Unraveling Genes Critical for Inner Ear Development

By Albert Edge, Ph.D., and Alain Dabdoub, Ph.D

The goal of the Hearing Restoration Project (HRP) is to determine how to regenerate inner ear sensory cells in humans to eventually restore hearing for millions of people worldwide. These sensory cells, called hair cells, in the cochlea detect and turn sound waves into electrical impulses that are sent to the brain. Once hair cells are damaged or die, hearing is impaired, but in most species, hair cells spontaneously regrow and hearing is restored. The HRP is aiming to enable this ability in humans. 

All cells develop through a chain of events triggered by chemical signals (proteins) from outside the cell. The signals kick off responses inside the cell that can change the cell’s ability to proliferate (grow and divide) and differentiate (take on specialized functions).

The Wnt signaling pathway, a sequence of events triggered by the Wnt protein, helps guide inner ear cell development, including the proliferation of cells that differentiate into the hair cells and supporting cells necessary for hearing and balance. But in mice and other mammals, inner ear cell proliferation does not continue past newborn stages.

Underscoring their importance in evolutionary terms, Wnt signals occur across species, from fruit flies to humans—the “W” in Wnt refers to “wingless”—and Wnt signaling is guided by dozens of genes. Albert Edge, Ph.D., Alain Dabdoub, Ph.D., and colleagues performed a comprehensive screen of 84 Wnt signaling-related genes and identified 72 that are expressed (turned on) during mouse inner ear development and maturation. Their results appeared in the journal PLoS One this February.

The Wnt signaling network has three primary pathways. Two are known to be integral to the formation of the mammalian inner ear, including the determination of a cell’s “fate,” or what type of cell it ultimately turns into. This is particularly significant because the inner ear’s sensory epithelium tissue is a highly organized structure with specific numbers and types of cells in an exact order. The precise arrangement and number of hair cells and supporting cells is essential for optimal hearing.

The relationship between the Wnt-related genes, the timing of their expression, and the various signaling pathways that act on inner ear cells is extremely complex. For instance, the composition of components inside a cell in addition to the cell’s context (which tissue the cell is in, and the tissue’s stage of development) will influence which pathway Wnt signaling will take. It is known that inhibiting the action of Wnt signaling causes hair cells to fail to differentiate.

The new research complements previous chicken inner ear studies of Wnt-related genes as well as a recent single-cell analysis of the newborn sensory epithelium in mice (conducted by HRP scientist Stefan Heller, Ph.D., and colleagues). Comprehensively detailing these 72 Wnt-related genes in the mouse cochlea across four developmental and postnatal time periods provides a deeper understanding of a critical component of hair cell development, bringing the HRP closer to identifying genes for their potential in hair cell regeneration.

Your Support Is Needed!

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.  

Brush with Hearing Loss Inspires Young Artist's Vision to Help Others

WESTPORT — A young artist appealed to people’s sense of sight at his first solo art show Saturday to raise funding for research to cure hearing loss.

Alex Mussomeli, 11, a student at Long Lots School, who uses a Cochlear implant in one ear and a hearing aid in the other, sold 51 of the 54 acrylic paintings he displayed in the show, titled, “The Wonder of Art Exploration!” It raised approximately $16,000 for the New York City-based Hearing Health Foundation. According to the HHF website, it is the largest non-profit funder of hearing research and its aim is “to prevent and cure hearing loss and tinnitus through groundbreaking research and to promote hearing health.”

“Our mission is to fund research through a consortium of scientists to find a cure for hearing loss. We have a Hearing Restoration Project,” said Roger Harris of New Canaan, an HHF board member, who attended Alex’s art exhibit at the Fairfield County Hunt Club.

Harris said Alex is “a very gifted young man,” and he is impressed with the boy’s philanthropic efforts. “It was his idea to have this show. To have someone at his age be so socially aware is really amazing.”

“This is my first time exhibiting. It feels pretty special and exciting,” said Alex, who worked for a year to produce his colorful paintings, prints and notecards, many of them with floral and spring themes: “Enchanted Flowers,” “The Tulip Playing the Note,” “The Dreamy Sunflower,” “The Bright Butterfly” and “A Spring Dream.”

