USH1B Stories


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Lia | california

Lia is 18 months old and lives in the Bay Area. We learned Lia was deaf 2 weeks after she was born and we were shocked and crushed as new parents. As we slowly began to come to terms with her diagnosis and just started to feel comfortable again, our geneticist revealed the cause of her hearing loss - Usher Syndrome Type 1B. We knew that meant Lia would start to lose her vision at a very young age and she would have significant balance issues for the rest of her life. Learning our baby daughter had a rare genetic disorder with such significant sensorial loss was surreal and heart breaking, were still learning how to cope with it as we raise this incredible little soul. We move on one day at a time with her as the center of our universe and the source of our happiness. We find hope in medical advances that will lead to a treatment. Don’t let that cute smile fool you, this little carb lover is stubborn as an ox and smart as a whip. When she’s not hugging up on her favorite stuffed animal - “hedge hog” - she’s barking orders at mommy and daddy to read more books to her.

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Jackson | holland

Jackson was 7 months old when he was diagnosed with Usher syndrome type 1B. At a point where we just started to accept his deafness, this was the one thing we did not see coming. Our son would go blind? We were devastated. But we had such a happy baby boy, we needed to be strong for him. If he was willing to deal with this head on, then so should we. And that’s how we choose to live our lives. Jackson has shown us that Usher syndrome is not game-over, it’s a game-changer. Despite his vestibular problems, he is walking and running. He loves the outdoors, animals and is doing really well in school. Although Jackson is progressing well, we worry about his vision every minute of every day: when and how will his vision start to be affected, is it affected now, how bad? It’s a like a looming dark cloud that never goes away. We do our best to raise awareness and raise money in order to find a cure. We are hopeful, but it’s a race against time. Please join us in our fight to save sight now. For Jackson, for Lia and for all others around the world living with Usher1B.

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Rebecca | Massachusetts

Rebecca is 3-1/2 years old and has Usher Syndrome type 1B, which is the leading cause of congenital deaf/blindness. Rebecca's sub-type, 1B, is the most aggressive type of Usher Syndrome, with symptoms occurring more rapidly than that of other types. Rebecca was born profoundly deaf in both ears, and has profound bilateral vestibular loss. Rebecca hears with the help of cochlear implants, she uses both spoken language and ASL. Rebecca has struggled with her balance since birth. Things that we take for granted such as walking on a sandy beach, are a challenge for Rebecca. Rebecca already has some night vision loss, and light sensitivity. Rebecca has a hard time seeing in poorly lit areas, and she also struggles to see on bright sunny days. If a cure is not found soon, Rebecca will continue to lose more and more of her vision. We are desperate to find a cure.

 
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Megan | Virginia

My name is Megan. I am 22 years old and have Usher Syndrome 1B. I am profoundly deaf, have severe balance issues and Retinitis Pigmentosa, which causes vision loss. I learned American Sign Language as my first language and received a cochlear implant at 2 ½ years old. I had speech therapy and completed it well before my class, in third grade. I read at a college level when I was in second grade and my love for reading and literature has continued into adulthood. I primarily speak and use English as my daily mode of communication. I graduated from the University of Mary Washington in 2019 with a Bachelor of Science in Psychology and a double major in Sociology. I am taking a year off to work before pursuing a Master’s. I am currently exploring career options in the therapeutic field. I am an advocate for disability rights for those with Usher Syndrome. The need for research is clear. The only way to achieve empowerment of those with disabilities is ensure equal access and opportunity. Research for treatments and a cure for Usher’s is one way to support us. Taking vitamins and supplements, eating pounds and pounds of fish and wearing sunglasses can only do so much. We need collective action, of which you can be a part

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Kadie | Pennsylvania

My name is Kadie Trauger and I was raised and reside in Pennsylvania.  I was born deaf and received my right side cochlear implant at age 5.  Mainstreamed with a sign language interpreter, had the both worlds of oral and sign language. At age 16, on the day of my drivers license test, I found out I had Usher Syndrome 1B.  They still gave me my license and I drove for 3 years until I decided to give it up for safety reasons. I then received my left side cochlear implant at age 18 to give me the surrounding sounds. Graduated from Rochester Institute of Technology (RIT) in 2008 with Business degree. I’ve gone through many challenges like many others with Ushers have.  It only made me stronger and want to overcome it with a positive attitude. I don’t often take the “I can’t” kind of attitude as I will find a solution. I married my childhood friend who is also deaf and we have two children. I’m blessed to be a stay at home Mom. I am very thankful to have such a great support system from my family and friends as well as the Usher Syndrome community. 

