Ask the Scientist: Gene Therapies and Hearing

By Peter G. Barr-Gillespie, Ph.D.

A DNA double helix

National Human Genome Research Institute

Recently, Hearing Health Foundation (HHF) has received several questions regarding the Reuters report on gene therapies for hearing. There are two separate but related topics raised in this article. As the scientific research director of HHF’s Hearing Restoration Project, which since 2011 has been uncovering concrete discoveries toward a biologic cure for hearing loss and tinnitus, I want talk about each individually, and then discuss what I interpret they mean together.

The article first presents the Science Translational Medicine paper from Jeffrey Holt’s lab. This is very much a proof-of-principle report, focused on an animal model and using a time for delivery of the corrected gene that is extremely early in development (equivalent to a 5-to-6-month-old human fetus). It is important to point out that their strategy will only correct one type of genetic hearing loss and genetic hearing loss from mutations in other genes will require related but different strategies. Nevertheless, this is an exciting example of modeling gene therapy in animals, and represents a logical progression toward that goal in humans.

The article then moves on to reference the Novartis trial. For this trial, they are using a similar technical strategy, viral delivery of a gene, but they are targeting people—those who have lost their hearing through non-genetic means, such as noise damage, aging, or infections. The gene they are delivering, known as ATOH1, may stimulate production of new hair cells; it is a gene that is essential for formation of hair cells during development, and in some experimental animal models, delivery of the gene can lead to production of a few hair cells in adult ears.

That said, many people who I have talked to in the field who work with experimental models of hair cell formation using ATOH1, including members of our Hearing Restoration Project consortium, believe that this trial is premature. By and large, the animal models do not support the trial; most suggest that there will be few hair cells formed and little hearing restored. While we can hope for a little bit of hearing recovery, we are concerned about toxic responses to the gene delivery using viruses. Personally, while I think it would be truly fantastic if the Novartis trial works, at this moment in time I don’t think the rewards yet outweigh the considerable risks being imposed on a human (include safety during the procedure and potential side effects afterward).

Still, the Novartis trial will tell us about the safety of viral delivery into the ears of humans, and knowing that is critically important. I think the most likely outcome is that we will learn whether the strategy the Novartis trial used to deliver the gene is safe. Unfortunately, if we don’t see improved hearing, we won’t know why—did the gene not get to the right place, or does it just not work?

Technical aspects of gene delivery are what ties together the Novartis work and the Holt lab work. Both use viruses for delivering genes, and together the results from these and others will let us know, from a procedural standpoint, how we can deliver genes to the ear. I think it is unlikely that delivering just ATOH1 will do the trick of restoring hearing; it may be that we need to deliver other genes or to use drugs to overcome the block we see to making new hair cells.

So while these are exciting reports to hear about, especially that Novartis is actually carrying out a trial in humans, it is still premature to think that this is going to be a viable strategy for restoring hearing. This is why Hearing Health Foundation's Hearing Restoration Project is doing everything possible to accelerate the pace of its research.

Hair cell regeneration is a plausible goal for the treatment of hearing and balance disorders. The question is not if we will regenerate hair cells in humans, but when. 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.

I Lost My Hearing in My Forties. Here's How I Handled It.

By Mary Louise Kelly

The interesting thing about going deaf is you don’t realize it’s happening. It’s impossible to pinpoint when everyone began to mumble, when you ceased hearing your own footsteps clicking down a hall.

“Is it the accents?” my husband asked when I complained that the actors on Downton Abbey spoke too fast. We started watching with subtitles. At the theater, I focused on the beauty of the sets and costumes because—though I would have denied this—I couldn’t follow the dialogue. Meanwhile, car horns and sirens dimmed. Packages didn’t arrive, yet the UPS man insisted he’d rung the bell three times. “Impossible,” I shot back. “I was home.”

My lowest moment came last spring at a reading to promote my first novel. A woman rose and recounted what I later learned was a risqué tale about a CIA spy (the book was an espionage thriller), then asked a question that had the audience in stitches. I squirmed, laughed along, and responded with what was surely a non sequitur, as I’d caught barely a word of what she’d said. In the taxi home, I thought: Enough.

Still, none of this prepared me for sitting in an audiologist’s office at age 43, being told that I suffered severe hearing loss. How severe? In one test, he stood across the room, spoke a series of words in a normal voice, and asked me to repeat them.

“Void,” he said.

“Void,” I repeated.

“Ditch,” he said.

“Ditch.”

Out of 20, I got 16. “Not perfect,” I sniffed, “but hardly severe.”

He repeated the test, now holding a sheet of paper before his face.

“Mumble,” he said.

“Um . . . repeat that one?”

“Mumble mumble.”

“Shoelace?”     

“Nope. Mumbledy mumble.”

This time, I got 6 out of 20. When I couldn’t see his lips move, I missed 70 percent of what he said. 

