By all accounts, Briella Wright has had some tough times during her first three years of life. During pregnancy, her mother fell into a three-week coma due to having both type 1 and type 2 diabetes. When Briella was born at 37 weeks in July 2014, she had multiple holes in her heart requiring surgical repair, needed a feeding tube and was almost totally deaf in both ears. “I could run a sweeper next to her, and she’d never hear it,” says Betty Radtke, Briella’s grandmother and guardian. “She could cry and babble but couldn’t talk.”
Briella’s profound hearing loss impaired her ability to develop language. “The sounds that she could make, like ‘ba-ba’ or ‘ma-ma,’ were what we might expect from an 8-month-old, not a 3-year-old,” says Lehigh Valley Health Network (LVHN) audiologist Alicia Kittle, AuD. “She had no concept of speech and would throw tantrums because she was not able to understand what was going on around her or follow simple directions.”
A surgical solution
Briella’s family equipped her with hearing aids around age 2. “They didn’t help at all,” Radtke says. “She still had no response to sound.” In fact, the devices seemed to annoy Briella. “She would constantly take them out and hand them to her grandmother or us, presumably because they weren’t doing anything for her,” Kittle says. The family faced the prospect that Briella would continue lagging developmentally.
“That’s when they came to see me,” says pediatric ear, nose and throat specialist Sri Chennupati, MD, with LVPG Pediatric Surgical Specialties. “We decided to evaluate Briella for a cochlear implant.”
Unlike a hearing aid, which amplifies sound so ears can detect it, a cochlear implant directly stimulates the cochlear nerve that transmits auditory signals to the brain. A microphone and transmitter worn on the head and a receiver/stimulator under the skin convert sounds into electrical impulses that reach the cochlear nerve. “The implant requires a two- to three-hour surgery,” Chennupati says. The surgery had not been available in the Lehigh Valley until Chennupati recently came to Lehigh Valley Reilly Children’s Hospital (Children's Hospital).
Evaluation and commitment
Kittle conducted numerous tests to learn more about auditory function in Briella’s auditory system, while imaging confirmed Briella’s hearing system had the structures necessary for an implant. “All her wiring was there,” Chennupati says.
Chennupati and Kittle also spoke at length with the family about life after surgery. “A child who gets a new bionic ear requires continuing speech therapy and audiology,” Chennupati says. “Briella’s family seemed very committed to that.”
Briella’s cochlear implant surgery in her left ear – Children's Hospital’s first – occurred in June 2017. For six weeks, the implant remained silent while Briella healed from the surgery. Then came the day she returned to have it activated.
Experiencing a new world
“When we turned it on, she just kind of stopped,” Kittle says. “She wasn’t sure what was going on.”
“When I spoke, she looked at me and started slapping me, like ‘Whatever you’re doing, stop,’” Radtke says. “I asked, ‘Can you hear Grandma?’” When people around the room spoke to Briella or someone clapped behind her back, the girl turned toward the sound. “She had never done that before,” Radtke says. “She knew we were saying things, and I started to cry.”
Briella quickly adjusted to the world of hearing. “Within two weeks, she was saying words,” Radtke says. Within two months, her vocabulary was more than 25 words. “She has phrases like ‘look at this,’ ‘watch me’ and ‘I want a binky,’” Chennupati says. Now when Briella hands people her headpiece, it means she wants it on.
“Briella regularly meets with an LVHN pediatric speech therapist,” Chennupati says. With ongoing audiology and speech therapy, she’s expected to be mainstreamed in school by fourth grade. Briella’s home life with a 4-year-old brother has changed too. “It used to be that she paid no attention, and that would frustrate her brother,” Radtke says. “Now they holler at each other.”