Sept 2017 Spokane Journal of Business

First launched in 2004, the Spokane Hearing Oral Program of Excellence (HOPE) has relocated to larger quarters. The nonprofit, which offers early intervention auditory and language therapy for children with deafness or hearing loss, previously occupied space in the University Hearing and Speech Clinic, jointly operated by Washington State University and Eastern Washington University.

HOPE educators say improved technology for cochlear implants and similar devices is coupled with the pre-kindergarten school’s therapy expertise to help children during early years, prior to kindergarten, when rapid brain development occurs. HOPE doesn’t rely on sign language, lipreading, or other visual cues, but emphasises continued auditory and oral language development.  “We’re the only listening and spoken-language program in the region for children who are deaf or have hearing loss,” says Danette Driscoll, HOPE executive director. “Our mission is to teach children with hearing loss how to listen and speak so they can be successful in our hearing world.” With an annual operating budget of $400,000, HOPE doesn’t receive any medical insurance reimbursement, so funding sources include money from local school districts, grants, donations, and its fundraiser.  

HOPE ProgramHOPE graduates Kassie Broers, left, and Mia Newell, with mothers Kim Broers and Luisita Francis, display their cochlear implants.

HOPE serves about 50 children each school year through two early-learning programs and its preschool. For the 16 students involved in HOPE’s preschool, Spokane-area school districts contract with HOPE to cover the cost of a student’s tuition, set at $9,000 per school year. 

“The school districts realise the benefit because the HOPE graduates come back to their school district ready to enter a general-education kindergarten class with little if any additional support needed,” Driscoll says.  Its Birth to 3 In-Home Therapy Program offers services from initial diagnosis, says Driscoll, who adds that children can be diagnosed with hearing loss at birth with a state-required newborn hearing test. HOPE also has a weekly toddler-and-parent program at its center for children 18 months to 3 years old to develop social, emotional, and collaborative learning. Meanwhile, the parents network about their shared experiences. Driscoll says, “Spokane is one of the smallest cities in the country to have a program like HOPE, and it’s because the Spokane community supports the financial needs through grants, and individual and corporate donations.”

Children supported by HOPE services can hear with hearing aids, cochlear implants, or bone-anchored implants. Those devices assist them in experiencing speech sounds to begin learning language, in some cases before the age of 2.  Families often are referred to HOPE by the Spokane Regional Health District’s Infant Toddler Network. The state Department of Early Learning and Office of Superintendent of Public Instruction provide funding that covers HOPE teachers of the deaf in-home services, so families don’t have to pay, Driscoll says.  Other referrals to HOPE are from Spokane Guilds School, which provides various therapies for young children. 

HOPE employs six people, including two teachers, Driscoll, a development director, and two instructional assistants.

Nine-year-old Mia Newell, a HOPE graduate, enjoys physics and science in an international school during part of the year in Europe. Mia’s hearing loss is a genetic condition, says her mother, Dr. Luisita Francis. Mia passed a newborn hearing screening and started talking, but at around 9 months, her speech stopped. A paediatrician and assistant dean at WSU’s Elson Floyd College of Medicine, Francis says she became concerned that Mia wasn’t meeting her speech milestones. She took her infant daughter to a paediatrician, and Mia then failed the hearing test. Mia could only hear sounds at a decibel level of 110, the equivalent of a jet airliner, Francis says. Before learning about what HOPE offers, she considered leaving Spokane for medical services in Los Angeles.

“All the resources actually kept us here in Spokane,” she says. She adds that HOPE also teaches children how to advocate for hearing needs. It works with families about what to do in the home, such as repeating words applied to actions and objects.  Before Mia’s cochlear implants, Francis knew about them as a doctor but hadn’t been exposed to newer technological advances.  “Now with cochlear implants, you can really pick up key sensitive sounds,” Francis says. “The frequencies heard through the cochlear implants are broader, and the external part gets updated about every five years. That’s what receives the sound and transmits to a head piece.” She adds, “You have this window of 0 to 5 years for really significant brain development.”

