UC Davis program hopes to facilitate follow-up for infants needing diagnostic audiology evaluations.

Infant Jack James rests in mother Michelle's arms while undergoing audiology testing via a telemedicine arrangement between Mercy Medical Center Redding and UC Davis.

Babies who do not pass newborn hearing screening tests require immediate diagnosis and intervention, but that can be a challenge for families living in the vast expanse of rural Northern California, where a dearth of pediatric hearing specialists, geographic isolation and the topography all conspire to create obstacles.

Those obstacles meant that in 2007, 40 percent of rural Northern California newborns who needed additional testing for a potential hearing loss did not receive it and were “lost to follow-up” care – giving Northern California the poorest lost-to-follow-up rate in the state, where the overall average was 8 percent.

“Bringing these babies back for testing is imperative to optimize their development, especially the speech development critical to acquiring language and learning,” said Anne Simon, senior pediatric audiologist in the UC Davis Department of Otolaryngology.

But Simon also said she understands that there are substantial barriers that discourage families in rural communities from making the trek to the audiologist so that their infant can receive additional testing.

“Making the three- or four-hour-long trip to a big city medical center with a four-week-old baby and may not be possible for many families,” Simon said.

To meet those families’ needs and improve the numbers of Northern California infants receiving follow-up care for hearing loss, UC Davis has entered into a unique new partnership with the State of California and Mercy Medical Center Redding. It will allow infants located throughout Northern California to be seen by a pediatric audiologist at UC Davis – via telemedicine.

Among the first of its kind in the nation, the new pilot program is funded by a three-year, $354,242 grant from the U.S. Health Resources and Services Administration Maternal and Child Health Bureau through the state Department of Health Care Services (DHCS), Children’s Medical Services.

“We are thrilled to be implementing this innovative approach to more quickly identify infants with hearing loss in Northern California,” said DHCS Director Toby Douglas. ”UC Davis is a leader in telehealth and pediatric audiology, and we are fortunate to have them as partners in this endeavor.”

Early identification of deaf and hard-of-hearing infants before 3 months of age and starting early intervention services before 6 months of age are the most important factors in developing age-appropriate language skills, whether families communicate using sign language or spoken language.

The program focuses primarily on infants living inland in the far northern counties in California adjacent to Shasta County where Redding is located, such as Glenn, Butte, Trinity, Tehama, Lassen, Modoc and Siskiyou counties. Participation in the program is by referral from the state Hearing Coordination Center.

The teleaudiology program is unique because, rather than consulting with audiologists or other clinicians at the remote location, who then diagnose and treat the patient, the UC Davis audiologists actually perform the hearing screening and making the diagnosis.

“We are very, very excited about providing this program, because central Northern California has the highest lost-to-follow-up rate in the state for newborn hearing screening,” said James Marcin, professor of pediatric critical-care medicine and director of the UC Davis Pediatric Telemedicine Program.

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UC Irvine’s licensing officers help campus researchers protect their inventions.

Alvin Viray, UC Irvine

They call it “the baby monitor,” but it’s nothing like the ones sold at Babies “R” Us that alert parents when junior’s crying in his crib.

Developed by UC Irvine pediatrics professor Dr. Dan Cooper, the sophisticated wireless device can detect subtle movements in infants that signal increased risk of cerebral palsy, autism and other neurological disorders. It’s outfitted with a sensor created by Pai Chou, UCI associate professor of electrical engineering & computer science, and it has a special application to warn of sudden infant death syndrome.

The baby monitor and sensor are just two of many important inventions conceived at UCI. The campus holds 316 active U.S. patents and 360 foreign ones for ideas and products that do everything from quieting jet noise (Dimitri Papamoschou’s Mach Wave Elimination) to restoring hearing (Fan-Gang Zeng’s cochlear implants).

And, no matter which lab or department they originate from, all fall under the careful eye of UCI’s Office of Technology Alliances. The OTA handles the patenting and licensing of the campus’s intellectual property. It helps UCI employees – primarily faculty and graduate students – protect and market their ideas.