“They’re full of life and my happiness,” he added, observing that painting is “my favorite thing to do.”

“Spring after the Storm” was fifth-grade classmate Caroline Motyl’s favorite. “I like the name and the color best because I think it’s really creative,” the 10-year-old said, adding that Alex’s project is “a really brave thing to do. It’s nice because he’s helping other people,” she said.

Alex said he was inspired to use his artistic talent for a good cause after seeing a “CBS Sunday Morning” story about a legally blind artist, Jeff Hanson, who has raised $1 million for charities like the Make-a-Wish Foundation and a children’s hospital through the sales of his paintings.

“I felt like ‘Why couldn’t I do the same for hearing so other kids like me can hear?’ I like to talk to my parents about my day and listen to music,” said Alex.

Alex with Roger Harris

Nada Mussomeli, Alex’s mother, said he was 3 months old when his hearing loss was detected in both ears due to an enlarged vestibular aqueduct, an inner-ear malformation. He received a Cochlear implant in the right ear when he was 3 years old and also has a hearing aid in his left ear.

“My parents wanted me to be mainstreamed so I got a hearing aid, but we learned that the implant is stronger,” Alex said.

Alex uses his senses of hearing and vision as he paints. In his artist’s statement Alex said, “Sometimes I get inspired by something I see.” He also listens to music, sometimes relaxing and sometimes stirring compositions. “I like music full of expressions. I always have music on when I paint.” Ideas dance in his head as his brush dances on the canvas, he said.

Hundreds of people turned out for Alex’s show, among them John Hansen of Fairfield, who said he appreciates the young artist’s use of color, diversity within in the field of plant life and positivity.

Family friend Carol Mueller of Westport said Alex “is an inspiration to all who know him. Alex loves art and enjoys visually expressing his optimism through his painting.”

Nada and Adam Mussomeli said they could not be more proud of their son. Nada Mussomeli said she is inspired watching him do his art and enjoy every minute. “It was a journey.” She also said her dream is that a cure for hearing loss is discovered in her lifetime, but if not in hers then in Alex’s.

This article originally appeared in Westport News on April 12, 2016. It was repurposed with permission. 

10 Signs and Symptoms of Ménière's Disease

By Catherine Roberts

According to the Mayo Clinic, Ménière's Disease affects the inner ear and causes bouts of vertigo due to fluid that fills the tubes of your inner ear. On top of the dizziness and nausea, flare ups can also cause some loss of hearing in one or both ears and a constant ringing sound. You might not be able to drive, work, or do regular daily activities at times, though this chronic condition varies greatly from person to person. Unfortunately, the disease is not curable, but there are many treatment options that successfully help manage the disease.

Keep an eye out for these ten signs and symptoms of Ménière's Disease, and see your doctor if you experience any of them…

1. Dizziness
   Dizziness can be caused by a lot of different diseases and infections, but it’s one of the main symptoms associated with Ménière's disease. Your inner ear is responsible for your balance and since the disease attacks the tubes in your inner ear, dizziness is bound to occur when experiencing a flare up of the disease. If you experience dizziness as a result of an attack of Ménière's disease, it may cause you to unsteady on your feet and feel like the world is moving unsteadily around you. In severe cases, dizziness can make you fall over or trip while walking, making it dangerous to be driving, working, or even simply walking.
   
   

2. Nausea
   Nausea is another common symptom. Feeling sick to your stomach and actually vomiting can easily occur when you’re experiencing severe dizziness as well. When attacks of the disease come on, symptoms of vertigo are felt for as little as half an hour to as long as a full 24 hours. Though these attacks are seemingly short-lived, nausea and other symptoms can be felt strongly during the whole flare up. Although a symptom like nausea is extremely inconvenient and unpleasant to deal with, there are over-the-counter medications for motion sickness and prescription anti-nausea medications that your doctor can provide that could greatly help manage the attack. There are also some antibiotics that can be injected into the ear to help relieve symptoms if necessary.
   
   

3. Hearing Loss
   Though usually temporary and in one ear, permanent hearing loss happen to some over time. Many patients describe the hearing loss as if their ear is plugged—sound can seem distorted, fuzzy, far away, or even tinny. Sensitivity to sound is also common, and finding a way to successfully remove the fluid helps improve any hearing problems the patient suffers from.
   
