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Ulrich Mueller

The Future of Deafness Research

Professor Ulrich Mueller, Ph.D., is one of the researchers at The Scripps Research Institute who is investigating the underlying causes and potential treatments of hearing loss. In fact, his passion is deafness.

"Deafness is so prevalent in humans, yet no pharmaceutical company is doing anything about it," Mueller says. "Also, of all the senses - vision, smell, and taste - it is the least well known.

Because of these factors, I feel I can make a real impact on health care through my research." Deafness is the most common form of sensory impairment in the human population, affecting over 30 million Americans and their families. More than half of all people over 75, and 80 percent of those over age 80, suffer some form of hearing loss. On the other end of the age spectrum, one in a thousand children is born with a hearing impairment. "Hearing loss leads to a significant decline in the quality of life and often to a sense of isolation," says Mueller. "To develop therapeutic strategies against hearing loss, it is critical that we understand how sound signals are processed by the ear and nervous system, and how such processing is perturbed in disease."

Opportunity for Interaction

Mueller - whose interest is in how the nervous system works, both from the perspective of basic science and possible treatment strategies - came to Scripps Research from the Friedrich Miescher Institute in Basel, Switzerland. Prior to his stint there, he received his Master's from the University of Cologne in Germany, earned a Ph.D. from Princeton University, and completed postdoctoral work at the University of California, San Francisco.

"I came to Scripps Research because of the opportunity for interaction with top biologists and chemists, both at the institute and other institutions in La Jolla," he says.

Much of his lab's attention has focused on the genes that cause Usher Syndrome. And about four in 10,000 babies born in the United States have Usher syndrome, a devastating genetic disease that causes deafness and blindness. Mueller's studies have shown this type of deafness is caused by defects in hair cells, which are situated in the inner ear and function as sensors for sound.

"Surprisingly, our recent findings show that one of the genes that is affected in Usher Syndrome is also linked to deafness from birth and age-related hearing loss," said Mueller. "We're targeting this one gene to deal with each of these diseases."

A Big Task Ahead

Mueller and others in the deafness research field have a big task ahead of them. Of the more than 400 genetic loci in the human genome that have been linked to deafness, only about 100 have been assigned to specific genes. According to Mueller, identifying relevant genes is critical for the development of diagnostic and therapeutic strategies.

"We're trying to get all this under control," said Mueller. "In my lab, we carry out positional cloning strategies to identify genes that are linked to deafness. We have recently linked several genes to different forms of hearing impairment, including age-related hearing loss. This should open up new therapeutics.

"We're also looking at ways to delay the deafness process. If we can eventually double the amount of time over a lifetime that it normally takes a person to go deaf, we won't need to develop a cure, since most people don't live until 130."

Mueller has seen much progress in the field, and he's hopeful for the future. "Deafness research is on the brink of some momentous breakthroughs," he says.

"Over the past decade, the field has gained momentum and we are now in a position to develop and test new therapeutic strategies that may lead within the next decade to advances in slowing age-related hearing loss, or even providing cures for some forms of deafness."

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 "Deafness is so prevalent in humans, yet no pharmaceutical company is doing anything about it," says Professor Ulrich Mueller. (Play video.)