Twice as Good: Bilateral Implants for Adrean Mangiardi

--Twice as Good: Bilateral Cochlear Implants for Adrean Mangiardi --

{caveat: not yet edited!}

The first device ever to replace a human sense received its stamp of approval in 1984. That year, cochlear implants were marketed as safe – as more than “experimental” – by the FDA. Today, the largest population of cochlear implant users found in one place congregates in Rochester, New York, at the world’s first and largest technological college for deaf students: The National Technolgical Institue for the Deaf.

This institute, better known as NTID, is home to over 11,000 deaf staff, students, and faculty, and of these, 180 can hear birds chirp better and footsteps advance faster than their deaf peers because they have chosen to get a cochlear implant. Now, three of these 180 implant users have taken yet another step; they’ve gone on to do something that’s practically unprecedented, even in the relatively young field of cochlear implants: they’ve gotten implant #2.

“Getting bilateral implants,” said Karen Black, director of communications at NTID, “is the newest of the new in cochlear implant technology.” This is impressive in a field whose modern history spans less than 25 years. True, a researcher named Volta put metal rods in his ears over 200 years ago, in 1790, but his attempts to stimulate hearing with electricity were not successful. And in 1961, when clinician William House performed the first modern implant surgery, much to the dismay of the neurophysiologists who wished he’d “keep [his] hands out of [their] cochlea,” scientists were skeptical. They doubted that the single electrode mechanism of an implant could cause anything beyond a buzz in the ear. As such, it wasn’t until about 20 years ago, with the FDA’s official accolade, that the cochlear implant was taken seriously by both the scientific and medical community. After this, research and advancements progressed rapidly.

But all study was directed at one effort: making sound perception and language acquisition better with a single implant. One implant for creatures with two ears. Great strides were indeed made; most notable was Graeme Clark’s 1985 invention of an implant that, unlike William House’s, used multiple electrodes. Clark’s was the first device in history that permitted even severely deaf people to understand speech. Previously, scientists had thought that providing quality speech understanding, even with the benefit of more than one electrode, was impossible since the inner ear requires 10-20,000 neurons to do so. Clark proved them wrong.

But amidst his and other advances, questions loomed in yet another place: the deaf population. To get an implant, or not to, many wondered. Getting even one was questionable, especially since benefits seemed variable from patient to patient. Today however, three students at NITD, including 25 year-old film major Adrean Mangiardi, have moved beyond wanting one implant and are happily sporting two. “My life is way better now,” Adrean says of his bilaterally-implanted days. He underwent a second surgery that was both painful and costly (implants are 40,000$ a piece). And I sought him out to learn why.

* * * * *

Adrean Mangiardi is technologically-savy. He studies film-making at Rochester Institute for Technology, supported by NITD, where he can maneuver video equipment with finesse. He also understands the in’s and out’s of the physics behind the bypass filters in his cochlear implants, in case he needs to troubleshoot. My interview with him – the self-proclaimed “brave man who’s desire to listen doesn’t stop [him], even though the idea of bilateral cochlear implants is fresh” -- was done via email, from my MAC to his.

The 25-year old Pennsylvania native, deaf since age __, got his first cochlear implant ten years ago, when he was 15. “Before I had the first one,” Adrean said, “I was losing my hearing each day. But I had a disgusted look on my face whenever I saw wire [from an implant] on somebody else’s body. I hated being deaf though and wished to be normal like everybody else.”

Adrean said that it was his mom who finally motivated him to go through with the first surgery. “One day she and I were driving to the mall and she started begging me to get the first implant,” he said. Adrean doesn’t recollect why; he had been able to communicate reasonably well with both of his hearing parents. He was actually surprised at his mother’s request. “I sighed and thought, why she would ask me that, knowing I didn’t want it?”

After the incident in the car though, he felt himself thinking about his mother’s words a lot more than usual. “From that moment on, I wondered what would it be like to wear the implant,” Adrean wrote. “I made a decision to go for it.” It is clear that his parents had a lot to do with this decison. “My parents know what’s best for me so I thank them for trying to help me out.” The second implant, however, which Adrean got just last year, was an idea that was completely his own.

