Brainstem implant offers a chance to hear

LOS ANGELES – Auguste is 3 years old, a charmer with big blue eyes, long lashes and a playful smile. He's wearing a T-shirt that says "Make some noise" and fiddling with his Etch A Sketch in a hospital exam room.

But when a doctor reaches toward his temple, he quickly releases the knobs and tilts his head. Behind each ear, under the skin, are cochlear implants — surgically implanted devices meant to help process sound. But they didn't help Auguste.

He was born without an auditory nerve, the wire that ferries sound from the ear to the brain. He points out planes in the sky but can't hear their engines roar. He's never heard the voices of his 4-year-old sister or 1-year-old brother. He doesn't know the sound of his own name.

Now, a team of Los Angeles doctors and researchers believes it can help Auguste hear. But the device the team is testing will require surgeons to go much deeper into Auguste's skull, all the way to the brain.

Sophie Gareau, Auguste's mom, often tears up when she thinks about what's ahead. "He's so perfect, you know?" she says. Her voice breaks. "I don't want anything to happen to him. But I am so convinced that it's going to work."

Sophie realized something was off when Auguste was 6 months old: Her baby wasn't babbling. Doctors in Montreal said he was fine. She insisted otherwise, but it was nearly a year later before audiologists played sounds as loud as an airplane engine in the toddler's ear and confirmed what his French Canadian parents had suspected: Auguste was profoundly deaf.

"I think it takes a moment to sink in and then you cry for a week," Sophie said.

Sophie heard about something called an auditory brainstem implant, or ABI. The device uses an external processor on the ear to send sound to a surgically implanted electrode array. A doctor warned her against it.

"He had mentioned that only the Italians were doing it and they were 'cowboys,' which in French means 'crazy people,' " she said.

The Italian was Vittorio Colletti, who began putting the devices in healthy deaf patients in the late 1990s. This was unheard of at the time: There had to be an urgent reason — such as removal of a dangerous tumor — to perform such a risky surgery.

Colletti was criticized. But he began reporting incredible results. The medical community was skeptical. So Colletti invited Robert Shannon, who helped develop the modern brainstem implant while working as a medical researcher at the House Research Institute in Los Angeles, to see for himself.

Shannon spent hundreds of hours testing patients in Italy.

"I was astounded," said Shannon, who now works at USC. "It was even better than he said."

A team of researchers that included Shannon wanted to introduce the procedure to children in the United States. But a risk-averse Food and Drug Administration denied the team's initial request, said USC audiologist Laurie Eisenberg, one of the lead scientists.

"After all," Eisenberg said, "it is brain surgery in children."

After some key FDA changes, the team received the go-ahead in 2012. The National Institutes of Health had agreed to pay for the team's clinical trial. Auguste is its first patient.

Auguste lies unconscious in the operating room at Children's Hospital Los Angeles.

The surgeons cut through skin, saw through bone and snip through the dura, the brain's protective covering. They drain cerebrospinal fluid so that the cerebellum relaxes, allowing them to move past the bulging tissue and reach the brainstem.

Colletti, the "cowboy" who pioneered the procedure, is sitting nearby. He has flown in from Italy to see the clinical trial begin. He can't wield a knife; all he can do is offer advice as he watches Auguste's brain on a large screen.

Neurosurgeon Marc Schwartz knows the path to the cochlear nucleus, the spot on the brainstem where the ABI will go. He's seen Colletti perform the procedure in Italy about half a dozen times. He follows the ninth cranial nerve to the boundary between the cerebellum and brainstem, then looks for a tiny nook called the lateral recess. That's where the cochlear nucleus should be.

But it isn't.

"There's nothing there. There's no hole there," Schwartz says. "No hole. Not even anything that looks like a hole. Remotely."

The chatter in the room has dropped to hollow silence, punctuated by the harsh beep of the monitors.

Schwartz retraces his steps. No luck. The surgeons debate what to do next. If they can't find the lateral recess, they can't put the device in. Schwartz is worried.

"I'm just afraid of hurting the kid," he said.

At last, Schwartz finds the entrance. Over several minutes, he carefully inserts the 8-millimeter-long electrode array into the right spot.

"Bravo," Colletti says. "Bravo, bravo!"

After a month of recovery, it's time to find out whether the surgery worked. Audiologists Margaret Winter and Jamie Glater — known as "child whisperers" — scrunch around the kid-sized table. As Auguste plays with his trucks, Winter uses her computer to remotely activate individual electrodes on the device.

Auguste is rolling a toy monster truck across the table. He freezes, mid-car crash. Something's happened.

Glater makes a crooked L with her fingers, the thumb pointed at her ear. "Listening! You're listening!" she says.

Then, it's time to try a human voice. They turn on the microphone.

Winter turns the signal up. Auguste winces.

That's perfectly normal, Winter tells the parents. It's a completely new experience. Whatever he felt, it "penetrated his world," Winter says.