The Path to Funding for Universal Newborn Hearing Screening

By Pranav Parikh

Due to the complexities of a multi-trillion-dollar federal budget, it can often be difficult to understand where all the money ends up. For recipients of Medicaid and their children, part of the government’s longstanding policy is to provide access to quality healthcare low-income communities could not otherwise afford. Medicaid recipients represent approximately 23 percent of the total U.S. population, with an enrollment of 74,550,529 individuals.

According to President Donald Trump’s Fiscal Year (FY) 2018 proposed budget, deemed the “America First” budget, and a nonpartisan CBO report, Medicaid will receive cuts totaling $610 billion USD over the next 10 years. In 2015, the U.S. Government spent $545.1 billion USD on Medicaid services. President Trump alludes to waste and redundancies as his justification of the proposed cuts.

One of the planned cuts will negatively impact newborn children and be detrimental to the well-being of infants across the country: Universal Newborn Hearing Screening. The terrifying impact is summarized below.
 

What exactly is being removed?
In his FY18 proposed budget, President Trump upheld his campaign promise by cutting what he deems “unnecessary and wasteful spending.” Unfortunately, one program that got the axe was the $18 million USD allocated towards newborn hearing screenings. This earmarked funding has doubled the percentage of newborns receiving hearing screenings before leaving the hospital from 46.5% to 97% just in the last decade. Without early detection, children will be at a distinct disadvantage in tackling hearing loss present at birth.

Why does this matter?
Every day, 33 children are born with some form of hearing loss, designating hearing loss as the most common congenital birth defect in the U.S. Reasons babies may have hearing loss present at birth include an inherited trait, ototoxic chemical, or a viral infection during a mother’s pregnancy. Challenges associated with having hearing loss can be overcome through early intervention, however it is imperative treatment and therapy are started as early as possible. As stated on the U.S. Government Department of Health and Human Services website, “If not identified early, [hearing loss] is likely to delay or impair a child’s development. Hearing problems are difficult to detect through observation alone, so almost all newborns have their hearing checked with special equipment.” 

What types of tests are done?
Aside from behavioral characteristics displayed by infants with hearing loss, there are two main tests conducted by physicians to determine any level of auditory impairment. The first of which is called Otoacoustic Emissions, a test designed to the test functionality of outer hair cells. A negative reading on this test is typically associated with cochlear dysfunction. The second test is called Auditory Brainstem Response (ABR) and determines activity of the auditory nerve through stimulation in the baby’s ear. A negative reading on this test indicates some issue with the vestibulocochlear nerve such as auditory neuropathy, but could also indicate problems with other parts of the ear. Both of these tests can be done while the baby is asleep and offer more concrete evidence to either rule out or diagnose infant hearing loss.

Have studies shown early intervention to be more effectual than later in childhood?
Yes, there are many studies that have shown that early intervention, especially for those receiving treatment within the first six months after birth, increases levels of cognitive function and advanced development. The control group of one study, led by Dr. Christine Yoshinaga-Itano at the University of Colorado-Boulder, showed that those who did not receive treatment or therapy within the first six months after birth had greater difficulty with oral communication and language comprehension.

What happens if children have undiagnosed hearing loss?
Hearing loss as a condition can present a number of symptoms associated with other disabilities, leading to improper diagnoses. For example, when children exhibit a lack of response to loud noise, or don't answer when spoken to, they sometimes are misdiagnosed by professionals as being autistic. If hearing loss is present and detected at birth, doctors will have access to necessary information earlier and children will be better off in the long run in developing their communication and learning abilities.

If funding for newborn hearing screening is decreased or removed entirely, what does that mean for those suffering from hearing loss?
At the moment, only 67.1% of those diagnosed with hearing loss receive early intervention before six months of age. With lower early detection and screening rates, this percentage will drop further. Without early intervention programs in place, children are at a noticeable disadvantage in developing hearing and speech functionality. After the age of three, it is considerably more difficult for children to develop the speaking and listening skills that are in line with their typical-hearing peers.

Would early intervention actually save money down the road in potential education costs?Some students with hearing loss utilize special education services, such as CART or note-taking, to ensure they don’t miss any of the materials and learnings while in the classroom. Access to the necessary technology and equipment, as well as highly trained teachers, is an expense incurred by school districts across the country.