“I noticed that wearing one cochlear implant was the standard way, but I’m not a standard guy,” Adrean said. “I seek rare possibility and make it happen for me.” He said his interest in film was one reason he decided to double up. “I cannot wait to work in the professional moviemaking business. Having bilateral implants will help me achieve my goal in filmmaking.”

Adrean also expressed that even though his single implant aided him greatly – letting him hear doors squeak and birds squawk as he’d never heard them before – he still had trouble localizing sound in large groups. He couldn’t pick out one voice amidst many, in other words, as he might need to do in a party situation, or in any large group scenario. “I wanted the second implant because I thought it could improve my life on a daily basis with my friends, too.”

His friends, in fact, were surprised by Adrean’s impulse. “One time, I was at a party at my friend’s apartment,” he recounted, “and I met a couple of girls who only wear one implant. I explained my plan of getting the second one and they were shocked.” When Adrean asked them what the “big deal” was if he only wanted to hear well, the girls explained that they wouldn’t want to go through that surgery again for anything.

“It was the surgery that scared them away,” he said. Adrean looked past it though. “I knew that it would be painful, but cochlear implant lasts forever, at least I hope, and I am the evidence that wearing two implants is a good way to improve your listening ability.” When he bumped into the skeptical girls a second time, outfitted with not one, but two implants, they asked him a billion questions about his new bilateral experience. “I think one day,” he said, “they will plan to get another one.”

But the road to bilateral victory wasn’t greased. There were other skeptics, besides Adrean’s friends, and there were uncertainties. Adrean’s physician didn’t actually know if the second implant – a $40,000 investment – would benefit they boy at all. “The doctor said that it might not help one bit or it might help a lot,” Adrean said. “But, what does he know? He’s not using it. He’s a doctor who can put things into our head and that’s it.” Adrean continued to express – in his written response – that with his second implant, and with some experience since the surgery, he is doing a lot better now than he had been when wearing just one. “Way better.”

There were mild side effects, however. After all, 2 implants is a lot of titanium and platinum for a brain accustomed to interacting with only bone. “I know that I didn’t get dizzy from the first implant,” he said, “but after I got the second one, I was dizzy for a few months because my inner balance was tampered with.” This dizziness is supposed to happen –a product of the ________ in the brain. But with Adrean’s second surgery, it lasted longer. “I didn’t know that the symptom would last that long,” Adrean said, “and I stumbled more than usual in the first few months after surgery and my eyes wouldn’t stay still when I tried to look at a something.” A year later, Adrean says that he is fine. The dizziness is gone and his vision is focused. “I’m very happy about that,” he confided. “I thought those problems would be permanent.”

And day to day, his life is richer. The biggest noticeable difference though, Adrean said, is not his ability to excel in filmmaking, or to distinguish a voice from across the room in a crowded party. “It is the birds. There a lot of noticeable differences now that I have two implants, but birds are one thing that I never heard before when I was a kid. Now, I hear them everywhere and it is so cool. I wish to film the life in a rain forest because there I will hear bird sound that I never heard before.”

Adrean also explained that though he’d been using his first implant for 10 years, and that had worked well enough, having a second one “gave me a boost to hear certain decibels, especially higher ones, that I couldn’t reach before.”

Oddly enough, there are ways in which having two implants, versus one, limits Adrean. “With one implant, I can wear a hat. I can’t with two because the hat would be pinching my head together. It’s not a good feeling.”

Regardless of any difficulty in donning a hat, Adrean says that the best way to explain the difference between having one implant and having two is that his life was fine before, “but now my life is improving everyday.” He’s especially happy with his ability to localize sound and no longer feels frustrated in large groups. He’s no longer overwhelmed by noise because he can now extract individual sounds better from it. He sees himself as a leader, too. “I hope that others will follow and understand the possibility of having a better chance of hearing things with two cochlear implants.”

