, a city 20 miles west of Milwaukee, to a family with strong German roots. From the time he was a preschooler, Les was encouraged by his mother, Evelyn, to entertain. Les recounted how when he was 5 years old the Rotarians would lift him to a tabletop during their meetings so he could sing for them. By the time he was 8, Les disassembled a harmonica he was given to see how it worked. After reassembling it, he filled the family home with the sounds of endless harmonica practicing.

Radio was brand new when Les was growing up and he couldn’t get enough of listening to the guitar-playing country singers. When the singers came to town, Evelyn took Les to see them perform. Les wanted to be just like the guitar player Pie Plant Pete, and when the performer came to Waukesha not only was Les in the audience, he was dressed just like Pie Plant Pete, who performed in “Showboat” in a sailor suit. In fact, years later Les changed his performance name from Red Hot Red to Rhubarb Red. (“Pie plant” is another name for rhubarb.)

But playing his guitar and harmonica, and singing and telling jokes, was not enough for Rhubarb Red. As a teen, Les loved his guitar but he was sure it could sound better. After inventing a harmonica holder so his hands were free, Les pondered how to improve his guitar so he could hear just the vibration of the strings. So Les stuffed socks, rags, and a tablecloth inside the guitar. The sound was different but not quite what he wanted. Next, he filled the guitar with plaster of Paris—and that was the end of his guitar. 

Les wanted the densest material he could find to build a guitar so that only the strings would vibrate. He tried a two-foot piece of discarded iron train rail. He stretched a single guitar string down the length of the rail and plucked it. What sustain! It was crisp and just the string vibrated and it vibrated a long time. It was exactly what he wanted. He ran to share his great discovery with his mother. The usually supportive Evelyn looked at her son and said, “The day you see a cowboy riding a horse with a piece of rail…” Les knew she was right, but he also knew he had the beginning of something big.

Teenage Les was playing all over Waukesha and the surrounding area. He wanted to hear what his audiences were hearing so he built a disk-recording machine using a flywheel from his dad’s car dealership, a rubber belt from his dentist, and aluminum disks for recording. By now, Les was performing on the radio and his mother would capture his performances on his recording machine. 

Just outside of Waukesha was Beekman’s Barbeque, a popular destination. Les became a regular, playing for tips. Les was constantly honing his guitar playing. He built his own amplifier using parts of his mother’s telephone and radio. When someone in the back said they couldn’t hear his guitar, Les created his first electric guitar with parts from the family’s phonograph and another radio.

In the mid-1930s Les was playing country (hillbilly) music on Chicago radio stations as Rhubarb Red. He spent his nights learning jazz at clubs in Chicago and soon was performing with the musicians. It was in Chicago that he took the name Les Paul when he played jazz.

As a teen, Les played at Waukesha’s band shell, now known as the Les Paul Performance Center. It is being renovated in time for the centennial anniversary of his birthday this June 9.

Learn more about the legendary Les Paul and the launch of the Les Paul Ambassador program, a partnership with Hearing Health Foundation to spread the message of hearing protection. The Les Paul Ambassdaors are guitarist Lou Pallo, saxophonist Chris Potter, jazz pianist John Colianni, and DJ and composer Chill Kechil who was in our magazine as well as in our blog. For the full list of events to celebrate Les Paul’s 100th birthday, see les-paul.com.

Sue Baker is the program director at the Les Paul Foundation. 

The Path to a Cure for Hearing Loss and Tinnitus

By Laura Friedman

On May 21, 2015, Hearing Health Foundation hosted its first live-video research briefing as part of our effort to provide regular updates on our research programs and progress. Through these briefings, our goal is for our attendees to obtain new information and understanding about hearing loss, prevention and research toward a cure.

During this inaugural research briefing, Dr. Peter Barr-Gillespie, Scientific Director, Hearing Restoration Project presented the Hearing Restoration Project (HRP). The HRP was founded in 2011 and is the first and only international research consortium focused on investigating hair cell regeneration as a cure for hearing loss and tinnitus. The overarching principle of the consortium is collaboration: open sharing of data and ideas. The HRP consortium consists of 14 of the top investigators in the audiological space, as well as a scientific director, Dr. Barr-Gillespie.

