Patient Stories | Brooklyn and Pia and Sensorineural Hearing Loss

Brooklyn and Pia: From Hearing Loss to Lifelong Friends

Brooklyn Ballein and Pia Rizek are besties. As active 6-year-olds, they enjoy using their imaginations, doing gymnastics and giggling during sleepovers.

But the two friends, and their families, have something else that bonds them—both girls were diagnosed with congenital cytomegalovirus (CMV) that resulted in sensorineural hearing loss. And as patients within the Division of Audiology, Brooklyn and Pia are thriving more than ever because of early action and dedication from their parents. 

Some babies born with CMV (i.e., congenital) have no symptoms. Others, like Brooklyn and Pia, can have short-term or long-term symptoms, such as hearing loss. Unfortunately, it’s hard to tell if a baby has CMV without testing. And oftentimes, testing doesn’t happen unless a baby shows specific symptoms.

In Ohio, all babies are required to complete a universal newborn hearing screening test. In the past, a failed hearing test wouldn’t necessarily result in a child being tested for CMV, even though it’s the leading cause of non-genetic hearing loss. For a confirmed congenital CMV diagnosis, a CMV test must happen within 21 days of birth. That’s what happened for both Brooklyn and Pia.

Pia and Brooklyn’s Diagnosis Journeys

Pia failed her hearing test on day one.

“It’s a very tricky situation because a lot of newborns fail their hearing screenings,” said Pia’s mom, Britt. “Hospitals are good at saying, ‘It’s probably nothing. Don’t worry about it. Come back in six to 12 weeks to get her screened again.’ We came back much sooner than that." 

Pia’s parents quickly noticed that she wasn’t responding to any noises. They were referred to Cincinnati Children’s for another more in-depth hearing screening.

“The whole time, you have your fingers crossed that she’s not deaf,” Britt said. “But you see the computer screen, and there’s just flat lines going across where you know it’s supposed to be going up and down when they have hearing." 

Four hours later, the test confirmed that Pia had complete hearing loss in both ears.

Brooklyn’s story is similar, but different. Her parents met her when Brooklyn was just five days old as her foster parents. At the time, they didn’t know she had hearing loss. Brooklyn’s social worker cited a failed newborn hearing test, and suggested that she get re-tested. Re-testing confirmed that Brooklyn had complete hearing loss on her right side and slight hearing loss on her left side. Over time, the hearing on the left side would get worse.

Their pediatrician referred them to the Division of Audiology at Cincinnati Children’s, where Brooklyn was also diagnosed with congenital CMV, in addition to the hearing loss. 

“I was in complete denial,” Brooklyn’s mom, Megan, admitted. “When they first told us that she had hearing loss, I thought, ‘No, she doesn’t. Look, she turns and looks at me. Clearly, she can hear me.’ That newborn screening definitely changed her life.” 

Finding a Support System, Deciding on Treatment for Hearing Loss

Both Britt and Megan did a lot of research after finding out their girls’ diagnosis. They also happened to join the same Facebook group for parents of children with CMV. 

“We quickly realized that the girls had a lot of similarities,” said Megan. “It was overwhelming and scary but so nice to have a mom who has a really similar experience and similar struggles.”

Later, the families reconnected through Ohio Valley Voices, a school that specializes in teaching children who are deaf or hard of hearing to speak. They also both received support at Cincinnati Children’s. 

Lori Garland, AuD, entered the picture and became like our fairy godmother,” said Megan. “She really changed the game for us. In that moment as a first-time mom and now a mother of a child with hearing loss, it was so helpful to have someone who was like our tour guide, leading us. She helped streamline the process and was there [for us] to vent, cry, or ask questions.”

After both families were referred to Cincinnati Children’s, they were presented with options and resources for how to approach their daughters’ hearing loss. Basically, they could either manage the deafness and learn sign language or try to use technology. Each family ultimately chose what would be best for them and their lifestyles. 

