Girl undergoes brain surgery to reverse total hearing loss
Katherine Mansfield/Observer-Reporter
Washington residents Anusha and her mother Jena Oberg pose for a recent picture at Little Lake Theatre.
Katherine Mansfield/Observer-Reporter
Anusha Oberg was born deaf in India. She recently underwent a surgery that has allowed her to begin to hear through electrodes implanted in her brain.
The brain surgery was successful.
Then five-year-old Anusha Oberg, doctors informed her mother, Jena, was heading to post-op to recover from the six-hour brain surgery, during which an auditory implant was attached to the little girl’s brainstem.
She would need to spend a week recovering at St. Louis Children’s Hospital and another three weeks healing at home in Washington, before returning to Missouri, where an audiologist would turn on 22 electrodes and Anusha would, hopefully, hear for the first time.
Anusha was born, deaf, in India. She was adopted by Jena Oberg last year.
Jena began the lengthy adoption process in 2019, a process that required approval from both the United States and Indian governments. After being approved by the United States, she received a list of children from an orphanage overseas.
The list contained only those children with disabilities.
“I saw Anusha’s face. I said, that’s my daughter. I just knew it. I knew she was supposed to come home,” Jena said.
Jena’s family has no history of hearing disability, but Jena, the artistic director at Little Lake Theatre who recently secured a partnership with the Western Pennsylvania School for the Deaf and state funding to produce a play starring an all-deaf cast, has a big heart for the deaf and hearing disabled.
“I learned (American Sign Language) in college,” she said. “A play that I directed, we had a deaf actress. I learned ASL then. It’s very bizarre and I can’t explain it – I think the universe was preparing me to be her mama. I always felt that I would have a deaf child. I just have always felt that that was going to happen.”
When Jena and Anusha were matched for adoption, Jena immediately began preparing for life with a deaf child. She scheduled assessments and toured schools, settling on the Western Pennsylvania School for the Deaf.
After spending three weeks in India, during which time the new mother and daughter bonded and adoption papers were signed, Jena and Anusha headed home to the United States. At the last minute, they changed their flight plans from a layover in China to a direct flight from India to New York, because Jena was unsure how Anusha would take to flying.
“The week we got home from India is the first week they found the first cases of COVID in Wuhan,” said Jena. “We would have been in China.”
At home, Anusha and Jena adapted to life together, and Anusha underwent several assessments.
“We knew she had hearing loss and that she was deaf, but not to what extent. We didn’t know what had caused the deafness,” said Jena. “Early intervention recommended placement for therapy, for school. We got our physical therapy, occupational therapy and speech therapy recommendations.”
Jena noticed how verbally motivated her daughter was and wanted Anusha to have the option of hearing.
“She would try to talk. She had pitch, she had range to her voice,” Jena said.
So she looked into cochlear implants, which are small, electronic devices that stimulate the ear’s hearing nerve. According to Johns Hopkins University, a cochlear implant improves hearing or restores hearing loss.
“Everyone was sure that she was going to be a great candidate for a cochlear implant. Here’s a kid who’s a shoe-in for a cochlear implant,” said Jena.
Anusha responded to hearing aids and the date for her cochlear implant was set. Shortly before her surgery date, however, Jena received a phone call.
“We got a call from the surgeon. He said, ‘You aren’t going to believe this, and I can’t believe what we saw. She is not a candidate,'” Jena said, admitting the news came as a shock. “She does not have cochleas. She’s missing both cochlear pieces. When I say it’s rare – they have about 100 kids on record since the 1800s with a complete absence of all four pieces.”
She spent days trying to process her daughter’s diagnosis of Michel’s Aplasia.
“It had seemed like everyone was so certain (the cochlear implant) was going to work. All of a sudden it was like, how do I support my child?” Jena said. “I spent several weeks saying, all right, what do we do? Is it even worth pursuing another step?”
Jena decided to pursue a next step because she wanted her daughter to be able to navigate a hearing world.
“I just wanted her to have awareness for sound, really for safety reasons,” Jena said. “I just wanted her to be able to say ‘help’ or ‘stop.’ I want her to be able to hear if a car is approaching, someone is nearby, have the ability to develop some speech to draw attention.”