A recent report released by the National Center for Hearing Assessment and Management states that treatment of hearing loss in children within the first three months of life can save up to $400,000 USD in eventual special educational costs by the time the hard of hearing student graduates high school. By bridging the gap early, and ensuring better interpersonal and cognitive skills in the first years of age, these children will require much less specialized instruction in future years. Essentially, early detection and intervention pays for itself.

Is there any legislation, not including the President’s proposed budget, that addresses this issue?
In March 2017, the Early Hearing Detection and Intervention (EDHI) Act was introduced on the House floor by Representatives Doris Matsui (D-CA-06) and Brett Guthrie (R-KY-02). A companion measure was also introduced in the Senate by Senators Rob Portman (R-OH) and Tim Kaine (D-VA). EHDI reauthorizes funding for Universal Newborn Hearing Screening for the next five (5) years, as well as establishes a database hub to collect information on the results of these tests. If the measure passes, parents will be assured of their child’s hearing health, and one of the nation’s largest public health concerns receives the necessary attention it deserves.

Undoubtedly, funding for newborn hearing screening is imperative. Hearing Health Foundation (HHF)'s Pranav Parikh spoke with Congresswoman Matsui’s staff on the reasons for proposing the legislation, and why she took the lead on tackling such an important issue. “So much of a child’s development happens in the first few years of their life, which is why early detection and intervention is so important,” said Matsui. “This bill will ensure that more infants have access to critical hearing screenings, so parents can be informed about the options for their children’s care.” It is comforting to know children suffering with hearing loss have an ally in our nation’s capital.

As Vickie Glenn, a Medicaid Coordinator for Tri-County Special Education recently stated in a New York Times article, “This isn’t Republicans or Democrats. It’s just kids.” Fortunately, President Trump’s proposed budget appears to be a “purely political document,” according to Peter Coy from Bloomberg BusinessWeek, possibly serving as a trial balloon and nothing more. Congress, even with a conservative majority consisting of many fiscal hawks, will likely reject many of the proposed cuts, as Texas Senator and chairman of the Freedom Caucus John Cornyn remarked, “we know the President’s budget isn’t going to be passed as is.” For now, at least, Universal Newborn Hearing Screening will receive its necessary and deserved funding.

And, finally, an urgent call to action from Nadine Deghan, CEO of HHF:
HHF has strongly supported Newborn Hearing Screening. In the 1990s, we championed legislation to encourage these simple but critical tests for our nation’s babies. For those who feel passionately about newborn screening funding, please contact your Congressional Representative and your Senator to let them know your views.

Welcome to HHF's New Website!

By Nadine Dehgan, CEO, Hearing Health Foundation

Hearing Health Foundation (HHF) proudly introduced a new website today, August 2. The contemporary design features an engaging storytelling format, clear calls to action, and mobile responsiveness, all of which will enable HHF to better serve and communicate with constituents.

Take a look around to familiarize yourself with HHF’s new virtual headquarters. Below are the site’s most exciting improvements:

Simplified Navigation Bar
We reduced our primary navigation bar to just six categories inspired by user analytics from our old website. Choose from About, How to Help, Research, News, Resources, and Hearing Loss. Between the home page and these six key sections, you will find everything you need.

Streamlined Donation & Partnership Hub
How to Help lists every single action that you can take to advance cures and treatments for hearing loss, tinnitus, and related conditions. The options shown here apply to both individual contributors and corporate partners.

Mobile-Friendliness
Did you ever visit the old HearingHealthFoundation.org on your cell phone? If you did, you probably quickly abandoned the page, frustrated by small text and the need to zoom in and out. Our new website fits perfectly on your smartphone or tablet. Try it!

Consolidation of Research Programs
The Research page provides information on our programs, Emerging Research Grants (ERG), Hearing Restoration Project (RFP), and, the newest, Ménière's Disease Grants (MRG), to keep you informed of our critical investigative work. The reorganization of the Research Programs will also more efficiently attract talented scientists who are researching cures and treatments.

Centralized E-Newsletter and Hearing Health Magazine Registration
Subscribing to HHF is no longer a two-step process. Conveniently opt into to our e-newsletter and free print magazine with fewer clicks on the Subscribe page. The modification will increase viewership and, therefore, hearing health awareness.

