As an individual with Usher syndrome, building awareness is important. Comprehending the invisible condition and knowing how to help or respond to individuals with the condition can be difficult.
Helpful Tips for Parents of Children With Hearing Loss
By Nadine Dehgan
You just learned your child has a hearing loss. It is common (and normal!) to feel overwhelmed, scared, and have many, many questions. Hearing loss is a loss. It’s not uncommon to grieve after receiving a diagnosis. Take some time to come to terms with the news, and know that there are professionals ready to help and there are many resources at your disposal. There are also countless families who have gone through this same experience and have found success.
Here are some helpful tips Hearing Health Foundation cultivated from parents just like you:
Accept your child and cherish them for who they are as a person. This will encourage your child to accept themselves and their hearing loss, providing them with the confidence and assurance that they will be accepted by their peers and community, despite their hearing loss.
Help your child develop a fun, but informative, answer to educate their friends and others about their hearing loss and assistive devices, should they ask when you’re not around. This will help them develop self-advocacy skills they will need later in life.
Discussing your child’s hearing loss with their siblings may be something you dread. Simple is key. Let them ask questions and answer them as honestly, and positively as possible.
If your child’s chosen method of communication is sign language, make lessons a family activity, if the rest of the family communicates orally. It’s important that your child with a hearing loss feels and is included in all conversations, especially those taking place in the household.
Ask your child’s audiologist for recommendations for a speech pathologist, education consultants, and materials to set up your child for success. (*Laura, HHF’s communications and programs manager, mother used and was very thankful for the free resources from the John Tracy Clinic).
If your child is school-aged, set up a meeting with the school district’s Department of Special Education Services and start the Individual Education Plan (IEP) process. Cultivate and maintain the relationships made with Special Services—your child’s academic success depends on it.
Know the laws related to disabilities and special education, so you’re properly equipped to be your child’s best advocate.
Have regular check-ins with your child’s teachers to identify any gaps and ensure they are developing appropriately for their age, both socially and academically.
Have a folder/binder and notebook where you house all the paperwork of test and reports, dates of appointments and procedures. This would be a good place to keep any and all hearing aid, BAHA or cochlear implant warranty information.
Hearing loss accidents happen and sometimes you just have to laugh them off: When Laura was in kindergarten, she used to take a bath after dinner, and her mother would scream down the hall, “Take out your hearing aids!” One day, she forgot to remind Laura, and into the bath those hearing aids went!
Take issues that arise from bullying and other social incidences and turn them into a life lesson about diversity and that being unique is not a bad thing, but something to embrace.
If you’d like to connect to others going through similar situations, or with those who have dealt with these circumstances before, please email us at info@hhf.org. We also encourage you to check out our Pediatrics page for more information and tips.
Nadine Dehgan is the former CEO of Hearing Health Foundation.
The mother of two boys who use cochlear implants, Alaine Jacobs shares “10 Ways to Be Encouraged When You’re Disappointed by a Hearing Loss Diagnosis” on her blog.
In the Midst of Numerous Natural Disasters, HHF's Emergency Preparation Guide Makes Headlines
HHF Communications and Program Manager's Laura Friedman's "Guide For Preparing For Emergencies When You Have Hearing Loss" recently made headlines in PRNewswire and many national news outlets.
As this year's vicious hurricane season carries on and, just yesterday, the effects of Mexico's devastating hurricane were felt in California, emergency preparation plans for individuals with hearing loss remain more critical than ever.
View the full press release, here.
New Study Suggests Serotonin May Worsen Tinnitus
Millions of people suffer from the constant sensation of ringing or buzzing in the ears known as tinnitus, creating constant irritation for some and severe anxiety for others. New research by scientists at OHSU shows why a common antidepressant medication may actually worsen the condition.
Researcher Discovers Gene Mutation Related to Usher Syndrome Type 3
By Pranav Parikh
Usher syndrome type 3 is an inherited disease in which an individual is born with typical hearing and develops hearing loss in the stages of early childhood. They will most likely develop complete hearing loss by the time they are an adult. Though cases of Usher syndrome type 3 (and its subtypes) are quite infrequent, representing 2 percent of total Usher syndrome cases, the onset symptoms have damaging and often irreversible consequences that severely disrupt the lives of those living with the condition. There is currently no cure for the disease, but cochlear implants have seen some success in providing partial hearing function in patients.
