Last November, 7-month-old Aida Little was able to hear her parents' voices calling to her for the first time.
"It was such a happy moment. She's such a little miracle," says Aida's mom, Melinda. "We were so excited that she could hear and that from that day on it was going to be a whole new life for us and for her."
Aida was born with Waardenburg syndrome, a rare genetic condition that can cause deafness, skin pigment changes, and pale blue eyes or eyes that don't match in color. The syndrome affects approximately 1 in 40,000 people, according to the National Institutes of Health.
Today, thanks to cochlear implant surgery at Mayo Clinic's Rochester campus, Aida enjoys listening to the radio, and she gets excited when she hears music. "She likes instruments and gleams with happiness when she hears different sounds coming together," Melinda says.
Aida was born prematurely and stayed in the Newborn Intensive Care Unit for the first weeks of her life. Before being released from the hospital, Aida failed two newborn hearing tests. After the second test, the infant's Mayo Clinic care team shared with her parents, Melinda and Matt, the difficult news that Aida was deaf. They explained that Waardenburg syndrome was most likely the cause.
"Our first reaction was, 'How is Waardenburg going to affect her development?'" Melinda says. "I'm a teacher, so I'm tuned into how kids learn."
Lisa Schimmenti, M.D., the Mayo Clinic geneticist who confirmed Aida's diagnosis, discussed the possibility that Aida could get a cochlear implant — a surgically implanted electronic device that bypasses damaged portions of the inner ear and directly stimulates the hearing nerve. Cochlear implants use an external sound processor that captures sound signals and transmits them to a receiver implanted under the skin behind the ear. The receiver sends the signals to electrodes implanted in the snail-shaped organ of hearing within the ear, called the cochlea.
Before the Littles could consider this option, however, Aida needed an MRI to make sure she was a good candidate for the procedure.
"Approximately 95 percent of children born without hearing have the necessary anatomy for an implant to be successful," says Matthew Carlson, M.D., a surgeon in Mayo Clinic's Department of Otorhinolaryngology/Head and Neck Surgery. "Based on Aida's overall development, and the anatomy of her cochleae and her cochlear nerves, we confirmed she would benefit from the implant."
While some centers wait to perform the surgery until a child is at least 1 year old, Mayo Clinic often places implants in infants as young as 6 months.
"The earlier the child gets sound, the better the brain will develop with it," Dr. Carlson says. "If implanted at a young age and followed closely by an experienced audiology team, it is highly likely that their speech and language development will be on par with other kids their age."
Before the surgery could be scheduled, Aida had to go through a series of hearing tests, including an auditory brain stem response test and behavioral testing, to confirm the severity of hearing loss. She also needed a three-month trial with hearing aids.
"The Mayo team explained that there are a lot of changes within an infant's inner ear, and they wanted to see if over time the hearing aids would work," Melinda says.
When hearing aids did not provide any significant benefit for Aida, the Littles started looking more seriously at the possibility of a cochlear implant. "Matt and I wanted Aida to hear and decided we would try anything to get her to do that," Melinda says.
During their first consult with Dr. Carlson, the Littles asked many questions about the procedure, and they were comforted by the answers.
"He was very warm and made us feel like there was a lot of hope. We watched a video of him doing the surgery on a 6-month-old," Melinda says. "He reassured us that Aida was going to be just fine and have a very successful life. Trust and respect built up really quickly."
Aida had cochlear implants placed in both ears on Oct. 29, 2018. That procedure involved putting electrodes inside the cochlea with the aid of high-powered microscopes. Two weeks later, the audiology team put the external sound processors around Aida's ears that allowed her to hear sound for the first time.
"The only expectation we had was that she was going to hear, and we were really thankful that we could come that far," Melinda says.
Aida has adapted to the implants well, according to her parents. She wears a headband to hold the external processors in place, and she seems to understand its significance already.
"She gets excited when she sees her headband come out in the morning," Melinda says. "Putting on her sound changes everything; she becomes more animated and focused. She likes playing with musical books and toy trains that have lights and sounds."
Aida works with a speech therapist twice a week, as well as a teacher from the deaf community. She also goes to an audiologist every three weeks to have the cochlear implant reprogrammed, tuning each of its 22 channels to improve sound.
"She can differentiate between a man's and woman's voice," Melinda says. "Now that she can hear, she feels very independent."
The implant hasn't only affected her hearing, it's strengthened Aida's bond with her 2-year-old brother, Dainen. "There's a deeper personal connection. They're now able to share something as simple as laughter," Melinda says. "They can share the sounds of life together and connect from there."
Being able to engage in conversation together as a family also has helped Aida bloom.
"We include Aida in our dinner conversation, and just with that little piece she has excelled with some milestones," Melinda says. "One example is learning how to wave or learning what 'so big' means. It seems like a lot of these things are coming out at the dinner table when we're having family time."
In just a few weeks, Aida will reach a major milestone: turning 1. And her parents can't wait.
"For all of us to sing 'Happy Birthday' — it's going to be amazing to see her reaction to it," Melinda says. "It's going to mean so much more to our family."
Learn more about Aida's story in this video:
Tags: Audiology team, cochlear implant, Dr. Lisa Schimmenti, Dr. Matthew Carlson, ENT/Audiology, Genomics, Hearing Loss, Mayo Clinic Children's Center, Newborn Intensive Care Unit, Pediatrics, Waardenburg syndrome