Parent Kim Broers says using HOPE services for her now 9-year-old daughter Kassie from infancy greatly enhanced early language and speech development. That included in-home services from the time Kassie was a baby to preschool with a certified classroom teacher who is trained in listening and spoken-language therapy. Broers says, “They don’t use sign language or any visual cues, and we do the same thing in the home, because we are building the auditory part of the brain during a critical time of brain development.” Kassie as an infant could hear at about 75 decibels, “equivalent of a lawnmower,” Broers says. Her daughter received hearing aids and then cochlear implants, one at age 2 and the other at 3. She had hearing loss because the inner ears’ stereocilia—sometimes called cilia—weren’t functioning properly, Broers says. The cilia as organelles of hair cells respond to fluid motion for various functions, including hearing and balance. Today, Kassie excels in fourth grade at Freeman Elementary School, Broers says. Her teacher wears a lanyard with a small transmitter that’s part of an FM sound-field system so the sound of the instructor’s voice goes directly into Kassie’s cochlear implants. Kassie can hear regular conversations without the transmitter, her mother says, but it helps in case the teacher turns facing away to write on a white board or is across the room.

Sept 2017 NZ Herald

Gethin Thompson's eyes lit up the first time he heard a sound. The 15-month-old was born profoundly deaf so when he said his first word it was even more special than for most. "We were sitting at the dining room table and he crawled over and said 'up, up, up'. We all looked at each other and said 'did you hear that?'. I just burst into tears," mother Rhian Thompson said.

When Gethin was a newborn he wouldn't sit still long enough for the newborn hearing test so it wasn't until Thompson took him in for a routine audiology appointment at 11 weeks old that they found out anything was wrong. "We got the diagnosis that Gethin was profoundly deaf. It was a very big shock. That wasn't even on our radar - there's no family history of it. We were completely devastated - it came out of left field," she said. "You automatically start thinking of all the things he can't do. I think I cried for a week."

But now, thanks to cochlear implants, Gethin is able to enjoy life like any other toddler. After his diagnosis, Thompson and her husband Michael, who also have a three-year-old daughter, learned he was eligible for cochlear implants in both ears. He had surgery to have the devices implanted when he was five-and-a-half months old and had a very strong response when they were switched on a couple of weeks later in November last year. "He had that eyes wide open, 'wow, what's this' look. That day his eyes were popping out of his head in wonderment," she said. "I think it was amazement and delight.” It was a big day for Thompson too. "It's just amazing. You go through all these mixed emotions. When we found out he was deaf we had quite a period of time where we though he might never hear. Just to actually see it in his eyes that' he's heard something it was like, 'wow, this is amazing'. After having a couple of months of it being really grim...it was just the beginning of a fresh start for us." 

Rhian Thompson   Rhian ThompsonRhian Thompson with her one-year-old son Gethin who was born profoundly deaf but now has cochlear implants which allow him to hear.

Almost 10 months later Gethin now understands basic instructions and about 80-90 words. He can say eight or nine words and is always trying to say new ones. "We're ecstatic about where he's at now. He doesn't miss a trick. He seems pretty switched on," Thompson said. She said it required them to be more aware of what background noise there was, focus more on repetition and give him longer to process words and instructions but they were hopeful he would be able to lead a normal life and be whatever he wanted to be.

Sept 2017 Duluth News Tribune

The red Under Armour skull cap Jaden Gronner wears beneath his football helmet has nothing to do with fashion, and everything to do with hearing. Tucked inconspicuously behind the Cromwell-Wright junior’s left ear is a cochlear implant. Gronner is deaf in his left ear and has minimal hearing in his right — the result, his parents believe, of a nasty brush with scarlet fever when he was 13 months old. The skull cap keeps the device firmly in place and relatively free of moisture during activity. Gronner, 17, received the implant three years ago.

 Jaden GronnerWhen the implant was switched on, Gronner’s left ear suddenly was exposed to the racket of daily life. It took some getting used to. Initially, sound generated by cochlear implants is discordant. Time and training are required to process and interpret the signals. “The first time it was really weird,” Gronner said during the Cardinals’ practice Tuesday afternoon in Cromwell. “After a couple weeks, I started hearing different sounds.” Gronner’s dad, Cromwell-Wright coach Jeff Gronner, said Jaden could understand words with his left ear a month after activation, a rapid turnabout.