   

4. Ringing In the Ear (Tinnitus)
   According to the Mayo Clinic, Ménière's disease can cause tinnitus, unfortunate sounds in the ear described as ringing, hissing, roaring, buzzing or whistling. Since the disease affects people in different ways and with varying symptoms, some patients may experience tinnitus without vertigo and hearing loss, or a combination of several symptoms. While not painful, any sort of foreign sound like ringing in the ear can be really difficult to cope with.
   
   

5. Uncontrolled Eye Movements
   Some people who have the disease experience a sort of jerky eye movement in one or both eyes, side to side, up and down, or in a circular motion. It varies depending on the patient and each attack. The inner ear directly affects balance, and in turn, the movement of your eyes. Tests are done to check the response of your eyes when your inner ear balance is changed.
   
   

6. Diarrhea
   Those who experience diarrhea might be more likely to also be experiencing vertigo during the same attack. To make matters worse, diarrhea can continue to occur after flare ups, not just during them. This makes it very important for you to stay hydrated when you’re struggling to cope with these symptoms of Ménière's disease. Since vertigo can cause you to throw up, the combination of vomiting and diarrhea will make you extremely dehydrated, and cause other health problems if not corrected. Along with diarrhea, abdominal pain and other gastrointestinal discomfort is possible.
   
   

7. Cold Sweats
   When in the middle of an attack, people who experience vertigo symptoms—nausea, dizziness, and even vomiting—could also develop cold sweats, adding another uncomfortable symptom to deal with. Since cold sweats are typically a result of vertigo in the case of Ménière's disease, vertigo medication could eliminate or greatly reduce this and other vertigo symptoms. Depending on how severe your episodes are, your doctor may prescribe an anti-nausea medication to reduce the harmful symptoms of the attacks.
   
   

8. Feelings of Fatigue
   It’s thought that those with Ménière's disease are more sensitive or susceptible to fatigue, increasing the risk of falling prey to an attack. Some patients have also shown that fatigue could be what causes flare ups, so people with the disease should adjust their lifestyle as needed to reduce the risk of becoming too tired. Work on sleeping well and not over-working or over-extending your capabilities.
   
   

9. Extreme Mood Changes
   People with Ménière's disease have claimed feeling a variety of changes in their mood both during episodes and in-between them, from anger and irritability to anxiety and fear. These mood swings and feelings of instability can be caused by many things, so they aren’t necessarily from Ménière's disease. It’s also worth it to note that mood changes and roller coaster-type feelings aren’t necessarily a physical reaction from the disease—the Mayo Clinic explains that it’s currently unknown if anxiety contributes to and causes episodes or if anxiety is a by-product of the disease, occurring after attacks.
   
   

10. Migraines
    A sign of Ménière's disease that’s easy to overlook are severe headaches, specifically migraines. There are so many dietary and lifestyle causes of migraines that it’s no wonder this sign can be overlooked. That said, once you really think about the disease and what it attacks, it’s not so hard to see the connection. It’s thought that migraines can cause damage to the inner ear, and those with Ménière's disease are more likely to get migraines during flare ups.

Excerpts of this article was repurposed with permission of the author and originally appeared on activebeat.com January 23, 2016.

7-Day Challenge for Better Hearing Health

By Maggie Niu

In honor of National Public Health Week kicking off April 4, Hearing Health Foundation has seven challenges for you to complete over the course of next week, all related to hearing loss and hearing prevention. Help us celebrate by completing our list of challenges below and sharing your experiences in with us in the comments.

On your mark… get set…GO!

Day 1: Make an appointment to get your hearing tested!

It is important to have your ears tested at least once a year, especially if you are experiencing any buzzing or ringing in your ears or unable to hear clearly. Don’t hesitate to make an appointment: Early intervention is key for preventing further damage.

Here is a directory for audiologists from the Academy of Doctors of Audiology. It is super-easy and quick to find an audiologist close to you. Simply type in your zip code and the radius you are willing to travel and bing, you have your list of audiologists.

Day 2: Reduce the volume on personal music devices to under 70% of the maximum.

