DiversityNursing Blog

New Hearing Technology Brings Sound To A Little Girl

Posted by Erica Bettencourt

Mon, Jun 01, 2015 @ 01:10 PM



auditory implant 1 resized 600Many of us are familiar with the cochlear implant, but did you know it doesn’t work for everyone? We came upon this article featuring information about clinical trials for a new technology that gives the hearing-impaired another option for the ability to hear.

Jiya Bavishi was born deaf. For five years, she couldn't hear and she couldn't speak at all. But when I first meet her, all she wants to do is say hello. The 6-year-old is bouncing around the room at her speech therapy session in Dallas. She's wearing a bright pink top; her tiny gold earrings flash as she waves her arms.

"Hi," she says, and then uses sign language to ask who I am and talk about the ice cream her father bought for her.

Jiya is taking part in a clinical trial testing a new hearing technology. At 12 months, she was given a cochlear implant. These surgically implanted devices send signals directly to the nerves used to hear. But cochlear implants don't work for everyone, and they didn't work for Jiya.

"The physician was able to get all of the electrodes into her cochlea," says Linda Daniel, a certified auditory-verbal therapist and rehabilitative audiologist with HEAR, a rehabilitation clinic in Dallas. Daniel has been working with Jiya since she was a baby. "However, you have to have a sufficient or healthy auditory nerve to connect the cochlea and the electrodes up to the brainstem."

Jiya's connection between the cochlea and the brainstem was too thin. There was no way for sounds to make that final leg of the journey and reach her brain.

Usually, the story would end here. If cochlear implants don't work, you turn to sign language. And the Bavishis did — for years they communicated with their daughter through sign language. But then they heard about an experimental procedure called an auditory brainstem implant.

It is a very rare procedure, according to Dr. Daniel Lee, director of the pediatric ear, hearing and balance center at Harvard Medical School. "There have been less than 200 of these implanted worldwide in children," he says. In the U.S., auditory brainstem implants are approved by the Food and Drug Administration for adults and teenagers who have lost their hearing due to nerve damage, but they have not been approved for use in younger children.

Surgeons in Europe have pioneered the use of the auditory brainstem implant in children who are born deaf and can't receive a cochlear implant, Lee says. "And those data look pretty encouraging."

So in 2013, the FDA approved the first clinical trial in the U.S. for young children. The Bavishis decided to apply for Jiya. It wasn't an easy decision. It would involve surgery to place a tiny microchip into Jiya's brainstem.

"The family was at a crossroads," Daniel says. Did they want to take a chance on a risky, experimental procedure to give their daughter a chance to hear? They decided to try the procedure and traveled from their home in Frisco, Texas, to Chapel Hill, N.C., for the eight-hour surgery. The University of North Carolina is one of four institutions investigating the implant.

Jiya's mom, Jigna Bavishi, pulls back her daughter's purple headband to reveal two of the three parts of the device.

There's the piece that sits on her ear, which works like a microphone to pick up sounds. That microphone is attached to a small black magnet that rests on her head. What you can't see is what the magnet is connected to. And this is what makes it different from a cochlear implant. Below the skin, there's a receiver, and down in the brain stem is the microchip. The idea is that the sounds picked up from the microphone on her ear end up in the implant in the brainstem.

"It's a rectangular shaped element," says rehabilitative audiologist Linda Daniel. "It has two rows of electrodes and each electrode is responsible for a band of frequencies." The electrodes transmit signals directly into the brain.

Daniel says we don't know exactly what Jiya hears.

"I think we could assume that it doesn't sound crisp, distinct, clearly interpretable," she says. "It would take longer to learn to interpret the sound."

Doctors told the Bavishis not to expect any changes for a year or two. But Jiya didn't take that long to start recognizing and mimicking sounds. On the day I visit, Jiya is playing with a yellow toy car. "Beep, beep," she says.

"They actually had to tell us, even though she's doing so good right now, we have to still be careful where we set our expectations," says Jigna.

Doctors will monitor Jiya, and four other children taking part in the study, for the next few years. They'll be studying how their brains develop and incorporate sounds and speech. There are two other clinical trials investigating auditory brainstem implants in children: one at Children's Hospital in Los Angeles, and the other at the New York University School of Medicine.


Topics: hearing, hearing loss, clinical trials, implant, cochlear implants, auditory brainstem implant, hearing aids

Healing the 'butterfly children'

Posted by Erica Bettencourt

Wed, Aug 13, 2014 @ 11:47 AM

By JoNel Aleccia

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By the time her third daughter was born last year, Gabriella McCann thought she was an old pro at handling new babies.

But nothing in her experience with Greta, now 6, or Stella, 3, could have prepared her for Elisa, who arrived missing a puzzling patch of skin on the back of one hand. Within 24 hours, that patch was followed by blisters that erupted on Elisa’s body — and stumped doctors who didn’t know what to make of the shocking condition.

“The baby was born and the whole world crashed,” recalled McCann, 40, a Minneapolis mom who was staying with family and friends in her native Palermo, Italy, when Elisa was born.

