Children born with severe hearing loss who can't or don't benefit from a hearing aid face an added challenge.
The brain starts processing sound at birth. Without this arousal, structures in the brain fail to develop in the best way. This can make it harder to acquire language and lead to a host of cognitive and social problems.
And then there’s Oliver Gruber.
“Oliver gets to be a beautiful boy and enjoy all the activities a 3-year-old should,” says David Chi, MD, Oliver’s doctor. “We expect that throughout his life, hearing will not hold him back from anything he wants to do.”
That’s thanks to cochlear implants, a loving family, and his care team at UPMC Children’s Hospital of Pittsburgh.
The Challenge: Auditory Neuropathy
Oliver was born near Detroit in October 2014 and adopted at birth by Michele and Jonathan Gruber of Hampton.
Although he had passed his newborn hearing screening in Michigan, his parents soon noticed something was wrong. Oliver didn’t seem to respond to the sounds of their voices or other stimuli.
Mrs. Gruber says that it was most noticeable because of their son Emeric. Emeric — whom they lost to Tay-Sachs disease on May 2, 2014 — had an increased sensitivity to sound as part of the disease.
“If I were to drop a pan or something,” she says, “I’d look at Oliver, and he was just oblivious.”
In January 2016, the Gruber’s brought 14-month-old Oliver to the Hearing Center at UPMC Children’s Hospital for testing.
A brainstem response test confirmed that Oliver had auditory neuropathy — a type of hearing loss often not helped by hearing aids. Luckily, these days, many kids with hearing loss have another option.
The Solution: Cochlear Implants
“Hearing aids still use the ear,” says Dr. Chi. “But cochlear implant devices do not. They bypass the inner ear and stimulate the nerve that sends sound to the brain directly.”
Doctors regard cochlear implants as the first electronic device to restore a human sense.
During a cochlear implant, the surgeon:
- Opens up the bone behind the ear.
- Finds the cochlea and makes an opening there.
- Places an electrode within the cochlea and closes it back up.
The implant works in tandem with an external device that receives and processes sounds. It then sends the sound through the skin to the implant.
Oliver’s family and health care team chose to give him two cochlear implant devices. Dr. Chi did the first implant on the right side in May 2016, and the second on the left in December 2016.
After turning the devices on, there was an a-ha moment as Oliver heard sound for the first time. And, there have been many wonderful moments since then.
“We were in the car after a trip to Disney,” says Mrs. Gruber. “Oliver kept saying ‘up, up, up, down’ and making the sign for a boat with his hand. Then he said, ‘Arrgggh!’ and I realized he was talking about the pirate ride. That was the first time I felt like he was telling me a story.”
The Result: Making Great Strides
Oliver goes to the preschool program at Pittsburgh’s DePaul School for Hearing and Speech and returns to UPMC Children’s for check-ups.
Oliver's audiologist, Rena Levy, AuD, fine-tunes his implants. His speech and language therapist, Jennifer Rakers, MSLP, works with him to keep on improving his skills.
He’s making great strides.
“In the past, someone with a cochlear implant wouldn’t be able to go swimming. But now there are covers that make them waterproof,” says Dr. Chi. “There are even attachments that use Bluetooth technology to let kids listen to music like other kids.”
Oliver is on track to progress just like his peers, which includes giggling at noises the human body can make.
Mrs. Gruber recalls one night at dinner when she got the hiccups.
“Oliver thought it was hilarious, and now he imitates me. If he hadn’t been able to hear, he would just see my body jerking, and it might be scary. The sound is what makes it funny. It’s just one of the little things he’d miss out on.
“Oliver would still have a full and happy life without hearing — but it’s pretty amazing with it,” she says.
About David Chi, MD
Dr. Chi is chief of the Division of Pediatric Otolaryngology and director of the Hearing Center at UPMC Children’s.
He also serves as vice-chair for the Pa. Department of Health’s Newborn Hearing Screening Program.