Our Research on Usher Syndrome
Thanks to the generosity of our donors, Hearing Health Foundation (HHF) funds groundbreaking research to advance our scientific understanding of the leading genetic cause of deafblindness, Usher Syndrome.
Grants focused on Usher syndrome are awarded annually to promising scientific investigators through the Emerging Research Grants (ERG) program.
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I’ve always been fascinated with how things work and have considered electrical and/or mechanical engineering. It would be very powerful for someone who has worn hearing aids for all his life to design hearing aid technology to fit various stages of life and lifestyles.
At the age of 36, I have less than three degrees of peripheral vision, very severe night blindness to the point where I almost can’t function without assistance, and some hearing loss that makes it necessary to wear hearing aids.
CI kids are not disabled. The handicaps are not the cochlear implants but rather the low awareness of the parents and caregivers around them. I believe immensely in the possibilities these children have and find that adversity is an opportunity. When CI kids push themselves to achieve greater things, the sky's the limit.
Meet Elizabeth “Elizzy” Galvan, a 40-year-old professional bodybuilder from Fargo, North Dakota, who doesn’t fit the stereotypical presentation of someone in her chosen sport. She is deaf, lives with Usher syndrome, and has one arm.
My longtime fascination with all things aerospace inspired my desire to work with computers for a living. But, at times, my hearing and vision loss caused some turbulence.
I’m thrilled we are moving toward better treatments and cures for hearing and balance conditions — together. I was diagnosed with a hearing loss at age 4 and with Usher syndrome (combined hearing loss and vision loss with balance difficulties) as an adult.
Our grantees’ research investigations seek to solve specific auditory and vestibular problems such as declines in complex sound processing in age-related hearing loss (presbycusis), ototoxicity caused by the life-saving chemotherapy drug cisplatin, and noise-induced hearing loss.
This retinal eye specialist—who was the first person to utter, “You have Usher syndrome” to me—had the worst bedside manner. Immediately after I left his office I cried—a lot—but then regained my composure and made a few calls to see a second retinal eye specialist doctor for a second opinion.
Recent research published in JARO by Emerging Research Grants (ERG) recipient Michelle Hastings, Ph.D., and colleagues shows that early administration of a genetic targeting treatment is critically important for repairing outer hair cells and thus rescuing hearing in those with genetic disorders like Usher syndrome.
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.
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Types of Usher Syndrome
Usher Syndrome Treatments
Our Research on Usher Syndrome
Bruna’s diagnosis at age 9 months is Usher syndrome type 1B. It is a rare disease, a recessive inherited disease that we, her parents, had given to her. It is a disease that we had bypassed, but not our daughter.