More Social Sharing Options
Share useful educational resources or inspiring blog posts with your friends and family seamlessly. Every page includes a sharing sidebar from which you can quickly send information through social media or email.

Ad Space
The new website is more customizable than the previous, allowing for greater advertising capacity. As a result, more organizations will be able to contribute to HHF’s life-changing research and education programs and show commitment to the many Americans with hearing loss, tinnitus, and related conditions.

We welcome your feedback about the new website in the comments section.

Early Detection Improved Vocabulary Scores in Kids with Hearing Loss

By Molly Walker

Children with hearing loss in both ears had improved vocabulary skills if they met all of the Early Hearing Detection and Intervention guidelines, a small cross-sectional study found.

Those children with bilateral hearing loss who met all three components of the Early Hearing Detection and Intervention guidelines (hearing screening by 1 month, diagnosis of hearing loss by 3 months and intervention by 6 months) had significantly higher vocabulary quotients, reported Christine Yoshinaga-Itano, PhD, of the University of Colorado Boulder, writing in Pediatrics.

The authors added that recent research reported better language outcomes for children born in areas of the country during years where universal newborn hearing screening programs were implemented, and that these children also experienced long-term benefits in reading ability. The authors said that studies in the U.S. also reported better language outcomes for children whose hearing loss was identified early, who received hearing aids earlier or who began intervention services earlier. But those studies were limited in geographic scope or contained outdated definitions of "early" hearing loss.

"To date, no studies have reported vocabulary or other language outcomes of children meeting all three components of the [Early Hearing Detection and Intervention] guidelines," they wrote.

Researchers examined a cohort of 448 children with bilateral prelingual hearing loss between 8 and 39 months of age (mean 25.3 months), who participated in the National Early Childhood Assessment Project -- a large multistate study. About 80% of children had no additional disabilities that interfered with their language capabilities, while over half of the children with additional disabilities reported cognitive impairment. Expressive vocabulary was measured with the MacArthur-Bates Communicative Development Inventories.

While meeting all three components of the Early Hearing Detection and Intervention guidelines was a primary variable, the authors identified five other independent predictor variables into the analysis:

• Chronological age

• Disability status

• Mother's level of education

• Degree of loss

• Adult who is deaf/hard of hearing

They wrote that the overall model was significantly predictive, with the combination of the six factors explaining 41% of the variance in vocabulary outcomes. Higher vocabulary quotients were predicted by higher maternal levels of education, lesser degrees of hearing loss and the presence of a parent who was deaf/or hard of hearing, in addition to the absence of additional disabilities, the authors said. But even after controlling for these factors, meeting all three components of the Early Hearing Detection and Intervention guidelines had "a meaningful impact" on vocabulary outcomes.

The authors also said that mean vocabulary quotients decreased as a child's chronological age increased, and this gap was greater for older children. They argued that this complements previous findings, where children with hearing loss fail to acquire vocabulary at the pace of hearing children.

Overall, the mean vocabulary quotient was 74.4. For children without disabilities, the mean vocabulary quotient was 77.6, and for those with additional disabilities, it was 59.8.

Even those children without additional disabilities who met the guidelines had a mean vocabulary quotient of 82, which the authors noted was "considerably less" than the expected mean of 100. They added that 37% of this subgroup had vocabulary quotients below the 10th percentile (<75).

"Although this percentage is substantially better than for those who did not meet [Early Hearing Detection and Intervention] guidelines ... it points to the importance of identifying additional factors that may lead to improved vocabulary outcomes," they wrote.

Limitations to the study included that only expressive vocabulary was examined and the authors recommended that future studies consider additional language components. Other limitations included that disability status was determined by parent, with the potential for misclassification.

The authors said that the results of their study emphasize the importance of pediatricians and other medical professionals to help identify children with hearing loss at a younger age, adding that "only one-half to two-thirds of children met the guidelines" across participating states.

This article was republished with permission from MedPageToday. 

Small Solution, Large Impact: Updating Hearing Aid Technology

By Apoorva Murarka

For many people, the sound quality and battery life of their devices are often no more than a second thought. But for hearing aid users, these are pivotal factors in being able to interact with the world around them.

