Regenerating sensory hair cells, which produce electrical signals in response to vibrations within the inner ear, could form the basis for treating age- or trauma-related hearing loss. One way to do this could be with gene therapy that drives new sensory hair cells to grow.

Researchers at Emory University School of Medicine have shown that introducing a gene called Atoh1 into the cochleae of young mice can induce the formation of extra sensory hair cells.

Their results show the potential of a gene therapy approach, but also demonstrate its current limitations. The extra hair cells produce electrical signals like normal hair cells and connect with neurons. However, after the mice are two weeks old, which is before puberty, inducing Atoh1 has little effect. This suggests that an analogous treatment in adult humans would also not be effective by itself.

The findings were published May 9 in the Journal of Neuroscience.

“We’ve shown that hair cell regeneration is possible in principle,” says Ping Chen, PhD, associate professor of cell biology at Emory University School of Medicine. “In this paper, we have identified which cells are capable of becoming hair cells under the influence of Atoh1, and we show that there are strong age-dependent limitations on the effects of Atoh1 by itself.”

The first author of the paper, Michael Kelly, now a postdoctoral fellow at the National Institute on Deafness and Other Communication Disorders, was a graduate student in Emory’s Neuroscience program.

Kelly and his coworkers engineered mice to turn on the Atoh1 gene in the inner ear in response to the antibiotic doxycycline. Previous experimenters had used a virus to introduce Atoh1 into the cochleae of animals. This approach resembles gene therapy, but has the disadvantage of being slightly different each time, Chen says. In contrast, the mice have the Atoh1 gene turned on in specific cells along the lining of the inner ear, called the cochlear epithelium, but only when fed doxycycline.

Young mice given doxycycline for two days had extra sensory hair cells, in parts of the cochlea where developing hair cells usually appear, and also additional locations (see accompanying image).

The extra hair cells could generate electrical signals, although those signals weren’t as strong as mature hair cells. Also, the extra hair cells appeared to attract neuronal fibers, which suggests that those signals could connect to the rest of the nervous system.

“They can generate electrical signals, but we don’t know if they can really function in the context of hearing.” Chen says. “For that to happen, the hair cells’ signals need to be coordinated and integrated.”

Although doxycycline could turn on Atoh1 all over the surface of the cochlea, extra sensory hair cells did not appear everywhere. When they removed cochleae from the mice and grew them in culture dishes, her team was able to provoke even more hair cells to grow when they added a drug that inhibits the Notch pathway.

Manipulating the Notch pathway affects several aspects of embryonic development and in some contexts appears to cause cancer, so the approach needs to be refined further. Chen says that it may be possible to unlock the age-related limits on hair cell regeneration by supplying additional genes or drugs in combination with Atoh1, and the results with the Notch drug provide an example.

“Our future goals are to develop approaches to stimulate hair cell formation in older animals, and to examine functional recovery after Atoh1 induction,” she says.

The research was supported by the National Institute on Deafness and Other Communications Disorders, the National Basic Research Program of China and the Natural Science Foundation of China.

The complexion of a $2 billion, 10-year settlement between the state of Virginia and the U.S. Department of Justice involving the closing of state training centers for people with disabilities could be significantly altered by a decision to allow families opposing the settlement to intervene.

U.S. District Judge John A. Gibney Jr. entered an order Wednesday that will allow into the case families who oppose removal of their loved ones from state training centers to community care facilities.

The families argued in legal briefs this year that the state is not equipped to handle community-based care of their family members and that to force them from state training centers where some clients have lived for decades amounts to a violation of their civil rights.

“This means a lot to all the parents and guardians but especially to those in their 70s, like me and my wife,” said Wriley Wood, whose daughter Peggy, 51, is the lead plaintiff among opposing families and has been receiving care at Central Virginia Training Center in Lynchburg, Va. for decades.

The proposed settlement, which was reached in January and must be approved by Gibney, would close three of four state training centers and downsize the fourth.

Some $2 billion would be pumped into community care and waiver slots that serve as the gateway for funding care to individuals. About 1,100 people reside in the Virginia centers, located in Lynchburg, Petersburg, Fairfax and Chesapeake.

Gibney concluded that the families “have a significant, protectable interest in receiving the appropriate care of their choice and protecting their rights” under the Americans with Disabilities Act.

The settlement is designed to gradually remove hundreds of patients from unnecessary institutional care, but the families approved by Gibney argue that a separate class of people would be harmed by removal because community-based care would impose on their loved ones a more costly and potentially harmful way of life.

Wood and other plaintiffs, many of them in Northern Virginia, liken the state-federal plan to an eviction.

However, state and national organizations that advocate for those with disabilities have fiercely defended the settlement that promises tens of millions of dollars in new money and a gradual shift away from institutional care and the state’s poor record of funding help.

“We remain very optimistic that the judge will approve the excellent and fair settlement and we look forward to presenting the views of our families and members to the court,” said Jamie Liban, executive director of The Arc of Virginia.

Gibney set out a 16-day schedule for filing arguments in response to the families’ motion to dismiss the federal-state agreement.