I asked Adrean one last question: You’re surrounded by efforts to improve hearing at NTID, so what do you see as the next big break-through in hearing technology? His answer kindled my imagination. “I once had a dream that there would be a cochlear transplant,” he had typed. “There’s a liver transplant, a heart transplant, a kidney transplant, and so on. Why not a cochlear transplant, too? I seek for something natural as possible instead of something electric and artificial.”

There is nothing artificial about Adrean Mangiardi, or his drive to accomplish what he seeks. We may see his films. We may find him in rainforests. Either way, he will clearly hear – and be worthy of – our accolades.


.MGW.

ExcerptWords

A lovely excerpt -- about runners and love -- from a Sharon Olds' poem:

They are like great runners:
they know they are alone
with the road surface, the cold, the wind,
the fit of their shoes,
their overall cardio-vascular health--
just factors,
like the partner in the bed,
and not the truth,
which is the
single body
alone in the universe
against its own best time.


.MGW.

VitalSignsWords

"Vital Signs"

http://www.jhu.edu/~jhumag/0206web/wholly2.html#vitals

Appeared in Hopkins Magazine's February issue

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Talpid2Words

--Mutant Chickens Grow Teeth--

By Meagan White
ScienceNOW Daily News
21 February 2006

Warning: Mutant chickens may bite. Researchers have identified a genetic mutation that creates incipient teeth in bird embryos. The discovery provides a modern day glimpse of a feature that hasn't been seen in avians for millions of years.

Birds lost their choppers 70 million to 80 million years ago. That's what made an experiment in 1980 so surprising: After scientists grafted oral tissue from mice onto a chicken's gums, the birds grew round, mouselike teeth. But because avians and mammals are not closely related, scientists doubted whether the experiment proved that birds had truly retained a genetic vestige of their forbearers' bite.

Now a group of developmental biologists has found a strain of birds that don't need outside help to grow teeth. While investigating a gene mutation known to affect organ development in chickens, Matthew Harris of the Max Planck Institute in Tübingen, Germany, noticed sharp protrusions on the jaw of a 16-day-old embryo. Scientists had never suspected a connection between tooth formation and the gene--known as talpid2--because embryos with the mutation rarely survive past 12 days. Further investigation by Harris and colleague John Fallon at the University of Wisconsin, Madison, indicated that the teeth were conical and saber-shaped, resembling those of an alligator or crocodile.

To see how tooth formation in these chickens compares to that of other animals, the team looked at the expression pattern of a gene called sonic hedgehog (shh), which is essential for tooth production in vertebrates. In normal chicks, shh was expressed in a region analogous to the sides of the gums, but in alligators and talpid2 mutants, shh appeared in the center of the gums. The mutant version of talpid2 thus appears to turn shh on in the right place for growing teeth. Over time, changes in the gene may have disrupted this ability, resulting in tooth loss, the researchers report 21 February in Current Biology.

The finding is a great example of how altering the location of gene expression can cause changes in body types over time, says biologist Scott Gilbert of Swarthmore College near Philadelphia, Pennsylvania. "The real wow here," adds Paul Sharpe, a biochemist at the Department of Craniofacial Development at Kings College London, "is that these guys essentially show a glimpse of what the teeth probably looked like in the first birds."

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BAHA.Words

-- BAHA in Baltimore: How John Niparko Inserts a Bone-Anchored Hearing Aid --

“It’s not pretty,” Dr. John Niparko declared, smiling and plucking purple gloves from his fingers after a successful surgery. “There’s a lot more blood here when I do it than you would typically see in a BAHA procedure. But I get the job done.”

Niparko – famous worldwide for his cochlear implant work, including implantation of Heather Whitestone, America’s first deaf beauty queen – is head of the Listening Center at The Johns Hopkins Medical School. The Baltimore physician performs about 10 bone anchored hearing aid (BAHA) surgeries a year. And even though, as Niparko said, the BAHA procedure has been considered “simple enough to be pulled off in the office,” it is indeed complex.

