The AHRF will be transitioning throughout the months of May and June to a non-profit management firm, to be selected in the coming weeks. Meanwhile, Sharon will continue to ensure that the Foundation continues to run smoothly, and she will be instrumental in managing the transition.

The Foundation remains commited to its mission to fund hearing research as it has been for more than 50 years. Research proposals can still be emailed to sparmet@american-hearing.org, and will be processed as usual. Stay tuned for information about our address change and email contact change.

Christina L. Runge, PhD, Associate Professor, Otolaryngology & Communication Sciences and Physiology at the Medical College of Wisconsin, Milwaukee, and colleagues will investigate the feasibility of a multi-institutional, multi-disciplinary genetic hearing screening program for newborns. The screening will look for the most common genetic markers known to cause hearing loss at birth or predispose children to permanent hearing loss from environmental factors. To watch a video about the research, go to the AHRF’s YouTube page.

Congenital hearing loss affects approximately one in 1,000 babies born in the United States each year, and it is estimated that half of the cases are genetic, or inherited. Since 2009, universal screening of newborns for hearing loss has been standard in all 50 states, and currently 97% of newborns are screened for hearing loss at the time of birth by measuring an infant’s responses to sound stimuli. However, these tests are only able to identify hearing loss present at the time of the screening, and they do not provide information about the cause of hearing loss. Several forms of hearing loss develop later in life, but so far, children at risk for these kinds of inherited hearing loss are not routinely tested and identified before the hearing problems start. By developing an effective protocol to genetically test newborns for mutations known to contribute to hearing loss at birth or later in life, appropriate follow up procedures, interventions and therapies can be started earlier in children who are at risk.

“The genetic markers of hearing loss we test for cause progressive and profound hearing loss, some of which would not necessarily be caught by the newborn physiological hearing screening performed soon after birth. Even if hearing loss was identified this way, we would not know the cause. By testing for these markers, we could have more information that would help us make the most appropriate treatment recommendations for the child. For example, we may identify children who might be candidates for treatments like cochlear implants. There is a relationship between the age of implantation and the ability to develop language skills; therefore, the earlier we can identify these children to get them involved in therapy and fully assess their treatment options, the better,” says Dr. Runge.

The screening will look for the most common mutations linked to congenital hearing loss and those that predispose patients to permanent hearing loss from environmental causes, such as certain ototoxic medications (medications that damage hearing cells in the inner ear). “It is well known that people who carry a specific genetic mutation are much more susceptible to medication-induced hearing loss,” says Dr. Runge. “Some antibiotics used to treat serious illnesses are known to cause significant permanent hearing loss, so knowing whether you have this genetic marker can be critical for your health care. A physician may be able to use a different antibiotic and hearing would be preserved.”

A cochlear implant consists of an external speech processor that picks up sound from the environment with a small microphone, and a transmitter/receiver that receives signals from the speech processor and transmits them as electrical signals to a thin, filamentous electrode array that is surgically threaded into the cochlea. The array has several electrodes or contact points along its length that deliver electrical impulses to spiral ganglion neurons in the snail-shaped cochlea. The auditory nerve carries the signals to the brain where they are translated into different frequencies of sound.

The American Hearing Research Foundation is funding Esperanza Bas Infante, PhD, an associate scientist at the University of Miami who is investigating how and why scar tissue forms along the cochlea after the electrode array is implanted. “When anything is surgically implanted in the body, there is an inflammatory response, which can lead to the formation of tough, fibrotic scar tissue,” Dr. Bas says. “If we can figure out how to interfere with the formation of scar tissue along the array, which happens in some people after cochlear implantation, it will improve the efficacy of the CI and the quality of life of the patient.” Scar tissue can also be a real problem if a CI fails to work properly and needs to be replaced through a second surgery. The AHRF grant is for $20,000 to support research for one year.

Dr. Bas’s research will focus on the molecular mechanisms behind fibrous tissue and bone formation (another form of scar tissue) that can occur after cochlear electrode placement in mice. Wound healing is a complex process involving a cascade of immune and chemical responses with scar tissue formation being one possible outcome. By better understanding this process, which is the aim of her AHRF grant, Dr. Bas hopes to then look at ways the immune response can be mediated using different factors to control scar tissue formation in the cochlea. To see a video of Dr. Bas talking about her research, click here.

The researchers, led by Sumit Dhar, PhD, Associate Professor in the Department of Communication Sciences & Disorders, Northwestern University, Evanston, Illinois, David A. Klodd, PhD, Professor of Audiology at the University of Illinois, Chicago, and Jonathan Siegel, Associate Professor in the Department of Communication Sciences & Disorders, Northwestern University, Evanston, Illinois hope that their new test will be able to significantly reduce the number of chemotherapy patients who experience hearing loss due to treatment.

The new test is actually a significant improvement on a currently-used testing technique called distortion-product otoacoustic emissions, or DPOAEs. DPOAEs deliver two tones to the ear and then measures the ‘echo’ from the interacting waves emanating from the outer hair cells of the cochlea in the inner ear. This test can assess hearing loss in a wide range of frequencies from low pitches to high pitches, but its efficacy at determining hearing loss in the high frequencies tops out at less than 6 kHz. The AHRF researchers have made dramatic improvements in the test hardware and test algorithm to enable measurement of DPOAEs up to 20 kHz.

Hearing loss in this range is usually not initially noticed, but losses in this range often lead to subsequent losses in hearing in the lower frequencies, so being able to determine hearing loss above 6 or 8 kHz, can help drive treatments and preventive measures to stop the hearing loss from “spreading” into lower frequencies, where they are much more noticeable to the patient and can negatively impact quality of life and communication. “Hearing loss in these ranges often indicates that the loss is going to start expanding through the lower frequencies where hearing loss is actually perceived as a problem. By identifying HL at these high frequencies, steps can be taken to stop its progression,” says Dr. Klodd.

The researchers are investigating the efficacy of their DPOAE test in patients undergoing chemotherapy containing the drug cisplatin, an ototoxic drug known to cause hearing loss that starts in the higher frequencies above 8 kHz. They plan to test the new DPOAE sensor in approximately 20 patients receiving cisplatin chemotherapy treatment (as well as 20 control patients not receiving cisplatin) at the University of Illinois at Chicago. “These are patients who are already undergoing extremely stressful treatment and are coping with the fact that they may have a life threatening, or at the very least, life altering diagnosis. The last thing they need is to develop hearing loss over the course of their chemo therapy, or in the months following treatment, as so often happens in these cases where the patient is sensitive to cisplatin,” says Dr. Dhar. “The goal is to detect the hearing loss before the patient does, and to begin treatment and prevention protocols so that these patients can enjoy a better quality of life once they get better.”