The Hearing Restoration Project: Update on the Seattle Plan and More

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

Hearing Health Foundation launched the Hearing Restoration Project (HRP) to understand how to regenerate inner ear sensory cells in humans to restore hearing. These sensory hair cells detect and turn sound waves into electrical impulses that are sent to the brain for decoding. Once hair cells are damaged or die, hearing is impaired, but in most species, such as birds and fish, hair cells spontaneously regrow and hearing is restored.

The overarching principle of the HRP consortium is cross-discipline collaboration: open sharing of data and ideas. By having almost immediate access to one another’s data, HRP scientists are able to perform follow-up experiments much faster, rather than having to wait years until data is published.

Regenerated hair cells from chicken auditory organs, with the cell body, nucleus and hair bundle labeled with various colored markers. Image courtesy of Jennifer Stone, Ph.D.

You may remember that two years ago, we changed how we develop our projects. We decided together on a group of four projects—the “Seattle Plan”—that are the most fundamental to the consortium’s progress. These projects, which grew out of previous HRP projects, have now been funded for two years, and considerable progress has been made. We have also funded several other projects that have bubbled up out of new observations and capabilities, and they have added considerably to our knowledge base. With this in mind, I am pleased to share with you the latest updates for our 2018–19 projects.

SEATTLE PLAN PROJECTS

Transcriptome changes in single chick cells
Stefan Heller, Ph.D.

• Found that all “tall” hair cells are exclusively regenerated mitotically in this animal model.

• Compiled evidence for different supporting cell subtypes.

• Obtained good quality single cell RNA sequencing (scRNA-seq) data and are in the process of evolving an analysis strategy for the baseline cell types (control group). Identified about 50 novel marker genes for hair cells, supporting cells, and homogene cells, including subgroups.

• Developed a strategy to finish all scRNA-seq using a novel peeling technique and latest generation library construction methods.

•  Established two methods for multi-color in situ hybridization (PLISH, proximity ligation in situ hybridization) and SGA (sequential genomic analysis) for spatial and temporal mRNA expression validation.
  
  

Epigenetics of the mouse inner ear
Michael Lovett, Ph.D., David Raible, Ph.D., Neil Segil, Ph,D., Jennifer Stone, Ph.D.

• Completed epigenetic, chromatin structure, and RNA-seq datasets for FACS-purified cochlear hair cells and supporting cells from postnatal day 1 and postnatal day 6 mice, and provision of these data sets to the gEAR (gene Expression Analysis Resource portal) for mounting on their webpage through EpiViz for access by the HRP consortium.

• Established a webpage (EarCode) so that HRP consortium members can access the current data directly through a University of California, Santa Cruz, genome browser.

• Discovered maintenance of the transcriptionally silent state of the hair cell gene regulatory network in perinatal supporting cells is dependent on a combination of H3K27me3 and active H3k27-deacetylation, and that during transdifferentiation, these epigenetic marks are modified to an active state.

Mouse functional testing
John Brigande, Ph.D.

• Defined in vitro and in vivo model systems to interrogate genome editing efficacy using CRISPR/Cas9.
  
  

Implementing the gEAR for data sharing within the HRP
Ronna Hertzano, M.D., Ph.D.

• Added scRNA-seq workbench for easy sharing and viewing of scRNA-seq data. Such data, which are now driving the field forward, have been particularly difficult to share

• Created additional public datasets to improve data sharing.

• Completely rewrote the gEAR backbone to be updated to the latest technologies, allowing the portal to now to handle a much larger number of datasets and users.

• Performed hands-on gEAR workshops at the Association for Research in Otolaryngology and the Gordon Research Conference, increasing the number of users with accounts to greater than 300.

Single Cell RNA-seq of homeostatic neuromasts
Tatjana Piotrowski, Ph.D.

• Optimized protocols for fluorescent-activated cell sorting and scRNA-seq; obtained high quality scRNA-seq transcriptome results from 1,400 neuromast cells; clustered all cells into seven groups; and performed analyses to align the cells along developmental time, providing a temporal readout of gene expressions during hair cell development.
  
  

OTHER PROJECTS

Integrated systems biology of hearing restoration
Seth Ament, Ph.D.