“It was really cool for us because we got to see somebody that didn’t have much hearing at all get a cochlear implant and suddenly be able to hear much better,” Drew Libbon, a junior quarterback and linebacker for the Cardinals, said. “That made it a lot easier for us as a team to be able to communicate with him.” Jaden’s expert lip-reading, sharpened out of necessity, hasn’t hurt, either. Time was, he could “hear” better with his eyes.

On the field, just as he does in the classroom, Jaden utilizes an FM system. Attached to a lanyard around Jeff’s neck is a microphone that carries every one of his directives wirelessly to Jaden’s implant. Life-changing as the cochlear implant has been, two specific situations curtail its usefulness: wind and excess background noise. Two of the most common characteristics of high school football games in Northeastern Minnesota? Wind and excess background noise. The FM system bypasses both, giving Jeff a direct line to Jaden.

Sept 2017 MIT Technology Review

Scientists are hopeful they can inject the gene-editing technology directly into the ear to stop hereditary deafness. We all know that CRISPR is the next big thing in gene-editing treatments. But how do you get the versatile genetic scissors into a person’s body? The usual way might be to load the gene-editing instructions into billions of viruses and use those to infect a person’s cells. But some scientists are experimenting with delivery tricks that could make treatments easier to pull off. One of them is Zhen-Yi Chen, a hearing researcher in Boston who has been treating mice by directly injecting the gene-editing components into their ears.  

CRISPRThe experiments in Chen’s lab at the Massachusetts Eye & Ear Infirmary involve mice who share a genetic defect that humans have that causes gradual hearing loss. One breed is called “Beethoven,” after the composer who eventually couldn’t hear his own music.

 “They saw a beautiful rescue of hearing,” says Xue Zhong Liu, an ear doctor who directs the centre for hereditary disease at the University of Miami, who learned of the results at a meeting this year. “It will be the first example to show how CRISPR is used for progressive hearing loss.” Actually, doctors aren’t clear on what physiological changes to ear cells these genetic errors actually cause. The inner ear is enclosed in the hardest bone in the body, says Liu, and doctors don’t often get to peek inside.

But knowing what genes are to blame may be enough. Chen says he’d been trying some more complex approaches to treat hearing when it dawned on him. “With CRISPR we can just do direct delivery, one shot delivery, and the correction is permanent,” says Chen. He and David Liu, a gene-editing specialist at Harvard University, have found ways to package the CRISPR cutting protein into fatty blobs called liposomes. These are injected into a mouse’s inner ear, home to delicate hair cells that sense vibrations and send messages to the brain. The target is a gene called TMC1. CRISPR’s job: put it out of commission with a quick snip to its DNA letters.

The animal’s hearing is tested by putting it to sleep inside a soundproof metal chamber, where it’s exposed to various noises. An electrode attached to its brain stem reads out whether the message got through. In the best cases, Chen and Liu have reported, mice treated with CRISPR retain significant hearing at two months of age. Otherwise, they’d be so deaf they couldn’t hear an 80 decibel noise—about what a food blender makes.

Each of your ears has, deep inside it, about 16,000 cells that sprout sensory hairs. That is how sound is picked up. You can damage those cells by standing in front of the speakers at a rock show. Some genetic conditions have the same result.  Chen works on so-called dominant genetic disorders, meaning just one parent needs to pass on an aberrant gene for the problem to arise. It causes the sort of hearing loss that develops gradually, over the years, starting before the age of 25 and leaving people profoundly deaf by 50. Chen’s next steps will take place in China, where scientists are enthusiastically pursuing gene-editing technology. He’s working with a team there to create pigs that are genetically modified to have the same gene mutations as people do. “The pig will absolutely go deaf,” says Chen, given how similar their ears are to ours. If the CRISPR injections can prevent that, Chen says, “we can move the endeavours into humans.” That’s another advantage of working in China. Hospitals there, he says, have already registered thousands of people with the genetic disorder, whereas in the U.S., very few affected families are being tracked. “It’s on a totally different scale,” says Chen. “That’s why we will do it there first.”