We all know that unwanted noise is a nuisance so we try everything in our power to drown it out—either by turning up the volume of the music we're listening to, or talking louder. In the long run, does it benefit our hearing health? The answer is no.

Noise-induced hearing loss can occur gradually over time by listening to loud music or being exposed to loud environmental noises. We can’t always control ambient noise, but we can control personal earphone volume. Next time you are using your earphones on a high volume, remember that you are damaging your ears!

Day 3: Plan a fundraiser to help us find a cure for hearing loss and tinnitus.

Need some ideas? See examples of past events and ideas for creating your own event. Individuals, companies, organizations, sororities, and fraternities of all sizes have joined in our efforts, and we hope you will too!

Day 4: Keep a journal of the foods you eat and note the loudness of the environment you’re in. You may be surprised at what you find.

Noise can affect many things. It can cause stress and affect our mood, but would you believe that noise can affect your palate? A Cornell University study found, "…that in an environment of loud noise, our sense of taste is compromised. Interestingly, this was specific to sweet and umami tastes, with sweet taste inhibited and umami taste significantly enhanced," said Robin Dando, an assistant professor of food science. "The multisensory properties of the environment where we consume our food can alter our perception of the foods we eat."

Day 5: Eat this! Incorporate certain nutrients into your diet for optimal hearing health.

Now we know that noise can affect the taste of food we eat, but are there foods that can help our ears? Check out these five nutrients that can prevent or delay hearing loss. 

• Omega-3 fatty acids and vitamin D: Usually found in fish, both can have positive effects on hearing loss. Studies have shown that adults who ate fish twice a week had a 42% lower chance of facing age-related hearing loss than non-fish eaters.

  • Recipe idea: Grilled Salmon and Zucchini with Red Pepper Sauce
    

• Antioxidants and folic acid: You can find this in spinach, beans, broccoli, asparagus, eggs, nuts and liver. They can reduce the number of free radicals in your body, which can damage the nerve tissue in your ears.

  • Recipe idea: Skinny Taco Stuffed Peppers.
    

• Magnesium and potassium: Bananas, potatoes, artichokes, and broccoli are rich in magnesium and potassium. Magnesium can protect you from noise-induced hearing loss. Potassium is responsible for regulating the amount of fluid in your body.

  • Recipe idea: Creamy Garlic Pasta with Shrimp and Vegetables.
    

• Zinc: Zinc can be found in dark chocolate and oysters. It is important to include zinc because it boosts the body’s immune system and is responsible for warding off germs that cause the common cold and ear infections.

  • Recipe idea: Baked Oysters with Wild Mushroom Ragout.
    

• Vitamins C and E and glutathione: Vitamin C and E can increase your overall health by reducing the chance of getting an ear infection. Vitamins C and E can be found in vegetables and in fruit. Glutathione is made naturally by the liver and is found in fruits, vegetables, and meat.

  • Recipe idea: Pomodoro Pasta with White Beans and Olives.

Day 6: Use everyday technology to enhance your hearing health. 

Take control of your hearing health with the technology you use daily: download a sound level meter on your smartphone or tablet to measure the decibel levels. In our Winter 2015 Hearing Health magazine, we listed apps that were vetted by the National Institute for Occupational Safety and Health, and they include: NoiSee by Noise Lab ($1), Noise Hunter by Inter•net2day ($6), and SoundMeter by Faber Acoustical ($20). These apps were cited as providing the most accurate A-weighted sound level measurements.

Other apps include: The Jacoti ListenApp, where you can test your hearing via earphones, and the LesserSound App, which allows the user to take sound readings and record the location from where the noise was recorded. 

Day 7: Share your story!

Share your story about living with hearing loss, tinnitus, or other hearing conditions and how it has affected you via our online scrapbook, blog, or magazine. Inspire others who are touched by similar conditions so that we can help raise awareness about the prevalence of hearing loss and other hearing disorders as well as our research to find better treatments, therapies, and ultimately a cure.

You can share your story by emailing us at info@hhf.org. It can be on ANYTHING related to hearing loss, tinnitus, or other hearing related conditions, such as funny stories, personal experiences, tips for our readers, or hearing health. If you would like to contribute but find that you're having writer's block, email us anyway! We're HEAR to get you through it! (Pun intended.) 