One possible way to update existing technology – which has gone unchanged for decades – is small in size but monumental in impact. Apoorva Murarka, a Ph.D. candidate in electrical engineering at MIT, has developed an award-winning microspeaker to improve the functions of devices that emit sound. Murarka sees hearing aids as one of the most important applications of his new technology.

The Current Problem – Feeling the Heat

Most hearing aids have long used a system of coils and magnets to produce sound within the ear canal. These microspeakers use battery power to operate, and lots of it. Valuable battery life is wasted in the form of heat as an electric current works hard to travel through the coil to eventually help produce sound. The more limited a user’s hearing is, the more the speaker must work to produce sound, and ultimately that much more battery is used up. 

As a result, research has shown that many hearing aid users in the United States use about 80 to 120 batteries a year or have to recharge batteries daily. Aside from the anxiety that can accompany the varying dependability of this old technology, the cost of constantly replacing these batteries can quickly add up. 

But battery life is not the only factor to consider. Because the coil and magnet system has not been updated in decades, the quality of sound produced by hearing aid speakers (without additional signal processing) has been just as limited. Even small upgrades in sound quality could make a world of difference for users.

The Future Solution – Going Smaller and Smarter

Apoorva Murarka has invented an alternative to the old coil and magnet system, removing those components completely from the picture. In their stead, he has developed an electrostatic transducer that relies on electrostatic force instead of magnetic force to vibrate the sound-producing diaphragm. This way of producing sound wastes much less energy, meaning significantly longer battery life in hearing aids. Apoorva was recently awarded the $15,000 Lemelson-MIT Student Prize for this groundbreaking development.

The biggest difference? Size. You would need to look closely to even see this microspeaker’s membrane – its thickness is about 1/1,000 the width of a human hair. 

Additionally, the microspeaker’s ultrathin membrane and micro-structured design enhance the quality of sound reproduced in the ear. Power savings due to the microspeaker’s electrostatic drive can be used to optimize other existing features in hearing aids such as noise filtration, directionality, and wireless streaming. This could pave the way for energy-efficient “smart” hearing aids that improve the quality of life for users significantly. 

This invention is being developed further and Apoorva hopes to work with the hard-of-hearing community, relevant organizations and hearing aid companies to understand the needs of users and explore how his invention can be adapted within hearing aids.

You can read more about Apoorva and his invention here. 

More People = Less Noise?

By Kathi Mestayer

Beautiful, open, echoey space.

In the summer, attendance at our church falls noticeably as people go on vacation and spend weekend mornings doing other seasonal things, like birdwatching. After the service on a recent Sunday, we all headed out of the sanctuary, toward the atrium. Normally, this is a time when it’s really difficult for me to talk with anyone because of the reverberant nature of our building. It’s an architectural masterpiece and wonderful for music—and an acoustical nightmare, at least for speech comprehension.

To be fair, our church is not the only one with a large, open worship space where sound bounces around for what can seem like…. forever.  It’s actually becoming more common; when churches get bigger, sound challenges follow. As the authors of a research paper on the topic point out, “We are witnessing a paradigm shift from small church enclosures to very large church auditoriums.  Most of these auditoriums fall short of providing good sound quality and… sooner or later it becomes a very serious problem because such buildings are places for communication to an audience.…”

So, I’ve gotten used to the reverberation, and just try to avoid conversation until we’re out of the sanctuary. That summer day, however, as I worked my way toward the exit, I noticed that the noise level was significantly louder than usual. “That’s weird,” I thought, "fewer people, but more noise?” I checked with a couple of friends, and they had also noticed that the noise level seemed much higher than usual. So it wasn’t just me.

When I got home, I told my (physicist) husband about it, and he asked me how many people were at the service. I said, "Way fewer, less than half the usual number…probably vacations.” He replied, "Oh, that’s probably why it was noisier. People absorb sound.” But at such a noticeable level?

Ask an Acoustician

In search of a second opinion, I contacted Rich Peppin, the president of Engineers for Change, a nonprofit acoustics and vibrations consulting firm. Rich had helped me with a Hearing Health article, “Caution: Noise at Work,” so I knew he’d have the answer. I posited our working hypothesis in my email to him: that a reverberant space would be noticeably noisier if there are fewer people in it.

