Deaf Toddler Hears For First Time With Device Implanted in Brainstem Hearing device is part of a clinical trial and not yet FDA-approved for children

March 5, 2014 -- At a hospital in Boston, sound registered in Alex Frederick's brain for the first time.

Alex, just 17 months old at the time, is deaf, but a device, not yet approved in the United States for children, is helping to change that. It was implanted directly into his brain.

Alex was born two months prematurely, weighing just four pounds and four ounces at birth. He spent the first month of his life in the neonatal intensive care unit of St. John Hospital in Detroit. His early arrival brought him into the world facing a host of challenges. Scans would show that Alex had a heart condition, while his vision appeared to be compromised. And then there was the matter of his hearing. Alex failed the hearing test given to all newborns. Then he failed a second hearing test two months later.

"That was a blow," said Alex's father Phil Frederick. "The most difficult part for me was that first time… he failed his first hearing test, because I really thought, 'this is going to go fine.' … that was the most, for me personally, most heart-wrenching point."

Alex has two older sisters, Evelyn, 6, and Izabella, 3. Phil was watching one day as Evelyn played with her brother.

"She wanted to get a response from him and something she was doing with a toy… and I was like, 'Well, Evelyn, he can't hear… he might not see what you are trying to hand him,'" Phil said. "She just said, 'no, I don't want that for him'… 'how's he ever going to play with us' or 'how's he ever going to play with anyone?'"

When Alex was 1 year old, his parents tried for a cochlear implant, a 40-year-old technology that uses electrodes to stimulate auditory nerves. The surgery commenced, but was halted in mid-operation when it became evident it would not work due to the irregular structure of the toddler's inner ear. The scar from that failure is still evident behind his right ear.

Through all this, life for Alex went on as a constant round of visits to specialists to undergo more tests for his heart, and then begin sign language classes, as it started to sink in for his parents what all this meant.

"There's things that were going to happen in his life that… I wouldn't know how to help him through those situations where he needed help," Phil said.

Phil kept looking for an answer, for some other technology that would help his son hear. In the course this research, he learned about an approach for children that had been pioneered in Italy by Dr. Vittorio Colletti, and it was about to undergo a series of clinical trials in the U.S. to win FDA approval.

It's called an Auditory Brainstem Implant (ABI), a small antenna that is implanted on the brainstem so that it can pick up signals from a tiny microphone worn on the ear and relay them back inside as electrical signals that reach the area of the brain associated with interpreting sound.

Dr. Colletti had some successful outcomes with his ABI surgeries, including a young woman, previously deaf, who can now carry on phone conversations without issue, but it took her years after the ABI was implanted to be able to get to that point. Another one of Colletti's patients was a boy named Andrea, whose case demonstrated that for children who have never had the ability to hear, it takes years to learn what hearing means. But years after having the surgery, Andrea learned to play the guitar.

After finding out about ABI, Phil found out which U.S. hospitals where hosting the clinical trials and emailed them all individually to get Alex on the list.

Last August, the family heard that there was an opening in a trial being conducted at the Massachusetts Eye and Ear Infirmary in Boston, under the direction of Dr. Daniel Lee.

"ABI surgery in the child… who cannot get a cochlear implant can result in meaningful sound awareness and speech perception with time, but it takes work," Dr. Lee said.

On Oct. 5, 2013, the Fredericks made their way from their home in Washington Township, Mich., to Boston. Alex's surgery cost hundreds of thousands of dollars, but was paid for by the family's insurance company.

His parents were by Alex's side as a team of doctors from Massachusetts General, Massachusetts Eye and Ear Infirmary, and even Dr. Vittorio Colletti who flew in from Italy, prepped the toddler for surgery. Even as he was wheeled down to the operating room, it was still uncertain whether the implant would even work.

After five and a half hours in the operating room, Alex was taken into intensive care. His head was wrapped in a cap of bandages, under which was a cluster of wire that doctors hoped would allow him to hear. He then went home to recover.

Several weeks after surgery, the big day arrived. Alex and his family returned to Massachusetts Eye and Ear Infirmary in November to have his ABI switched on for the first time. The wires connected the device inside his head to a sound generator controlled from a computer, where a doctor could manipulate the sound level on the device.

Alex's parents decided that they wanted the first sound their son to hear to be his sisters' voices, so after the device was turned on, the girls started talking, but Alex didn't elicit a reaction. Others in the room tried raising the sound level, but still nothing at first.

Then, to everyone's surprise, a doctor in the room slammed her keys into the side of a desk, and Alex turned towards the sound.

"All of the sudden he just looked," Stephanie said. "He stopped everything that he was doing and he looked." "I felt right away, 'oh he definitely heard that,'" Phil added. "I knew, he was completely focused on his toy and then he just-- he looked."

With that little turn of his head, Alex had made the connection to sound for the first time, but it was the first step in a long journey.

Alex and his parents now return to Boston every month where doctors continue to test Alex's hearing response as they fine-tune the electronics inside his skull. But Alex still has to go through the long process of learning what sound actually is and how it has meaning, even meaning as words.

"It takes speech therapy, it takes audiology to continue to tweak the program," Dr. Lee said. "And it takes a lot of commitment on the part of the family, and we are very lucky that our patient has an unbelievably dedicated parents that are going to see this to a successful outcome."