• Discovered 29 novel risk loci for age-related hearing difficulty through new analyses of genome-wide association studies of multiple hearing-related traits in the U.K. Biobank (comprising 330,000 people), and predicted the causal genes and variants at these loci through integration with transcriptomics and epigenomics data from HRP consortium members.

• Generated scRNA-seq of 9,472 cells in the neonatal mouse cochlea and utricle (postnatal days 2 and 7).

• Conducted systems biology analyses that integrate multiple HRP datasets to characterize gene regulatory networks and predict driver genes associated with the development and regeneration of hair cells. These analyses utilize scRNA-seq of sensory epithelial cells in mouse, chicken, and zebrafish hearing and vestibular organs, as well as epigenomic data (ATAC-seq) from hair cells, support cells, and non-epithelial cells in the mouse cochlea.

Comparison of three reprogramming cocktails
Andy Groves, Ph.D.

• Created and validated transgenic mouse lines expressing three different combinations of reprogramming transcription factors.

• Demonstrated these lines can produce new hair cell–like cells in the undamaged and damaged cochlea of the immature mouse.

• Compiled preliminary data showing Atoh1 and Gfi1 genes can create ectopic hair cells in the adult mouse cochlea.

Signaling molecules controlling avian auditory hair cell regeneration
Jennifer Stone, Ph.D.

• Identified four molecular pathways (FGF, BMP, VEGF, and Wnt) that control hair cell regeneration in the bird auditory organ. These pathways were identified in Phase I (gene discovery) as being transcriptionally dynamic in birds, fish, and mice during regeneration, which indicated they may be universal regulators of hair cell regeneration.

• Determined that the Notch signaling pathway (a powerful inhibitor of stem cells) also blocks supporting cell division in the chicken auditory organ after damage. This discovery shows that Notch is a negative regulator of regeneration, conserved in birds, fish, and mice.

• Identified signaling molecules in birds that are correlated with either mitotic or non-mitotic modes of hair cell regeneration, and are now exploring how these signaling molecules interact to determine which mode of regeneration occurs. Since mammals only exhibit non-mitotic regeneration, we are particularly interested in determining how this mode is controlled.
  
  

UP NEXT

We look forward to our annual meeting, which will be held in Seattle in November. There we will discuss and integrate these data to develop our plans for our 2019–20 projects.

As always we are very grateful for the donations we receive to fund this groundbreaking research to find better treatments for hearing loss and related conditions. Every dollar counts, and we sincerely thank our supporters.

HRP scientific director Peter G. Barr-Gillespie, Ph.D., is a professor of otolaryngology at the Oregon Hearing Research Center, a senior scientist at the Vollum Institute, and the interim senior vice president for research, all at Oregon Health & Science University. For more, see hhf.org/hrp.

Empower the life-changing research of the Hearing Restoration Project and other scientists today.

ReSound LiNX Quattro: More Access to Sound; Rechargeable Convenience

By Dr. Laurel A. Christensen

In September, GN Hearing launched ReSound LiNX Quattro. Described as the world’s first “Premium Plus” hearing aid, ReSound LiNX Quattro has generated strong interest among the hearing loss community. As ReSound’s Chief Audiology Officer, I’ve answered many questions about this latest innovation in hearing to facilitate informed decision-making. Here are two of the most common questions I receive.

Can you share the latest features and improvements in ReSound LiNX Quattro? What makes it “Premium Plus”?

ReSound LiNX Quattro is the fourth generation of the LiNX hearing aid family. LiNX streamlined technology with Made for Apple hearing aids in 2014, and brought remote fine-tuning capabilities to audiology in 2017 with ReSound Assist, which allows for adjustment without an additional clinic visit. Both of these breakthrough features are included with ReSound LiNX Quattro, plus more.  

Built on a newly designed, powerful microchip platform, it brings users an unprecedented combination of benefits, while enabling hearing capabilities never before possible. Putting sound quality first, ReSound LiNX Quattro technology enables patients to hear more “Layers of Sound,” delivering an extended range of sounds never before heard clearly through hearing aids. The sound quality is natural; soft sounds are clear and loud sounds are rich, full, and distortion-free. Users enjoy an especially marked improvement when listening to music.