A 3D model of the HARS enzyme, including the catalytic site (where the reaction occurs) and the anticodon site (the part that starts protein synthesis through RNA transcription).
Susan Robey-Bond, Ph.D., a 2012 Emerging Research Grants scientist, and her team at the University of Vermont College of Medicine were able to isolate a mechanism involved in the development of Usher syndrome. Histidyl-tRNA synthetase is an enzyme that is instrumental in protein synthesis. This enzyme, given the acronym HARS, is thought to be involved in the presentation of Usher syndrome type 3B in patients. The early symptoms of temporary hearing and vision loss, hallucinations, and sometimes sudden fatal buildup of fluid in the lungs may be triggered by a fever-causing illness. The hearing and vision loss are eventually severe and permanent.
A graphical representation depicting temperature variation between the wild-type and mutant version of the HARS enzyme.
Usher syndrome type 3B is autosomal recessive, meaning children of parents carrying the gene but who do not display symptoms have a likelihood of developing the disease. It is caused by a USH3B mutation, which substitutes a serine amino acid for a tyrosine amino acid in HARS. The team studying the biochemical properties of the gene compared the Y454S mutation in the HARS enzyme with its wild-type (non-mutated) form and found similar functional biochemical characteristics, as stated in the researchers’ recent paper in Biochemistry.
The amino acid activation, aminoacylation, and tRNA binding functions were all consistent between the mutation and wild-type genes. In later analysis, though, the team found that at an elevated temperature the Y454S substitution was less stable than the wild-type. More specifically, cells from patients containing the Y454S mutation displayed lower levels of protein synthesis, which could explain the onset of deafness these patients experience. How these proteins are implicated in the hearing processes will eventually help develop cures or better treatments for Usher syndrome.
Susan Robey-Bond, Ph.D., was a 2012 Emerging Research Grants recipient. For more, see her Biochemistry paper:, “The Usher Syndrome Type IIIB Histidyl-tRNA Synthetase Mutation Confers Temperature Sensitivity.”
HHF Attends Cantor Fitzgerald’s Charity Day, a Star-Spangled Effort to Build Hope on Tragic 9/11 Anniversary
By Yvonnie Phan
Hearing Health Foundation (HHF) was among the dozens of charities selected to participate in yesterday’s Cantor Charity Day—an annual, celebrity-filled philanthropic event held around the anniversary of September 11—at Cantor Fitzgerald’s New York City headquarters.
From left: HHF Board Vice Chair Paul Orlin, Cantor Fitzgerald Relief Fund President Edie Lutnick, Former Hockey Star Martin St. Louis, HHF CEO Nadine Dehgan
HHF’s attendance marked the organization’s fifth year of participation. HHF was represented by Hockey Legend Martin “Marty” St. Louis, HHF Board member Paul Orlin, and Nadine Dehgan, HHF’s CEO.
As Marty’s own foundation has supported HHF’s life-changing hearing and balance research in the past, HHF was especially thankful that he also dedicated his time to serve as an ambassador.
Marty St. Louis and Robert De Niro
The HHF trio had a particularly memorable encounter with Robert De Niro and another hockey star, Mike Richter, who were also among Charity Day’s celebrity roster. Marty told Mr. De Niro that in his days as a player (for Tampa Bay Lighting, Calgary Flames, and the New York Rangers), he impersonated De Niro’s film roles in the locker room to energize his teammates before big games—and the movie star found that amusing.
All proceeds from Cantor Charity Day will benefit the Cantor Fitzgerald Relief Fund, which aids the families of the firm’s 658 employees who perished in the World Trade Center attacks. Since its 2001 establishment, the Relief Fund has expanded its reach to also support victims of terrorism, natural disasters, and emergencies, wounded military personnel, and other charitable causes worldwide, including HHF.