“We’re the liaison between the lab bench and the marketplace,” says Doug Crawford, senior licensing officer for UCI. “We also create alliances with companies in the private sector so that campus research has the greatest positive impact.”

The OTA’s seven officers meet frequently with investigators to learn about their latest projects. “It’s fun to see all this great new stuff,” Crawford says, “and how excited the researchers are about what they’ve invented and what their creations can do for people.”

He recently began working on a patent for a wastewater treatment devised by Betty H. Olson, civil & environmental engineering professor. “It’s not the most glamorous invention – it’s a kit for sewage,” Crawford says. “It detects bacteria that bloom in the water early, before it grows out of control and becomes a lot more expensive to treat. Her technology saves both energy and water.”

Dr. J. Stuart Nelson developed UCI’s No. 1 revenue-producing invention, the Dynamic Cooling Device, which boasts more than $40 million in royalties. The attachment allows medical lasers to penetrate deep into the skin without burning, substantially reducing pain.

“It’s great,” Crawford says. “They did a test spot on my hand with the cooling device. Then they used the laser without it, and – ow! – that hurt.” Nelson created the product for treating birthmarks and port-wine stains. Now it’s standard in all kinds of laser procedures, such as tattoo removal and wrinkle reduction.

Other leading inventors at UCI include Hans Keirstead, who holds worldwide patents for his work with stem cells and the regeneration of damaged spinal cords; Frank LaFerla, director of UCI’s Institute for Memory Impairments & Neurological Disorders (UCI MIND), who has pioneered therapies for cognitive disorders; and Jean-Claude Falmagne, professor emeritus of cognitive sciences and creator of a software program called ALEKS (Assessment & Learning in Knowledge Spaces), which helps children develop learning skills.

While benefiting people by advancing health care, technology and other fields, inventions also benefit the University of California by generating revenue for further research and education.

Intellectual assets belong to UC. Patent income is divided three ways, with UC receiving 50 percent, the inventor pocketing 35 percent, and 15 percent going to the academic department where the idea originated.

All UCI employees must file a record of invention disclosing their creation to the OTA. In 2010-11, the campus had 180 new ROIs. “We review them for patentability and commercial viability,” Crawford says.

Each licensing officer has a different specialty – such as medical devices, microbiology or engineering – to facilitate the complex patent application process. Some have degrees in law or business. “We’re in each other’s offices on a regular basis,” Crawford notes.

His background is in plasma physics. An inventor himself, he holds patents for electrodeless lighting – an alternative to fluorescent bulbs – which he came up with as a researcher at Lawrence Berkeley National Laboratory.

Once OTA enters into negotiations with a company for licensing an invention, the office must tread carefully:

“We don’t want them sitting on it to protect their own [possibly competing] product,” Crawford says. “We make sure they intend to get our invention out to the broadest market.”

In addition, the office assists faculty in launching startup companies to manufacture an invention, as with the HIPerWall.

The OTA continues to manage and protect UCI patents until they expire 20 years from the date of filing – and sometimes beyond if an idea is still commercially viable.

“We’re here from cradle to grave,” Crawford says.

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UC Berkeley neuroscientists’ findings offer hope to those who suffer from tinnitus.

Neuroscientists at the University of California, Berkeley, are offering hope to the 10 percent of the population who suffer from tinnitus – a constant, often high-pitched ringing or buzzing in the ears that can be annoying and even maddening, and has no cure.

Their new findings, published online last week in the journal Proceedings of the National Academy of Sciences, suggest several new approaches to treatment, including retraining the brain, and new avenues for developing drugs to suppress the ringing.

”This work is the most clearheaded documentation to this point of what’s actually happening in the brain’s cortex in ways that account for the ongoing genesis of sound,” said Michael Merzenich, professor emeritus of otolaryngology at UC San Francisco and inventor of the cochlear implant, who was not involved with the research. “As soon as I read the paper, I said, ‘Of course!’ It was immediately obvious that this is almost certainly the true way to think about it.”

