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"Thjs is a medical miracle for many patients," says Dr. Joseph Chen. "They go from silence to hearing speech immediately at switch-on"

Sunnybrook is not only Ontario's go-to centre for cochlear implants, its physicians are working on new ways to restore sound to people facing deafness

A cochlear implant didn't just give Faryn Wegler the ability to hear; it gave her a voice.

And that voice will help her find a career she loves. "Before my implant, I was very shy," says this 24-year-old Thornhill, Ont., student. "I purposely avoided conversations because I couldn't hear what people were saying. I thought I'd have trouble finding work."

"Now," says Faryn, "I know what's going on around me and I connect better with people. I'm confident I'll get a job in my field."

Faryn, who is a postgraduate student in Fashion Management and Promotions at Humber College, has a progressive and mysterious type of hearing loss that was discovered when she was 13 years old. Because she was such a good student, she made it through elementary and high school with "my own accommodations," such as sitting in the front row and learning to read lips. But, still, she often felt embarrassed by her secret. "No one was ever directly mean to me, but I felt left out. I would ask people to repeat themselves and they'd say, 'Oh, never mind.'"

"This is a medical miracle for many patients," says
Dr. Joseph Chen. "they go from silence to hearing
speech immediately at switch on."


In 2013, the hearing in her right ear had deteriorated to the point that she qualified for a cochlear implant. Faryn came to the right place – Sunnybrook, which performs about 120 cochlear implants a year. It offers the largest adult cochlear implant program in Canada and is one of the top three in North America.

"Cochlear implantation is a fast-expanding biomedical marvel that combines a sophisticated microprocessor and an electrode system in the inner ear, to restore hearing," says Dr. Joseph Chen, director of the Cochlear Implant Program and chief of the department of otolaryngology (head and neck surgery) at Sunnybrook. Until recently, cochlear implants were only considered for the most profoundly deaf patients, who had no hearing at all. But because of improvements in technology, they are an option for a much larger number of people, including those with moderately severe hearing loss. "Now," says Dr. Chen, "people who struggle with hearing aids are candidates for cochlear implants."

Sunnybrook, which is fully affiliated with the University of Toronto, is the co-ordinating site for all four of Ontario's cochlear implant centres, including the Hospital for Sick Children. Sunnybrook has worldwide stature in the field and is hosting an international cochlear implant symposium in 2016.

"As a group, we've moved the program forward by increasing its scope and size. Greater funding in recent years has reduced our waiting list to six months, from up to three years," says Dr. Chen.

Faryn's surgery was conducted in May 2013 by Dr. Chen. Like most people who live with hearing loss, Faryn's auditory nerve was still functioning, but the tiny hair cells inside the cochlea in her inner ear were damaged.


HOW IT WORKS

Here's how cochlear implants work: The surgeon implants a small receiver into the bone behind the ear and feeds an array of electrodes into the snail-shaped cochlea in the inner ear. Externally, a small speech processor is attached behind the ear. The patient is given a month to recover from the surgery before the device is turned on. Once activated, sounds enter a microphone and travel to the processor. There, they are converted into digital information that is sent to the electrodes, which stimulate the auditory nerve to send information to the brain.

Some patients have to do hearing exercises for weeks or even months to relearn sound, while others hear normal sound as soon as the device is activated.

Paul Wheeler, the recipient of Sunnybrook's 1,000th cochlear implant in 2012, was choked up with emotion when he heard normal sound on the day of his activation. His implant restored 90 per cent of hearing to his left ear, which had been virtually deaf. "This is a medical miracle for many patients," says Dr. Chen. "They go from silence to hearing speech immediately at switch-on. They start to cry."

For Faryn, the miracle arrived gradually, propelled by hard work. At activation, she heard only beeps; after a week, the beeps started sounding like words. Three months of listening to audio books and working with a verbal therapist gave her back the hearing she had lost as a child. "Now, I go to movies with my friends, and I can hear about 80 per cent of what's going on," she says.

Before her implant, Faryn was depressed and unsure of herself. Now, she's thrilled by her prospects and is already doing an internship as a fashion editor. She is so pleased with her implant that she's on the waiting list for one in her left ear.

"It is extremely satisfying to be able to help these people," says Dr. Chen. "It really gives patients a new lease on life."


CAN HEARING BE REGENNERATED?
MILESTONES IN HEARING

More than 300,000 people have been implanted around the world, 80 per cent of them in the last decade. Because of the modern technology's massive global impact, its developers were awarded the Lasker-DeBakey Clinical Medical Research Award (known as the baby Nobel) in 2013.

Sunnybrook has been active in cochlear implants since 1984, when Dr. Julian Nedzelski pioneered the program. Now part of Sunnybrook's leading Brain Sciences Program, the otolaryngology team has enjoyed many "firsts" in Canada: The first-ever cochlear implant in a patient with single-sided deafness (see sidebar) and the first-ever Bonebridge surgery in a patient with single-sided deafness. The Bonebridge procedure involves surgically implanting a device behind the ear, where it produces vibrations through the bone and reroutes sound from the good ear to the deaf ear.

