Two sons born without hearing get the gift of sound

From: Baltimore Sun - Oct 3, 2012

The Duick family turned to GBMC for double cochlear implants for both sons

By JIll Rosen, The Baltimore Sun

Shannon Duick brushed away tears as a technician fixed electrodes onto the head of her infant son, but as a machine sent beeps and clicks into his ears, the baby slept in her arms, unaware.

He slept as the machine came up empty, as it showed his brain registering none of those sounds and as they moved to a quieter room to try again. He slept as they took the clock down from the wall in case the ticking was interfering. He slept as they tried a different room, farther away from the noisy elevator.

With each beep, each click, Duick's anxiety grew. The tech tried again and again, over and over for several hours, to detect a positive sign. And finally, somehow, he saw whatever flicker on the monitor he needed.

"We left that hospital under the assumption that our son can hear," Shannon Duick recalls. "I remember that sigh of relief, that exhale."

It would be months before they figured out that Zachary was profoundly deaf. He couldn't hear beeps. He couldn't hear clicks. If a jet landed right next to him, he wouldn't even turn his head at the noise.

Zachary would become Greater Baltimore Medical Center's first patient to have cochlear implants placed in both ears.

The Food and Drug Administration approved implants for adults in 1985, and children have been getting them since 1990.

Putting implants in both ears is slowly becoming more common. Experts say being able to listen with two ears can help children better understand speech and make it easier to determine where sounds are coming from. Since Zachary got his first implant in 2007 and the other side in 2008, GBMC has done only 34 more double implants.

And one of those was Zachary's younger brother.

The birth of their first child was a long-anticipated landmark for Bill and Shannon Duick. The York, Pa., couple relished decorating a nursery and filling it with toys that rattled and squeaked and played the baby Celine Dion lullabies every day. In their blissed-out days after Zachary arrived on Christmas Eve 2005, the couple, who both have normal hearing, hardly considered that anything could be wrong — even after the harrowing results from the hearing test. With big brown eyes, smooth skin, and charming pink ears, Zachary seemed perfect.

In time, the doubts crept in. Their pediatrician asked if Zachary jumped when the dog barked or when someone dropped a pot. They went home to clang and slam things, and sometimes, it seemed, the baby turned his head. But he was never startled. And at the age when toddlers start saying "mama," "dada" and defiantly shrieking "no," Zachary only babbled.

The audiologist who retested Zachary never said the word "deaf," but he suggested the Duicks hurry the child to a specialist. When they got the diagnosis at GBMC, Shannon said, it was like having the wind knocked out of her.

But someone at GBMC had mentioned the possibility of a cochlear implant. And though Shannon hardly knew what it meant, she latched onto it because it sounded like hope.

Cochlear implants are essentially synthetic ears, devices that allow a person with no natural ability to hear — in some cases, almost normally.

By 2010 more than 42,000 people in the country had them, the FDA estimates, about 28,000 of them children.

Cochlear implants are nothing like hearing aids, which merely turn up the volume for people whose hearing needs a boost. Implants skip right over the ear, ferrying sound frequencies right to the aural nerves and onto the brain.

The device comes with three tiny, complex parts. The implant itself is surgically placed behind the ear, just beneath the skin and hair. It has a long tail that reaches into the core of the inner ear, where it anchors a cluster of electrodes. A processor hugs the back of the ear to pick up sounds and send them to the implant through radio waves. Then a remote control that looks just like an iPod can adjust the settings for particular environments.

Regina Presley is the senior audiologist at the hospital's cochlear implant center. The Duicks first met her there with Zachary when he was about 18 months old. Presley says children who get an implant before turning 2 can catch up to their hearing classmates by kindergarten. Babies must be at least 12 months old to get an implant, according to FDA rules.

The first thing visitors see walking into Presley's narrow, sunny office is the latest in implant technology — it's on a shelf right by the door. The pieces aren't necessarily a discreet, hearing-aid beige. There are pinks, blues and greens, swirls and checks — even soccer balls. It's to let people know, kids in particular, that these things are not something to be ashamed of. They can even be a sort of style statements.

A shadowbox hangs in a prominent spot on Presley's wall. Hands shaped from clay sign a message, "Thank you." An inscription at the bottom says, "Thank you for giving me the gift of sound." In the middle is a picture of a smiling Zachary, plastic parts peeping from behind each ear.

Once a cochlear implant is placed and turned on, the patient, particularly if it is a baby who has never heard a thing, has no idea what sound is, let alone how to talk or communicate. Just as someone with a hip replacement might go to rehab to learn how to walk again, someone with a cochlear implant needs to learn, from scratch, how to make sense of sound.

"Somebody wouldn't know what a knock at the door was," Presley says. "People are surprised that when you pour soda into a glass, there's sound."

They have to learn each knock, buzz, bam and fizz — the difference between the beep of a car and a siren, a fire alarm and a ringing phone, and in Zachary's case, his mother's voice and his dad's.

When doctors turn on an implant for the first time, they call it an activation. The moment is epic, yet in the case of a baby or a toddler like Zachary, subtle. The Duicks recorded it.

Playing back the video, one sees Zachary seated at a toy-filled table in Presley's office. He's squishing colored dough, he's banging bells with a plastic stick, he's yanking on the wires that dangle from his head, pulling them out as fast as Presley attaches them.

Presley tells the Duicks she's going to flip the switch. Zachary continues to swat the stick and, after smacking a bell, he looks up with what could be surprise. That's it, the first sound he's ever known.

When the Duicks had a second son two years ago, they asked that the hospital's most experienced technician perform the mandatory hearing test. When Ethan didn't pass, they knew. (Though they didn't know it when Zachary was born, the couple later learned there were distant instances of deafness on both sides of the family, increasing their odds of having deaf children.)

The couple arranged for his cochlear implant surgery to take place as soon as it was legally possible, right on the 12-month mark; Ethan's second operation came a few months later.

Before Ethan's first activation, the Duicks asked Zachary what was the first thing he wanted his brother to hear. It was "I love you," and he was the one to say it.

In the Duicks' subdivision, there's a sign in front of their corner lot that says "Hearing-Impaired Child." The couple might tell you that aside from that, they're about as ordinary as a suburban family gets.

Zachary, who's 6 now, loses himself in video games, plays goalie in his soccer league and just recently started getting into music, tuning into the pop hits on this mom's iPod. He went to regular day camp at the Y this summer and just started first grade, where he's in a special class for hearing-impaired kids in the morning, then moves in with everyone else for the rest of the day.

Ethan is 2, an energetic bundle who communicates half in sign language, half in talking — and he's becoming chattier by the day. Because of his head start with the technology, everyone expects speech and communication to come to Ethan far easier than it did for Zachary, who's still perfecting articulation.

The family calls the boys' implants their "ears." They put their ears on every morning, first thing out of bed.

Zachary never talks about them. They're just a fact, no more interesting to him than his nose or his toes.

Bill calls it "our new normal."

Two years ago, the family started what's becoming a holiday tradition. They bought one of those recordable ornaments and had Zachary say "Merry Christmas." When they did it again in 2011, Zachary's message was so much clearer. This year, they can hardly wait to hear.

jill.rosen@baltsun.com

Copyright © 2012, The Baltimore Sun