Implanted electrodes offer hope to hearing-impaired children

From: Virginian Pilot - Norfolk,VA,USA - Jun 13, 2004

Associated Press

Madalyn Hopkins’ favorite stance is with her hands on her hips.

The 4-year-old leans slightly forward, as though about to stride confidently into the world, scold her twin brother or reorder those Barbie dresses for the millionth time in her cool new pink closet.

Two pigtails are high on her head, brushing down across a neat network of scars above her ears – scars reminiscent of the lightning bolt on the forehead of the magical book character Harry Potter. They’re the mark of someone who’s special in a way she doesn’t even understand yet.

Someone who has taken the lead in the plucky way of the young and imperfect. And in so doing, pulled along anyone who happens down the same path.

First, her brother, whom she beat into the world by a solid minute.

Second, her parents, Megan and Paul Hopkins, a couple in their early 30s who’ve barely stayed in step with the vibrant little girl who holds her tongue in the corner of her mouth when concentrating.

And finally, a committed band of hearing specialists at Children’s Hospital of The King’s Daughters, who ventured out on the edge of a pioneering effort with the sandy-haired, blue-eyed girl.

Without even knowing it yet, Madalyn has become a scout of sorts for the 3 in 1,000 children born every year in this country with hearing impairments and for the 10,000 children who have turned to electronic devices called cochlear implants as a way out of silence.

The Williamsburg girl is quiet, somewhat shy. She goes sure-footedly on her way, looking at newcomers askance for just a second, checking them out, then running on with a spirit that says: “You comin’ or not?”

In the beginning, it was her brother, Jarrett, who was hot on her heels. Right from the beginning, they’d had quite a ride, born very early, weighing 3 pounds a piece, and finding themselves in the scary neonatal world of preemies.

But they passed all the important milestones, all the screening tests. They went from the monitor-beeping hospital unit to the warm, relaxed light of home. For a moment, anyway, their parents – both schoolteachers – breathed easy.

In baby pictures, the twins have their arms hooked around each other in their sleep. They toddled in tandem. Danced together to “Blue’s Clues” songs. Chased after balls in the family den. Laughed at their parents’ tickles.

About the time they were 15 months old, though, Madalyn began to veer slightly onto a different path.

Jarrett would answer to his name. Madalyn would keep right on playing at the sound of hers.

Jarrett said “up” and “ball” and a handful of other words.

Madalyn said one word, “Mama.”

Soon, she stopped saying that.

Since the twins each had passed newborn hearing tests, the Hopkinses thought their hearing was fine. When Madalyn was 2, though, they revisited the issue.

Madalyn failed the test. Completely.

She was profoundly deaf.

Her parents decided to have Jarrett’s hearing tested, too, and found he had a moderate loss in one ear, a severe one in the other. Megan quit her job teaching English, knowing the twins would need her at home. Paul continued his job teaching history at Hampton Roads Academy in Newport News, wondering what would come next for his family.

Both children were fitted with hearing aids. Jarrett thrived with his. His vocabulary multiplied. He paid closer attention. He insisted on having his aids on at all times to pick up what he struggled to hear before.

Madalyn peeled hers off and tossed them aside.

While Jarrett was learning more words, Madalyn remained silent. She learned some sign language, cradling her arms to ask for her doll.

Her parents worried Madalyn’s silence would shrink the world she moved in outside their home on a quiet cul-de-sac near Kingsmill resort. They fretted about her limitations without realizing that she would be pulling them into a larger world, on an adventure filled with new technology and research, set against a backdrop of the science of the brain – specifically, Madalyn’s.

That journey would draw them past the tried and true and onto a new frontier. The Hopkinses knew their spirited young girl wanted more, knew she ran to keep up with Jarrett, not lag behind him, in that “Whatever you can do, I can do better” nature of twins.

Which is why Madalyn next stood at the door of Dr. Barry Strasnick at Eastern Virginia Medical School.

In Hampton Roads, he’s the go-to guy for a cochlear implant, the next step the Hopkinses had decided to take for Madalyn.

For Strasnick, professor and chairman of the otolaryngology department at EVMS, implanting a cochlear device was nothing new. He’d been doing the procedure since it was first approved for children by the Food and Drug Administration in 1990.

At that time, it was controversial, with some deaf people saying it was unfair to put implants in children too young to decide for themselves. Others, particularly the hearing parents of deaf children, saw the device as a way out of silence. Studies were showing that the earlier a child receives an implant, the better the chance of normal language and speech.

The procedure, which costs $40,000 to $50,000, works with electrodes placed inside the spirals of a child’s cochlea, the inner ear structure containing the hearing nerve. An external microphone picks up sounds, and a speech processor converts the sound into electrical signals. Those signals are transmitted to the electrodes via a device that connects the processor to the inner coil and stimulates the hearing nerve fibers. Those fibers deliver messages to the brain to interpret into sound.

Strasnick, an upbeat but straightforward man in his mid-40s, has witnessed the wonder of what implants can do. In some cases, they have helped children go to regular classes, speak clearly with others, hear well enough to talk on the telephone. One of his early patients, who received an implant at age 2, is now 10 and hears well enough to dance ballet.