We wanted to share with you highlights from the presentation, which is available to watch with live captioning or to read with notes summarizing each slide. 

1. History of Hearing Health Foundation

   • Founded in 1958, established reputation for pioneering breakthroughs in hearing and balance research.

     • Early supporters of the revolutionary cochlear implant. Today, over 220,000 children and adults benefit.

     • Advocated for the passage of Universal Newborn Hearing Screening legislation in the 1990s. Today, 97% of newborns are tested for hearing loss at birth.

     • The Emerging Research Grants Program provides seed funding for researchers in hearing and balance science such as discoveries in hair cell regeneration, tinnitus, hyperacusis, and Ménière’s research. 

2. The Challenge

   • In the past century, the primary treatment for hearing loss has been hearing aids and cochlear implants. While these have been very successful treatments, they have limitations.

   • For this century, we have a number of different avenues for more effective therapy. 

     • Preventing the damage to the hair cells to preserve hearing. By generating greater awareness of the effects of hearing loss, we aim encourage people of all ages to protect their ears.

     • Gene therapy, targeting those who have lost hearing due to genetic disorders.

     • The majority of people who have lost hearing have done so through noise damage or aging, and may be candidates for hair cell regeneration/restoration.

3. HRP Consortium History & Model

   • One of the key facets of the HRP’s approach is that we use three different animal models for studying hair cell regeneration

     • Two of those models, the chick and the zebrafish, show robust hair cell regeneration.

       • f you damage the hair cells of a chick or a fish, within a short time—only a day or two for the fish, a few weeks for the chick—the hair cells come back; new hair cells are formed.

         • So, that's spectacular, because it tells us that animals are capable of regenerating hair cells.

     • y contrast, the mouse is our other experimental model. Like in the human, the mouse shows no hair cell regeneration after a few days following birth.

       • You can damage the hair cells in the mouse and as far as we can tell, nothing much happens in terms of restoring hair cells. So, if we can figure out how to regenerate hair cells in the mouse, then we will be able to regenerate hair cells in people.

4. HRP Strategic Research Plan

   • Our strategic plan consists of three separate phases. We have already made a lot of progress on Phase 1 and we have initiated Phase 2:

     • Phase 1 – Discovery research:  Compare the fish, chick, and mouse to discover pro- or anti-regeneration pathways and determine supporting cell fates.

     • Phase 2 – Pathway validation: Verify pathways using fish, chick, and mouse models and describe regeneration strategies.

     • Phase 3 – Develop therapies and treatment options: Identify drugs that trigger hair cell regeneration in the mouse model.

5. Progress To-Date

   • Progress on Phase 1: We've identified a variety of candidates for hair cell regeneration and the pathways that are necessary. 

     • We have too many, so we really are continuing to use bioinformatics methods to winnow down and determine which are most important.

     • We have definitively shown, at least in the mouse, the specialized supporting cells remain.

     • We know now what our target cells are for triggering hair cell regeneration. 

   • Phase 2 has begun, but we haven’t stopped Phase 1: 

     • We've got multiple approaches to try and see whether or not we can block regeneration in the fish and chick or stimulate regeneration in the mouse.

   • Phase 3 is in sight:

     • Experimental models from Phase 2 will be used to screen for drugs—using the mouse first

6. The Next Five Years

   • With your help, we can continue to quicken the pace towards a cure. Here’s our plan for the next five years: 

     • Phase 1 will continue: more candidate generation for Phase 2

     • Phase 2 (pathway verification) already initiated in zebrafish, mouse, chick (low throughput)

     • Phase 2 must be scaled up: many more genes, combinatorial approaches; cell lines for screening

     • Phase 3 (drug screening) requires the right screening model, which will come out of Phase 2.

The Future is Very Bright – But we need your support!

Hair cell regeneration is a plausible goal for eventual treatment of hearing and balance disorders. The question is not if we will regenerate hair cells in humans, but when.  However, we need your support to continue this vital research and find a cure! Please make your gift today.  

Advocacy at Work

By Kathi Mestayer

Ever had a hard time hearing in the hospital? Or visited someone who did? You're in good company. Hospitals can be very noisy, with alarms, footsteps, noise from machines, televisions, and clanging carts and food trays. For starters.