The powerful radio provides more reliable, faster streaming and connectivity to any wireless accessory or mobile device. Using the ReSound Smart 3D app, users can take advantage of on-the-go sound personalization such as changing hearing aid programs, adjusting volume, decreasing the level of background or wind noise in the environment, and adjusting streaming sounds from a mobile phone. Also included is a geo-tag function for frequently visited locations so users can return to their preferred location-specific settings as desired.

Finally, ReSound LiNX Quattro is the world’s most advanced rechargeable solution. As many hearing aid users know, changing batteries weekly can be cumbersome, especially for those with impaired dexterity and eyesight. The built-in lithium-ion batteries eliminate the weekly need to change batteries with a rechargeable battery that lasts up to 30 hours. The recharging case holds 90 hours of portable power, greatly reducing the fear of depleted batteries.

How does ReSound LiNX Quattro actually extend the range of hearing? 

ReSound LiNX Quattro introduces four newly designed microchips that combine to deliver twice the memory, 100 percent more speed, and 30 percent more computing power—with 20 percent power consumption reduction.

The new chipset allows for an increase to 116 dB of input dynamic range so that sounds enter the hearing aid without distortion. In addition, the frequency bandwidth has been extended to 9.5 kHz both for the hearing aids and for sounds streamed to the devices.

In many other hearing aids, sounds outside these ranges are not heard or are heavily distorted. With ReSound LiNX Quattro, sounds typically missed such as birds singing, higher-pitched speech, or music are clearly discerned.

And by expanding access to sounds, especially higher frequency sounds, we observe improved spatial perception in users, with more cues for localization.

Learn more about ReSound LiNX Quattro.

Laurel A. Christensen, Ph.D. is the Chief Audiology Officer of GN ReSound Group.  In this role, she leads Global Audiology & User Experience in Research and Development.  She holds adjunct faculty appointments at Northwestern and Rush Universities and is a former member of the Executive Board of the American Auditory Society and a member of the Advisory Board for the Au.D. Program at Rush University.  In 2015, she received the Distinguished Alumna Award from the Department of Speech and Hearing Sciences at Indiana University.

Breaking The Silence

By Joe Mussomeli

If there's one thing my brother, Alex, and I love, it’s spending time with our cousins in Maryland. We’ve been visiting them for years now—each stay more fun than the last.

These visits have left us all with happy memories of holding Mario Bros. competitions on the Wii, playing tag downstairs, watching funny movies, and, most importantly, telling stories before bed. When we were little, we used to tell stories to each other all the time. Together we’d create ridiculous parodies of fairy tales taking place in obscure settings, including our own versions of Jack and the Beanstalk and The Jungle Book. We loved telling these stories.

One time, we finished telling our stories and readied ourselves for bed. As usual, my brother Alex took his hearing aid and cochlear implant off in preparation for sleep. After this, our cousin Lara, who was only five years old at the time, asked Alex a question. When he didn’t reply, she repeated her question. To her confusion, he didn’t say anything once more. Lara then called for my mom and asked why Alex wasn’t answering. My mom explained to Lara that when Alex takes off his hearing aid and implant, he cannot hear anything.

“He can’t hear anything?” Lara asked.

“He can’t hear anything,” my mom confirmed.

Lara was silent for a few seconds before she said something; “You know, it’s challenging, but maybe it’s peaceful at times, not hearing a sound. Maybe it’s relaxing for him.”

Though our visits to Maryland are enjoyable, Alex’s hearing loss has presented challenges for our family when we go swimming with our cousins. When Alex was little, he was unable to wear his hearing devices while swimming, for they were not waterproof. This meant that he could not converse with our cousins in the pool; he couldn’t join in on the conversation in a meaningful way. He could talk, but he couldn’t respond. He could swim with them in the pool, splash water in their eyes, and laugh along with them. He just couldn’t hear his own laughter.

We all worried that Alex would get hurt while swimming without his hearing technology.  My cousins and I tried our best to help Alex when we were in the pool. We would always swim near him, making sure he was safe. I, in particular, would answer questions anyone was trying to ask Alex when he was in the water. I hope I did my best to help him out during these early years.

All of this changed when something marvelous entered our lives, a waterproof cover for his cochlear implant that makes it usable for swimming. This has made swimming so much better for Alex. He can now hear in the pool and can socialize with others. He can talk with our cousins, splash them with water, and hear his own laughter. Now, whenever someone asks him for his name, he can confidently say, “I’m Alex, what’s your name?”

