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Brian Hubbard has always loved to gather people around him. But in his mid-50s, a rare genetic disorder threatened to cut him off.
At the age of 56, Brian J. Hubbard felt his world shrinking.
When he went outside, he held the elbow of his partner, Sharron, and he couldn't tell if they passed someone he knew. He might hear a voice, and he'd ask her, "Who was that? Did someone say something to me?" But Sharron, shy and self-effacing, was not on the lookout for every chance to chat.
Hubbard found himself getting anxious in crowds. He could no longer discern voices amid noise. In restaurants, at parties, he didn't know when someone was talking to him. He was lost in the babble.
At home, a little white house in Newport, he perched at his computer for hours. A mechanical voice read each word of his e-mail, and the same voice bleated each letter as he typed replies. To his friends, a lifetime of friends, he wrote pages and pages dense with news.
But how could e-mail be enough?
That's why, in 2003, Brian Hubbard made up his mind: he would have the surgery. He sent a message to a doctor he knew at the UMass Memorial Medical Center, in Worcester, to say he was ready to get going.
He had no idea the kind of journey he was about to take.
YOU COULD say that Brian Hubbard's world had been shrinking since he was born, but he never saw it that way. A hearty mixture of denial and exuberance propelled him into life as if there were no limits. He would never be happy with limits.
Barely 4 years old, he snatched his sister's bike and taught himself to ride. He tied on ice skates and spun across the pond, faster than anyone.
But his mother knew something was wrong with her youngest child. Brian could bike and skate, but he could not speak. A doctor blamed emotional problems. A mother knows better: Brian could barely hear.
She resolved to teach him how to speak. In the kitchen she'd interrupt her cooking, bend down and grasp his chin. "It's not 'jicken,' Brian," she'd say, her face close to his, "it's 'chicken.' Chicken."
Brian attended Lynn, Mass., public schools. When he was 8, he was outfitted with a hearing aid: a bulging ear button with a yellow cord that snaked to a silver box half the size of a cigarette pack. The box rested in a pocket on a chest harness.
His fists quickly dispatched the few kids who made fun of this getup, so teasing was not a problem. Besides, his athletic skills won him respect. His hockey coach made him a chest shield to protect the hearing aid. Everyone marveled at the way Brian pursued the puck, plowing through players as if he were alone on the ice. But why did he keep running into the boards or getting hit from the side?
When, at age 15, yet another hockey injury sent him to the hospital with bleeding on the brain, the doctors discovered why he'd always been so accident prone. Brian had no peripheral vision. He could see only what was directly in front of him.
Brian cried bitterly when the doctors insisted he quit hockey.
But neither the doctors nor his parents told him all they knew about his newly diagnosed eye disease, retinitis pigmentosa, in which the light-receiving cells at the back of the eye gradually die off. Brian had always been night-blind, counting streetlights to find his way home on winter nights (and once or twice getting lost when a streetlight was out). The eye condition, together with the hearing loss, resulted from a genetic disorder called Usher Syndrome.
A disease that progresses slowly leaves room for denial, and Brian Hubbard elbowed his way into the future. His near-perfect speech revealed no hints of hearing trouble. As a young man, "Hubby" was known for his gift at making parties happen, making people want to be with him.
He graduated from the University of Massachusetts at Amherst, earned a master's degree in social work, and worked as a therapist and social worker in clinics and a hospital. He founded Counseling for Independent Living, an agency specializing in psychotherapy for people with disabilities. He married, had a daughter, divorced and remarried a couple of times, and skied some of the toughest slopes in America.
Over the decades, darkness closed in inch by inch. By the time Hubbard was 40, he saw the world as if looking through a very slender straw.
Around 1995, shortly before Hubbard turned 50, darkness finally overtook all. The glimmer at the end of the tunnel flickered -- some days, if the light was just right, he could make out a face; other days, nothing. Eventually, there was always nothing.
It was a time of many losses. Hubbard's third marriage was breaking up. His counseling service was shutting down, the money gone. He got prostate cancer, and traveled to California for treatment.
By the time Hubbard moved into the white ranch house in Newport, it was clear that total blindness had an especially high cost: He couldn't read lips anymore. As his hearing continued to fade, it got harder and harder to have a conversation, to stay in touch. What could be worse?
