PALO ALTO, CA (KGO) -- A first of its kind surgical procedure pioneered by a Bay Area surgeon is changing the life of an 11-year-old boy from Mexico. Diego Neumaier was born without ears, but thanks to the generosity of a neurotologist, a plastic surgeon, and some compassionate donors, the young man now has what he's always wanted.
Neumaier's most valued possessions are a handful of gold medals he has earned. They are a symbol of his victory in Mexico's national competition for young gymnasts and they are something he wants to give to his doctor, neurotologist Joseph Roberson. Neumaier says the metals are like gold and Roberson is like gold to him.
Neumaier was born without ears or ear canals, although his middle ear bones and inner ear are intact.
"Somewhere in development as the ear is starting to form or fold, and the ear canal starts to open or dissolve, that stops," says Roberson.
The condition is known as atresia and microsia and typically requires several surgeries to correct, but Roberson and plastic surgeon Dr. John Reinisch have pioneered a way to perform multiple operations the same day; it's about 10 hours in surgery, working together to create a new ear canal and ear.
This chance something Neumaier and his mom have dreamed of.
"It's a miracle for me, it's been 12 years since I've wanted my ears," says Neumaier.
He will do it even if his newly-constructed ears might bring him some discomfort.
"They say that if I get bit by a mosquito, my ear will swell just like everyone else's ears," says Neumaier.
"I've been so nervous about everything, but calm because I know he'll be in good hands with Dr. Roberson," said Neumaier's mother.
The surgery began early in the morning. First Reinisch began the construction of the outer ear. Then Roberson works to create an ear canal and eventually an ear drum for Neumaier, while Reinish continues his sculpting of the ear.
It was a team effort that combined seven surgeries into one day. Four weeks later, Neumaier was ready to have the packing removed and was given a chance to hear for the first time.
"If you had a light cotton ball in your ear canal, that's what he's hearing right now. For him that's a huge change because he's never heard normally, but we'll see over the next several weeks and months is that will improve and come up to an even better level," says Roberson.
Neumaier's indebted to both doctors, but had another favor to ask. He wanted the doctors to not forget his other operation since he plans to be back at Christmas time for his left year, which means he'll soon hear the roar of the crowd at his gymnastic competitions -- a distraction he welcomes.
Roberson's Let Them Hear Foundation covered the cost of the surgery and the expenses for Neumaier and his mom. Both doctors donated their time, but as Neumaier mentioned, he needs his other ear.
Some kids seem to have near-constant ear infections. Even after the pain is gone, a parent's got to wonder: Are there lasting effects from all that muffling of sound in the formative years?
Research in rats just published in the journal Neuron suggests there might be effects in the brain that, while not permanent, can last for years. Apparently, hearing loss in one ear during critical periods of brain development can rewire the auditory cortex, changing the way it processes sound.
Neurobiologist Dan Polley, who recently moved to Harvard and the Massachusetts Eye and Ear Infirmary in Boston, conducted the research with a colleague, Maria Popescu, while at Vanderbilt University.
Polley says that while we don't need two ears to hear sound, figuring out where that twitter of birds or the shout from a friend is coming from requires the sort of depth perception that input from two ears provides. Plus, there are other benefits from a nuanced fusion of the two signals in the brain.
"Our ability to hear speech in a noisy background; to hear the wonderful compliments that your date is paying when you've taken her out to dinner; or when you have multiple people talking to you at once, and you try to home in on one speech source -- all these phenomena depend critically upon integrating signals from each ear," he says.
Polley wondered if the kind of periodic, months-long hearing loss experienced by some children with chronic infections and resulting blockage of the middle ear might actually affect the wiring of the brain. So he and his colleague tried a little test in rats of different ages: In each animal, they blocked the sound in one ear for a couple of months, and then unblocked that ear.
The result: In young rats, the ear that had remained open and clear made a sort of real estate grab in the auditory cortex, developing a much richer network of neural connections. The blocked ear lost influence. And even after both ears were once again sending clear signals to the brain, the imbalance in the brain persisted.
It's the sort of thing, Polley says, that could make triangulating the source of a sound harder, he says, and create subtle, but important deficits in hearing.
"When you don't correctly identify the position of a sound a in space, you may not know it," he says. When you're not able to hear in a noisy background, you may just not go out to dinner as often. You may end up isolating yourself from the environments that really require good hearing."
A child with that sort of problem might withdraw in a noisy classroom, Polley says, or--depending on when the imbalance occurs--might miss milestones in language or learning.Other studies have shown that's just the sort of thing that's been reported among some children with chronic middle ear infections.
Here's some comfort for parents: Though it can take a while, the brain is pretty good at developing workarounds, Polley says. Restore hearing, and the brain will eventually catch up.
The human is a complex body organ that begins development
early in a mother’s pregnancy. Those who have
been pregnant know the ear works by the last third of
pregnancy and that babies respond to sounds in their
environment by startling, by kicking or by stopping
movement.
For the ear to form correctly, structures grow from the skull base
and must join with growth from the outside of the head which
progresses in toward the inner ear. The two processes meet at the
eardrum and middle ear bones. A part of the process involves formation
of the outer ear (called the pinna) as several small folds of
skin merge to give our ears their normal appearance. Download Complete Newsletter