The success stories led him to launch a drive to require hearing screening of newborns in Virginia. That screening began in 1999, and Virginia is now one of 38 states to have mandatory tests.

Strasnick ’s next battle is to make sure that, once identified, children get the services they need . They don’t always, because of a lack of money or insurance.

Or sometimes they don’t have the proper follow-up in terms of speech therapy or efforts to make sure an implant works. Madalyn’s parents, being teachers with insurance, were in a good position to make sure that happened.

In July 2002, Strasnick implanted a cochlear device in Madalyn’s left ear, making her one of some 10,000 children in the country to have an implant and one of more than 100 to be implanted at CHKD since the early 1990s.

A month would pass before the device was turned on. A month before she had her first inkling of what Jarrett had that she didn’t. A month before her brain picked up the sound of Claire Jacobson: “Madalyn, can you hear me?”

Jacobson, on this day in August 2002, wore a rhinestone-studded tiara in honor of Madalyn’s love of Cinderella. As the CHKD audiologist on the cochlear-implant team, she’s the one who turns the implant on and adjusts frequencies to meet the child’s needs. Jacobson always starts out with very low stimulation in the beginning because the sensation is completely foreign – and sometimes frightening – to the child .

Madalyn was seated in her mother’s lap, not understanding the day’s significance. Jacobson gradually increased the stimulation until Madalyn heard a beeping sound.

Madalyn’s face had a puzzled look as she forgot the toys she was playing with and climbed higher in her mother’s lap, pushing her head against her mother’s stomach.

“Madalyn, ” her mother said. “Can you hear us? Look, Claire is wearing a tiara.” “Maddy, can you hear me?” Madalyn’s father said, looking at his daughter’s face.

Madalyn looked sideways from the safety of her mother’s lap. Her eyes teared up. She buried her face deeper into her mother.

Her parents and Jacobson spoke in soft voices, but there was another sound in the room, one getting louder and louder and higher and higher and more and more hysterical.

One day her parents would explain. It was the sound of Madalyn’s own cry.

The Hopkinses were struggling with understanding what was going on in Madalyn’s head. They had talked with adult implant recipients who described the sensation as incomprehensible electronic beeps at first, then hard-to-distinguish voices as the brain makes sense of the stimulation.

One person described voices as sounding like Donald Duck talking under water. In the days and weeks to come, the Hopkinses would find that Madalyn’s adaption to this new dimension in her life would be tough. Jarrett’s hearing aids amplified sound, but Madalyn’s brain now had to translate electrical impulses into sense.

Her parents talked with her constantly. Megan practically narrated the day, talking in the car, in the grocery store and at home. If Madalyn would use sign language at home, Megan would respond: “What? I can’t hear you.”

At night, Megan would go to bed exhausted. Breakthrough moments began to surface. One day, about a month after the implant was turned on, Madalyn was in the kitchen with her grandmother when she heard a certain distinctive tone. She followed the noise to the piano, where Jarrett was banging away.

Madalyn also began babbling during the next few weeks after that, then saying words in the coming months, then putting them together. At a once-a-week therapy sessions, CHKD speech therapist Susan Adam would teach her listening and speech skills.

“Can you find the pink marker, Madalyn? ” Adam said, covering her mouth, so Madalyn couldn’t watch her lips. “Not the purple one, the pink one.”

Madalyn covered her mouth, too, and answered back, “Pink one.”

Adam reported back to Jacobson which sounds Madalyn wasn’t getting, and Jacobson adjusted the frequency at monthly “mappings.”

At bedtime, Madalyn soon begged to have her implant, which she called her “ear,” left on instead of turned off to conserve battery power.

It would not be long before she would pose a question that would pull her parents to the next leg of the journey. Jarrett had two ears.

Why didn’t she?

For Madalyn, it was a practical question. She and her brother each took gadgets off their ears for bath and bedtime. Jarrett had two; Madalyn, one.

Megan had long wondered why hearing-impaired children received only one cochlear implant instead of two.

What she learned was that typically, only one ear is given an implant. The procedure’s cost is one reason. And the operation has some risks, as any surgery does.

Doctors have preferred to “save” one ear for future advancements. Technology is constantly evolving. The processor has gotten smaller over the past decade, for instance. A new device is being developed in which the external part is implanted under the skin . Research is even under way to regrow hair cells in the ear to restore hearing.

Since the cochlea and inner ear don’t grow like the outer ear, there has been no reason to replace a device once implanted. Megan was willing to go along with the mainstream thinking – to hold back an ear for her daughter – until she attended a conference in Washington in April 2003.

A German surgeon named Dr. Joachim Muller spoke there about “bilateral implants,” in which cochlear devices are placed in both ears. Muller had been performing such implants in Germany. He also had published studies showing that the implants improved ability to figure out the direction sound was coming from and also helped wearers better distinguish speech in noisy settings.

The studies were small, but the research looked promising. Nature provided two ears, so it made sense that two implants would create more natural hearing.