IN 1790, Alessandro Volta, inventor of the electric battery, stuck a pair of metal rods in his ears, and closed an electrical circuit attached to them. As the electricity surged into his head, he felt as if he'd been struck, and then he heard something: a sound like the bubbling of thick soup.
Volta was the first person to use electricity to produce the sensation of hearing. Scientists experimented with the concept many times over the decades, but only after the 20th century brought advances in microsurgery and microcircuits could researchers develop a practical application: a tiny electrode placed deep inside the inner ear -- the cochlea -- to stimulate the auditory nerve. They called it the cochlear implant.
Tested widely in the 1970s and approved for general use in 1984, the cochlear implant is the first device to replace a human sense.
The first implants had only one electrode. Users were able to detect sounds, such as a fire alarm or a microwave's beep, but usually could not discriminate speech. For many, hearing any sound was blessing enough, preventing them from being cut off from their environment.
In the 1980s and 1990s, implants with multiple channels -- more than one electrode, each corresponding to a different pitch -- came into use, along with more sophisticated speech processors, the tiny computers that rest behind the ear. Now people could recognize speech.
But there remains a key drawback: The process of inserting the electrode into the cochlea destroys any natural hearing a person may have. The implant is thus offered only to people who are severely hearing impaired -- for whom the implant's tinny tones are clearly superior to what their own ear can produce.
"These cochlear implants are very specialized and they don't help everyone," said Dr. Daniel J. Lee, the UMass Memorial ear surgeon who worked with Hubbard. "We want to make sure that the patient can potentially benefit."
So when Hubbard went to the Cochlear Implant Program at UMass, he faced a lengthy process with surgeons, audiologists and social workers. There was interview after interview: to explain what the implant could do, to assess his ability to adapt to it, to make sure he understood its limitations. The implant audiologists, Sarah Poissant and Dayna Hume, confirmed that his hearing loss was severe enough to justify the surgery.
"He was otherwise in good health. He had significant hearing loss in both ears and he was extremely motivated. And he had realistic expectations," Lee said.
Hubbard remembers Lee telling him, with surprising confidence, "I know it will help you."
Hubbard, of course, brought the added complication of blindness. At one time, doctors were reluctant to put cochlear implants in blind people because they thought that implant users needed visual cues to understand speech. But experience proved otherwise. In 2001, a University of Michigan study of eight deaf-blind people who received cochlear implants found that they did even better at understanding speech than a comparison group of patients who were only deaf.
In Lee's view, Hubbard's blindness was not a deterrent. Just the opposite: it made improving his hearing a priority. A blind man needs his ears.
SURGERY WAS set for Aug. 11, 2003.
Lying on the operating table, Hubbard suddenly realized what a huge moment this was. It wasn't just that he might get some hearing back. For the first time, somebody was going to actually make things better. For the first time, Hubbard faced not loss, not the need to accept yet another limitation -- but the chance to gain, to grow. As the anesthesia kicked in, tears filled his eyes.
Lee began the delicate procedure. He made an inch-and-a-half incision behind Hubbard's right ear and gouged out a portion of the mastoid bone with surgical drills. He threaded a wire with electrodes through the opening to the cochlea, the snail-shaped organ of hearing deep in the inner ear. Under the skin, behind the ear, he secured the receiver, the slender box containing the electronics.
Hubbard had chosen an implant made by the Med-El Corp., of Innsbruck, Austria, one of three companies that make cochlear implants approved for use in the United States. The Med-El implant has 12 electrodes (the other versions have 16 and 22) and extends deeper into the cochlea than the other brands. If all went well, the 12 electrodes, strategically spaced, would allow Hubbard to hear 12 different pitches, the higher ones near the opening of the cochlea, the lower ones deeper inside.
It took Lee two hours to place the internal portion of the cochlear implant. Now, Hubbard was totally deaf in his right ear, and reliant on the hearing aid in his left. He went home to heal for three weeks.
Later, he would get the outside components: a tiny computer with microphone that rests behind his ear, called the speech processor. Only after these were turned on would Hubbard find out what the implant could do.
THAT DAY came Tuesday, Sept. 2, 2003.
Holding Sharron's elbow, Hubbard made his way to a tiny office in the implant clinic at UMass Memorial. He took a seat, his blue eyes looking straight in front of him as if they could see. His square athlete's face had gone soft and fleshy, but his dark hair showed scarcely a strand of gray (he once joked that he's entitled to one good gene).