Within days, the problem was clear. Doctors in Rome diagnosed Elisa with a form of epidermolysis bullosa, or EB, a genetic disorder in which layers of the skin don’t anchor together properly, causing friction that leads to massive, painful blistering at the slightest scrape or bump. 

“They actually look like burn blisters,” McCann said. “She had blisters surrounding her fingers so that they looked like hot dogs in a bun.”

Today, however, 18-month-old Elisa’s fingers look almost normal. Her mother has taken off the gloves and wraps that covered her hands and feet and she’s even allowing the toddler to experiment with walking — even if it means she falls.

That’s because in May, Elisa became the 28th child enrolled in a ground-breaking clinical trial at the University of Minnesota that uses bone marrow from healthy donors — in this case, Elisa’s sister Stella — to repair the symptoms of the devastating disorder.

Led by Dr. Jakub Tolar, director of the university’s Stem Cell Institute, the trial begun in 2007 is still considered experimental but has become the treatment of choice for the most severe cases of EB, he said.

“This is one of the most difficult to treat disorders and a very painful disorder,” Tolar said. “Before we started, there was absolutely nothing that would change the outcome of these children.”

EB is caused by a defect in the genes that make collagen, a connective tissue that allows skin layers to adhere to each other, Tolar said.

“It’s a protein that makes loops that are like Velcro that attach the upper layer to the lower layer,” he said. “When that Velcro is missing, the layers slide against each other and cause blisters.”

About 20 in every 1 million babies born in the U.S. have EB, which comes in several forms and can range from mild to severe, experts say. That means between 25,000 and 50,000 people in the U.S., mostly children, are living with the condition. Left untreated, EB results in painful disfigurement and early death, typically before the age of 30. Earlier victims of EB often developed deadly skin cancer spurred by the rapid cell turnover the condition causes. 

Babies like Elisa, those born with severe EB, develop lesions everywhere: on their hands and toes, on their bottoms, in their mouths and throats. They’re often called “butterfly children” because their skin is so fragile — as fragile as butterfly wings, some say.

But when Elisa was born, McCann wasn’t interested in sentimental labels. Like many modern parents facing rare diseases in their children, she turned to experts and the Internet to demand answers.

“I said I want to know the No. 1 doctor in the world who can do something about this,” she said. Over and over, Tolar’s name came up.

“I got in contact with him and we were in contact every day,” she said. “Right away, he got very protective of Elisa.”

McCann and her husband, Dagan McCann, a travel writer, moved to Minneapolis to be close to Tolar and his treatment, she said.

Transplanting bone marrow from Stella to Elisa populated her blood with healthy cells. First, though, scientists had to give Elisa chemotherapy and radiation to make room for the new cells to “set up shop,” Tolar said. An extra boost of full-body irradiation appeared to allow the cells to engraft, or take hold, even better.

At the last measure, more than half of the donor cells from Stella had partially engrafted, a high percentage, Tolar said. In previous protocols, about a third of the children who received bone marrow transplants had some improvement, but not the dramatic results Elisa has shown. In many of the children, however, Tolar has been able to show that cells from the donated bone marrow actually gravitate to the skin, healing the injuries. 

For Elisa, the real-life results have been impressive, Gabriella McCann said.

“Before the transplant, I was walking everywhere with a needle or scalpel to open the blisters and drain them,” she said. “They would get humongous in a few minutes.”

Now, Elisa still gets blisters, but they’re smaller and they heal by themselves. She’s still fed through a tube because of the blisters that scarred her throat, but that’s getting better. And just this week, she got to go out to lunch for the first time because she’s healthy enough to be just another baby out with her mom in public.

“My daughter didn’t have a life before,” McCann said. “Now my daughter has a life and they’re pretty sure she won’t blister as much as before.”

Funding for work by Tolar’s team and other leading EB experts who are investigating stem cell therapy and gene transfer therapy at Stanford University is a huge concern. EB is classified as a rare disease, and paying for the research can be a challenge. But the scientists are boosted by organizations such as the EB Medical Research Foundation, headed by Andrea Pett-Joseph and her husband, Paul Joseph. Another prominent foundation is DEBRA of America, the Dystrophic Epidermolysis Bullosa Research Association. 

The Josephs' son, Brandon, was born 10 years ago with EB, galvanizing his parents into taking over the all-volunteer nonprofit that has raised $5 million for EB since 1991. They’ve attracted a star-studded honorary board of directors that includes actors Courteney Cox, Jennifer Aniston, Brad Pitt and Adam Sandler, plus other sports and political luminaries.

But with successes like Tolar’s bone marrow transplant and a natural gene therapy program and new research into gene transfer therapy at Stanford, the Josephs say the investment is paying off.

“A lot of families are feeling more hopeful that this is happening,” said Andrea Pett-Joseph. “It’s happening at a good pace and people can see the evidence of it. Sometimes, science is so far away.”

Source: www.today.com

Topics: University of Minnesota, epidermolysis bullosa, EB, blisters, clinical trials

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