Sunnybrook also performed the first MED-EL auditory brainstem implant (ABI) in North America. This highly intricate, groundbreaking surgery is an option in people whose inner ear is not accessible for a cochlear implant because of a tumour or severe inner ear abnormality. The idea is to bypass the inner ear and stimulate the brain directly. Dr. Chen and colleague Dr. Farhad Pirouzmand, who specializes in skull base surgery, conducted this historic implant in July 2012, in a procedure that took nine hours. ABI will benefit about 50 Canadian patients a year.

For patients like Faryn and Paul, Sunnybrook's cochlear implant program is life-altering, as it helps them connect with a world of sound they were missing. "It's not only about hearing improvement," says Dr. Chen. "Cochlear implants can be transformative in getting people back to work, increasing their productivity and quality of life."


CAN HEARING BE REGENERATED?

Sunnybrook's state-of-the-art Sonja N. Koerner Hearing Regeneration Laboratory is exploring new scientific frontiers in reversing hearing loss.

One of the main reasons for deafness is thought to be damage to tiny hair cells in the inner ear. These hair cells detect sound and convert it into neural signals that travel to the brain. "Once they are damaged or lost, these vital cells are capable of regenerating in birds and fish, but they are never recovered in humans. Why?" asks internationally renowned hearing researcher Dr. Alain Dabdoub, who has moved his laboratory from San Diego to Sunnybrook to help answer this question.

"The goal of the laboratory is to understand how these cells develop in the first place during embryonic development and then learn to regenerate them, thus reversing hearing impairment," he says.

Dr. Dabdoub and clinician-scientist Dr. Vincent Lin are focusing on several cutting-edge areas of research: Activating molecular pathways that play a role in early development of these hair cells, using gene therapy or medications to turn on hair cell development and reprogramming cells that have been damaged.

Recent work at Dr. Lin's lab has found that, with aging, there is a definite decline in the density of blood vessels in the cochlea. He is studying what happens at the cellular level during that decline, in the hopes to reverse it one day. This will be good news for the three million people across Canada who live with some kind of hearing disability. – Celia Milne


SINGLE-SIDED DEAFNESS

Joseph Sathananth is making history. He is the first adult patient in Canada to receive a cochlear implant for deafness in only one ear.

On a cold winter day, he is at Sunnybrook to have his implant, which was surgically inserted a month before, turned on for the first time. This 56-year-old research analyst at an investment company will find out whether the device has reintroduced sound to his left ear. He has not heard a thing out of that ear since February of 2012, when medication he was taking for dental pain damaged his inner ear, resulting in complete hearing loss on that side.

"At home and at work, you feel embarrassed because you can't hear what people are saying and doing," says Joseph, who lives in Brampton, Ont. "My sister-in-law came to our house to visit. She rang the bell for half an hour, and I didn't hear her. I felt terrible." Balance problems and tinnitus (ringing in the ear) are also common deficits in single-sided deafness.

Up until now, cochlear implants (CI) have been reserved for cases of profound deafness in both ears. Sunnybrook is one of the leading sites in North America for CI, having done about 1,300 operations. Dramatic improvements in technology and surgical techniques have opened up these devices to people with partial deafness.

"It is very exciting that we can now offer this technology to a wider group of patients," says Dr. Vincent Lin, the otolaryngologist (head and neck surgeon) who conducted Joseph's surgery. "In the past, the only option for these patients was hearing aids, but now early research in Europe has shown that sound quality can be very, very good with a combination of natural hearing on one side and a cochlear implant on the other."

After surgery, patients are given about a month to recover before they return to the hospital to have the device turned on for the first time, a process called "activation." Sunnybrook audiologist Tara Millman, who is doing Joseph's activation in her office at Sunnybrook, tempers his expectation by explaining that patients shouldn't expect much on the first day. It usually takes months of rehabilitation and therapy to train the brain to hear in this new way.

Activation involves first programming each of the 16 electrodes that have been surgically implanted in Joseph's inner ear. There's a different pitch for each electrode. Joseph's job is to tell Millman when the beeping reaches a comfortable level. "Way too low," he says as she adjusts the volume on the first electrode from her computer, then, "Too low," then, "Ya, that's enough."

Finally all of the electrodes are set. The hope is that when they are all working together, Joseph's brain will interpret the noises as familiar sounds.

Before activating the device, Millman puts an earplug in Joseph's right ear, the good ear. She says something, and he cannot hear her.

She turns on the cochlear implant.

"What did you have for breakfast?" she asks Joseph.

"A Subway sandwich," he says.

"When is your birthday?" she asks.

"Next Friday!" he says triumphantly.

Joseph is not only the first adult patient in Canada to receive a cochlear implant for single-sided deafness, but also lucky enough to experience hearing on the very first day. There's an echo in the background, but this is expected and will diminish over the course of his follow-up appointments.

"I'm feeling great," he says before heading home in the snow. "I am born again, after two years!" – Celia Milne


This content was produced by The Globe and Mail's advertising department, in consultation with Sunnybrook. The Globe's editorial department was not involved in its creation.

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