Crowded into the room with them were the audiologist, Dayna Hume; one of Hubbard's fraternity brothers, Peter Rossetti; and Melissa Waller, a Med-El representative.
Hume placed a disk-shaped magnet behind his ear, where it grasped the electronic device embedded next to his skull. She then hooked the 2-inch, L-shaped speech processor over his ear, but didn't connect it. Instead, a wire from the magnet connected to a computer.
The first task was to activate each of the 12 electrodes. Looking at a computer screen, Hume used the mouse to click on "Activate," thus sending a signal to the first electrode.
Hubbard heard a deep tone. Hot damn! he thought. This thing works!
Hume punched the keyboard to adjust the loudness. Then she clicked on the second electrode, and the third -- each tone higher, each tone heard.
When she activated the fourth electrode, Hubbard was thunderstruck. Never, in all his life, had he heard this pitch. This sound was new to his brain. It felt like a hallucination.
One by one, eight more electrodes gave birth to eight novel sensations, one by one ringing in his new reality. These were the high-pitched notes Hubbard had been missing all his life, the sounds squeaky and sibilant, the chirp, the tinkle and the hiss.
But that was only the beginning. Next, Hume attached the wire to the speech processor, the miniature computer hooked on his ear. The processor picks up sounds through a microphone and translates them into patterns of electrical impulses.
As the speech processor cranked up, Hubbard heard a sound like a vacuum cleaner running underwater (perhaps reminiscent of Volta's bubbling soup). He didn't know what to make of it.
I took my leap of faith and listened, Hubbard would write two days later in a letter to his friends. Still nothing was making too much sense. . . . It was as if a giant auditory puzzle had been dumped out of the box onto the table and I had to find a way to put this puzzle together, bit by bit. . . .
All of a sudden . . . I think I hear a recognizable sound. I listen patiently now, just taking it in. Is this sound I am hearing what I think it is? I listen carefully. . . . It is my name. Someone is saying "Brian."
I start to ask if someone is calling me, except that when I utter the first syllable I nearly jump out of my skin. I heard my voice for the first time through the processor, and man, did it sound different. . . .
Now I am distinguishing some more words. "Brian, can you hear me?" I hear this sentence repeated at least three times. It reminds me of the line from the rock opera Tommy by The Who. . . .
Finally I say, "Yes, I think I hear you" but again jump to the sound of my own voice. Then I say something to the effect, "Why does everything sound like a bunch of chipmunks in heat?"
Then I hear a cacophony of sounds that resemble a canary that got caught in a fan, but I decipher it to be laughter.
So I laugh, and again I jump.
HUBBARD LEFT UMass with his speech processor still on, his brain scrambling to unscramble every new sound. He went out to dinner with Pete Rossetti and Sharron. Rossetti amused himself trying different sounds -- shaking the ice in his glass -- and quizzing Hubbard on what he heard.
But, as Hubbard wrote to his friends with embarrassed delight, the high point came after dinner, when he went to the men's room.
If you can see and hear, it might not be obvious why simple things can bring such happiness. A blind man, of course, can cane-tap his way to the men's room and he can, of course, position himself where he needs to be. But a blind man who can't hear much has no way to verify his aim.
So on Sept. 2, 2003, a deaf-blind man was filled with joy, a joy so great that tears came to his eyes even as he laughed, when he discovered that -- finally, thankfully -- he could hear the tinkle.
As the days went by, Hubbard's brain continued to adapt to the device. He reveled in all he could now hear. The cries of his Siamese cat, Minky. The mourning doves cooing in his backyard. The creak of the swing chair. The jangle of the computer booting up. Even unwelcome sounds caught his attention -- the hiss of traffic, the drone of the washing machine.
Conversations flowed naturally. People could stand anywhere in the room, and Hubbard could hear them. He could understand speech even when there was background noise. That meant -- oh, joy! -- he could listen to the Super Bowl. It was miraculous.
So miraculous that Hubbard wanted more.
STORY of his life, you could say: Hubbard wanted more. He knew that if he had two good ears, he could better tell where a sound was coming from. This is especially important when you don't have sight. He felt that with two ears, it would get even easier to separate conversations from background noise.
But mostly, he had this thought: if one cochlear implant could so dramatically improve his life -- just imagine what two could do!