With the summer pool season approaching, a battle is brewing between advocates for people with disabilities and hotel owners over how to make public swimming pools more accessible.

At the center of the dispute is a new regulation that requires hotels and recreation centers that operate public pools and spas to install or order permanent lifts — costing between $2,500 and $6,500 each, plus installation — by May 21. The requirement also can be satisfied by pool ramps, which are much more expensive.

Better pool access is long overdue, said James Moses, a paralyzed Vietnam War veteran from San Pedro, Calif. At age 67, he said, he is too frail to get into a pool lift without help. But he would like hotels to offer access to all guests with disabilities.

“We want to be acknowledged,” he added. “I want to go to a hotel and say I had a good time.”

Hotel owners say they want to improve pool access. But many are resisting the new regulation and are supporting federal legislation to extend the deadline. Introduced in Congress in March, the legislation would let pool operators comply by buying portable lifts and delay implementation for 12 months.

Permanent and portable lifts cost nearly the same, but installation of permanent lifts greatly increases the cost, experts say. Pool manufacturers estimate that the law applies to about 256,000 pools and spas across the country. Only a small percentage of those pools are equipped with lifts, they estimate.

The lifts resemble small cranes that stand at a pool’s edge. They basically lower swimmers in a chair into the water. Portable lifts can be rolled away from the pool when not in use. The cost to install a permanent lift ranges from $1,000 to $3,000, in addition to the cost of the lift itself, according to industry officials.

Hotel industry representatives say they are concerned that permanent lifts that are left unattended may be a safety hazard for children, who will be tempted to play on them.

A 12-month delay is also needed, they said, because a backlog of orders for permanent lifts makes it impossible to meet the May deadline.

“It gives us an opportunity to work to assure we have the equal access to pools at all our hotels,” said Kevin Maher, senior vice president of government affairs for the American Hotel & Lodging Association, the trade group for the nation’s hotel industry.

Meanwhile, some hotel chain operators are rushing to install permanent lifts.

Marriott International Inc., which operates more than 3,400 hotels under 13 brands in the U.S., is “in the process of ordering the lifts” in time to meet the deadline, said company spokeswoman Laurie Goldstein. “We are moving ahead to get them installed.”

Disability advocates say the hotel industry has been the loudest opponent of the law. They say hotel owners knew about the requirements for nearly two years and should be prepared to meet the deadline.

“I think it needs to be abundantly clear that these rules need to be enforced,” said Lara Schwartz, vice president of external affairs for the American Association of People with Disabilities. “We can’t just kick this can down the road.”

But some people with disabilities said they think portable lifts would be enough.

Scott Souza of Manteca, south of Stockton, Calif., supports the use of portable lifts. He became paralyzed in a motorcycle accident 10 years ago, and he said hotels should not be required to spend the extra money to install permanent lifts when portable lifts work just as well.

“I understand where the law is coming from,” he said. “But it seems like it’s going too far, particularly for small mom-and-pop hotels that can’t afford the cost.”

And he said he doesn’t mind having to ask a hotel worker to bring out a portable lift every time he wants to swim. “I’m a mid-level quadriplegic,” Souza said. “I have to deal with that stuff every single day.”

The Americans With Disabilities Act calls for the owners of public pools to install lifts or underwater ramps to provide access for people with disabilities. Many in the hotel industry assumed they could comply with the law by using portable lifts. That changed when the Justice Department issued an interpretation of the law Jan. 31, saying the law calls for permanently affixed lifts where feasible and affordable.

In response to an outcry from hotel owners, the Justice Department delayed the deadline to comply until May 21. It initially was March 15.

Pool operators who do not comply with the regulations may be vulnerable to lawsuits, according to hotel industry experts.

To avoid litigation, some hotel owners who are not prepared to meet the May 21 deadline may close their pools, said Maher, the hotel trade group representative. “The concern is that we could be vulnerable to drive-by lawsuits,” he said, referring to suits by lawyers seeking only to collect attorney fees.

Often reviled for encouraging kids to spend too much time in front of screens, new research suggests that some video games may actually benefit those with cerebral palsy.

The finding comes from a new study in which researchers observed 17 children with cerebral palsy as they played four “active games” on the Nintendo Wii — Bowling, Tennis, Boxing and Dance Dance Revolution.

They found that the games encouraged repetitive movements, while providing positive feedback in a fun environment, according to the study published online this week in the Archives of Physical Medicine and Rehabilitation.

Significantly, the researchers said children with cerebral palsy who typically utilized one, dominant side of their body were engaging their full body when playing the games, suggesting that the activity could be a low-impact way of achieving therapeutic goals.

“While our results did not show that (active video) game play can be regarded as a replacement for more vigorous physical activity or muscle strengthening, we found that some games may provide targeted therapy focused on specific joints or movements,” said Elaine Biddiss of the University of Toronto who led the study.

“Future development and optimization of AVG technologies may usher in a new age in physical rehabilitation where virtual environments provide an arena for neuroplastic change in the comfort of one’s home,” she said.