Rich replied: “Yes. Because people absorb sound and hence reduce reflections. We can calculate the reduction of reverberation if we know before and after numbers of people.” Now, we’re getting somewhere.

The calculations Rich was talking about are based, in part, on how much sound humans absorb. In addition to the sound absorption by human bodies, there are other variables that impact reverberation, such as: what the people are wearing, whether they are sitting or standing, whether there are padded seats in the room, and the size and shape of the room.

In my church example, however, most of the major variables were unchanged between winter and summer: lightly padded seats with metal frames; hard floor, walls, and ceiling; and no drapes. And everyone was standing up, walking out to the atrium, where conversation is a little more possible.

So, how much sound can people absorb? The study Rich shared with me had the results of controlled tests of sound absorption with different numbers of people (zero, one, two, three). The results varied widely for different frequencies (more sound absorption per added person at the higher frequencies tested).  

Human speech, however, was the source of the sound in our church sanctuary, and its frequencies range from an average of 125 Hz (for males) to 200 Hz (for females).  

And the result? Sound absorption increased by about 5 to 20 percent (depending on the frequency) with each person added to the test chamber.

Even though I didn’t know the exact numbers of people at my church, it was a big difference between the winter months, when it’s close to full, and that summer day, with its small attendance.  I estimate at least 75 fewer people. So it was not so surprising that the sanctuary was noisier the day that I, and a few others, noticed it. The bottom line? My husband was right—again. Oh, me of little faith!

Kathi Mestayer is a staff writer for Hearing Health magazine.

An Animal Behavioral Model of Loudness Hyperacusis

By Kelly Radziwon, Ph.D., and Richard Salvi, Ph.D.

One of the defining features of hyperacusis is reduced sound level tolerance; individuals with “loudness hyperacusis” experience everyday sound volumes as uncomfortably loud and potentially painful. Given that loudness perception is a key behavioral correlate of hyperacusis, our lab at the University at Buffalo has developed a rat behavioral model of loudness estimation utilizing a reaction time paradigm. In this model, the rats were trained to remove their noses from a hole whenever a sound was heard. This task is similar to asking a human listener to raise his/her hand when a sound is played (the rats receive food rewards upon correctly detecting the sound).
 

FIGURE: Reaction time-Intensity functions for broadband noise bursts for 7 rats.

The rats are significantly faster following high-dose (300 mg/kg) salicylate administration (left panel; red squares) for moderate and high level sounds, indicative of temporary loudness hyperacusis. The rats showed no behavioral effect following low-dose (50 mg/kg) salicylate.

By establishing this trained behavioral response, we measured reaction time, or how fast the animal responds to a variety of sounds of varying intensities. Previous studies have established that the more intense a sound is, the faster a listener will respond to it. As a result, we thought having hyperacusis would influence reaction time due to an enhanced sensitivity to sound.

In our recent paper published in Hearing Research, we tested the hypothesis that high-dose sodium salicylate, the active ingredient in aspirin, can induce hyperacusis-like changes in rats trained in our behavioral paradigm. High-dose aspirin has long been known to induce temporary hearing loss and acute tinnitus in both humans and animals, and it has served as an extremely useful model to investigate the neural and biological mechanisms underlying tinnitus and hearing loss. Therefore, if the rats’ responses to sound are faster than they typically were following salicylate administration, then we will have developed a relevant animal model of loudness hyperacusis.

Although prior hyperacusis research utilizing salicylate has demonstrated that high-dose sodium salicylate induced hyperacusis-like behavior, the effect of dosage and the stimulus frequency were not considered. We wanted to determine how the dosage of salicylate as well as the frequency of the tone bursts affected reaction time.

We found that salicylate caused a reduction in behavioral reaction time in a dose-dependent manner and across a range of stimulus frequencies, suggesting that both our behavioral paradigm and the salicylate model are useful tools in the broader study of hyperacusis. In addition, our behavioral results appear highly correlated with the physiological changes in the auditory system shown in earlier studies following both salicylate treatment and noise exposure, which points to a common neural mechanism in the generation of hyperacusis.