"I wanted to double the wows," he said.
The Cochlear Implant Program at UMass rarely places implants in both ears, because the benefits of bilateral implants have not been proven to outweigh the costs and risks. But in Hubbard's case, Dayna Hume, the audiologist, and Daniel Lee, the surgeon, were enthusiastic about the idea. "The guy's pretty amazing," said Lee. "He's unique."
They were impressed with how nimbly Hubbard's brain had adapted to the first implant, and how motivated and smart he seemed. Besides, his need was great: He had little useful hearing left, and he was totally blind. In their eyes, there was every reason to believe that a second implant would bring huge benefits to Hubbard.
Sharron stood by, silent, incredulous, filled with foreboding. She could not imagine anything more foolhardy. Brian was hearing better than ever. He had the best of both worlds -- his left ear with a little natural hearing and a hearing aid; his right ear with a cochlear implant. Why rock the boat?
Sharron knows something about deafness. She was deaf, herself, for 15 years, with a condition affecting the tiny bones in the middle ear. It was Brian who told her that her condition could be corrected with surgery, and who connected her with the surgeon -- the very UMass surgeon who originally suggested a cochlear implant to Brian.
Those folks at UMass, Sharron thought grimly, took such pains to make sure Brian was ready for the first implant -- and now they're rushing into the second. They pushed for it -- they pushed too hard in her opinion. Why couldn't they see that this step was equally as big? Brian, a blind man, would have no natural hearing left. He would never again hear a sound except filtered through a tiny computer. And that fallible, man-made device would be his only connection to the world.
Sharron, who had changed her last name to Hubbard although the pair never legally married, had been living with Brian for several years. She knew that if she said anything to discourage him, Brian would feel, for the rest of his life, that she kept him from something he wanted to do. Any regrets would fall into her lap.
So, of course, would the task of caring for Brian if things went badly.
Brian Hubbard was not without misgivings. As a blind man, he felt his situation was unique. He searched for another person who was blind and had obtained cochlear implants in both ears -- but couldn't find one. (He later connected with Rudy Vener, who is thought to be the first deaf-blind man to get a second cochlear implant in a 2001 operation in New York City.)
As he headed into surgery, Hubbard believed he was the first. Much as he relished being a pioneer, he wished he could know more. He got cold feet and delayed the surgery for a week.
But here was a guy who used to ski double-diamond trails with a pinhole view of the slopes. He'd position himself at the top of the slope, wearing his "BLIND SKIER" vest, and tilt his skis downward to gauge the steepness. Then, peering at the swaying specks of his guide's boots in front of him, he'd plunge forward.
In the end, this was the deciding factor: If he didn't get a second implant, he would always wonder what he might be missing. It would dog him the rest of his life. He plunged forward.
On March 12, 2004, Brian Hubbard checked in to UMass Memorial. Sharron, who drove him there, immediately fell ill with a migraine.
RAY BASTARACHE, Hubbard's friend since their days as youngsters playing ice hockey in Lynn, noticed something missing in the spring of 2004: his e-mail contained no messages from brian@brianjhubbard.com. Bastarache, now the deputy superintendent of Lynn Public Schools, was accustomed to regular megabytes of discourse from his old buddy.
Worried, he placed a call to Newport. Hubbard had little to say. This was unheard-of. Hubby with few words? Not only that, he broke down.
Bastarache had witnessed Hubbard leaping so many hurdles over so many years -- being forced off the hockey team, his father's suicide, going blind, prostate cancer. Always, he kept up a certain bravado.
But now, Hubbard sounded nothing short of desperate, and he wasn't hiding it.
"I'd hang up the phone and say to my wife, 'Mackie, he doesn't want to talk. He admits to me that he's drinking.' It went on for a long time. We'd call the house. Nobody would pick up. We were very, very nervous about it."
Dayna Hume, the audiologist, did receive e-mails from Hubbard, but they were just as worrisome as the silence Bastarache faced. The first one, 12 days after the second surgery, bore the subject line, "Reassurance needed." A few days after that, Hubbard wrote, "Sometimes I feel fatigued, like my life batteries are low."
Nothing could recharge him. As the months went by, and the occasional e-mails from Hubbard brought only more distress and disappointment, Hume got scared. Had they made an awful mistake? Had they led Hubbard down the wrong path?
