June 2017 CBS Minnesota WCCO

A White Bear Lake woman hears her daughter’s voice for the first time, and it’s all thanks to the new cochlear implants she was fitted with by the University of Minnesota. Nathalie Carrigan was finally able to hear her 3-year old speak. Her cochlear implants are working better than she could have ever imagined. “Things sound exactly how I remember them, songs sound exactly like I remember them,” she said.

Nathalie had progressive hearing loss diagnosed in childhood and got worse over the years. Her profound inner ear hearing loss made her a great candidate for cochlear implants. “It’s just so different from hearing with a hearing aid,” she said. “That’s just kind of more muffled noise and the cochlear implant is so clear and so crisp.” Nathalie’s first sounds were those of her 3-year-old, Maeve. “It’s amazing, like noises,” she said. “Like, you guys all sound alien.” Nathalie says she can now enjoy life with her family, without barriers. “I can listen to the radio, I can listen to my kids talking in the car,” she said. “I think it’s just the daily stuff that I’m most excited about.”

Audiologist Dianna Hart says Nathalie is experiencing a new way of  hearing. This visit, six weeks after surgery, is to make sure Nathalie’s implants are working properly. “We are looking at each individual electrode,” Hart said. “Each electrode is a different region of pitches so we are playing different pitches to Nathalie and make sure each pitch is set comfortably.” Hart says Natalie is doing better than expected because she remembers how things use to sound, making it easier to adjust to the cochlear implants.

May 2017 Newsweek

More than 5 percent of the world’s population suffers from profound hearing loss, and about 60 percent of the deafness in infants is caused by gene mutations. That’s why scientists at the Boston Children’s Hospital and Harvard Medical School have spent several years refining a technique to repair one of the common genetic disorders that cause deafness, offering hope to millions. The genetic disorder they repaired is Usher Syndrome. The disorder stems from an abnormality in a gene called ush1c, which encodes the proteins in the inner-ear hair cells. These hairs convert sound vibration into electrical signals for the brain. In people with Usher Syndrome, that conversion does not happen, leaving them with devastating deafness, loss of balance and sometimes even blindness.

The researchers wanted to see if tackling the syndrome at its root—the abnormal gene—could reverse the damage. For this, they turned to a pioneering approach called adeno-associated viral vector, which uses a harmless virus to deliver new genes. They replaced DNA from a virus with a healthy ushc1 gene from a mouse. That altered virus was multiplied in a petri dish, then injected into the inner ears of deaf mice suffering from Usher Syndrome. After six weeks, the mice had almost perfect hearing and full recovery of sensory hair growth, brain auditory function, balance and sound sensitivity down to a whisper. (High-pitch frequency sensitivity was only partially restored.)

Dr. Margaret Kenna, a specialist in genetic hearing loss at Boston Children’s Hospital who wasn’t involved in this study, says that while there are devices that can bolster hearing, nothing beats nature. “Cochlear implants are great, but your own hearing is better in terms of range of frequencies, nuance for hearing voices, music and background noise, and figuring out which direction a sound is coming from.” Exactly the kind of results achieved with the deaf mice.

After this study, written up in two papers published in Nature Biotechnology, the Boston team started preparing for human trials. In March, they exposed human outer-ear hair cells in the lab to the same ush1c-carrying virus. After 10 days, the virus penetrated about 83 percent of the targeted cells. Although cell function was not tested, the team’s ability to reach so many cells has brought real hope that this technique might be able to cure not just a human ush1c gene defect but other hearing disorders caused by genetic mutations.

Brad Welling, chief of otolaryngology at Massachusetts Eye and Ear, wasn’t part of that team but is cheered by their work. “The day is growing closer to human trials which will correct defects in the outer ear cells and potentially other areas of the inner ear…and we look forward with great anticipation to human application.”

May 2017 Business Insider Australia

Cochlear plans to use artificial intelligence to more accurately calibrate its devices. It announced an exclusive licensing and development agreement with Otoconsult for its artificial intelligence fitting assistant FOX (Fitting to Outcomes eXpert). The company says the technology is expected to enable a faster and more consistent fitting of cochlear implants. “FOX’s artificial intelligence assistant will provide clinicians, no matter where they are in the world, a platform to speed up the cochlear implant fitting process while also helping them achieve the best possible patient outcome,” says Cochlear CEO Chris Smith.

William H. Shapiro, clinical associate professor at the NYU School of Medicine in New York, says the technology will change how cochlear implants are programmed. “The audiologist can perform a set of simple, yet critical tasks, where the patient is an active participant, to provide the evidence for target-based fitting much like hearing aid verifications today,” he says.

May 2017 Greater Dandenong Leader

Being blind and nearly deaf are no barriers to Alex Sar’s artistic pursuits. The Springvale man lost his sight aged 13 and relies on a cochlear implant for his limited hearing. He’s one of about 330,000 Australians to have lost both senses. But the 26-year-old hasn’t let that slow him down and said he wants to be known for his paintings, rather than his impairments. “Stevie Wonder is a good example,” he said. “People can see past the disability and think of him as an artist.” Art is just one of many projects on Mr Sar’s busy schedule, which includes public speaking, learning Auslan, and organising camps for fellow blind-deaf clients with Able Australia. 

Alex sarThe painting was suggested to him as a form of therapy a few years ago.

“It isn’t really something I focus on day to day — it’s more something I enjoy doing”, he said. His portrait of newsreader Peter Hitchener was entered into the 2013 Archibald Prize.

Describing how that artwork came together, said it involved a lot of prodding of the Nine news presenter’s face. “Peter was very patient,” he said. “We had him sitting next to the canvas for two sessions (four hours). I had one hand on the paint brush and the other on the top of Peter’s head, and was just making my way down. “The nose was probably the hardest bit,” he said. Mr Sar has since turned his focus to painting dogs, which he says presents its own challenges. “They’re a lot less patient and they’ll just jump up and run off on you,” he said.

Mr Sar has been a client of Able Australia for seven years and said the organisation has helped him gain confidence to pursue his passions. Mr Sar said losing his sight on top of his hearing was a lot to deal with, particularly at a young age. “I had a glycauma since I was born but previously I had a fair amount of vision. I totally lost my sight at the age of 13 which is a pretty crappy age to deal with that,” he said. “I’d just started year 7, I was two weeks in. I was walking down the corridor and couldn’t see properly at all. I started bumping into doors. I woke up one day and couldn’t open my eyes properly.”

Mr Sar said his paintings, public talks and use of social media have helped him express himself and advocate on behalf of the deaf-blind community. “It’s about putting myself out there, just sort of getting involved in different activities. My hope is that people can see past the disability and appreciate people for what they do.”

Able Australia is this year marking 50 years of operation. Details are available on their website.