Although people with hyperacusis rarely attribute their hyperacusis to aspirin, the use of the salicylate model of hyperacusis in animals provides the necessary groundwork for future studies of noise-induced hyperacusis and loudness intolerance.


Kelly Radziwon, Ph.D., is a 2015 Emerging Research Grants recipient. Her grant was generously funded by Hyperacusis Research Ltd. Learn more about Radziwon and her work in “Meet the Researcher.”

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Turning Fourth of July Into a Science Lesson

By Kelly N. Barahona

In most cities if not towns of a certain size in the U.S., a grand display of fireworks for the Fourth of July is part of the celebration of America’s birthday. But just how loud are the fireworks people have come to expect every summer? Unfortunately fireworks can measure from 140 to as high as 165 decibels, easily a hearing-damaging event if you are sitting too close.

This doesn’t mean you can’t enjoy the festivities. With the abundance of decibel-reading apps for smartphones it’s easier than ever before to learn how much noise is in the world around us. Most apps use the smartphone’s microphone to give a reading of the decibel level. As with a professional-grade meter, most apps can also show how the noise fluctuates over time, in real time, and provide numerical reference points that users can compare to their own sound levels. Some apps even let you geo-tag the decibel level to a specific location, like your local coffee shop or favorite restaurant.

Parents, camp counselors, and teachers can turn the Fourth of July into a science lesson. On the night of the fireworks show, Hearing Health Foundation recommends staying at least one block away from where the fireworks are being displayed and using a smartphone app to measure the decibel level.

If you want to be closer to the action, protect your hearing by using foam earplugs or over-the-ear earmuffs for the youngest children. A fun but loud activity like this can be a good segue for conversations about how listening to music at too loud a volume and participating in noisy recreational activities may be harmful, as well as how to incorporate better hearing health practices in your daily life.

Fourth of July should be a time of fun and enjoyment, but as with anything, it is necessary to take precautions to make the holiday safe as well. Teach your loved ones about the noises and sounds around them to hopefully encourage everyone to take active measures to protect their hearing on a regular basis. Remember, noise is the most preventable cause of hearing loss.

Some 1 Like You

By Makayla Allison

Our 6-year-old daughter, Lily, was recently diagnosed with Ehlers Danlos Hypermobility (EDS-HT). We received this diagnosis only after she acquired more than three dozen symptoms and diagnoses of uncertain significance, ranging from global joint pain and muscle weakness to tinnitus, and over the course of nearly five years.

It was a long and isolating time for our family as we tried to figure out what was going on. We so badly wanted to connect with someone who understood what our little one was going through, and when I asked Lily’s specialists if they could connect us with anyone, the answer was never yes due to privacy laws. Without a diagnosis it is nearly impossible to find groups of people in the same situation to talk to. And it can be even more frightening when the uniqueness of your symptoms isolates you even more.

The discovery of how Lily’s condition affected her hearing was both transparent and innocent. When Lily was 4 years old she asked me if the invisible bumblebees were going to sting her. She was so confused why she couldn’t see the bees that buzzed around her ears. It was shortly after she was referred to an ENT that we learned about tinnitus and that the sounds she hears come from inside her head.

Our daughter had a big desire to find a friend like her, but looking for someone else experiencing the same health challenges online, without posting them in great detail, was proving to be an impossible task. Her dreams inspired us to create Some 1 Like You (S1LY), an organization that connects people privately based on whatever health conditions they are experiencing, regardless of whether or not they’ve received a diagnosis.

According to the documentary film Undiagnosed: Medical Refugees, “The total number of undiagnosed patients is unknown but considered to be vast.... It takes an average of 7.6 years in the U.S. to uncover a rare disease diagnosis. Worldwide there are an estimated 350 million people living with a rare disease; add to [that number] patients still waiting for a diagnosis, patients who have been misdiagnosed, and adults and children who have diseases not yet named or recognized. Being ‘undiagnosed’ is not commonly considered to be an identity, but it should be. Helping people who are ill to feel that others are supporting and advocating for them, and know that they exist, can make all the difference in the world.”

Our mission for S1LY is to privately connect people across symptoms and diagnoses to empower the individuals facing these complex challenges. S1LY is unique because we can perform that search for people, while also keeping their health information private: To make these matches we take only their email address, as well as the health qualities, or groups of qualities they possess and are looking for in someone else.

