Brainstem implant offers a chance to hear

  • Article by: AMINA KHAN , Los Angeles Times
  • Updated: August 2, 2014 - 7:57 PM

In a first-of-a-kind U.S. surgery, doctors implant device into brainstem.

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Sophie Gareau, right, looks in on her son Auguste Majkowski during a visit as the three-year-old recovers in his crib at Children’s Hospital Los Angeles on May 11, 2014. Surgeons implanted an auditory device into Auguste’s brain stem. (Brian van der Brug/Los Angeles Times/MCT)

– 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.

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  • Auguste Majkowski, 3, who has been deaf since birth, laughs with his mother Sophie Gareau on May 3, 2014. The family moved to Los Angeles for a rare surgical procedure that involves inserting a device into Auguste's brain stem in the hope that it will help him hear. (Brian van der Brug/Los Angeles Times/MCT)

  • Three-year-old Auguste Majkowski reacts as he hears a series of tones during testing of his new auditory brain stem implant device with his mom Sophie Gareau, right, his dad Christophe Majkowski, left, and audiologists at USC Center for Childhood Communication on June 13, 2014 in Los Angeles. Auguste was diagnosed as being profoundly deaf when he was a year and a half old, and the test was part of his participation in a National Institutes of Health clinical study. (Brian van der Brug/Los Angeles Times/MCT)

  • Sophie Gareau touches her son Auguste Majkowski's face as he wakes up from surgery in the ICU at Children's Hospital Los Angeles on May 6, 2014. Surgeons implanted an auditory device into Auguste's brain stem during a roughly six-hour surgery. (Brian van der Brug/Los Angeles Times/MCT)

  • Three-year-old Auguste Majkowski concentrated as he participated during the testing of his new auditory brainstem implant device. At top, he laughed with his mother, Sophie Gareau, and was consoled by her after he recovered from surgery at Children’s Hospital Los Angeles.

  • Three-year-old Auguste Majkowski's head is taped with wires and electrodes that monitor his brain and nerve functions during a surgery at Children's Hospital Los Angeles on May 6, 2014. Surgeons implanted an auditory device into Auguste's brain stem during the six-hour operation. (Brian van der Brug/Los Angeles Times/MCT)

  • Deaf three-year-old Auguste Majkowski communicates with his mother Sophie Gareau using French Canadian sign language at their rented home in Los Angeles on May 3, 2014. The family, from Montreal, is in Los Angeles for a surgical procedure that involves inserting a device in Auguste's brain stem in the hope that it will help him hear. (Brian van der Brug/Los Angeles Times/MCT)

  • Auguste Majkowski,3, with his mother, listened for a series of tones during the testing of his new brainstem implant device.

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