Imagine Winter 2014 - A Sound Solution

The first federally funded clinical study on auditory brain stem implants offers Auguste Majkowski, deaf since birth, a chance to hear.

Auguste Majkowski had had quite enough. He shook off the speech processor that was curled over his right ear, and in its path came the built-in microphone and the transmitter attached to it. The entire apparatus that would allow him to hear for the very first time -- he tossed it to the ground and tried to give it a good stomp. Thankfully, he missed. It was a tantrum suitable for a 3-year-old, which is what he was.

All the adults who were there to see it, including a surgeon, three audiologists, and his mother and father, were delighted. This wasn’t a straight-to-YouTube, “Eureka! He hears!” moment, but it was evidence he experienced something, even if it was a sensation -- sound -- that his brain didn’t recognize and wasn’t ready to accept. “It’s hard to know whether for him that could qualify as hearing at this point,” says audiologist Margaret Winter of the Keck School of Medicine of the University of Southern California (USC), where Auguste’s new hearing device was activated, “but we know he got stimulation that penetrated his world.”

Five weeks earlier, in a six-hour operation at Children’s Hospital Los Angeles, Auguste, deaf since birth, received his auditory brain stem implant, or ABI, the initial case in a clinical trial launched at USC to assess the safety of the device in young children. It’s the first ABI study to have the backing of both the U.S. Food and Drug Administration and the National Institutes of Health. The ABI offers an option to patients whose cochlear nerve, which ushers sound from the inner ear to the brain, is either damaged or, like Auguste’s, absent. The ABI sidesteps the ear altogether, converting sound waves to electrical signals and transmitting them to electrodes implanted right at the brain stem.

“You’re not actually putting a sound into the brain,” explains Mark Krieger, MD, head of Neurosurgery at CHLA, who alongside neurosurgeon Marc Schwartz, MD, and neurotologist Eric Wilkinson, MD, of the House Clinic in Los Angeles, operated on Auguste. “You’re coming up with an electrical stimulation pattern that the brain Auguste had already received cochlear implants, one in each ear, which stimulate the hearing nerve. They hadn’t worked, it was discovered afterward, because he did not have a hearing nerve.

For his mother, Sophie Gareau, that episode had settled things. Residents of Montreal, she and her husband, Christophe Majkowski, accepted Auguste’s deafness and were establishing a life that accounted for it. The whole family was taking sign language classes, in-laws included. “We had 12 people in our basement learning sign language,” Sophie says. “Life went on.”

But not without some loss, Christophe admits. “When I get my kids from school,” he says, “I can ask my daughter, ‘How was your day?’ But with Auguste, how do you do that? In sign language, the feelings... ” His voice falls off. “It’s hard.”

Auguste’s audiologist, however, approached Sophie after the cochlear implants failed. “She told me, ‘You know, there’s something else that could be possible. It’s called an ABI.’ That’s all she said.”

It’s a technology that in the U.S. has been used almost entirely on adult patients with neurofibromatosis 2, or NF2, a tumor that can grow on the auditory nerve. To remove the tumor, the nerve has to be sacrificed, costing patients their hearing.

Outside of a few clinical studies, the ABI has not been approved in the U.S. for use on anyone under age 12, though among the congenitally deaf only the very young can benefit from it. Past age five or six, if the brain’s native language mechanisms have not been stimulated, they atrophy and can’t be restored. But in Europe -- specifically, Italy -- ABIs have been implanted in kids for years.

Sophie queried the doctor who performed Auguste’s cochlear implant surgery. “He said, ‘Yes, I know about the ABI. It’s done in Italy, but those guys are cowboys.’ “I said, ‘Oh, well, forget about it.’ I erased it from my mind.”

But soon after, she read of the FDA’s approval of the clinical trial in Los Angeles. “I thought, ‘If the Americans are doing it, it must not be that bad. Maybe those Italian guys aren’t such cowboys after all.’”

Verified in Verona

It wasn’t really “those guys,” but that guy. Being called a cowboy is gracious compared with other charges the colleagues of Vittorio Colletti, MD, launched at him after he began doing ABI procedures on children in the late 1990s at the University of Verona, where Colletti is chairman of the Otolaryngology Department.

