A family’s refusal to give up on girl’s hearing – and the MUSC doctors who proved them right

*Rattle-Rattle*

*Clap-Clap*

Doctor of Audiology Amy Noxon continues making different sounds as she attempts to get the attention of Zlata Kuzmina, her almost-three-year-old patient at the MUSC Health Cochlear Implant Program at R. Keith Summey Medical Pavilion. At first, while Zlata taps away on her little rainbow xylophone, it doesn’t seem like she is hearing anything, but Noxon explains she has the settings very low. Then, suddenly, as Noxon raises the sound level, Zlata turns toward her father with wonder in her eyes. She motions to her ears. For the first time in her life, the angelic blonde is hearing sounds louder than ever before. Her new cochlear implant is doing its job and providing sound to the young girl. 

While MUSC performs over 170 cochlear implant surgeries each year, with many going to young patients like Zlata, this particular implant is attached to both a smaller-than-normal cochlear nerve and a long journey that brought Zlata and her family from Ukraine to South Carolina.

 

Zlata, a joyful and animated toddler, loves lacey dresses and neon orange socks. She skips everywhere she goes and adores her doting big brother, Filip. Her enormous blue eyes twinkle while she nibbles on her Lucky Charms. After “playing” the piano with Filip, she can’t decide if she wants to blow bubbles or help her fuzzy lamb go night-night. 

Zlata was born in Ukraine, which has been under attack by Russian forces for over a year now. Her mother, Diana, is from the Donbas region, which is where much of the fighting has been concentrated. Diana was a family physician in her homeland and enjoyed her life in Ukraine. But when Russians fought to annex Crimea back in 2014, Diana left and moved to Odesa, Ukraine. What was first a temporary move turned out to be permanent after meeting her husband.

In 2022, the family moved to the United States to join an aunt who came to the states five years prior. This turned out to be incredibly fortunate for them, as the years-long immigration process had only just been finalized, months before these most recent attacks started. 

“The whole country is in war now, but in the Donbas region, war started in 2014, and that’s why we started this process – because we couldn’t come back to Donbas because we feel a big difference between Ukraine and Russia,” Diana said.

After stops in the Czech Republic, Germany and New York, the family finally settled down in Boiling Springs, South Carolina. But prior to moving to the United States, Diana noticed that Zlata wasn’t speaking or reacting to sound as her mother might expect. Once in the states, they had an ear, nose and throat (ENT) doctor examine her and scheduled audiology tests.

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That doctor referred them to MUSC, where Zlata underwent an MRI to check on her cochlear nerves – the nerves that run from the inner ear to the brainstem and are associated with hearing. An MRI indicated that Zlata likely had no cochlear nerve in her left ear and a very small nerve in her right ear. 

When the family met with Noxon, the audiologist, she had them sit in a sound booth to see if Zlata would react to any noises. The sounds on her left went completely unnoticed, but loud sounds on her right seemed to be a different case. “We didn't expect her to have any hearing,” Noxon said. “So when I turned on a loud sound, she startled to the sound in her right ear. And that gave us some hope that she did have some hearing.”

This was welcome confirmation for Diana, who was convinced that her daughter did indeed have some hearing. Visits to other physicians were met with disappointments, when doctor after doctor at other prestigious medical centers around the nation said there was no hope. 

“We had a few doctors in America that said there’s no chance she will hear, and the implant wouldn’t help,” Diana said. “We continued to search for other doctors, and at MUSC, Dr. Noxon and Dr. McRackan confirmed what I saw – that she reacted to sounds on her right.” 

After these tests, Noxon suggested that Zlata might be a candidate for a cochlear implant. Ted McRackan, M.D., medical director of the Cochlear Implant Program in the Department of Otolaryngology – Head and Neck Surgery, is a big believer in this procedure. It’s a surgery he’s very familiar with, as he performs as many as three a day and runs a cochlear implant research program. He also said that research suggests that people find greater benefits from the cochlear implants the earlier they’re implanted.

 

“In the United States, we are very, very proactive about getting children cochlear implants as soon as possible, based on data showing that the earlier you get implanted, the better you perform,” McRackan said. “So if a child is found to have severe or profound hearing loss at birth, the earlier they get the implant, the more likely they are to catch up with their peers at an earlier date, with regard to speech and many academic outcomes.”

When McRackan met with the family and agreed to do Zlata’s cochlear implantation procedure, an interesting connection emerged: McRackan’s great-grandmother was Ukrainian. It was a coincidence that bonded the group and gave the family an added measure of comfort.

 

The implantation, which took McRackan and his team two hours to complete, was successful. At that point, everything would ride on the activation.

Noxon mentioned that there can be some misconceptions when it comes to cochlear implants. Poignant viral videos pop up on the internet showing people appearing to gain full hearing post-activation, instantaneously perceiving the world around them in an exhilarating new way. But, she explained, those kinds of reactions are rare, and often, the new stimulus of sound is strange and scary for young children. 

 

“It's hard to predict how a child will react on the first day,” Noxon said. “We typically like to start it at a soft level so we do not scare her and then kind of turn it up slowly, looking for reactions to sounds. But reactions to sound for kids can vary from giggles to tears and anything in between.”

Even before the implant was turned on, Zlata could communicate. The whole family is learning American Sign Language, and here in the exam room, it’s how they communicate with each other. After a quick examination, Noxon escorts Zlata and her parents to her office, where she fits the processor around Zlata’s ear as the little girl plays with her new blinking wand toy. At first, the volume is too low, and Zlata does not react. As the volume increases, Zlata reacts the way Diana hoped she would … with surprise and joy written all over her face. 

 

As Zlata looks around to see where the sound is coming from, she lifts her hands inquisitively. As Noxon continues to adjust the implant, she explains that newly implanted deaf children don’t have any assigned meaning to sounds – more often, they pay attention to some sounds and completely ignore others. 

Everyone is grateful for Zlata’s results and incredibly optimistic for her future.

 

“She already has language through American Sign Language, so it’s just in understanding spoken language she will need to progress and develop,” Noxon explains. “She has such a great support system so I know she will be successful in her own way.”

And Diana has high hopes for her daughter. “I pray to God that she will hear. That she will speak. And also, I pray that she will sing. All our family sings. I hope she will sing with us in her own voice."