And I would argue that the hospital is one of the most important places to hear things right. The two phrases "So, are you in any pain now?" and "You know what to do if you're in any pain, now?" can sound really, really similar to a person just out of surgery and who uses a cochlear implant.

In this actual situation, the patient—my father—answered, "Yes," which meant two very different things to each party. The nurse thought "yes" meant “Yes, I know what to do if I'm in pain," but what it really meant was, "Yes, I am in pain now." Over the course of 45 minutes, after multiple attempts by the patient to get help via the intercom system to contact the nurse, and the nurse using the intercom system to ask what the problem was, it became clear that:

1. He was now in a lot of pain.

1. The intercom system doesn't work for people who are very hearing impaired. He never heard a single word through the intercom system from the nurse's station, and was reduced to moaning louder and louder until help arrived, in person.

This is the kind of problem that the Department of Justice (DoJ) program called the Barrier-Free Healthcare Initiative is intent on addressing. The DoJ, which oversees compliance with Americans with Disabilities Act (ADA), has committed resources and attention to the important work of providing effective communication for patients with hearing loss in hospitals, pharmacies, rehab facilities, and doctors’ offices.

My home state, Virginia, has jumped on board with this initiative, and passed its own initiative to assist hospitals with ADA compliance. Signed by Governor Terry McAuliffe earlier this year, Chapter 113 reads as follows:

Be it enacted by the General Assembly of Virginia:

That the Department of Health shall (i) work with stakeholders to develop guidelines for hospitals to ensure that hospitals are complying with requirements of the Americans with Disabilities Act and that patients and family members with sensory disabilities are able to communicate effectively with healthcare providers and (ii) report on its progress in developing such guidelines to the General Assembly no later than December 1, 2015.

The bill would not have made it to the floor, much less the printer, had it not been for the efforts of Arva Priola, the outreach coordinator for the deaf and hard of hearing at the disAbility Resource Center in Fredericksburg, Virginia. Priola, who wears cochlear implants, saw the need firsthand from her experience in the recovery room. “I always direct the nurses to mark my cochlear implants so they are put into the correct ears. That’s also true for hearing aids. It allows us to use our hearing sense when we wake up from a procedure,” she says.

Virginia’s initiative is intended to help hospitals comply with the requirements of the ADA. Those requirements include (in layman's terms):

• assessment of each patient's communication needs

• provision of the technology or other assistance (for patients who are deaf, this is often ASL interpreters)

• covering the costs, if any, of providing that assistance

Priola not only saw the need for such an effort by the state but also consulted with agencies and stakeholders, such as the Hearing Loss Association of America's Virginia Chapter, the Virginia School for the Deaf and Blind, Association for Late-Deafened Adults, the Virginia Association for the Deaf, and the Virginia Department for the Deaf and Hard of Hearing (where I serve on the advisory board). She then approached Delegate Robert "Bobby" Orrock (VIrginia House of Delegates) to sponsor the measure. He agreed.

The rest is history. Strong support (unanimous, in fact) was received in both legislative chambers, and the governor signed it in March. The ball is now in the Virginia Department of Health's court, since it is designated as the agency to make it happen (along with the stakeholders and cooperating agencies that will be involved in implementation).

It's a positive note, not only for Virginia and its governing bodies, but for demonstrating what we can do as advocates when we really put our minds, and energies, and focus, to work. And when we have a mover and shaker like Arva Priola.

The DoJ's ADA website is full of information about its national initiative. Click here to see some of its recent actions in enforcing the ADA for communications issues in all healthcare settings, not only hospitals.

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. She writes about the science of how our brains make sense of sound at BeaconReader.com.

Ages and Stages

By Maureen Plain

Effective communication is one of life’s greatest pleasures, and for those of us with typical hearing, we so often take this gift for granted. The process of verbal communication starts with being able to hear what is said. We then apply knowledge of the words we hear, so we can decipher its message.

When crafting a response, we must have the words in our repertoire of vocabulary readily available, so we can express our thoughts and then pronounce the words clearly enough to be understood by others. As we verbalize our response, grammatical markers for our words, intonation patterns, and understanding the social setting helps us share our thoughts effectively. This process of verbal communication begins in infancy with the use of eye contact, sounds, and body language, and continues throughout our lives. 