Merzenich is also chief scientific officer at Posit Science, which develops software to retrain the brain, primarily to improve learning and memory but more recently to address problems like schizophrenia, Alzheimer’s Disease and tinnitus.

“Two million Americans are debilitated by tinnitus; they can’t work, they can’t sleep. Its life destroying and a substantial cause of suicide,” he said. “These experiments have led us to rethink how we attack the tinnitus by our training strategies.”

According to coauthor Shaowen Bao, adjunct assistant professor in the Helen Wills Neuroscience Institute at UC Berkeley, tinnitus – pronounced TIN-it-tus or tin-NIGHT-us – is most commonly caused by hearing loss. Sustained loud noises, as from machinery or music, as well as some drugs can damage the hair cells in the inner ear that detect sounds. Because each hair cell is tuned to a different frequency, damaged or lost cells leave a gap in hearing, typically a specific frequency and anything higher in pitch.

Experiments in the past few years have shown that the ringing doesn’t originate in the inner ear, though, but rather in regions of the brain – including the auditory cortex – that receives input from the ear.

Bao’s experiments in rats with induced hearing loss explain why the neurons in the auditory cortex generate these phantom perceptions. They showed that neurons that have lost sensory input from the ear become more excitable and fire spontaneously, primarily because these nerves have “homeostatic” mechanisms to keep their overall firing rate constant no matter what.

“With the loss of hearing, you have phantom sounds,” said Bao, who himself has tinnitus. In this respect, tinnitus resembles phantom limb pain experienced by many amputees.

One treatment strategy, then, is to retrain patients so that these brain cells get new input, which should reduce spontaneous firing. This can be done by enhancing the response to frequencies near the lost frequencies. Experiments over the past 30 years, including important research by Merzenich, have shown that the brain is plastic enough to reorganize in this way when it loses sensory input. When a finger is amputated, for example, the region of the brain receiving input from that finger may start handling input from neighboring fingers.

Bao noted that retraining the ear has been tried before, but with limited success. Most such attempts have taken patients with some residual hearing and trained their ears to be more sensitive to the affected frequencies. This wouldn’t work for patients with profound hearing loss, however.

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UC Irvine team develops iPhone app that mimics a hearing aid and produces basic hearing tests.

UC Irvine doctors and a medical student are preparing to release the second version of EarTrumpet – the only iPhone hearing assistance application that combines adjustable volume controls with a hearing test that lets users tailor sound enhancement to their needs.

The idea grew from a lecture Dr. Hamid Djalilian gave about hearing aids last spring in Dr. Brian J.F. Wong’s biomedical engineering class. Noting the high price of hearing aids, Djalilian and Wong chatted afterward about the need for a low-cost alternative and how iPhones, iPads and iPod touches are stigma-free fashion accessories. Wong met with medical student Allen Foulad, who saw the potential in such a device and had the computer skills necessary to develop it.

They set out to create a tool with the functionality of a commercial hearing aid and the sophistication of a professional audiology test. Djalilian offered advice about features, and Foulad programmed the app and designed the user interface.

“Other ‘hearing aid’ applications simply make sound louder at all frequencies,” says Djalilian, director of otology, neurotology and skull base surgery at UC Irvine Medical Center. “We wanted it to work just like a hearing aid, where one can choose which pitches to amplify. People usually have high-pitch hearing loss and don’t need to amplify low-pitch sounds.”

Allowing the user to adjust pitch volumes, however, led to another challenge, he says: “Most people don’t know which pitches need amplification or by how much. So we added a hearing test that permits the user to identify and then fine-tune just the pitches where there is hearing loss.” Commercial hearing aids rely on audiologists to adjust the pitch amplification.

Other features include an intense volume boost, preset equalizer profiles that reduce background noise and enhance common frequencies, and adjustable volume for each ear.

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UCSF study reveals that softened bone leads to hearing loss; restoring hardness restores hearing.