Once a match is made, the email addresses of those members are shared with each other, and communication is then done only between members. It is our hope that this vast sharing of knowledge and resources among patients will make its way back to physicians and impact treatments as a whole across diseases.

S1LY has developed a Gifted Membership program to cover the lifetime membership fee to Some 1 Like You for constituents of qualifying organizations. 100 gifted memberships have been donated to the Hearing Health Foundation community. The first 100 people to submit their Connect Contact Forms to S1LY with the code “HHF100” will receive lifetime memberships to privately connect with Some 1 Like You members.

If you would like to explore gifted memberships for your patients or members at no cost, please email Makayla at gifted@some1likeyou.com. A portion of the proceeds of every S1LY membership goes to funding research on Ehlers Danlos Syndromes.

NIH Researchers Show Protein in Inner Ear Is Key to How Cells That Help With Hearing and Balance Are Positioned

By the National Institute on Deafness and Other Communication Disorders (NIDCD)

Line of polarity reversal (LPR) and location of Emx2 within two inner ear structures. Arrows indicate hair bundle orientation. Source: eLife

Using animal models, scientists have demonstrated that a protein called Emx2 is critical to how specialized cells that are important for maintaining hearing and balance are positioned in the inner ear. Emx2 is a transcription factor, a type of protein that plays a role in how genes are regulated. Conducted by scientists at the National Institute on Deafness and Other Communication Disorders (NIDCD), part of the National Institutes of Health (NIH), the research offers new insight into how specialized sensory hair cells develop and function, providing opportunities for scientists to explore novel ways to treat hearing loss, balance disorders, and deafness. The results are published March 7, 2017, in eLife.

Our ability to hear and maintain balance relies on thousands of sensory hair cells in various parts of the inner ear. On top of these hair cells are clusters of tiny hair-like extensions called hair bundles. When triggered by sound, head movements, or other input, the hair bundles bend, opening channels that turn on the hair cells and create electrical signals to send information to the brain. These signals carry, for example, sound vibrations so the brain can tell us what we’ve heard or information about how our head is positioned or how it is moving, which the brain uses to help us maintain balance.

NIDCD researchers Doris Wu, Ph.D., chief of the Section on Sensory Cell Regeneration and Development and member of HHF’s Scientific Advisory Board, which provides oversight and guidance to our Hearing Restoration Project (HRP) consortium; Katie Kindt, Ph.D., acting chief of the Section on Sensory Cell Development and Function; and Tao Jiang, a doctoral student at the University of Maryland College Park, sought to describe how the hair cells and hair bundles in the inner ear are formed by exploring the role of Emx2, a protein known to be essential for the development of inner ear structures. They turned first to mice, which have been critical to helping scientists understand how intricate parts of the inner ear function in people.

Each hair bundle in the inner ear bends in only one direction to turn on the hair cell; when the bundle bends in the opposite direction, it is deactivated, or turned off, and the channels that sense vibrations close. Hair bundles in various sensory organs of the inner ear are oriented in a precise pattern. Scientists are just beginning to understand how the hair cells determine in which direction to point their hair bundles so that they perform their jobs.

In the parts of the inner ear where hair cells and their hair bundles convert sound vibrations into signals to the brain, the hair bundles are oriented in the same direction. The same is true for hair bundles involved in some aspects of balance, known as angular acceleration. But for hair cells involved in linear acceleration—or how the head senses the direction of forward and backward movement—the hair bundles divide into two regions that are oriented in opposite directions, which scientists call reversed polarity. The hair bundles face either toward or away from each other, depending on whether they are in the utricle or the saccule, two of the inner ear structures involved in balance. In mammals, the dividing line at which the hair bundles are oriented in opposite directions is called the line of polarity reversal (LPR).

Using gene expression analysis and loss- and gain-of-function analyses in mice that either lacked Emx2 or possessed extra amounts of the protein, the scientists found that Emx2 is expressed on only one side of the LPR. In addition, they discovered that Emx2 reversed hair bundle polarity by 180 degrees, thereby orienting hair bundles in the Emx2 region in opposite directions from hair bundles on the other side of the LPR. When the Emx2 was missing, the hair bundles in the same location were positioned to face the same direction.

