Two-year-old Natalie Su last week toddled among her peers – exploring, talking and listening to the lively cacophony. But for Natalie, the sounds are newer; the preschooler was born with hearing loss.
When she was 10 months old, Natalie received bilateral cochlear implants, which replace the function of the damaged inner ears. Her parents, Los Altos residents David and Charlene Su, didn’t take the decision lightly as they weighed their options.
“She could be deaf and lead a wonderful, rich life,” Charlene Su said. “We wondered whether we’d be forcing something upon her.”
After months of tests, deliberation and research, the couple decided that cochlear implants would give Natalie the fullest spectrum of choices. When she’s older, she can choose for herself to be part of the deaf community or keep the cochlear implants.
Unlike hearing aids, which make sounds louder, cochlear implants do the work of damaged parts of the inner ear to provide sound signals to the brain.
“It’s a different way of hearing – a semblance of sound,” Su said. “You have to retrain your brain to recognize sound.”
Su – who’d never heard of the device prior to her daughter’s birth – has since become somewhat of an expert in the technology. But that initial revelation following Natalie’s newborn screening came as a shock.
“You leave feeling really vulnerable and scared,” Su said. “We have no family history.”
Steps to surgery
Dr. Kay Chang, a surgeon who practices at Lucile Packard Children’s Hospital, first saw Natalie when she was 4 months old.
“She had previously been evaluated and several tests indicated significant hearing loss, but the tests weren’t totally consistent,” said the cochlear implant specialist.
Chang confirmed bilateral profound hearing loss, meaning Natalie couldn’t hear speech at any level. Su noted that Natalie did respond to louder noises like a lawn mower.
“The whole goal of hearing, especially at this age, is you want to be able to hear speech clearly,” she added. “If they don’t hear, they’ll never be able to understand or produce clear speech.”
One to six out of every 1,000 children in the U.S. may be born with a severe to profound hearing loss, according to the American Speech-Language Hearing Association.
The most common cause of congenital sensorineural hearing loss, like Natalie has, is a genetic mutation that disrupts an ion pump in the inner ear that powers the hearing cells. However, patients with the genetic mutation have normal anatomical structures and nerve endings in the inner ear.
“With cochlear implants, we’re inserting an electrode into the inner ear, which is able to directly stimulate these nerve endings,” Chang said.
Surgically implanted, a cochlear implant contains a computer chip, removable magnet and electrode array. An external magnet attaches to the coil on the sound processor located outside the skin. The electrode array follows the natural curve of the cochlea and directly stimulates the hearing nerve to provide sound.
Chang said three different companies – Cochlear, Advanced Bionics and MED-EL – offer implants. With each boasting unique specialty features, he likened choosing one over the others to someone selecting an iPhone over a Samsung.
The Su family opted for the implant made by Cochlear.
Chang performed Natalie’s cochlear implant surgery when she was 10-and-a-half months and, after the site healed, activated the device just before she turned 1. One month later, Natalie spoke her first word – “mama.”
Chang said two aspects determine how well a patient responds to cochlear implants: the age of implantation and – “perhaps more importantly” – parent involvement.
“Just sticking in an implant isn’t going to work,” he said. “The brain has to learn how to respond to the electrical stimuli. These electrical impulses generated by the implant do not resemble in any manner the nerve patterns from normal functioning inner ears.”
Fortunately, as Chang put it, “the brain is the best pattern-recognition device that we know in the world. If implanted early enough, and with appropriate auditory and speech rehabilitation, the brain is able to distinguish sounds electrically transmitted through a cochlear implant as well as it would have been able to had there been an intact normal ear.”
Twice weekly, Natalie attends a cognitively based preschool, Weingarten Children’s Center in Redwood City. She also goes to two hours of speech therapy per week, plus an additional hour weekly with a hearing teacher through Santa Clara County’s Early Start Program. Natalie also takes gymnastics, ballet and music classes.
“She’s super busy – very overscheduled,” Su joked.
When Su realized the extent of rehabilitation – especially in the early years – she elected to leave a career in marketing and brand management to stay home full time.
“The therapy is there to teach the parent what to do when they go home,” she said. “It’s not like that one hour is going to make all the difference.”
Therapy has become part of the family’s everyday life: observing and explaining, reading aloud and narrating activities to create meaning between sounds and words.
“At first, it felt like work – I’m talking about everything,” Su said. “Now it’s just our life. It’s not even work anymore.”
Natalie is now just past 2 years old. The Su family recently received results from an auditory test and found that her expressive vocabulary is on par with 3-and-a-half year olds.
Even with Natalie’s remarkable rehabilitation, Su still finds herself marveling at the simpler successes. She recalled a time the family was indoors and Natalie heard a car driving past. She pointed to her ear and said, “Vroom, vroom.”
“I had this expectation that she would never hear these sounds,” Su said. “It’s the everyday things that are really huge for our family.”
She encouraged other parents of children with hearing loss to hold onto the dreams they have for their children.
“It’s not going to be the hearing loss that limits who they can be,” she said. “They can realize those dreams even with hearing loss. It takes a lot of work, but there’s really no limit to what they can do.”
For more information on Cochlear implant systems, visit cochlear.com.