These are just some tips and advice that can help your hearing and the broader hearing health community. For any additional questions please contact your audiologist, email us at info@hhf.org, or visit our website.

7 High-Tech Reasons You Should Finally Deal with Your Hearing Loss

By Laura Friedman

Lifting your mood, boosting your energy, protecting your earnings, super-charging your social life — and even keeping your mind sharp. These are just some of the many spoils that come with facing and dealing with a noise-induced hearing loss that has been slowly but persistently creeping up on you.

The quality-of-life and feel-good benefits of treating even just mild hearing loss brought on by years of loud music, power tools, high-volume headphones, motor-sport engines, crowded night clubs and bars, noisy restaurants, and raucous sporting events are plenty. But in this digital age of smart phones and wearable technologies, the draw for many solution-minded consumers may be in the technology itself. Super-smart, super-sleek, super-convenient, and super-sophisticated — today’s hearing aids give you a multitude of reasons to address that hearing loss you’ve been trying so hard to ignore.

Consider these inspiring facts about today’s highly functional, high-powered hearing aids. They just may get you to finally do something about your hearing loss and make your life easier.

1. They’re cool, sleek, discreet and virtually invisible. The latest hearing aids offer functionality, style and effortless living. The designs are incredibly attractive and they’re much smaller than even conventional Bluetooth earpieces. Many of the latest hearing aids are so tiny; they sit discreetly and comfortably inside the ear canal, out of sight. Aesthetically, hearing aids have had a complete makeover.
   

2. They cut out background noise so you hear what you want to hear. Hearing aids now scan the listening environment and automatically adapt to it—even in the wind. There are even hearing aids that can actually “geo-tag” a location. So if it’s convenient for you to network at a certain coffee shop, your hearing aids will know when you’re there and adjust themselves accordingly.
   

3. New technologies not only help you decipher speech details in music and noise, but they better preserve and clarify the more subtle sounds of language — like the consonants B, S, F, T, and Z — so you can really follow what someone is saying. No faking.
   

4. You can hear from all directions — even when scoping out what’s in the fridge. Advanced directional microphone technology lets you hear from the back and side — something really important when driving a car. But it also makes it easier to hear voices more clearly in other everyday settings — like when your head is in the fridge and your significant other is talking at your back. Yes, that’s one great feature.
   

5. Digital, Bluetooth, and wireless capabilities in hearing aids are the now the norm. Many new technologies let you stream sound directly into your hearing aids — at the perfect volume — from your smartphone, laptop, conference-room speakerphone, home entertainment system, and other Bluetooth devices. Using a wireless mini-microphone — with cool, contoured designs, some even looking like a pen— placed on the restaurant or conference-room table, or near anyone you want to hear, makes it feel like they’re speaking directly and clearly into your ears, no matter how noisy the setting.
   

6. State-of-the-art hearing aids can do a lot for the person. They offer no whistling due to advances in digital technology. Most are hypoallergenic with nanotechnology coating to keep them clean and dry. Some are fully waterproof so you can swim or shower with them in, and some have rechargeable batteries.
   

7. There are even more disruptive hearing technologies on the horizon. Totally out-of-sight, semi-permanent hearing aids that stay in for two to three months let you shower and sleep in them, no fuss. Hearing aid manufacturers are deep in the trenches working to create future breakthrough technologies that will make it as easy as possible for the brain to decode speech and other sounds. After all, we really do hear with our brains and not with our ears. Some hearing aids with these technologies are already available.

The content for this blog post originated in a press release issued by The Better Hearing Institute. For a list of hearing aid models check out the Hearing Health Foundation's New Technology page. 

Are Hair Cell Regeneration Genes Blocked?

By Yishane Lee

On March 8, 2016, Hearing Health Foundation hosted a live-video research briefing, as part of an ongoing effort to provide regular updates on our research programs and progress. Through these briefings, our goal is for our attendees to learn new information and achieve a greater understanding of hearing loss, prevention, and to o develop effective therapies for hearing loss and tinnitus.