“They said I was doing something that had no possibility of development and was very risky,” he says. “But I am a tough Italian, and if I’m convinced of something, if I have the rationality to do it, I pursue it.”

— Vittorio Colletti, MD

The rationality amassed in his favor, as non-NF2 ABI patients -- including children -- excelled, showing greater success developing speech and hearing than NF2 patients.

Colletti’s greatest advocate has been Robert Shannon, PhD, of the Keck School of Medicine of USC. An eminent auditory scientist who participated in the ABI’s original development, Shannon met Colletti at a conference in 2004, and Colletti urged him to come to Verona to verify his work for all the doubters.

“He said, ‘I have these brain stem implant patients -- adults -- who are talking on the telephone, and nobody believes me,’” Shannon recalls. “I said, ‘Yeah, I don’t believe you either.’

“I was skeptical,” he says, turning to his old friend. “I’m always skeptical. I’m a scientist.” Colletti shoots back, “I’m a surgeon. I’m a believer!”

Shannon visited Italy and confirmed the data. “He had more than one good patient, he had many good patients, and they were talking on the phone without any difficulty. Then he said, ‘Oh, I have some children, too.’ Then I saw Andrea.”

An ABI recipient at 2½, Andrea was 7 in the video Shannon saw of him conversing boisterously with his audiologist. That was also about the same age the boy was when he came home from school with a note admonishing him for talking too much.

“He was getting success we had never seen,” Shannon says of Colletti. “He was changing the face of treatment for children.”

Things That Go Boom

Colletti is now nearing 100 young ABI patients. His next one was nearly Auguste Majkowski.

After learning of the clinical study at USC, Sophie submitted her son’s application. But as a fallback, she was corresponding with Colletti’s group in Verona and had sent Auguste’s medical file to Italy.

Sophie applied for the L.A. study in November, and spring was nearing without an answer. Auguste was turning three, which in ABI years translates to middle age. “This was prime time,” she says. “We were getting antsy. We were heading to Italy if they said no.” In March, she received an email from USC. Auguste was in.

Still, he would have the benefit of Colletti’s presence. Schwartz, Wilkinson and Krieger together had been to Verona to watch Colletti operate on children without hearing nerves. Now Colletti would be at CHLA to attend Auguste’s surgery.

In addition to observing Colletti, the three surgeons collaborated on four ABI surgeries on teenagers with NF2 in advance of the clinical trial. That only added to the extensive experience Schwartz and Wilkinson had accumulated at the House Clinic, a center for ear disorders where more than 300 ABI procedures have been done. Wilkinson says he does cochlear implant procedures on children routinely, and the only difference is the placement of the electrodes.

Of course, he adds, that’s a powerful difference. “If you make a mistake, the consequences are much more severe when you’re working on the brain stem than in the cochlea. We’re going to an area in a child that no one goes to.”

That risk kept Auguste’s Canadian doctors from recommending the surgery, thinking it was unnecessary to do on a healthy child. But a two-day visit to L.A. to consult with the entire team buoyed Sophie and Christophe -- particularly Shannon’s assurances that Auguste would hear. At the very least, he was confident Auguste would recognize reference sounds -- car horns, sirens, things that go boom.

That was enough for Sophie. “If your kid is running in the street and there’s a truck coming, you want him to hear that truck,” she says. “For me, that’s it. Anything beyond that is a bonus.”

Interestingly, not so for Schwartz, who wants to see some amount of language acquisition before he’ll call the case a success. “I’ll be extremely happy helping this kid be more connected to the world,” he says, “but I’m hoping for more than that. I’ll only be satisfied if he develops some speech perception.”

Auguste’s brain has work to do before that happens, as it learns to decipher the electrical codes it receives through the implant. “If he learns to distinguish language and speech, and even speak himself, only the future will tell,” Sophie says. Her initial despair over learning Auguste was deaf has been replaced by a kind of tenacious objectivity.

“You do think, oh my God, he’ll never hear the ocean. You grieve, but then you get over it. It’s not like he’s lost something. He’s never heard it, you know? He just knows how beautiful it is. He can feel the breeze around it, he can look at it. He can feel it in different ways. We’ll do everything possible to train Auguste to distinguish these sounds. I’m not saying he’ll never hear the ocean, but if he doesn’t, it’s fine. He’ll appreciate life in a very beautiful way, no matter what.”

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