Joe Mussomeli is a 10th-grade student who lives in Westport, CT. His younger brother, Alex, has been featured in Hearing Health magazine and is a participant in HHF’s “Faces of Hearing Loss” campaign.

ERG Grantees' Advancements in OAE Hearing Tests, Speech-in-Noise Listening

By Yishane Lee and Inyong Choi, Ph.D.

Support for a Theory Explaining Otoacoustic Emissions: Fangyi Chen, Ph.D.

It’s a remarkable feature of the ear that it not only hears sound but also generates it. These sounds, called otoacoustic emissions (OAEs), were discovered in 1978. Thanks in part to ERG research in outer hair cell motility, measuring OAEs has become a common, noninvasive hearing test, especially among infants too young to respond to sound prompts..

There are two theories about how the ear produces its own sound emanating from the interior of the cochlea out toward its base. The traditional one is the backward traveling wave theory, in which sound emissions travel slowly as a transverse wave along the basilar membrane, which divides the cochlea into two fluid-filled cavities. In a transverse wave, the wave particles move perpendicular to the wave direction. But this theory does not explain some anomalies, leading to a second hypothesis: The fast compression wave theory holds that the emissions travel as a longitudinal wave via lymph fluids around the basilar membrane. In a longitudinal wave, the wave particles travel in the same direction as the wave motion.

Figuring out how the emissions are created will promote greater accuracy of the OAE hearing test and a better understanding of cochlear mechanics. Fangyi Chen, Ph.D., a 2010 Emerging Research Grants (ERG) recipient, started investigating the issue at Oregon Health & Science University and is now at China’s Southern University of Science and Technology. His team’s paper, published in the journal Neural Plasticity in July 2018, for the first time experimentally validates the backward traveling wave theory.

Chen and his coauthors—including Allyn Hubbard, Ph.D., and Alfred Nuttall, Ph.D., who are each 1989–90 ERG recipients—directly measured the basilar membrane vibration in order to determine the wave propagation mechanism of the emissions. The team stimulated the membrane at a specific location, allowing for the vibration source that initiates the backward wave to be pinpointed. Then the resulting vibrations along the membrane were measured at multiple locations in vivo (in guinea pigs), showing a consistent lag as distance increased from the vibration source. The researchers also measured the waves at speeds in the order of tens of meters per second, much slower than would be the speed of a compression wave in water. The results were confirmed using a computer simulation. In addition to the wave propagation study, a mathematical model of the cochlea based on an acoustic electrical analogy was created and simulated. This was used to interpret why no peak frequency-to-place map was observed in the backward traveling wave, explaining some of the previous anomalies associated with this OAE theory.

Speech-in-Noise Understanding Relies on How Well You Combine Information Across Multiple Frequencies: Inyong Choi, Ph.D.

Understanding speech in noisy environments is a crucial ability for communications, although many individuals with or without hearing loss suffer from dysfunctions in that ability. Our study in Hearing Research, published in September 2018, finds that how well you combine information across multiple frequencies, tested by a pitch-fusion task in "hybrid" cochlear implant users who receive both low-frequency acoustic and high-frequency electric stimulation within the same ear, is a critical factor for good speech-in-noise understanding.

In the pitch-fusion task, subjects heard either a tone consisting of many frequencies in a simple mathematical relationship or a tone with more irregular spacing between frequencies. Subjects had to say whether the tone sounded "natural" or "unnatural" to them, given the fact that a tone consisting of frequencies in a simple mathematical relationship sounds much more natural to us. My team and I are now studying how we can improve the sensitivity to this "naturalness" in listeners with hearing loss, expecting to provide individualized therapeutic options to address the difficulties in speech-in-noise understanding.

2017 ERG recipient Inyong Choi, Ph.D., is an assistant professor in the department of communication sciences and disorders at the University of Iowa in Iowa City.

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

A Fast Track to Hearing Damage

By Andrew J. Guralnick

Millions of commuters using the New York City subway system know it can be noisy, but just how loud is it? As a 2018 Hearing Health Foundation (HHF) intern, I set out to measure the danger that the NYC Metropolitan Transit Authority (MTA) subway system presents to riders and employees.