Peter Barr-Gillespie, Ph.D., the scientific director of the Hearing Restoration Project (HRP), began the webinar with announcing the newest HRP consortium member, Ronna Hertzano, M.D., Ph.D., from the University of Maryland. Ronna is a clinician as well as a research scientist, a rare combination and an asset for the HRP. She also developed a bioinformatics platform, gEAR, that the HRP is using to efficiently compare large, complex genetic datasets between species.

Dr. Barr-Gillespie went on to outline a year in the life of the HRP—how the investigators collaborate, discuss, and develop research projects. He then provided an overview of a currently funded project focused on examining whether genes can be manipulated to overcome a block to hair cell regeneration in mammals, including humans. The advancements in technologies, such as CRISPR gene modification, provides the HRP with the ability to study hair cell regeneration in different species and at a level of detail and manipulation unheard of before.

We invite you to watch the video with captioning, or read the presentation with summary notes. We are excited to share this discussion of the HRP’s progress to date and our plans for 2016 and beyond.

Your Support Is Needed!

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.

Defining Auditory-Visual Objects

By Molly McElroy, PhD

If you've ever been to a crowded bar, you may notice that it's easier to hear your friend if you watch his face and mouth movements. And if you want to pick out the melody of the first violin in a string quartet, it helps to watch the strokes of the players' bow.

I-LABS faculty member Adrian KC Lee and co-authors use these examples to illustrate auditory-visual objects, the topic of the researchers' recently published opinion paper in the prestigious journal Trends in Neurosciences.

Lee, who is an associate professor in the UW Department of Speech & Hearing Sciences, studies brain mechanisms that underlie hearing. With an engineering background, Lee is particularly interested in understanding how to improve hearing prosthetics.

Previous I-LABS research has shown that audio-visual processing is evident as early as 18 weeks of age, suggesting it is a fundamental part of how the human brain processes speech. Those findings, published in 1982 by the journal Science, showed that infants understand the correspondence between sight and the sound of language movements.

In the new paper, Lee and co-authors Jennifer Bizley, of University College London, and Ross Maddox, of I-LABS, discuss how the brain integrates auditory and visual information—a type of multisensory processing that has been referred to by various terms but with no clear delineation.

The researchers wrote the paper to provide their field with a more standard nomenclature for what an audio-visual object is and give experimental paradigms for testing it.

“That we combine sounds and visual stimuli in our brains is typically taken for granted, but the specifics of how we do that aren’t really known," said Maddox, a postdoctoral researcher working with Lee. “Before we can figure that out we need a common framework for talking about these issues. That’s what we hoped to provide in this piece.”

Trends in Neurosciences is a leading peer-reviewed journal that publishes articles it invites from leading experts in the field and focuses on topics that are of current interest or under debate in the neuroscience field.

Multisensory, especially audio-visual, work is of importance for several reasons, Maddox said. Being able to see someone talking offers huge performance improvements, which is relevant to making hearing aids that take visual information into account and in studying how people with developmental disorders like autism spectrum disorders or central auditory processing disorders (CAPD) may combine audio-visual information differently.

"The issues are debated because we think studying audio-visual phenomena would benefit from new paradigms, and here we hoped to lay out a framework for those paradigms based on hypotheses of how the brain functions," Maddox said.

Read the full paper online. This article was republished with permission of the Institute for Learning & Brain Sciences at the University of Washington. 

Ross Maddox, Ph.D. was a 2013 General Grand Chapter Royal Arch Masons International award recipient. Hearing Health Foundation would like to thank the Royal Arch Masons for their generous contributions to Emerging Research Grantees working in the area of central auditory processing disorders (CAPD). We appreciate their ongoing commitment to funding CAPD research.

We need your help supporting innovative hearing and balance science through our Emerging Research Grants program. Please make a contribution today.

Proud Grandparents

By Darel Sorensen, Ed.D.

When our grandchild Mikaela, now 15, was born, newborn hearing screening was not yet an option at their hospital in California. She was diagnosed with a sensorineural hearing loss at age 23 months, after we noticed she had delayed speech.