Looking to other animals to see if Emx2 played the same role, they found that Emx2 reversed hair bundle orientation in the zebrafish neuromast, the organ where hair cells with reversed polarity that are sensitive to water movement reside.

These results suggest that Emx2 plays a key role in establishing the structural basis of hair bundle polarity and establishing the LPR. If Emx2 is found to function similarly in humans, as expected, the findings could help advance therapies for hearing loss and balance disorders. They could also advance research into understanding the mechanisms underlying sensory hair cell development within organs other than the inner ear.

This work was supported within the intramural laboratories of the NIDCD (ZIA DC000021 and ZIA DC000085).

Doris Wu Ph.D. is member of HHF’s Scientific Advisory Board, which provides oversight and guidance to our Hearing Restoration Project (HRP) consortium This article was repurpsed with permission from the National Institute on Deafness and Other Communication Disorders. 

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The Connection Between Hearing Loss and Dementia

By Alycia Gordan

June is Alzheimer's & Brain Awareness Month and Hearing Health Foundation would like to shine light on the effects untreated hearing loss can have on our brains and memory. Hearing loss is often linked with dementia, and research is being conducted to establish the exact link between the two. Evidence suggests that by treating hearing loss, the risk of dementia can be mitigated.

Dementia is a medical term that is used to describe a host of symptoms, characterized by a deterioration in a patient’s cognitive abilities. The degeneration of brain cells causes neurons to stop functioning, leading to a series of dysfunctions.

A person may have dementia if at least two of his mental faculties are affected: the loss of memory and focus; difficulty communicating; short or interrupted attention spans; impaired judgment; or an inability to perform everyday tasks.

Frank Lin, M.D., Ph.D., an associate professor of otolaryngology and epidemiology at Johns Hopkins University, conducted a study in 2011 in which the mental abilities of 639 cognitively stable individuals were supervised regularly for 12 to 18 years. The results indicated that volunteers with normal hearing were much less susceptible to acquiring dementia while those with mild, moderate, and severe hearing loss were two, three, and five times more susceptible to the disorder, respectively.

Another study conducted by Lin in 2013 involved observing the cognitive abilities of 1,984 older adults over six years. The research concluded that older adults with hearing loss tended to experience 30 to 40 percent accelerated cognitive dysfunction and were at a higher risk of developing dementia.

What Is the Cause?

Since the exact link between hearing loss and dementia is still a mystery, there are theories about how the former may aggravate the latter.

One of the theories suggests that if the brain struggles to cope with degraded sounds, its resources are allocated to processing these sounds and this “cognitive load” causes a decrease in overall cognitive functioning. Moreover, hearing loss accelerates atrophy in the cerebrum which is not exclusive to processing sound as it also plays a role in memory. In addition, it is speculated that social isolation that results from hearing loss causes stress and depression and exacerbates cognitive deterioration.

What Is the Solution?

Not many studies have been conducted to check the influence of treating hearing loss for treating dementia. However, the studies that have been conducted so far do provide considerable hope.

One way to improve profound hearing loss is receiving cochlear implants. French researcher Isabelle Mosnier, M.D., of the Assistance Publique-Hôpitaux de Paris, evaluated the effect of cochlear implants on cognitive functioning in 94 elderly people who had profound deafness (in at least one ear).

Mosnier found that hearing rehabilitation improved not only cognitive functioning of the elderly, but their speech perception as well.

The most direct link between auditory impairment and memory loss is the brain. Thus, any stimulus that helps the brain remain alert will keep the person active too. Hence, researchers are considering the use of music therapy to restore cognitive functions in people who suffer from memory loss.           

Concetta Tomaino, a cofounder of the Institute for Music and Neurological Function, found that music stimulates parts of the brain made inactive by dementia. In a pilot study, music therapy sessions were conducted with 45 individuals with chronic dementia and the results showed that neurological and cognitive abilities improved significantly for those in the music group.

This research shows there are techniques that can aid individuals with dementia and hearing loss. If you or a loved one has hearing problems, please see a hearing health professional to get a hearing test in order to potentially prevent future cognitive issues. 

Alycia Gordan writes for Brain Blog.

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