Usher Syndrome

Shock and Then Purpose

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.

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A Future in Engineering

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.

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From Denial to Pride

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.

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We Believe ‘CI Kids’ Can Do Anything

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.

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Bodybuilding Against All Odds

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.

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Flying My Way

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.

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With Your Support for Hearing Research, We Can Do Great Work Together

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.

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Introducing the 2018 Emerging Research Grantees

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.

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You Are a Masterpiece

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.

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Prenatal Intervention May Be Necessary for Usher Syndrome Treatment

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.

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