Cochlear implants click for Isabelle

Isabella-IVFive-year-old Isabelle Labriola loves sound. She eagerly chats with her twin sister, loves to sing along to holiday songs and enjoys dancing to music. Sounds click with Isabelle, even though enlarged vestibular aqueducts (the tiny canals in the inner ear) resulted in moderate-to-severe hearing loss in her right ear and severe-to-profound hearing loss in her left ear.

Isabelle’s hearing loss was identified at birth, and she was fitted with hearing aids at 6 weeks of age. Cheryl Edwards, AuD, interim director of Diagnostic Audiology in the Department of Otolaryngology and Communication Enhancement at Boston Children’s Hospital, provided testing and hearing aid management every few months. Results of these periodic hearing tests showed the hearing loss was progressing.

By 2 years, Isabelle lost hearing in her left ear to the point where the left hearing aid no longer helped. She managed remarkably well and developed good speech skills with a single hearing aid in the right ear until 4 years of age, says her mother Vicki Labriola.

“We started to see progressive hearing loss in her right ear,” says Vicki. Isabelle’s otolaryngologist, Greg Licameli, MD, director of Boston Children’s Cochlear Implant Program, together with her care team suggested that Vicki and her husband, Jason, consider cochlear implants for Isabelle, because it was likely that she would lose all hearing in the right ear. She would then be deaf in both ears.

Because Isabelle consistently wore hearing aids from a young age and had excellent age-appropriate speech and language skills, she was an ideal candidate for cochlear implants. Her parents understood this, but they needed to get Isabelle on board with the plan.

Introducing cochlear implants

Vicki and Jason introduced the idea to Isabelle and began preparing her for the change by reading the book “Abby Gets a Cochlear Implant.” They also started the preliminary evaluation process.  Sarah Thomas, MHA, coordinator of the Cochlear Implant Program, helped Vicki and Jason to schedule the necessary appointments.

Isabelle-IDenise Fournier Eng, MA, CCC-SLP, a speech/language pathologist in the Deaf and Hard of Hearing Program, evaluated Isabelle’s speech and language skills. Jennifer Harris, AuD, CISC, an audiologist on the Cochlear Implant Team, began presenting options for various devices. Terrell Clark, PhD, pediatric psychologist and director of the Deaf and Hard of Hearing Program, began preparing Isabelle and her family for the possibility that hearing aids might not always meet her communication needs. Licamelireviewed the fine details of the surgery, and he made sure that Isabelle had all the required medical testing to ensure that she was a good candidate from a medical standpoint.

When asked what she thought about getting cochlear implants, Isabelle balked, and Vicki and Jason didn’t push the matter too much.

A sudden turn of events

Everything changed when Isabelle bumped her head in a sledding accident on Dec. 28, 2013. “In less than half an hour, it was clear that she wasn’t hearing,” recalls Vicki. She rushed Isabelle to Boston Children’s emergency room, where the on-call audiologist tested Isabelle’s hearing and confirmed what they feared: bilateral profound hearing loss. The on-call otolaryngologist administered steroids to attempt to reverse the loss.  Over the next two weeks, Edwards monitored Isabelle’s hearing, but unfortunately her hearing did not return.

Isabelle’s frustration was sky-high. Although she had decent speech reading skills, her family rarely used sign language and at age 5, Isabelle was just beginning to learn to read. They suddenly found themselves with limited ways to communicate with their daughter. “It was clear that we needed to go forward with implants as soon as possible,” recalls Vicki.

Isabella-IIIThe family relied on visual cues and pictures to help Isabelle understand about what had happened, the upcoming surgery and the gradual process of learning to hear through the cochlear implants.

Isabelle’s team already had determined that she was a good candidate for cochlear implantation, her surgery could be scheduled without delay. Licameli performed bilateral cochlear implant surgery on Jan. 16, 2014.

When it all “clicked”

“She fully expected to hear when they took her surgical bandages off,” says Vicki. But the sense of sound is not stimulated until after healing has begun and the devices are activated, which for Isabelle was still another three weeks later. Even then, it takes time, as well as ongoing and appropriate auditory therapy, to begin understanding speech through a cochlear implant.

Isabell-IIAfter the initial activation of Isabelle’s cochlear implants in early February, Isabelle started hearing something. She tapped her water bottle and scraped her boots on the ground. “On the third day, something clicked in her brain. It was clear she was understanding language,” says Vicki.

During her second appointment with the audiologist, Isabelle snuggled on Vicki’s lap and sailed through the tasks needed for programming the devices. During that appointment, Harris continued to help Vicki and Jason learn to use and care for the cochlear implant equipment.

“Boston Children’s Cochlear Implant Program makes sure the family is involved in the whole process, which is really wonderful especially at Isabelle’s age. This is a lifelong relationship—not just a surgery. It’s the continued follow-up, guidance and support that you need through the whole process,” says Vicki

Isabelle’s journey is far from over; in fact, this part of her journey is just beginning. Her Boston Children’s team will follow her through childhood to help maximize her success in every aspect of her communication.