One Chance to Hear

Two-month-old Eleanor Nolen was up against the clock. Bacterial meningitis was turning her inner ear to bone. She had lost all hearing. Le Bonheur Otolaryngologist Joshua Wood, MD, knew that if Eleanor was going to have a chance to hear again, he would need to conduct cochlear implant surgery before time ran out.

But the FDA-approved age for cochlear implants is 9 months. And no documented patients younger than 3 months had ever undergone the surgery. Wood, with Eleanor’s parents, Jacob and Rachel, had a decision to make.

“Once the ossification process starts, it’s a time-sensitive situation,” said Wood. “Many places wouldn’t attempt cochlear implants this young, but that would mean she would be totally deaf her entire life with no later opportunity for cochlear implants. We knew we had the skill and training to change that for Eleanor.”

Thanks to the expert team at Le Bonheur Children’s, Eleanor became the youngest child ever documented to receive cochlear implants.

First Alarm

After celebrating Eleanor’s first Christmas with their families, Jacob and Rachel noticed that their daughter was lethargic and unresponsive. Their pediatrician in Jonesboro, Ark., sent them to Le Bonheur, just to be on the safe side. A spinal tap revealed that Eleanor had meningitis.

At first, the Nolens felt fortunate — Eleanor’s bacterial meningitis was diagnosed early.

But events began to snowball for Eleanor. Her hospital stay stretched to 27 days. She developed a fever of 102 degrees. She had two strokes. And then, a hearing test revealed that she was completely deaf.

“We were having breakfast and drinking coffee, and the audiologist said she had bad news. She wasn’t picking up any signals even on the highest pitches,” said Jacob. “It was like everything froze and took my breath away. We were in total disbelief and shock — we knew it was a common side effect but didn’t think it would happen to our baby.”

An MRI revealed that Eleanor’s inner ear structures were beginning to turn to bone — a condition known as labyrinthitis ossificans. The cochlear fluid was infected due to the meningitis, which was causing the ossification of those fluid-filled channels.

The clock was ticking.

“If the cochlea turns to bone, there is no way to put in a cochlear implant,” said Wood. “We had to decide if we would implant much younger than is normally done.”

Time to Act

The U.S. Food & Drug Administration (FDA) has approved cochlear implants for children as young as 9 months with “normal” hearing loss. But if Wood waited until 9 months to perform cochlear implant surgery for Eleanor, it would be too late.

Cochlear implants function by bypassing the eardrum, middle ear and, to some extent, the inner ear structures. During the surgery, electrodes are placed through the cochlea to directly stimulate the hearing nerve. But if these inner ear structures turned to bone, no surgical approach could reach the hearing nerve.

After weighing the risks, the Nolens decided that to give their daughter the best chance to hear, they needed to proceed with the surgery.

“As a parent you want what’s best for your kid. This was a tough decision as no one this young has ever  undergone this surgery,” said Jacob. “We had faith in Dr. Wood and the way he explained the process to us. We felt comfortable with him and trusted him and his opinion when he said he could successfully perform the surgery.”

On Jan. 21, 2022, Eleanor Nolen became the youngest documented child to undergo cochlear implant surgery.

“For an infant, the approach for cochlear implant surgery is a little different than traditional surgery, but Eleanor’s surgery went smoothly with no issues,” said Wood. “Without this surgery she would be deaf for her entire life. It’s great that we were able to successfully do the surgery, but it’s even more exciting to think about what this means for Eleanor for the rest of her life.”

Even after a successful surgery, the Nolens still had to wait several long weeks to activate the implants to see if Eleanor could hear their voices again.

Moment of Truth

Three weeks later it was time to find out — did the cochlear implants work for a child as young as Eleanor?

Eleanor’s case was unique in that she could hear for the first month of her life and then lost all hearing. Normally, cochlear implants are placed in a child who has never heard in their life. The implant activation process can be startling.

On Feb. 11, 2022, Jacob and Rachel watched their daughter’s cochlear implant processors light up as she turned her head to the sound of their voices for the first time in weeks.

“Activation day was amazing. We were so excited to see  Dr. Wood again and get started on Eleanor’s hearing,” said Rachel. “We were wearing masks, so the fact that she turned and tracked my voice was incredible.”

And now the Nolens are enjoying introducing Eleanor to sound all over again, from the lilt of their voices to the barks of the family dog.

The Future

Eleanor will continue to meet with a Le Bonheur audiologist long-term to adjust her implants as needed so she can better hear the world around her. In the meantime, Eleanor’s case opens the door for other children who need cochlear implants at a young age.

“The fact that we performed this surgery successfully goes a little bit against traditional teaching of when is ‘too young’ to get cochlear implants,” said Wood. “It shows that this is a possibility for very young children.”

Wood believes that Eleanor’s long term prognosis for getting cochlear implants even at this young age will have a great outcome. She’ll go to the appropriate speech therapies and should reach the same milestones in speech as other children her age. Rachel and Jacob are looking forward to what their daughter’s future will look like, pursuing her passions and doing everything another child her age would.

“We’re so thankful for the cochlear implants, and we want her to be proud of those — not letting them stop her from doing certain things because she’s technically deaf,” said Rachel. “Our plan is to let her find her passions and support her 100%.”