“How are you even functioning?” he inquired, genuinely mystified.

As a reporter, I’ve spent time on aircraft carriers, in helicopters, in war zones. For two decades, I’ve edited stories on deadline through headphones cranked too loud. But the most likely explanation for my hearing loss? Genetics. My father is hard of hearing. So are his sisters and 96-year-old mother. I’ve long known what loomed in my future—I just hadn’t expected it so early.

My first day with hearing aids, I went about my routine with a sense of wonder. It was astonishing to rediscover that pop songs had words I could sing along to. “Have been bopping to an ’80s dance mix all morning,” I posted on Facebook. “I challenge anyone to deny Debbie Gibson was a genius ahead of her time.” (To which came the inevitable reply: “You need to get your hearing checked.”)

By day two, I was on sensory overload. Starbucks left me near tears—I’d had no idea frothing milk made such a racket. I jogged in Rock Creek Park and for the first time in years didn’t jump every time cyclists whizzed past, because I could hear them coming.

The doctors can’t say whether my hearing has stabilized or will worsen. And hearing aids are an imperfect solution. The experience is different from, say, getting glasses and instantly being able to see. It takes time for the brain to adjust, to relearn the pathways it once knew. You almost never recover all that has been lost.

But you do learn to savor small triumphs. The other day, the UPS driver rang my doorbell and I heard him—and tipped big. I still can’t watch TV without subtitles. But at a play recently, the curtain rose and I slumped in sheer relief at being able to follow the words. Not every line, but enough. I’m holding onto that theater program, a memento of a pleasure once dimmed, now mine once more.

Mary Louise Kelly is a contributing editor at The Atlantic. She has spent two decades reporting on national security and international affairs for NPR and the BBC. As NPR’s intelligence and defense correspondent, she covered wars, terrorism, and rising nuclear powers. Kelly has also anchored NPR programs including “Morning Edition” and “All Things Considered.” She has taught national security and journalism at Georgetown University. Kelly’s writing has appeared in The New York Times, The Washington Post, Newsweek, Politico, and others. She is the author of two novels: Anonymous Sources and The Bullet.

Taking Hair Cell Regeneration Down a Notch

By Andy Groves

Hearing Restoration Project (HRP) consortium scientists, Andy Groves and Stefan Heller had their research published in Frontiers in Cellular Neuroscience on March 31, 2015. Below is a summary of their research:

Sensorineural hearing loss is most commonly caused by the death of hair cells in the organ of Corti, and once lost, mammalian hair cells do not regenerate. In contrast, other vertebrates such as birds can regenerate hair cells by stimulating division and differentiation of neighboring supporting cells.

During the development of the inner ear, newly-born hair cells send signals to their neighbors that instruct them to not become hair cells, but to become supporting cells instead. Hair cells are the mechanosensitive cells of the ear. Supporting cells surround them and as their name implies, physically support them and help regulate some of the properties of hair cells. One of these signals is an evolutionarily ancient pathway, the Notch signaling pathway. We and others have shown that if you block the Notch signaling pathway in the cochlea or balance organs of young mice, the supporting cells no longer get the message to stay as supporting cells, and instead they transform into hair cells. This process also happens during hair cell regeneration in birds - supporting cells transform into hair cells, which then send a Notch signal to their neighbors and prevent too many hair cells from being formed.

These observations suggest that it might be possible to block Notch signaling in mature, deafened animals as a means of getting new hair cells to form. We performed a simple experiment to test this in progressively older and older animals. To our surprise, we found that once mice are more than a week old, blocking Notch signaling has no effect on the cochlea any more, and no new hair cells are made.  We showed that this was due in part to components of the Notch signaling pathway being switched off in the ear as the animals get older. Viewed this way, the Notch signaling pathway can be thought of as a “Scaffold” - it is used to allow the cochlea to be built in the first place, but is then dismantled once the cochlea becomes functional.

What does this mean? It suggests that inhibiting Notch signaling alone is unlikely to be an effective means of hair cell regeneration in mammals. It is possible that other factors will be required, and some HRP members are busy testing these other pathways right now. It will also be of great interest to understand HOW the Notch pathway is dismantled with age, whether we can exploit this in future therapies.

Read more about this research proposal here: http://hearinghealthfoundation.org/hrp-consortium-projects-groves-segil-stone.

This work was supported by Department of Defense Grant DODW81XWH-11-2-004(AKG) and Hearing Restoration Project consortium grants from the Hearing Health Foundation (AKG and SH), NIH grant DC004563 (SH), NIH grant P30DC010363 (SH, JSO), and NIHR01DC014450 (JSO).

Hair cell regeneration is a plausible goal for the treatment of hearing and balance disorders. The question is not if we will regenerate hair cells in humans, but when. 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.

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.