I found that the system significantly breaches the threshold of what is safe for our ears. To protect hearing, both the U.S. Environmental Protection Agency and the World Health Organization recommend an average exposure limit of 70 decibels (dB) over the course of 24 hours. But what we measured exceeds that limit: Our samples show the average noise levels on all subway platforms and on all subway rides (inside subway trains) is between 72.5 and 76.5 dB and between 74.1 and 75.8 dB, respectively. And, with maximum readings actually as high as 119 dB on platforms and 120 dB on rides—based on actual recorded data within the sample—the NYC subway is likely an auditory minefield. (See hhf.org/subway for full data.)

Using our data’s sample averages, I determined ranges as to what the actual averages are on all subway platforms and rides through the MTA system. Based on the data, we are 99 confident about our results.

Collecting and Analyzing

From January to August 2018, three data collectors used Decibel Meter Pro, a smartphone app on iPhones and an iPad to collect 120 samples from platforms and rides. All 60 platform samples were equally represented at five minutes each. The 60 ride samples were assigned random recording lengths from 10 to 30 minutes. Samples on Saturday and Sunday or between 11 p.m. and 4:45 a.m. on any day were excluded. Random sampling was utilized as much as possible to help ensure generalizability on behalf of all platforms and rides.

The analysis examined potential harm to hearing from loud noises on subway platforms and loud noises during subway rides. For platform noise, the main variable is the number of trains that pass; for subway ride noise, the main variable is the number of local stations the train passes. We also investigated the number of seconds the subway noise level reached 75 dB or higher.

When measuring subway rides, we noted train travels between Manhattan and another borough or vice versa; whether a train runs above ground; whether the sample was collected during rush hour; and whether a local train ever becomes an express train, with fewer stops.

The statistical method of multiple regression was used to predict dangerous noise exposure on both platforms and rides. We can predict that each train that enters or leaves a platform will expose a rider’s ears to 16.53 seconds of noise at 75 dB or higher. For example, if a rider waits at a platform where two trains come and go before their train arrives, that would be a predicted exposure of 82.65 additional seconds of noise at 75 dB or higher.

We can also predict that each subway stop that is passed will expose a rider’s ears to 36.06 seconds of noise of 75 dB or higher. For example, if a rider passes 10 local train stops on their trip, the predicted exposure of noise at 75 dB or higher is 360.60 additional seconds—or 6.01 additional minutes.

Cumulative Effect

HHF’s recommendation for commuters, MTA staff, and platform retailers such as newsstand operators is simple: Wear ear protection. MTA staff and platform retailers are at elevated risk given the hours they spend underground and on the trains. The tendency for many commuters to block noise by raising the volume of their headphones is not a helpful approach and could in fact damage hearing even more.

The subway is merely only one of many sources of daily noise. “Noise-induced hearing loss can result from a single, sudden noise event and from constant exposure to loud noises that has a cumulative effect (not unlike sun exposure) and can lead to related negative health effects when unknown and untreated,” says Lauren McGrath, HHF’s marketing manager.

The MTA appears aware of the issue of subway noise. The newly built Second Avenue subway line uses effective noise-reduction measures such as “low vibration tracks and sound absorbing panels.” We hope the MTA will continue to use these quieter, low vibration tracks when making subway and station upgrades, especially since they are more cost-effective than traditional wooden tracks.

2018 HHF intern Andrew J. Guralnick is pursuing a master’s in public administration at Baruch College in New York City.

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Study Points to Possible New Therapy for Hearing Loss

University of Rochester Medical Center research associate professor Patricia White, Ph.D. (2009 and 2011 ERG), with Hearing Restoration Project member and Harvard Medical School professor Albert Edge, Ph.D., and team have been able to regrow the sensory hair cells found in the mouse cochlea. The study, published in the European Journal of Neuroscience on Sep. 30, 2018, builds on White’s prior research that identified a family of receptors called epidermal growth factor (EGF) that is responsible for activating supporting cells in the auditory organs of birds. When triggered, these cells proliferate and foster the generation of new sensory hair cells. In mice, EGF receptors are expressed but do not drive regeneration of hair cells, so it could be that as mammals evolved, the signaling pathway was altered.

