Ellie Pokrzywinski is like any other toddler.
She cruises around the cul-de-sac of her Fargo, North Dakota, neighborhood on her scooter as fast as she can. She’ll play hockey in her toy room. She loves playing notes on her two-times-as-big-as-she-is piano mat that she can walk over.
But when she was younger, her family didn’t know if she’d ever hear the notes she so excitedly plays.
First hearing screenings
Tara Pokrzywinski is Ellie’s mother. She said everything about her pregnancy, labor and delivery with Ellie was normal. She was born healthy.
“When we were getting ready to be discharged from the hospital at 24 hours, we had learned she failed her newborn hearing screening. We didn’t think much of it. I know it’s a fairly common thing with fluid in the ears and stuff,” she said.
At a follow-up appointment a few weeks later, Ellie failed another hearing screening. Tara and her husband were referred to Kayla McClellan, an audiologist at Sanford Health in Fargo, by Ellie’s pediatric provider.
McClellan conducted an auditory brainstem response (ABR) on Ellie, which indicates how well the inner ear, or cochlea, and the brain pathways are working together.
The logistics of an ABR for a newborn:
- Bring the child in as hungry and as tired as possible.
- Feed the child and wait for them to fall into a deep sleep.
- While sleeping, providers will test brain activity reactions to different frequencies.
- The whole test takes roughly an hour.
“An ABR is great, but it’s not an exact science given the circumstance of the baby’s sleep schedule. We were told Ellie had anywhere from mild to moderate hearing loss, to moderate to severe,” said Tara.
“We got her fitted with hearing aids and continued to monitor her hearing,” said McClellan.
Learn more: Hearing loss, hearing aids and preventing further damage
Ellie was still only 3 months old at this time. A sedated ABR is the most accurate form of the test, but due to Ellie’s age, providers weren’t comfortable doing the sedated form of the ABR.
They recommended Ellie come back when she was 6 to 7 months old.
“They did a sedated ABR at that time and an MRI, and they told us that day that she was profoundly deaf in both ears,” recalled Tara.
“Eventually it was indicated that hearing aids weren’t going to be very beneficial anymore,” added McClellan.
Cochlear implants were needed
Amanda Johnson is an ear, nose and throat nurse practitioner at Sanford Health in Fargo. She said it’s hard to know what specifically caused Ellie to be born deaf.
“Did she have any risk factors, like maternal infections? CMV (cytomegalovirus) is a big cause of hearing loss. Genetics cause (hearing loss) about a fourth of the time. We never do find a reason as of right now,” she said.
All of Ellie’s imaging looked normal, and there were no indications of genetics being a factor.
Ellie had an entire team of providers on her side.
“Kayla McClellan is our audiologist that worked with her closely. She also had her zero to three team, working with her speech therapist, a genetics evaluation, the MRI and CAT scan in little children often require anesthesia. So, it’s just this whole multidisciplinary team that it takes,” explained Michael Shinners, M.D., a neurotologist at Sanford Health in Fargo.
A neurotologist, if you’ve never heard that, is a doctor who specializes in diagnosing and treating disorders that affect not only the ear but also balance and the skull base.
Despite many providers working with Ellie, Pokrzywinski said there were only a few options left for Ellie.
The first was learning sign language as a family and looking at deaf communities and deaf school opportunities. The other was cochlear implants.
Dr. Shinners said cochlear implants provide a very small electrode that goes inside the cochlea.
“When you wear it, the sound coming in goes into the inner ear and directly stimulates the nerve endings. So, it bypasses the ear canal, the eardrum, the hearing bones that all normally vibrate,” he said.
“It’s not acoustic hearing anymore. It’s electric hearing.”
Related: 7-year-old’s hearing restored with cochlear implants
Dr. Shinners said it takes time for the brain to learn how to process the signal.
“We have found that when a child has very significant hearing loss, that the younger you implant them, the better they do because it’s earlier and their brain is still developing. It can use the signal better,” he said.
It’s completely different from a hearing aid, Johnson explained.
“A hearing aid can amplify the sound, which is great, and it’s what a lot of people need for various levels of hearing loss. But this works in a different modality to treat severe neural hearing loss, or profound (neural hearing loss) in Ellie’s case,” she said.
The family made the decision to try cochlear implants, and Dr. Shinners did the procedure.
Dr. Shinners said cochlear implants are a great option for a number of people, depending on their severity of hearing loss.
“It’s from kids being born with a significant hearing loss, to the whole other (end) of the spectrum of adults who have onset hearing loss and they’ve progressed over time enough that they have their hearing aids, but they just don’t help that much anymore. It’s a whole spectrum of very young children to very elderly people,” he said.
Tara said to help Ellie learn, she and her husband were “almost narrating our lives for a full year.” Ellie also continues to work with a speech therapist.
“It’s kind of training a brain that spent the first year without sound what’s happening,” Tara said.
Hearing perfectly
Two years after the procedure, Ellie had a “lengthy” hearing assessment and found out she was tracking at all development levels to that of her hearing peers.
“Just those small moments of stuff where she probably didn’t hear my voice for the first year of her life. She never heard me say ‘I love you,’ or sing her to sleep. As a parent, those are hard things to feel.
“So, the fact that now she can hear a car pulling into the driveway when she’s in the backyard or hear an airplane in the sky that she can’t see. It’s just wild. It’s been really, really fun to see,” said Pokrzywinski.
She said she couldn’t be more thankful for the all-hands-on-deck care Ellie, and she, received from her providers at Sanford.
“I think back to the initial diagnosis, I was just in an absolute state of shock. Every emotion all at once, but almost a numbness went over me. They approached it in such a way where it was like, we don’t have to talk about this today. If you need to sit with us and we need to talk tomorrow, or the day after that, or if you feel like you just need time, let’s do time.
“If you want to carve out an hour of our day to ask us a million questions, let’s do that. It was just whatever we needed, they showed up in that way.”
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Children’s, Ear, Nose & Throat, Fargo, Specialty Care