(Left) Tamara Alliston and Jolie Chang, UC San Francisco

Scientists are reporting the first direct evidence that a subtle change in the physical properties of a tissue can affect its function. The finding has immediate implications for understanding several rare hearing disorders, they said, and ultimately could offer insight into such conditions as osteoporosis, arthritis, cardiovascular disease and cancer.

In their study, the scientists discovered that blocking the function of a particular molecule in the ear bone of mice decreased the hardness of the bone, causing hearing loss. Reactivating the molecule restored the bone’s hardness — and the animals’ hearing.

The research likely explains the previously unknown cause of hearing loss in the human disease cleidocranial dysplasia, a genetic bone syndrome, said co-author Dr. Lawrence Lustig, UCSF professor of otolaryngology, and may explain hearing loss associated with some other bone diseases.

More broadly, the finding reveals the molecular pathway that regulates the physical properties of extracellular matrix — the interlocking mesh of molecules between cells — in the ear’s cochlear bone. The matrix is responsible for the hardness of human tissues, ranging from stiff bone and enamel to compliant brain and skin.

Perhaps most intriguing is the discovery that variations in the physical properties of extracellular matrix affect tissue function. This finding should lead to insights into abnormal matrix properties in the tissues of diseases throughout the body, the researchers said, including osteoporosis and arthritis.

“Our finding demonstrates that establishing and maintaining the proper calibration of physical properties is essential for healthy tissue function,” said the senior author of the study, Tamara Alliston, assistant professor of orthopaedic surgery and a member of the Eli and Edythe Broad Center of Regeneration Medicine and Stem Cell Research at UCSF.

Scientists have known that physical cues, such as extracellular matrix stiffness, direct the differentiation of stem cells into specific cell types, such as heart, liver and brain cells. They also have known that disruption of these cues underlies a wide range of diseases, such as osteoarthritis, cardiovascular disease and cancer.

However, they have not known the molecular mechanisms that establish the physical properties of extracellular matrix, nor the link between these properties and tissue function.

In the current study, recently reported in EMBO (online Sept. 17, 2010), the team, led by Dr. Jolie L. Chang, a resident in the UCSF Department of Otolaryngology and Head and Neck Surgery, set out to investigate the mechanisms involved.

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African American and Hispanic children are at increased odds of not being able to afford prescription medications, not having medical insurance and not being able to see a specialist.

Nina Shapiro, UCLA

Ear infections are one of the most common health problems for children, with most kids experiencing at least one by their third birthday. Annual costs in the United States alone are in the billions of dollars.

When these infections are left untreated, complications can include hearing loss, speech problems and more severe infections that can spread to bone and brain, causing meningitis. But not all kids have the same access to medical specialists and medicines.

A new study by researchers at the David Geffen School of Medicine at UCLA and Harvard Medical School has found that racial and ethnic disparities among children with frequent ear infections can significantly influence access to health care resources.

The findings, published in the November 2010 issue of the journal Otolaryngology—Head and Neck Surgery, show that compared with white children, African American and Hispanic children are at increased odds of not being able to afford prescription medications, not having medical insurance and not being able to see a specialist.

The study also shows that African American and Hispanic children are more likely than white children to visit the emergency room for an ear infection.

“Our goal was to provide an accurate demographic picture of the U.S. so that we could identify disparities to target for intervention,” said study co-author Dr. Nina Shapiro, director of pediatric otolaryngology at Mattel Children’s Hospital UCLA and an associate professor of surgery at the Geffen School of Medicine. “Clearly, we found that children of certain ethnicities who suffer from frequent ear infections are more likely to face greater barriers to care. This information provides an opportunity for improvements in our current health care reform.”

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Janice Chang’s interest in hearing began at age 10, when she became fascinated by a classmate’s cochlear implant. It continued as she earned a bioengineering degree at UC Berkeley — coming home to Irvine each summer to work in the lab of UC Irvine’s Fan-Gang Zeng, a world-renowned hearing and speech disorders researcher.

