Brain implant allows baby girl, born deaf, to hear for the first time
Jane Weaver TODAY
May 27, 2014
Sitting on her mother’s lap, a 1-year-old baby girl suddenly turns her head to hear the clicking in a black box next to her — the first sounds she’s ever heard. The child’s serious expression reflects a remarkable moment of discovery.
The parents of Elise Bradshaw, who was born profoundly deaf, shared the moments of discovery after Elise received an innovative procedure called an auditory brain stem implant (ABI).
Elise was diagnosed with Charge syndrome, a rare birth defect that left her deaf due to missing auditory nerves. “Her world was smaller, things that were happening left and right weren’t necessarily something she was aware of,” her mother Jill Bradshaw of Texas told TODAY.
Without auditory nerves, Elise wasn’t a candidate for a cochlear implant. But doctors at Massachusetts Eye and Ear in Boston thought the new procedure might help the little girl.
A cochlear implant bypasses nonfunctioning “hair cells” of the cochlea and stimulates the auditory nerve. But an ABI bypasses an absent or damaged cochlea and auditory nerve to directly stimulate a portion of the brain involved in hearing called the cochlear nucleus, Dr. Daniel Lee, director of Massachusetts Eye and Ear’s Pediatric Ear, Hearing and Balance Center, told TODAY.
The device is already being used in adults and has been implanted in older children, but Elise is the youngest patient in the United States to receive the ABI in a joint clinical program between Mass General and Eye and Ear, a Harvard Medical School teaching affiliate.
“She is neurologically normal, is age appropriate for her developmental milestones, and has incredibly supportive and dedicated parents who are committed to seeing her succeed with the ABI,” Lee said of the decision to include the child in the trial.
In late March, the girl underwent a right-ear craniotomy surgery and placement of auditory brainstem implant (ABI) at the Massachusetts General Hospital. And then on April 15, the audiology team of surgeons activated the implant for the first time, with Elise’s family recording her reactions on video.
A similar implant was given last year to 3-year-old Grayson Clamp, whose father captured the moment the boy heard his father’s voice for the first time in a video that went viral.
Now Elise can hear noises, although doctors are not sure if she’ll eventually be able to understand spoken word.
“As she becomes older, and with appropriate audiology and speech therapy support, we hope that she will be able to understand patterns of sounds and ultimately, speech,” Lee said. “Her ultimate hearing outcome is not known, however, but she is showing good progress thus far.”
Elise’s parents are hopeful.
“Now some of those dreams, careers and so forth, might be an option,” says Jill Bradshaw.
The little girl’s parents say she’ll learn sign language as she grows up and she’ll be part of the hearing world and the deaf community.
Revolutionary procedure gives baby gift of sound
By Amanda Brandeis
May 22, 2014
ROUND ROCK, Texas (KXAN) — Baby Elise could feel her father’s warmth. She could see her mother’s smile. But something was missing.
“We went from shock to immediately, what do we need to do?” said her mother, Jill Bradshaw.
Elise was diagnosed with CHARGE syndrome, which in her case left her profoundly deaf due to missing auditory nerves in the cochlea.
“Her world was smaller. Things that were happening left and right weren’t necessarily something she was aware of,” said Bradshaw. “They said they didn’t see the nerve and that she wasn’t a candidate for cochlear implants.”
However, there was another option, an auditory brainstem implant. The Bradshaw’s traveled to Massachusetts General Hospital for the procedure. Massachusetts Eye and Ear Infirmary and Mass General doctors did the surgery together, as well as a specialist from Italy. She was the first infant in the United States under the age of one to have the procedure. One week before Elise’s first birthday, the family traveled back to Boston to activate the device. The activation went according to plan, and Elise heard sound for the first time in her life.
“She did amazing that first day of activation, and then the subsequent day in the hearing booth,” her father Jason Bradshaw said. “When she was responding to the various frequency ranges in the speech spectrum, I was just truly amazed. We had data that said, ‘hey she’s actually hearing these different frequency ranges and responding to each one.’ So, we know her brain is getting the information.”
“I just became stupidly happy! I couldn’t stop smiling,” said Jill.
As for education, the Bradshaws say Elise is lucky. Live Oak Elementary has a program uniquely designed for deaf children. Of the 550 students enrolled at Live Oak Elementary, roughly 60 are either deaf or hard of hearing. Her parents are teaching her Signing Exact English (SEE), which is a sign language system that represents literal English.
“Now some of those dreams, careers, might be an option for her,” said Jill.
The road ahead will require hard work, but so far Elise has proven to be a fighter.
According to the CHARGE Syndrome Foundation webpage, CHARGE syndrome is a recognizable (genetic) pattern of birth defects which occurs in about one in every 9,000-10,000 births worldwide. It is an extremely complex syndrome, involving extensive medical and physical difficulties that differ from child to child.