Marty making trades on the phone
HHF CEO Nadine Dehgan reflects on the day: “Being part of this event where everyone gets together for the sole purpose of helping others is so inspiring. I am honored HHF was selected to participate again and grateful for the folks who make it happen, especially the Relief Fund’s President, Edie Lutnick, whose energy is unmatched. Marty was a superstar on the phone helping make trades!”
The Fund’s prioritization of wounded veterans is especially compatible with HHF’s work; tinnitus and hearing loss are the most common disabilities that afflict returning American military members.
“HHF will use the funds received from this event to continue to be a resource for veterans with hearing loss and to further research for treatments and cures for the 360 million people around the world who have hearing loss,” Nadine pledges.
A Guide for Preparing for Emergencies When You Have Hearing Loss
By Laura Friedman
“By failing to prepare, you are preparing to fail.” -Benjamin Franklin
With the recent devastation caused by Hurricane Harvey in Southern Texas, the wildfires in the Midwestern United States and California, and impending Hurricane Irma, a Category 5 storm predicted to hit the coast of Florida, Hearing Health Foundation (HHF) encourages everyone to have an action plan in place for the various emergencies one can face.
For people with hearing loss, emergencies can bring extra challenges and anxiety. As someone with a hearing loss myself, I'm nervous I'll sleep through my building's fire alarm or miss my subway stop due to not hearing an announcement. The latter may sound trivial, but it means I'm always hyper-aware of my surroundings, which is exhausting and also a little nerve-racking. So, when it comes to natural disasters and situations where my safety may be at stake, a predetermined action plan puts me (and my loved ones) at ease.
Here are some of HHF's tips for navigating one's hearing loss needs during emergencies:
1. Stock Up On the Basics and Know Your Power Sources
Keep extra batteries in your emergency kit for hearing aid and/or your cochlear implants, as well as for visual or sensory alerting systems.
For hearing aids, it's recommended to have a month's worth of batteries on hand.
For cochlear implants, consider a portable battery charger, especially because batteries generally last at most about eight hours. The chargers themselves need to be charged, so use them sparingly.
Procure a waterproof/sealed container that is large enough to hold hearing aids or cochlear implants to protect them in cases of extreme weather or susceptibility to damage/displacement, e.g., in an earthquake.
Remember your car can be a power source. Depending on the model and whether you have gas, your car may have some power for charging things like a cochlear implant battery pack or a cellphone through its USB port.
2. Emergency Contact List
Have a contact list printout with phone numbers for those to reach out to in case of emergency, as well as local text-9-1-1 services.
Keep your audiologist/ENT's number on hand, in addition to the phone number of the hearing aid or cochlear implants company in case the device needs to be repaired or for emergency support.
For those who live in urban areas, contact your city's Mayor's Office for People with Disabilities (MOPD) to inquire for emergency services and alerts; services vary greatly by city. For example, in New York City, there's a phone "tapping system" for connecting with local police and fire departments if vocalizing your needs is a challenge.
For those in suburban and rural areas, contacting your local Mayor's office may also be useful, as well as introducing yourself to the local police and fire departments. If you belong to a church, synagogue, or other place of worship, your religious leader may have local resources available or know the right people to connect you to.
3. Communication With Authorities and Loved Ones
When the power's out, it's important to have access to a regular landline phone (not cordless), battery-powered amplifier, or battery-powered TTY.
Buddy systems are not just for toddlers. It's recommended to have two or three friends locally who agree in advance to check up on you in case of an emergency.
One contact should be out of state in case the local power and/or telephone lines are down.
If your smartphone and laptop have internet access and power, use social media to stay in touch and ask friends for help, as well as mark yourself safe, through Facebook's Safety Check.
Reverse 911 is available in some communities; This service will call YOU in an emergency. Check with your local emergency management office to find out if Reverse 911 is available and if they have TTY capabilities.
Key Phrases Card: Have a card printed out that has key phrases to help you communicate with local authorities.
4. Emergency Alerts
Visual Fire Alarms with Strobe Lights: Many cities and states, such as Houston, offer free visual fire alarms for the D/d/HoH. You can also visit the National Fire Protection Association's website for a list of brands that meet that U.L. standards for smoke alarms.
