SAN DIEGO — Music saved Uri Manor’s life growing up. He was born with severe to profound hearing loss, and the guitar helped him find joy.
“Every teenager, not just me, you got all this angst, and whether it’s depression or anxiety or just stress, music is an amazing outlet,” Manor said. “And it’s something that you can do alone. So music has always been a kind of therapeutic for me.”
What You Need To Know
- According to the Salk Institute, roughly 80% of deafness cases that occur before a child learns to speak are because of genetic factors in developed countries
- One of these genetic components leads to the absence of the protein EPS8, which coincides with improper development of sensory hair cells in the inner ear
- Dr. Michelle Hu identifies as hard of hearing and is a pediatric audiologist
- She now uses her experience to help other people navigate hearing loss
He now wears powerful hearing aids, but admits he still can’t play very well by ear. He said he idolized musicians like Led Zeppelin, Metallica and Joe Satriani and became adept at reading sheet music and playing intricate songs. He remembers playing up to six hours a day.
“Being able to play an electric guitar has some advantages, like you can turn up the volume really loud as long as your parents don’t kick you out of the house,” he said. “At one point, I had my stuff set up in the basement or just in my room with the door closed and I could play really loud, which was amazing.”
Manor grew up from that angsty teenager to become a talented scientist at the Salk Institute for Biological Studies, with a firm belief that the right tools can improve our lives.
He is currently leading a study that shows promise for developing gene therapies to repair hearing loss.
According to the Salk Institute, roughly 80% of deafness cases that occur before a child learns to speak are because of genetic factors in developed countries. One of these genetic components leads to the absence of the protein EPS8, which coincides with improper development of sensory hair cells in the inner ear. These cells normally have long hair-like structures, called stereocilia, that transduce sound into electrical signals that can be perceived by the brain. In the absence of EPS8, the stereocilia are too short to function, leading to deafness.
Manor found they could regrow the delicate sensory hairs in the inner ear necessary for hearing by injecting deaf mice with a virus containing EPS8.
“What we’ve done is we’ve removed all of the harmful genes from these viruses and replaced them with the genes that these mice are missing,” Manor said. “And then we can surgically inject these viruses into the inner ear and get those cells to start expressing the missing genes.”
Manor said this research would not help restore his particular kind of hearing loss, but he is excited to give people more choices.
“I’m all about having more options. I’m that crazy person when you go to the restaurant and you see two desserts and you can’t decide which one you want. I just order both,” he laughed. “I want people to have the choice to have whatever they want. And if someone wants to be able to have better hearing as they get older or if they were born with some level of hearing loss, that they could have the option to have better hearing.”
Dr. Michelle Hu identifies as hard of hearing and is a pediatric audiologist. She wore hearing aids until she got a cochlear implant as an adult, and believes making that choice for herself gave her the courage to move to San Diego and start her own practice, Mama Hu Hears. She now uses her experience to help other people navigate hearing loss.
“I did not have confidence on the phone before I got this technology. I wouldn’t have heard somebody running behind me in a dark parking garage after work at night,” Hu said. “With [my cochlear implant], I’m able to have access to those sounds. I don’t need them, but you know, for my life, living in a hearing world, it does help me navigate that.”
Hu said it is a personal decision for anyone to choose between hearing amplification or visual language.
“I don’t like that word ‘cure’ for hearing loss or hearing impairment; being born Deaf absolutely can be welcomed and seen as a beautiful thing,” Hu said. “And each individual or each family gets to make a choice for their children or themselves.”
She is currently learning American Sign Language with her whole family, while monitoring Salk to see what will come out of Manor’s lab that might be a good fit for her and her patients.
“These studies that Uri is doing, it’s so fun to see what’s available out there,” Hu said. “Maybe he can provide an option, a choice for people later. And having options, having choices can be a wonderful thing.”
These days, Manor finds his passion for music challenged by his obsession with science.
“Get to have fun in the lab, just solving puzzles,” he said. “Thinking about these really amazing billion-year-old systems and, as a happy outcome, maybe even help some people.”
Manor says future research will include looking at how well EPS8 gene therapy might work to restore hearing during different developmental stages, and whether it might be possible to lengthen the therapeutic window of opportunity.
“I hope that in the next five years, maybe 5-10 years, we’re going to start to really understand better just how much can we do in humans,” he said. “And I’m actually pretty optimistic.”