This hidden complexity has left room for the reknown Hopkins surgeon to customize the way he approches insertion of bone anchored hearing aids. Niparko inserts them differently than most otolaryngologists across the nation, which has yielded him greater success, as measured by the fact that only 5% of his patients return for replacements.

“That’s a tremendously low rate,” said Steve Hazard, manager of CochlearTM, one of only three companies to make cochlear implants and other hearing devices. Hazard had come all the way from Colorado, Cochlear’s headquarters, to photograph the surgery in Baltimore the day that I observed. At each step of the way, Hazard took pictures of Niparko’s signature moves for a much-needed medical manual that his company will distribute to surgeons around the world.

“In a BAHA surgery, it all comes down to detail,” explained Dr. Marc Eisen, the young doctor who had worked alongside Niparko to perform his very first BAHA procedure the February morning that I visted the Hopkins Outpatient Center. “There is this feeling out there among other surgeons that the BAHA procedure is simple,” he continued. “This is because there are really no vital structures -- like facial nerves -- put at risk. Also, the steps sound simple: raise up the skin, thin the skin, drill a pilot hole, place the device, and place the thinned skin down over it. And in the short-term, these steps work well.”

Niparko doesn’t just think short-term, however. “What I learned working with him,” Eisen said, “is that for long-term success of a bone anchored hearing aid, three details matter: the size of the skin flap, its thinness, and the angle at which you place the part of the hearing aid that’s outside the head. These details are not well described in the BAHA literature.”

Bone anchored hearing aids work through bone conduction, one of two ways – the second being air – that sound is conducted to the inner ear. The cochlea, the part of the inner ear which receives the sound and converts it to the electrical signals that the brain can understand, is still functional in BAHA recipients, unlike in cochlear implant patients, for example. Therefore, the job of a BAHA is simply to get the sound to the cochlea, and it does so by enhancing the already naturally occurring transmission of sound to the bone.

Bone anchored hearing aids benefit people who cannot wear traditional air conduction hearing aids – placed in the outer ear – due to outer or middle ear deformities. People with microtia, a disease in which the outer ear is either severely deformed or entirely absent, as well as patients with chronic ear infections, inflammation, and astresia – the absence of the ear canal – are good candidates.

And as far as which hearing device – air or bone – improves hearing to a greater extent, “there is no clear cut winner,” Eisen said. However, the advantages of the BAHA are several-fold in the right situation. In cases of a patient with severe, unilateral deafness, for example, a conventional hearing aid offers little benefit, but the BAHA, explained Eisen, “seems to route the sound to the good ear, giving the patient some benefit of binaural (two-ear) hearing.”

Hearing via a bone anchored device involves a three-part system. A small box called a processor is attached to a screw that is drilled into the skull, behind the ear. The screw attaches to the third part of the hearing aid, a titanium implant. Both the implant and the screw must be inserted surgically, as I watched Niparko and Eisen do. And while the implant is lodged below the skin flap, the screw – meant to stay completely in the bone – is drilled into the skull afterwards. At 3 or 4 millimeters deep, the screw is not at risk of penetrating to the other side of the bone or piercing the dura, the fibrous coat which protects the brain. “This would be a sure route for meningitis,” Eisen explained.

Over time – about three months after the surgery – the titanium implant naturally integrates with the skull bone in a special process known as osseointegration. During this process, the implant bonds with the surrounding bone tissue, making a direct structural and functional connection. This lets vibrations from the sound processor be transmitted via the bone to the cochlea, on the other skull’s other side. The long term success of Baha rehabilitation is based on titanium’s unique ability to integrate with tissue and on the fact that an active bond between tissue and implant is created at the molecular level, such that the implant is not only accepted but also incorporated within the bone.

After successful osseointegration, the final part, the sound processor is attached to the screw. At this point, sound vibrations can be picked up from the air and travel through the screw to the implant, which vibrates. This initiates vibrations within the skull and inner ear, and these vibrations stimulate the nerve fibers of the cochlea directly, allowing hearing.