Sign up for weather and other emergency alerts through The Emergency Email & Wireless Network at emergencyemail.org.
NOAA Weather Radio: Provides one of the earliest warnings of weather and other emergencies and is programmed to alert you to hazards in your specific area. Some have a warning light, while others have LCD screen for alerts.
Notify Community Emergency Response Team (CERT), neighborhood watch, community block associations, and other local groups and authorities about your needs.
For more information, contact:
Federal Emergency Management Agency (FEMA): fema.gov
Ready (part of Homeland Security): ready.gov
Department of Labor’s Disability Resources: www.dol.gov/general/topic/disability
All of us at HHF—the staff, Board of Directors, consultants, and volunteers—have the victims of recent natural disasters in our thoughts and hope for swift relief for those affected and displaced.
With Her Ears On, Sherri Can Enjoy the Sounds of Her Grandchildren
By Sherri Ely
I lived a normal life in the hearing world until about 18 years ago when I started noticing I had a harder time following group conversations and talking on the phone. I discovered I had a moderate hearing loss in all the mid and high frequencies. For several years, I benefited greatly from hearing aids. I had a successful sales career in the Professional Beauty industry and was doing well.
One day, I began noticing that my hearing aids no longer helped with the clarity of speech. I could hear the sound of someone speaking, but had no idea what they were saying.
A day that is forever etched in my memory is the day I resigned from my job. I pulled up to go into one of my accounts at a noisy salon and literally could not get out of my car. The thought of walking into that business and not being able to understand what was being said to me over the background noise was overwhelming. My employer was wonderful and tried to place me in a position within the company where I didn’t need to rely as much on my hearing, but I just wasn’t able to continue working.
Because my hearing aids were no longer benefiting me, I was evaluated to see if I was a candidate for a cochlear implant (CI). While I met the criteria for a CI, I was concerned about losing my residual hearing. I felt I would be giving up normal hearing in one level to gain hearing in another. So I chose to wait.
In the following years, I lived between two worlds.
I no longer fit into the normal hearing world because I could not have social conversations in a group or talk on the phone, or hear children's voices at all.
And I didn't fit into the deaf community because I did have some hearing and I could not understand sign language or lip read very well.
About this time we received some great news. Our daughter was expecting our first grandchild! I was overjoyed to be a “Gammy,” but overwhelmed that I would never hear her little sounds, her cry, her little voice!
This sent me on a desperate search for help. This is when I found information about the EAS (electric-acoustic stimulation) implant from MED-EL. I read everything I could find and had hope again for the first time in many years. This new technology sounded perfect for me because it incorporated cochlear implant technology with an acoustic component. At the time, EAS was still being tested, and I was able to enroll in a clinical trial.
I was implanted with the EAS in 2010, and my granddaughter was born 6 months later. I was there when she was born and HEARD every cry and little sound she made. Today she is a chatty six-year-old that loves to ask me hundreds of questions. Her favorite question, when I don't answer her quickly enough, is, “Gammy, do you have your ears on?”
Today, because of my EAS, I can say, “I have my ears on and I'm enjoying all the sounds of my new grandson and my very talkative granddaughter.”
EAS implant recipient Sherri Ely, 58, from Charleston, SC, shares her story just in time for Grandparents Day, September 10. A recent survey from MED-EL found that 66% of adults said that a major life event (e.g., marriage, birth of a child/grandchild, health scare) would motivate them to get their hearing checked.
Celebrating Your Birthday in September? Learn How You Can Help HHF!
By Lauren McGrath
Sharing birthday presents is a popular custom. Most Americans report positive emotions when giving gifts. 83 and 78 percent of people feel joyful and generous, respectively, when sharing a present with a loved one, Pew Research finds.
The receiving side of the gift-giving process is much different. The question, "What do you want for your birthday?" often triggers a mental blank, especially when multiple friends or family members inquire simultaneously. To think of one tangible item on the spot can be difficult.