The new study aimed to unblock the regeneration of hair cells and also integrate them with nerve cells, so they are functional, by switching the EGF signaling pathway to act as it does in birds. The team focused on a specific receptor called ERBB2, found in supporting cells. They used a number of methods to activate the EGF signaling pathway: a virus targeting ERBB2 receptors; mice genetically altered to overexpress activated ERBB2; and two drugs developed to stimulate stem cell activity in the eye and pancreas that are already known to activate ERBB2 signaling. The researchers found that activating the ERBB2 pathway triggered a cascading series of cellular events: Supporting cells began to proliferate and started the process of activating other neighboring stem cells to lead to “apparent supernumerary hair cell formation,” and these hair cells’ integration with the network of neurons was also supported. —University of Rochester

A Birthday Wish

By Beth and Jeremy Hochheiser

In Beth’s words

Our son Jeremy recently turned 29, and on his Facebook page for his birthday he asked friends and family to donate to Hearing Health Foundation (HHF) to help reach his fundraising goal, which he exceeded.

Jeremy introduced us to HHF when he discovered its commitment to hair cell regeneration. He has a profound hearing loss and has been using hearing aids successfully since childhood.

We did not realize there was a problem with Jeremy’s hearing until he was 14 months old, mainly because he had been making sounds like a typical baby. Even as a baby, Jeremy had an infectious belly laugh and was always very attentive to what was going on around him.

But when we discussed a potential hearing issue with our pediatrician, he didn’t seem concerned. It was only after we went to see an audiologist that we finally got a diagnosis of profound congenital bilateral sensorineural hearing loss.

Throughout Jeremy’s childhood we visited audiologists and doctors regularly. We found an otolaryngologist in New York City who specialized in hearing disabilities, and we kept up with the latest technology in hearing aids to understand options for Jeremy. As he got older, we always encouraged him to ask people to repeat themselves if he couldn’t hear or understand them.

Since Jeremy was diagnosed with quite a bit of residual hearing, the audiologist suggested an unconventional means of learning speech, the auditory-verbal approach introduced by Helen Beebe. Children learn to use the hearing they still have by being bombarded with speech consistently. I read books and introduced pictures by sitting next to Jeremy, not facing him, so that he could learn how to repeat words and speech sounds by hearing them rather than watching my face and lip-reading (speech-reading).

As a result, Jeremy’s speech developed as a typical hearing child’s would—by listening and repeating. We as his parents knew that developing Jeremy’s hearing to its fullest potential was imperative—the “if you don’t use it, you’ll lose it” theory—and that lip-reading could come later, as a supplement.

With his hearing aids Jeremy thrived, learned to play piano at age 7, and was even presented with an award at Carnegie Hall. He excelled at school and showed us that his hearing was not an issue or a factor that would get in the way of his education or competitive spirit. The natural belly laugh he had as an infant translated into a great sense of humor and positive outlook.

As a kid Jeremy liked to place one of his hearing aids on his tummy and say, “Mom, I’m so hungry, I can hear my stomach growling!” We’re so proud of him and his studies, the many activities he has thrown himself into, his thriving career, and his own family and baby to come.

In Jeremy’s words

Be Bold

My advice to anyone with hearing loss (and their loved ones) is to be bold, brave, and up front about your hearing. Accept it and wear it proudly, otherwise others may misinterpret who you really are, or even bully you. Hearing loss isn’t who you are.

I also tell those new to hearing loss to never stop using your mind. Your brain is your most powerful tool. I play chess and compete in Brazilian jiu jitsu, plus I love being a software engineer and doing math, exploring art, and enjoying nature. I love to learn. Being fully engaged keeps your brain active and fights off feeling down from hearing challenges.

Your Voice Matters

I remember being afraid to ask others to repeat themselves, but as I got older I learned to ask, even if I had to do it more than once. In this way, I show I am involved and can contribute meaningfully to the conversation. I earn respect for that. Your voice and opinions really do matter. What definitely won’t work is to hold back.

I consider myself outgoing and social, and sometimes when I ask someone to repeat themselves it can break the flow of the conversation, or cause frustration in a new acquaintance who doesn't understand. But it is infinitely more frustrating if you can’t fully participate.