A surgeon suggested Jena look into an experimental hearing treatment called an auditory brainstem implant. An ABI bypasses the inner ear — the cochlea — and stimulates the auditory nerve directly. The nerve sends signals to the brain, which transforms those signals into sound.
Most ABI candidates undergo surgery because a tumor has formed on their cochlea, and they are no longer cochlear candidates.
“They started using it on children who had severe to profound hearing loss, who were not cochlear candidates, probably within the last 10 years,” said Jena. “They’ve really only implanted one- to two hundred kids worldwide who do not have this tumor. She was five, so we had to make a decision pretty quickly — she was already considered geriatric (for the implant).”
Near the beginning of September 2020, the Obergs headed west to Children’s Hospital in St. Louis, one of five hospitals in the nation that performs ABI brain surgery. After meeting with the surgeon and staff, Jena felt an ABI was right for her daughter.
Surgery was scheduled. As the date approached, nerves set in.
“Your mamma fear senses kick in. They’re cutting open your child’s head. They have to go over all the ‘Big bads’ — risk of spinal fluid leaking, the risk of brain bleeds, all the things that could go wrong,” said Jena. “If the paddle isn’t placed correctly, it can … cause heart or breathing problems. (Anusha wasn’t) able, really, to be involved in the decision-making process in a way that can be informed. Am I doing the right thing?”
Anusha’s surgeon assured the family that Children’s Hospital staff knew “the roadmap very well,” and their professionalism and experience helped ease Jena’s fears.
The operation went smoothly. After a full recovery at home in Washington, Jena and Anusha made a third trip west — this time, for an audiologist to turn on Anusha’s ABI.
There was no guarantee Anusha would be able to hear, and the ABI had to be turned on in a surgical setting in case an electrode had slipped and stimulated the wrong part of the little girl’s body.
“They turn on each electrode — there are 22 electrodes, one of them is a ground — one electrode at a time, at a low level,” Jena said.
The surgery is considered a success if the patient responds to between nine and 11 electrodes.
“We ended up getting a response on all 21 electrodes,” said Jena. “She actually really liked it. We got a huge smile when she heard the first beep. She looked up with big, wide eyes. She had never heard before. She didn’t know that there was … sound.”
Anusha has spent the last year discovering sound, figuring out the optimal noise level for her ABI and working on speech.
“Last time we programmed (the ABI) in St. Louis, her audiologist turned on the electrodes and didn’t prepare Anusha. All of a sudden, she looked up and pointed to her ear and said, ‘beep, beep, beep,'” Jena said.
Anusha, Jena explained, doesn’t process sound the way those without hearing disability hear, and there is no certainty she’ll ever be able to fully understand spoken language.
“I like to think of it a little like Morse code. It’s coming in in beeps, different frequencies of beeps that she has to identify,” Jena said. “It’s a whole different mechanic of evening having sound impulse.”
But Anusha, a sassy, imaginative six-year-old who loves playing, dancing and admiring the holiday show lights at Phipps Conservatory in Pittsburgh, is making progress.
“We aren’t sure to what extent she’s going to hear or be able to use sound. She may get a combination of reading lips and identifying sounds,” said Jena, who communicates with her daughter primarily through ASL. “She’s starting to match sound. If you say, ‘Moo’ behind her, recently she’s been turning to the sound and copying the sound ‘moo.’ But she’s only doing that for a few select sounds right now.”
Jena said Anusha has to choose sound awareness, and though her progress is remarkable — Anusha is a fast learner and a diligent student — she still has a long road ahead on her hearing journey.
“We’re still just identifying the difference between quiet and loud noises,” Jena said.
Anusha doesn’t wear the exterior portion of her ABI all of the time. Jena said they take it out at night, and sometimes her daughter simply does not want to hear.
If Anusha opts not to hear as she gets older, Jena said she will fully support her daughter’s decision, but for now, Anusha said — in American Sign Language — that she enjoys the sounds around her.
“We’ve had a very special bond since day one,” said Jena. “(The ABI has) given us more tools to communicate, which is good, and I think it’s given her some more tools to understand the world around her. She has the most brilliant imagination. I don’t ever want her to think that in order for us to feel she’s successful or whole she has to hear.”