More people in America were born in September than in any other month, according to a study shared in Reader's Digest from the U.S. National Center for Health Statistics, so it follows that this is the most popular birthday gift-giving time of the year, too.
Should you fall among the millions of Americans celebrating a birthday during this ninth month of 2017, Hearing Health Foundation (HHF) has a suggestion for you. Did you know that you can dedicate your birthday to a nonprofit organization of your choice on Facebook, inviting your friends to give directly to the cause?
A new feature on Facebook prompts users two weeks before their birthday to select a nonprofit organization's page. The individual whose birthday is approaching can set a goal amount and enter a custom message. Friends will see the public post, which expires at midnight on the user's actual birthday.
HHF relies on the generosity of individuals to propel forward its critical hearing and balance research, awareness efforts, and advocacy work. If you are a September-born HHF supporter who considers our work personally valuable, please consider creating a fundraiser of any size. Every dollar makes a difference.
Help your friends contribute to HHF through the following steps:
1. Once logged into Facebook, go to the Fundraisers page.
2. Click Raise Money.
3. Click Get Started.
4. Choose Nonprofit.
5. Type or Choose Hearing Health Foundation.
6. Personalize your message and click Create.
Happy birthday! And thank you for considering donating your special day to HHF.
Labor Day and Working with Hearing Loss
By Katelyn Serpe
Since 1894, when Labor Day was named a federal holiday, the United States has celebrated the labor movement and the many contributions that workers have made in improving the lives of Americans across the country. Establishing a minimum wage and child labor laws are just a few of the accomplishments that have been made since the start of the labor movement.
Among these accomplishments are various workplace safety regulations, including recommendations for occupational noise exposure. Yet despite these regulations, the National Institute for Occupational Safety and Health (NIOSH) estimates that 30 million U.S. workers are exposed to noise levels high enough to cause hearing loss. With approximately 150 million Americans in the workforce, nearly 1 in 5 U.S. workers are exposed to noise levels high enough to cause hearing loss.
According to the Centers for Disease Control and Prevention, an estimated 24% of hearing loss in the United States has been a result of workplace exposure, yet people with a hearing loss are often hesitant to disclose their hearing loss in the workplace due to the associated stigmas. Some people with a hearing loss may not seek treatment immediately if they view their hearing loss as an unimportant issue. Yet others may not even be aware they have a hearing loss due to the low number of people who regularly receive hearing screenings.
People with hearing loss are less likely to be in the labor force with nearly half (47%) of people with hearing loss not in the labor force compared with 23% of typical hearing individuals. And the unemployment rate for those with a hearing loss is nearly double that of the typical hearing population.
Having a hearing loss comes with its own challenges but an undisclosed hearing loss in the workplace can increase work-related stress and cause problems in the workplace. Most jobs require some form of verbal communication and an undisclosed hearing loss can decrease efficient verbal communication. This can result in incorrect work due to a lack of comprehension of verbal instructions or a general decrease in productivity. A person with undisclosed hearing loss may, therefore, lose out on promotions and possibly face a loss in annual wages. People with untreated hearing loss can lose as much as $30,000 in annual income and earn approximately 25% less than their typical-hearing peers.
Fortunately, the use of hearing aids has been shown to nearly eliminate the risk of income loss for those with milder hearing loss, and reduce the risk of income loss significantly for those with moderate to severe hearing loss. But while it may seem that the use of hearing aids is a solution for the risk of income loss for people with hearing loss, only 1 in 4 people who could benefit from hearing aids currently owns them.
Employers can help reduce the economic costs of hearing loss by creating environments where people with a hearing loss are unafraid to acknowledge their hearing loss. Making hearing tests part of a company wellness program, making sure hearing aids are covered by company insurance, and providing reasonable accommodations for those with hearing loss are some steps that employers can take to decrease the stigma of having a hearing loss in the workplace.
Through education and raising awareness about the prevalence of hearing loss among Americans, Hearing Health Foundation hopes to erase the stigmas surrounding hearing loss.
We hope you will join Hearing Health Foundation this Labor Day in celebrating the achievements made so far to improve the lives of working Americans while also encouraging further progress to improve American lives both in and out of the workplace.