At age 2 she began attending an “early start program” and preschool at age 3. Two years later Mikaela was joined in preschool by her younger brother Christian Joseph (CJ), now 13. He had passed the newborn screening test, but by age 2 1/2 he began to lose his hearing. After an alert teacher suggested testing, CJ was also diagnosed with sensorineural hearing loss.

To attend school, Mikaela and CJ traveled for one hour each day. The bus ride is a long one for kids who are toddlers, but our anxiety was tempered knowing that Mikaela and CJ would be getting specialized assistance with their hearing, speech, and language skills as well as learning how to sign. It would help prepare them for mainstream school classes.

Before she was 3 years old, Mikaela had cochlear implant (CI) procedures in both ears. A few years later, also at age 3, CJ also received a CI for his left ear and a hearing aid in his right.

Since the deaf and hard-of-hearing (DHOH) program in their school district spans kindergarten through high school, they have benefited from learning communication and coping skills from the same DHOH and speech-language therapists as they got older. They know to ask to sit on the side of the classroom, so they can more easily rotate to face whoever is speaking, and to be specific about what part of a conversation they missed when asking for something to be repeated.

Mikaela and CJ - 2 1/2 and 1 years old

Thanks to this consistent help, Mikaela and CJ were able to be mainstreamed into their local schools. Now in middle school, CJ has tried his hand at sports and now plays clarinet in the marching band; he was also selected to play in the concert band. Mikaela has played on the school basketball team for four years and received this commendation from her DHOH specialist:   

“Mikaela exudes confidence in class, never shying away from raising her hand, offering her insights, speaking up about her ideas and opinions. She advocates for herself by talking with her teachers about what works best for her. She is energetic, personable, and hardworking. In addition to her own success, she looks out for her fellow hard-of-hearing peers in order to help them succeed as well.”

Recently there was talk that the DHOH program may be moving from its current locations. Mikaela was quick to contact the administration to tell them how much the program meant to her and to her success: “Both DHOH and mainstream teachers… helped me understand everything, and now I’m a straight-A student because of them,” she wrote.

“The DHOH campuses are safer, friendlier, and better than most schools…. If you move the program you’ll be losing the teachers, staff, and students who treated us like family [and] the hearing students at those schools will be losing the ability of learning another language (sign language) and being friends with the deaf. PLEASE KEEP THE PROGRAMS WHERE THEY ARE!! Thanks for reading.”

These steps toward self-advocacy, as well as their self-confidence and concern for others, will serve them well. We could not be more proud. We wanted to share this story about our grandchildren because we believe in HHF and its mission of research, education, and prevention of hearing loss.

Darel Sorensen, Ed.D., is a retired educational psychologist and director of special education services. He lives with his wife, Betty, in California. 

Engineering Music to Sound Better With Cochlear Implants

By Columbia University Medical Center

When hearing loss becomes so severe that hearing aids no longer help, a cochlear implant not only amplifies sounds but also lets people hear speech clearly.

Music is a different story.

“I’ve pretty much given up listening to music and being able to enjoy it,” says Prudence Garcia-Renart, a musician who gave up playing the piano a few years ago.

“I’ve had the implant for 15 years now and it has done so much for me. Before I got the implant, I was working but I could not use a phone, I needed somebody to take notes for me at meetings, and I couldn’t have conversations with more than one person. I can now use a phone, I recognize people’s voices, I go to films, but music is awful.”

Cochlear implants are designed to process speech, which is a much simpler auditory signal compared with music. People with severe hearing loss also have lost auditory neurons that transmit signals to the brain.

It’s not possible to tweak the settings of the implant to compensate for the loss of auditory neurons, says Anil Lalwani, MD, director of the Columbia Cochlear Implant Program. “It’s unrealistic to expect people with that kind of nerve loss to process the complexity of a symphony, even with an implant.”

Instead, Dr. Lalwani and members of Columbia’s Cochlear Implant Music Engineering Group is trying to reengineer and simplify music to be more enjoyable for listeners with cochlear implants. “You don’t necessarily need the entire piece to enjoy the music,” Dr. Lalwani says. “Even though a song may have very complex layers, you can sometimes just enjoy the vocals, or you can just enjoy the instruments.”

Right now the group is testing different arrangements of musical compositions to learn which parts of the music are most important for listener enjoyment. “It’s not the same for somebody who has normal hearing,” Dr. Lalwani says, “and that’s what we have to learn.”