Brooklyn’s family opted to use technology only, meaning they had her fitted for hearing aids at first and then went through surgery to get cochlear implants by 22 months of age. 

“It felt like a really huge decision at the time,” said Megan. “It felt like a really personal decision for her that we were making as her parents, and I wanted so badly to ask her what she wanted.”

They also enrolled Brooklyn at Ohio Valley Voices to start speech therapy.   

Pia’s family decided on a hybrid treatment approach. They opted to get Pia cochlear implants and teach her sign language. The family toured Saint Rita School for the Deaf and Ohio Valley Voices. They started going to deaf community events and taking classes in American Sign Language.

“I remember being 16 and doing everything opposite of what my mom said,” explained Britt. “So, my fear has always been that when she turned 16, she would say, ‘Mom, I’m no longer going to wear implants. I’m going to be deaf with a capital D.’ So, we sign, which is super fun.”

Getting Cochlear Implants

With cochlear implants, the first step is to get fitted for hearing aids, which is part of the process for confirming eligibility for cochlear implant surgery. A cochlear implant is a hearing device with two parts—one that’s surgically implanted on the inside of the ear and another that’s worn on the outside of the ear. These parts work together to provide sound to those with severe hearing loss who wouldn’t benefit from using a hearing aid.

Neither Brooklyn nor Pia could hear using a hearing aid. So, they each received the cochlear implant surgery. The entire surgery lasts about eight hours, with patients going home that same day. Then, in a few weeks, they return to have the device activated. 

The surgery was a success for both girls, who now see an audiologist at least once each year for monitoring and fine-tuning of their cochlear implants. As equipment ages and technology advances, they’ll receive upgraded devices about every five years. 

Understanding the Effects of Hearing Loss

Brooklyn and Pia both received the resources they needed at the appropriate times, which is a huge contributor to their successful outcomes. With most conditions, especially hearing loss and CMV, early intervention is critical. 

“The brain is at its greatest potential for development, and more specifically language acquisition, very early in life,” explained Mike Scott, AuD, who serves as the auditory implant team coordinator at Cincinnati Children’s. “From the point that an infant starts to hear and see, they are developing building blocks for language. The longer a child goes without language input, the harder it becomes to develop these skills.”

Any degree of hearing loss can have a huge impact on development. Both Pia and Brooklyn have gone through, and continue to go through, intensive speech therapy. They’ve also received treatment for other symptoms associated with CMV. Megan can’t imagine what it would be like for Brooklyn, had they not tested her early.

“We would not have discovered this until she was older or starting kindergarten in a mainstream school,” she said. “I can’t imagine how behind she would be. The fact that this was detected so early, and she’s had years of intervention already, is miraculous.”

Influencing the Future of Congenital CMV

Although both families still have struggles raising their young girls, they’re thankful they got involved while their daughters were so young. They continue to support each other and other families in their situation.

“I feel like [cochlear implants] have propelled [Brooklyn] into reaching her full potential,” said Megan. “It’s not held her back at all. She’s very determined. Now, looking back, I don’t doubt for a minute that we made the right decision.”

“I always say that [Pia’s] deafness was the best thing that ever happened to us,” Britt said. “We have a superpower, which is sign language, now, and it’s been a phenomenal thing for us as opposed to a detriment.”

Since Brooklyn and Pia were tested for CMV, there have been a lot of efforts at Cincinnati Children’s to require testing if an infant fails their hearing test. Dr. Garland credits families like Brooklyn’s and Pia’s who have shown how important this testing can be for early intervention and better outcomes.

“We can understand the issues, the laws and early intervention with CMV, but to live it is where families give back to us,” Dr. Garland said. “We get to improve what we do based on each new family’s experience. When Brooklyn and Pia were really little, even though it wasn’t that long ago, there wasn’t as much known about CMV. And now we’re pushing for more CMV testing. These families really inspired us.”

(Published March 2023)