New Challenges

My wife Lauren has typical hearing and sees me as a typical hearing person. But when I am tired and my hearing is down, I have to ask her to repeat herself or let me see her lips. Then when I am less tired and my hearing is better, I get frustrated again if Lauren is still making accommodations for me that I don’t think I need—and that can frustrate her, constantly having to switch! I love my wife for going with the flow and understanding what I need to hear.

Managing Hearing Loss

I find that there are days where I can understand what people are saying without looking, and then sometimes I have to rely on speech-reading. People have said to me, “You can hear better with your glasses on!”

The company I work at now has wonderful benefits and accommodations for their employees with needs. They hold meetings throughout the year among those who have requested accommodations in order to foster an inclusive environment, and they are proactive about making sure I have everything I need. It’s been very welcoming.

How I Discovered HHF

When I was in middle school, my parents and I went to a support group, and I met a couple of kids my age who also have a hearing loss. One of them happened to end up working for HHF, Laura Friedman (HHF’s former communications and programs manager).

I also followed news on the development of hair cell regeneration in the inner ear. I am encouraged about HHF’s Hearing Restoration Project consortium bringing together multiple labs and scientists. All of this is why I wanted to do my part to raise money for research toward the cure.

Beth Hochheiser lives in New Jersey, and Jeremy Hochheiser lives in Pennsylvania. HHF sincerely thanks the Hochheisers and their family and friends for their support.

Painting for a Cure

By Nicolle Cure

My art is the fuel that ignites my passion for helping others. I use my art as a tool to create so that I can support the causes I believe in. Throughout my life, I have created several collections, for the most part biographical. To date, I've been blessed to have the opportunity to collaborate with animal welfare campaigns as well as education and health research initiatives. I am now proudly raising awareness about a particular cause that is dearest to my heart—hearing loss and vestibular (balance-related) disorders—after experiencing these conditions myself.

Nicolle painting in her studio. Credit: Lia Selfridge

On August 4, 2017, I woke up and noticed that the right side of my head was numb. I felt a strong pressure in my right ear and couldn’t hear anything as my ear felt blocked and full. It was really scary and very sudden.

Since that day, I have been in and out the hospital trying to decipher what is wrong with me and how to cure it. My first audiology appointment showed a profound hearing loss in my right ear, and after steroids injected into the middle ear for two months, I was able to recover the ability to hear low frequencies. However, the high frequencies only improved to severe (from profound), which is why I now suffer from tinnitus and I am extremely sensitive to environmental sounds.

My hearing loss was only the beginning. During the initial months, I also suffered from BPPV (benign paroxysmal positional vertigo), debilitating vertigo episodes, chronic migraines, constant nausea, and dizziness. My balance was completely off and I swayed to the right when walking. It felt like I was walking on quicksand. Another symptom that persisted for months was chronic fatigue, to the point that I could not get out of bed on certain days. My body felt heavy as if I had a slab of concrete on top of me.

These “invisible conditions” can really affect patients an emotional level. I was completely isolated from the world, I didn’t want to see anyone, and I avoided phone calls and going out. I’ve always been a very independent person and the fact that I couldn’t do anything or go anywhere made me feel frustrated most of the time.

My boyfriend Felipe, a communications professional and music producer, has been the greatest companion, helping me thrive and heal with his patience and love, and for that I am truly grateful. We share a passion for music and going to concerts, but from the time of my hearing loss I avoid loud places and crowds in general. I know music to him means as much as art to me, so I now wear custom musician’s earplugs. I am also investigating a hearing aid for my right ear, which my audiologist recommended after a recent tinnitus assessment to manage my tinnitus and sound sensitivity. Vestibular rehab therapy helped me regain my balance, as I had difficulty walking or even just standing still.

And of course my art has been my most powerful coping mechanism. While I am in the process of creating, I can focus better and forget about my symptoms. Painting makes me ignore my tinnitus even for a short period of time.

This experience has given me the opportunity to create awareness about invisible conditions. It is a fuel that continues to ignite my passion for the arts and for helping others. It has given me a sense of purpose—I truly feel the need to wake up and create something beautiful to deliver a powerful message of positivism in spite of my symptoms.