For those with hearing loss, all of this can be especially challenging.

Imagine the challenge for a 3-year-old girl who has just been diagnosed with a moderate to severe sensorineural hearing loss in both ears. For three years until diagnosis, she communicated entirely by reading body language and other physical cues. But how did this affect her ability to effectively communicate with others in the hearing world?

In the first couple years of life, caregivers (i.e. parents, nannies, and family members) are acutely aware of small children and often times face them when speaking. This girl’s caregivers did not realize she was solely dependent on reading body language and other physical cues, including lips, to communicate. As she got older, her mother noticed that when faced away, her daughter was unresponsive. After many misdiagnoses, this little girl and her family were told she had hearing loss.

Fortunately this child has a dedicated family that responded to her diagnosis with unwavering commitment, and immediately started designing a team of professionals to support the best learning possible, which included top-notch audiologists and a speech-language pathologist/auditory therapist. The school district provided great support and I was fortunate to be this child’s speech/language pathologist from the very beginning, working with her a few times each week from age 3 until she graduated from high school. 

Vocabulary growth and clear and proper pronunciation of words were always a crucial components of our therapy, as well as benchmarking and tracking progress. A toddler needs vocabulary to engage in play and to learn, just as a teenager needs vocabulary to navigate school and friendships.

At every age, building vocabulary with a person with a hearing loss is multifaceted and hard work. First we worked on hearing and saying each word until it became familiar enough to begin the process of developing listening for each word. We paid extra attention to words with syllables extremely difficult for her to hear, in this case those that are considered high-frequency (S-, SH-, CH-,TH-, -ECT.).

As the child grew older, she spoke intelligibly but would often leave off the ending syllables, such as a plural “s” because she was repeating only the sounds she heard. We worked on learning grammatical marker patterns so she would pronounce a plural “s” based on grammatical rules. Practicing the articulation for each sound component in a word is also very challenging, since a hearing loss makes it difficult to hear your own speech sound errors and correct them. We also used an oral motor approach to complement our work on sound production. We focused on how a sound felt when it was pronounced; where her lips, tongue and jaw belonged to articulate different sounds properly. We also practice oral vs. nasal air flow and practiced making sounds in front a mirror to see how it looked. Through this process, she developed speech that is clear and understood by all listeners, in addition to being able to use verbal cues and grammatical markers that she has been trained to watch for, but was otherwise unable to hear.

We also integrated speech therapy with auditory training exercises. Listening practice included her looking and listening when I was visible, and repeating the sounds she heard back to me. Once she mastered that, I then moved farther away from her sight to advance this skill. We then moved to improving listening without looking; while still facing her, I covered my mouth with a paper to develop better listening and discrimination of sound. As accuracy improved, I then would sit behind her and continually moved farther away until I was 6 feet away, with her back facing toward me. We also practiced listening to the word so it can be recognized in quiet and in noise.

Listening skills were paired with our work on vocabulary comprehension to enhance communication. Beyond pronunciation and listening, learning the definition of each word to build comprehension is crucial. Vocabulary shapes our conversation, and since mishearing words is a constant challenge for those with hearing loss, they must rely on their vocabulary knowledge to fill in the gaps for greater conversation participation and quality of life.

These are just a few of the many strategies that we used to improve speech, language, and listening during our 15 years together. To succeed she, as well as her parents and family, needed to fully trust the process and integrate these strategies into her everyday life. While therapy was only 2-3 hours a week, speech and verbal communication is a chosen part of her everyday life.

Therapy was a lot of hard work and sometimes frustrating for her, as not every milestone was easy. Until there’s a cure for her type of hearing loss, she will not have the ability to hear as seamlessly as those with typical hearing or have perfect speech. 

May is Better Speech and Hearing Month and serves as reminder that verbal communication is a shared pleasure that brings us laughter, learning, and love. We should take this month to treasure the gift of communication and celebrate the hard work and success of all individuals whose ability to communicate is challenged, for whatever reason.

Maureen Plain M.S., CCC-SLP, is a speech-language pathologist with 37 years of experience in the greater NY area. She is currently a Program Director for Sunny Days Consulting Services in New York.