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Linnea | florida

Linnea is 19, graduated high school in 2018 Magna Cum Laude and is now starting her sophomore year of college at the University of Central Florida. She is striving to be in the computer animation program and someday work for Disney. Linnea also rides on the school equestrian team. Linnea was just under 2 when she received her cochlear implants. It wasn’t until age 13 that we learned about her Usher Syndrome diagnosis. One day Linnea stated she couldn’t see the floor or her feet. Soon after we had genetic testing done and confirmed that Linnea had Usher 1B. Everything that happened while Linnea was growing up now made sense: her running into things, not seeing well at night, we all thought she was clumsy or had bad balance. As a parent we worry about her every day, what will her future look like, will she be independent and have a happy fulfilling life? We have hope that one day there will be a cure or at least a way to stop the progression of vision loss.

 
 
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ethan & gavin | toronto

The USHbrothers, Ethan (17) and Gavin (15) are originally from Toronto Canada, and currently live in Scotch Plains, New Jersey. These two proud Canadian boys were born profoundly deaf and diagnosed with Usher syndrome type 1B ten years ago when they were seven and five years old. They met this new challenge head-on by being early advocates for themselves, spreading awareness, raising money for Usher Syndrome causes and eventually becoming Student Ambassadors for Ava’s Voice, a non-profit organization that empowers youth with Usher syndrome. These two “Deaf Blind guys“ are competitive swimmers for the Lightning Wheels Paralympic sports club, their local YMCA and high school teams.

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Larissa | New Jersey

My name is Larissa DePamphilis, I am 24 years old. I was born profoundly deaf and received my first Cochlear Implant at 19 months old and my second at the age of 9. I was diagnosed with Type 1B at a very young age. I went through mainstream education system in my hometown - New Hope, PA, as well as Orientation and Mobility training when I was young. However, it wasn’t until I was in college when I realized just how much my vision had deteriorated and that I needed help. I received my first Seeing Eye Dog, Acadia, (Cadie) in the Summer of 2016. With her, I graduated from The College of New Jersey with a Bachelor’s Degree in Mechanical Engineering in 2017. I moved on to New York University Tandon School of Engineering and received a Master’s Degree in Mechanical Engineering this past May (2019), while working full time in New York City. Our next phase is Rutgers University where I will be pursuing a Ph.D in Biomedical Engineering beginning in the fall. I am very grateful for my current support system, which includes my family, friends and my boyfriend. I hope that one day I will be able to contribute to the Usher Syndrome Community by creating assistive technology and/or working toward a cure. 


 
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Aiden | Pennsylvania

Aiden is a wild 3 year old from Pennsylvania. Born at 27 weeks we found out a few months later he was profoundly deaf. He received his bilateral cochlear implants at 16 months. We learned he had Usher Syndrome Type 1B at the age of two. He already has peripheral vision loss, balance issues, has orthopedic inserts and just received his white cane. There are tough days - I worry about his future and his vision - but the Usher Syndrome community gets us through them with their help and support. Aiden currently has 5 different therapists that work with him. He is just starting to say 3 or more words and I was warned you want them to talk, but once they do they don’t stop and that is definitely true with Aiden. His bear and cane go everywhere with him and he is your typical paw patrol loving, fruit snack eating, temper tantrum throwing 3 year old, but the best thing in the world is the first time he told me he loved me and now he says it every night before bed. I hope and pray that one day there will be a cure or treatment that will stop the progression of vision loss, but for now I couldn’t be more proud of Aiden and grateful for the community we are a part of.

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Kaileb | East of England uk

Kaileb is now 9, he was born profoundly deaf. At 16 months old he went through bilateral cochlear implant surgery. During this time Kaileb had been diagnosed with a different genetic cause for his hearing loss. As a Mum I always knew we had more to find out. Kaileb didn't walk until he was 22 months old. 3 1/2 years after our genetic tests we learned Kaileb had Usher Syndrome Type 1B. We knew he would need an ERG test to confirm, but in my heart I already knew. October 2016 our world came crashing down, our profoundly deaf son was also going blind and he had been since birth. Kaileb was already night blind and had extensive loss of vision, he was 6 and registered as partially sighted. He started cane training within weeks and learning touch typing. We do all we can to protect his eyes. As a family we have been building visual memories... He is amazing and the most determined child you will ever meet, he is caring and loving, funny and charming. He now rides a bike and at 7 years old and he climbed Scafell Pike and reached the summit with no balance and little peripheral vision. To be born profoundly deaf and then have your eyesight taken from you day by day is devastating, there are no words that can describe how we feel, but we know he will be stronger because of the hurdles he has to overcome as he refuses to let Usher 1B define who he is.