Now, as an M.D.-Ph.D. student at UCI, Chang is focused on finding new treatments for tinnitus. More than 50 million Americans have some degree of prolonged ringing in the ears, and — like snowflakes — no two cases are the same. Frequency and intensity vary widely, from subtle background noise to a debilitating din requiring medical attention.

There is no cure, and management strategies work only for some. In addition, the number of sufferers is expected to grow: Studies indicate that long-term use of earbuds can cause irreversible hearing damage potentially leading to tinnitus, and the affliction is common among veterans returning from the Middle East.

Chang is trying to determine whether cochlear implants can help in the most severe cases, and she has found that electronic stimulation of the auditory system shows promise in suppressing the persistent ringing.

“Tinnitus can be devastating, and it affects so many people,” says Chang, who was recently awarded a $10,000 Public Impact Fellowship from UCI’s Graduate Division. “Hopefully, our efforts to identify an effective pattern of stimulation will yield an approach that can dramatically improve a person’s quality of life.”

Chang is in the right place. Besides Zeng’s groundbreaking work on cochlear implant design and his clinical research on sound suppression of tinnitus with UCI otolaryngologist Dr. Hamid Djalilian, UC Irvine Healthcare boasts one of the nation’s premier tinnitus treatment centers.

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Loss of spiral ganglion neurons or hair cells in the inner ear is the leading cause of congenital and acquired hearing impairment. Researchers at the UC San Diego School of Medicine and the National Institutes of Health found that Sox2, a protein that regulates stem cell formation, is involved in spiral ganglion neuron development. The study was published in the Jan. 13 issue of the Journal of Neuroscience.

“These findings may provide the first step toward regenerating spiral ganglion neurons, the nerve cells that send sound representations to the brain,” said Alain Dabdoub, Ph.D., co-investigator and assistant professor of surgery with the division of otolaryngology at the UC San Diego School of Medicine. “This has significant implications for advances in cochlear implant technology and biological treatments for hearing loss.”

In the cochlea, auditory neurons transmit sound vibrations conveyed by hair cells. These vibrations are then converted to nerve impulses that communicate with the brain. If the neurons are lost or damaged, hearing loss occurs. Existing therapies for hearing loss are based on either increasing hair cell stimulation with hearing aids or introducing an electronic substitute for the hair cells with cochlear implants. In either case, the presence of functional spiral ganglion neurons is required for a successful outcome.

Prior research shows that as few as 10 percent of the normal number of spiral ganglion neurons is sufficient for the success of cochlear implants.

“The identification of factors that induce functional neurons has important implications for hearing restoration,” said Chandrakala Puligilla, Ph.D., a research fellow at the National Institutes of Health. “The ability to induce even a small number of cells with gene-based therapy could be enormously beneficial.”

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Ava Martin seems less nervous than her parents as the three sit in an audiologist’s office at UC Irvine Medical Center a few days after Labor Day. In August, the 6-year-old had surgery to place a cochlear implant in her right ear. Now Ava plays with toys while Ginger Stickney describes to Dave and Gabrielle Martin the tests that will gauge how their daughter’s auditory nerve is responding to the implant. But first Stickney must activate the device that could restore function to Ava’s right ear – an ability lost years ago due to a congenital inner-ear defect that’s also destroying the hearing in her left ear.

While a hearing aid amplifies sound, a cochlear implant is designed to bypass nonfunctioning or damaged cells in the inner ear and directly stimulate the auditory nerve. A receiver, electrode system and magnet – all the size of a quarter – are placed under the skin behind the ear. Electrodes are surgically inserted into the cochlea, the part of the inner ear that converts sound waves into electrical impulses that are conveyed to the brain and processed as sound. An external speech processor affixed to the scalp by magnets completes the device.

Originally intended to enhance speech recognition – as an aid to lipreading – cochlear implants these days contain multiple electrodes, enabling users to hear a range of pitches. “Now we can get people to recognize 80 to 90 percent of words,” Stickney says, “without visual cues.”

UCI treats an estimated 5,000 hearing-impaired people annually, with about four receiving cochlear implants each month, says Dr. Hamid Djalilian, who operated on Ava.

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