In “The Colors of Sound” painting collection, I am trying to capture emotions and moods in sound. Using his recording equipment, Felipe showed me the range of frequencies that I was not able to hear anymore. It was a bizarre experience to be able to see the sound waves and frequencies that I could no longer hear. These ink paintings replicate the energy and movement of what was now missing.

Behind every invisible illness there are wonderful individuals with the will to thrive and heal. Helping others has been incredibly therapeutic for me, and I gained so much support from people, too. I want to create a space for dialogue so people can be open about their conditions and find treatments and relief and know that they are not alone in this journey.

Nicolle Cure is an artist based in Miami. “The Colors of Sound” appeared at Art Basel in Miami Beach (December 2017–February 2018).

A better quality of life for Nicolle—and so many others—is dependent on research funding for HHF’s groundbreaking scientific programs.

Please, if you are able, make a contribution to the research that will someday make it possible for Nicolle and millions of others to reclaim their independence.

Specially Timed Signals Reduce Tinnitus Symptoms

In a double-blind clinical trial with 21 subjects, University of Michigan Medical School professor Susan Shore, Ph.D. (1987 and 1992–95 ERG) and team showed an experimental device could help people with tinnitus (hearing ringing or buzzing in the absence of an external sound source). Fusiform cells, the main neurons in the brainstem’s dorsal cochlear nucleus region, help the brain focus on where sounds are coming from, and help tune out sensations that result from the movement of our own head and neck (known as somatosensory inputs). The team’s previous work in animals showed that loud noise can trigger a change in the nerve cells’ activity—altering its timing so that the cells fire off synchronized signals spontaneously instead of waiting for an actual sound from the environment.

This phantom signal is transmitted into other centers where perception occurs. To stop the signal, the device uses “bimodal auditory-somatosensory stimulation,” which plays a sound into the ears, alternating it with precisely timed, mild electrical pulses delivered to the cheek or neck. Both are aimed at pushing the damaged nerve cells back to typical activity. In the trial, whose results were published in Science Translational Medicine on September 2018, participants using a sham treatment experienced no effect, but those who used the device daily for four weeks reported a decrease in tinnitus and an improved quality of life. —University of Michigan

Meet the Researcher: A. Catalina Vélez-Ortega

By Yishane Lee

2018 Emerging Research Grants (ERG) awardee A. Catalina Vélez-Ortega received a master’s in biology from the University of Antioquia, Colombia, and a doctorate in physiology from the University of Kentucky, where she completed postdoctoral training and is now an assistant professor in the department of physiology.

IN HER WORDS:

TRPA1 is an ion channel known for its role as an “irritant sensor” in pain-sensing neurons (nerve cells). Noise exposure leads to the production of some cellular “irritants” that activate TRPA1 channels in the inner ear. The role of TRPA1 channels has been a puzzling project, with most experiments leaving more questions to pursue. My current project seeks to uncover how TRPA1 activation modifies cochlear mechanics and hearing sensitivity, in order to find new therapeutic targets to prevent hearing loss or tinnitus.

My father, our town’s surgeon, fueled my desire to learn. When I asked him how the human heart works, he called the butcher, got a pig’s heart, and we dissected it together. I was about 5 when I learned how the heart’s chambers are connected and how valves work. He also set up an astronomy class at home with a flashlight, globe, and ball when I asked, “Why does the moon change shape?” My father’s excitement kept my curiosity from fading as I grew older. That eager-to-learn personality now drives my career in science and teaching.

My training in biomedical engineering guided my interest into hearing science. The field of inner ear research mixes physics and mechanics with molecular biology and genetics in a way I find extremely attractive. Analytics also intrigues me. People who work with me know how complex my calendar and spreadsheets can get. I absolutely love logging all kinds of data and looking for correlations. I also like to plan ahead—passport renewal 10 years from now? Already in my calendar!

I take dance lessons and participate in flash mobs and other dance performances. But I used to be extremely shy. As a child I simply could not look anyone in the eye when talking to them. I was also terrified of being onstage. It was only after college that I decided to finally correct the problem. Interestingly, taking sign language lessons was very helpful. Sign language forced me to stare at people to be able to communicate. It was terrifying at first, but it started to feel very natural after just a few months.

Vélez-Ortega’s 2018 ERG grant was generously funded by cochlear implant manufacturer Cochlear Americas.

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