Down the road, Dr. Lalwani thinks software will be able to take an original piece of music and reconfigure it for listeners or give the listener the ability to engineer their own music.

“Our eventual goal, though, is to compose music for people with cochlear implants based on what we’ve learned,” Dr. Lalwani says. “Original pieces of music that will possibly have less rhythmic instruments, less reverb, possibly more vocals—something that is actually designed for them.”

The study is titled, “Music Engineering as a Novel Strategy for Enhancing Music Enjoyment in the Cochlear Implant Recipient.” The other contributors are: Gavriel D. Kohlberg, Dean M. Mancuso, and Divya A. Chari.

This blog was reposted with the permission of Columbia University Medical Center. 

Anil K. Lalwani, M.D. is the Head of Hearing Health Foundation's Council of Scientific Trustees.

Take It From Me: Auditory Processing Disorder in Class

By Eliza Uberuaga

My legs grow tense as a classmate’s whispers flood my ears. My breath becomes short as another taps his foot against the desk. My stomach lurches as I watch two students turn in their tests. Why can’t I block out the noise? Why can’t I answer the questions faster? Why am I the only one struggling? I must run while they walk, work while they sleep and prepare while they rest.

When I was diagnosed with a learning disability, my parents brought me to the most respected doctors in New York and enrolled me at one of the most prestigious schools in the country. However, that did not cure, fix or help me. Why? Because no doctor, teacher, parent or friend can change the world that tells me I have a problem. I am labeled with Auditory Processing Disorder (APD), characterized as slow and viewed with pity.

I am not asking for sorrowful looks, sympathy hugs or uplifting pep talks. I am asking you to understand that the student in your classroom who needs extra time wasn’t daydreaming during the test. The girl who needs directions to be repeated is listening. The boy who is last to raise his hand has the answer.

These kids likely have APD, a learning disability that slows the comprehension of information. (It is also known as central auditory processing disorder, CAPD.) It is not their hearing that is impaired, but their auditory pathways. Information that is spoken can be difficult to process if said too quickly, in a loud place or in large chunks of speech. Having APD is like listening to a voicemail on a busy street while everyone else is listening to it in a quiet space. While most people can block out that background noise, people with APD hear that noise as if it is the message itself. When given directions, most brains organize the information, as if putting it into filing cabinets. Those with APD take longer to find the filing cabinets, which slows the pace at which they comprehend.

APD affects students in a variety of ways, but students with APD (and most other students) could benefit if we looked at our classrooms the way we look at our world: valuing everyone's uniqueness—in this case, the unique ways in which they learn. Here are some techniques that helped me.

1. Stimulate the Senses
   In an art history class, we learned about the making of a blind arch. Rather than looking at a diagram, my teacher had four kids (including me) make an arch with our arms. Putting pressure on our formation and watching it collapse taught us how to make the most effective structure. I learned about arches by listening, watching and feeling, as opposed to simply listening and writing.
   

2. Teach With Variety
   In a science class, my teacher gave us an outline of the notes, wrote them on the board and lectured us on them—supporting auditory, visual and kinesthetic learners. He allowed each student to retain the information in whatever ways that worked. In this class, no kid was left behind because every kid was supported.
   

3. Create a Quiet Learning Place
   In order for all students to be able to focus, especially ones with APD, it is best to minimize all noise when students are working or trying to concentrate. Although it may seem helpful to speak in a quieter voice, for a student with APD, hearing whispers while working can actually be worse than hearing words spoken at normal levels. Although it may seem helpful to speak in a quieter voice, it is best to not talk at all.

I hope that, by writing to teachers and sharing my story, I can help the 10-year-old girl who cries when she gets home from school and tells herself she will never be smart. Although she may not feel intelligent when she goes to the library to finish a test, she must understand that she does not have a problem. She only feels like she has a problem because the world around her is unable to understand her intelligence. The day will come when she feels the way she learns is truly all right, but maybe that day will come sooner for her than it came for me.

Eliza is a high school senior in the Bronx, New York. Originally published on Teaching Tolerance, and is reprinted by permission of the author.

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