Patients who receive BAHAS are typically happy. Few of them, studies site, prefer their old hearing aids, and the majority report increased comfort and function. This wasn’t always the case for bone conduction hearing aids, however; “If you simply saw the old ones,” Eisen explained, “you would immediately have your answer as to why people found them uncomfortable. They weren’t implants, but headbands that held the hearing aid tightly against the skull someplace. They were also cumbersome and noticeable.”

Unlike the old bone conduction hearing aids, BAHAs do require surgery, but it is typically a short, minimally invasive procedure, lasting not much more than 30 minutes. As I observed in February though, Niparko takes his time, performing a BAHA in an hour, from start to finish.

“This process should take about 15 minutes – in and out,” joked a young resident in the operating room as he pulled on his face mask moments before the surgery. But he was wrong; Niparko – a tall, lean man with a careful eye – takes longer than average, and this is just one aspect of his BAHA surgery strategy that strays from the norm.

For starters, the size of skin flap made at the initial incision is larger -- that of an orange versus a ping pong ball – when Niparko is at the helm. “I don’t mind taking time to make the larger cut,” Niparko explained to Hazard, as the man watched, and took shot after shot.

Incision size is crucial; sufficient subcutaneous tissue – or fat – needs to be removed from under and around the skin flaps so that once the screw is drilled into the skull, it is less likely to be disrupted by creeping tissue. This tissue, though only millimeters in thickness, often grows back and pushes the screw out of line.

On the day of the surgery, Niparko spent calculated time using a pick to pull thin lumps of yellow, fatty tissue from under perimeter skin flaps of his large incision. After pulling an especially wide tissue lump from the patient’s head, Niparko plucked a ruler from the pocket of his green standard-issued Hopkins scrubs: “4 inches!” he said, commenting on its diameter. The tissue was so large that when the nurse dropped it in on a steel table nearby, it made a thud loud enough for me – on the other side of the room – to clearly hear.

Beyond incision size, the second detail Niparko focuses on is the angle of processor attachment. “I’ve never heard a better insight into the success of this surgery,” Hazard commented, after hearing Niparko explain his emphasis on a drilling the screw, perfectly straight, into the skull to avoid complications with the processor. Apparently, it does not function as well when it touches skin – when it’s angled up or down because of bad screw alignment, in other words. That’s because if the processor touches flesh, its vibrations are dampened, making the transfer of those vibrations – from processor to screw to implant – less efficient. “Remember,” Eisen reminded me later, “it is all about physics. No electrical stimulation like a cochlear implant.”

Eisen explained that surgeons do not use a device to measure the angle at which they enter the skull. “We just have our line of sight to guide us. This is why it’s important for the surgeon to anchor his arms.” As Eisen drilled the screw 4 millimeters deep into patient’s skull that day, Niparko loomed over him and lectured on angles. “Don’t let it be anything but 90 degrees,” he said. Meanwhile, Hazard watched closely, seemingly amazed at what I found to be a simple idea.

Niparko then did something which captivated all in the room. To determine how well the screw was affixed to the skull, he bent low to the patient’s head and hit the screw with a small metal pole. He listened carefully to the pitch generated. “A sharper ring should mean more solid fixation,” Eisen later explained. Niparko was dissatisfied with the high pitched ring that chirped quickly in the silent room, and he instructed Eisen to turn the screw again. To make it tighter.

Then Niparko tapped, listened, and -- still dissatisfied -- asked Eisen for yet another twist of the screw. After the final turn of screw into skull, Niparko was happy with the high pitch ting generated from careful insertion.

“Niparko’s strategy is great,” Hazard said. “Especially because he cuts a large flap when making the incision. Larger than most and large enough so that the tissue around the BAHA implant doesn’t become an issue and push the implant out of line. This takes more time and most surgeons won’t do it. They cut small flaps. Or they do a dermatrome. But Niparko doesn’t care. He’s takes his time and makes a big cut.”

By paying attention to tissue removal and drilling angle, then, Niparko has customized the BAHA surgery. His signature moves have yielded him success about which the world will hear with the coming of Hazard’s manual. Surely, Niparko’s patients will hear of it, too.

.MGW.