A key question yet to be answered was whether the second implant improved hearing and speech significantly enough to be worth the extra expense in surgery.

The evidence from clinical trials that were under way would nail that down and help determine whether insurance companies would agree to cover a second implant. Muller’s theory is that the earlier two implants are used, the better. The brain has a unique plasticity in young children, when new pathways best form. If you wait too long, that advantage is lost as the brain becomes less malleable.

He felt so strongly that time was of the essence, he offered to do the surgery for Madalyn, who was now 3 years old, if she could travel to Germany.

“He made so many compelling arguments, I left feeling guilty,” Megan said. “I felt as parents, it was something we had to do.” Paul initially was reluctant. What if something went wrong? He wanted more research results.

But the couple was also well aware that Madalyn, right now, was at a crossroads. The most critical window of opportunity for language and brain development is birth to five years of age.

As teachers, the Hopkinses had known that theoretically for years; as the parents of a deaf child, that concept was unfolding before their eyes with heart-stirring urgency.

Madalyn had passed more than halfway through that small window.

The couple turned to Strasnick. He had been following the dual-implant idea. But since clinical trials in children were just beginning in the United States, he hadn’t felt there was enough evidence to move forward.

At the Hopkinses’ request, he looked further into the matter. He was impressed by the early studies and became confident that the second procedure would be no less safe than the first.

Strasnick said he would do it. “He took a chance on us,” Megan said.

When the Hopkins family found out their insurance company would cover the surgery, the last hurdle was removed.

On Jan. 26, Strasnick placed a cochlear implant in the right ear of Madalyn, who was now 4, making her the first bilateral implant recipient at CHKD and one of about 80 children in the country to have two implants instead of one.

One day in April, Madalyn sat on her dad’s lap in a CHKD sound booth and blew bubbles until she heard the voice of Jacobson through a microphone in the corner.

“Madalyn, ” Jacobson said. “Are you too busy blowing bubbles to listen?”

Madalyn turned her eyes to the corner where the voice was coming from.

“Which side am I on now?” Jacobson asked, her voice moving to the other corner. Madalyn turned her eyes toward the sound of the voice, then returned to her bubbles.

This slight movement – not even a head turn, just an eye shift – may not look dramatic to the casual observer. But to Madalyn’s parents and the specialists who have been working with her, it shows that Madalyn can place where sound is coming from, giving her a better sense of her place in the world.

Adjusting to the second implant has been hard work. When it was turned on in February, she heard the electronic-type sounds again as a new part of her brain began to make sense of the stimulation. Jacobson slowly turned up the intensity, and Madalyn’s eyes welled with tears.

“Off!” Madalyn yelped.

But by the end of the session, she was on the phone – a difficult task for people who can’t hear well – with Strasnick to deliver a message: “Thank you for my two ears.”

Comfortable with the old one, she struggled during the next few weeks and months with the new implant and reverted somewhat to sign language. At times, she wanted the security of just the old ear.

But she also liked showing off the new one to friends and family, because now she had two ears, just like Jarrett.

So far, the results have been gratifying. Her speech is getting clearer, and she’s growing more confident.

It’s hard to separate her normal development from the added help of the second implant, but Jacobson, who tracks her progress at monthly visits, is convinced of the advantage.

“I’d love to go back and have all the kids implanted with bilaterals,” Jacobson said. “So many are doing well with one, but with two, we make the world clearer, crisper.”

Now, Madalyn wants both implants turned on, even in her ladybug-decorated bed.

Two years of wearing hearing aids have helped Jarrett nearly catch up to his peers. Madalyn is about a year behind in what she understands and a little more than that in what she can say. “She’s so competitive, she doesn’t want him to learn something she doesn’t know,” Megan said.

Madalyn’s hands-on-her-hips “You comin’ or not?” question will be answered in time. Clinical trials of bilateral implants are still under way, some being done on both ears simultaneously. And Strasnick performed his second bilateral implant on a local child last week.

Meanwhile, in the preschool class that the twins attend at the Williamsburg-James City County school district, Jarrett keeps up a steady chatter with his classmates, while Madalyn quietly goes her own way. He’s good at anything with balls and running, and she’s better with fine motor skills like writing her name and beading a necklace.

Madalyn occasionally looks to Jarrett for cues during class if she doesn’t get all the words in a song. But she also keeps Jarrett in line. When she thinks he’s talking too much, she puts her hands on her hips, turns and says, “Quiet!”

After preschool, Madalyn’s mom helped her sort out why she prefers having two implants.

“Why do you like two ears – is it better?” Megan asked.

“Jarrett,” Madalyn answered.

“Jarrett has two ears, doesn’t he?” Megan said.

Madalyn nodded.

“How many does Madalyn have?”

The girl held up two fingers.

Before Megan could say, “What? I can’t hear you,” Madalyn jumped in with a quiet two-word sentence: “Two ears.”

“Right,” her mother said. “Madalyn has two ears.”

Reach Elizabeth Simpson at 446-2635 or elizabeth.simpson@pilotonline.com

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