Late last spring, a few weeks after young Auguste Majkowski returned home to from experimental surgery to give him the sense of sound, a dog barked nearby and he looked up, startled. Hearing was at hand for a deaf little boy. His family rejoiced.
Then the dogs and all other noises seemed to go silent for three-year-old Auguste, who lives in the Montreal neighbourhood of Ville-Émard.
Auguste's mother and indefatigable advocate, Sophie Gareau, always knew the road to functioning electronic hearing would be long, but the setback was discouraging. "In the past few weeks and months, progress went stagnant. We felt like we'd hit a plateau," she said. "It almost felt like the thing didn't work. We didn't see any more reactions in everyday life. In therapy every once in a while we'd see something, so we'd wonder if it's working or if it's not."
In November, the family headed back to the Los Angeles hospital, where the experimental procedure was performed. Once again, they started with the battery of tests and adjustments similar to the ones that followed Auguste's surgery in May. This time, doctors started with a strategic retreat, turning off four of the probes leading into Auguste's brain, hoping he would have an easier time deciphering a simplified set of signals from seven wires instead of 11.
Then, Auguste headed into the sound booth with some of the world's top educational therapists. Once again, he started to react to sounds.
"He responded to every sound he could hear, it was unbelievable," Gareau said. "The therapist and I were looking at each other with these huge eyes."
Auguste's treatment is part of a groundbreaking clinical trial sponsored by the United States to test the auditory brainstem implant on children. Auguste had his operation at Children's Hospital Los Angeles, one of a handful of U.S. centres that started installing the ABI to transmit audio signals from a sensor behind the ear straight into the brain stem through a series of wires and probes.
A major challenge with Auguste is that he's a three-year-old boy who likely doesn't even understand what hearing is, or what he's supposed to feel and how he's supposed to react, according to his mother. He's at an age where rebellion is common. He's only starting to learn language through sign, so communication is a major challenge.
"He wants to set his own rules. Like any 3 1/2-year-old boy, he doesn't like to stay sitting down. That's what was happening," Gareau said. Educational therapy teaching him to sit still and react to auditory sensations is almost as important as the implant itself.
The ABI operation is a leap forward in technical difficulty and danger from the now-common cochlear implant, the device used to stimulate the auditory nerve of some 300,000 deaf people. Test subjects such as Auguste have no auditory nerve, so sensors stimulate the area of the brain stem that processes hearing.
Auguste returned to Montreal in December, where he resumed attending his specialized daycare, where he's learning sign language. He also resumed working with his audiologists. His mother held her breath at the first session. Would he still hear, as he had a week earlier in California?
"I was a little worried about how it was going to go, but we sat down, and he started to hear," Gareau said. "I guess I could say I'm flabbergasted. I'm really blown away."
And just a few days ago, Auguste was at a big Christmas party where a DJ worked a microphone. Three times, the disk jockey's booming, amplified voice made Auguste look up and point at his ear. "It was really clear he could hear something," Gareau said. "Now let's hope with time he'll make something out of it."
Gareau said she and her husband, Christophe, have worked hard to keep expectations in check. A signpost of success to the couple would be if Auguste could hear a honking horn or a fire alarm. His doctors have said they hope he can have a telephone conversation some day.
For now, real-world hearing is limited to the thunder of a DJ's voice, but the family remains optimistic. "You know when you hit a wall in life, and you have to find a way out? You have to get creative. Our team of doctors and therapists got creative," Gareau said.
"It's really fascinating to see so many people putting so much energy into one little boy and to figuring out how do we make this work."