Keck Medicine of USC’s Caruso Department of Otolaryngology – Head and Neck Surgery is revolutionizing the treatment of hearing loss.

Profound hearing loss affects every aspect of life. The inability to hear the sounds of nature, a loved one’s voice or the familiar beat of a favorite song often leaves patients feeling helpless and isolated. Whether caused by illness, injury or genetics, hearing loss not only affects one’s physical well-being, but also social, psychological, and cognitive health. In the last 20 years, the options available to help those locked in silence have grown tremendously with new technologies and surgical techniques. Keck Medicine of USC is at the forefront of these innovations.

The Caruso Department of Otolaryngology – Head and Neck Surgery offers one of the most comprehensive programs to manage hearing loss in both adults and children. The department is also involved in several clinical trials exploring cutting-edge technologies and research. In the past year alone, physicians and researchers have significantly expanded the depth and breadth of the facility’s diagnostic, clinical and therapeutic services, building on its world-renowned clinical and scientific expertise.

“We have an incredible team of scientists, and our goal is to continue to give them the tools they need to drive their success,” says John K. Niparko, MD, professor, chair of the Caruso Department of Otolaryngology – Head and Neck Surgery, and the Leon J. Tiber and David S. Alpert Chair in Medicine at the Keck School of Medicine.

Niparko is an internationally renowned otoneurologic surgeon and a leading authority on implantable technologies that enhance hearing for profoundly hearing-impaired individuals, including cochlear implants. He joined Keck Medicine of USC in 2013 from John Hopkins University.

Niparko was in high school when he started reading about the emergence of an implantable inner-ear device that made sounds audible to the profoundly deaf. The untapped possibilities available by combining advanced technology and clinical research to restore hearing captured his imagination. Niparko, past president of the American Otological Society, is currently the leading authority on cochlear implants, an electronic device that stimulates the auditory nerve allowing the user to recognize sounds.

His 12-year National Institutes of Health-funded study examining the effects of cochlear implantation on language, behavioral and societal outcomes in children, now in the analysis phase of outcomes, is the longest observation time to date. Early indications demonstrate that “implantable devices can have a remarkable impact. We have an incredible opportunity to impact the quality of life for children and adults with severe hearing loss or deafness,” says Niparko.

Childhood Deafness

Watching children born deaf as they hear everyday sounds, learn to talk, read and thrive at school among hearing peers is one of the most rewarding parts of Niparko’s career. The recent launch of the USC-Children’s Hospital Los Angeles Center for Childhood Communication (C3) has been an exciting part of the tremendous growth within the department this past year. The Center provides audiology and speech language pathology services to children with hearing loss from birth to adulthood, including access to national clinical trials and state-of-the-art rehabilitative care.

Profound hearing loss occurs in one in 1,000 children at birth and one in 500 by age 5, according to Niparko. Childhood deafness impacts social, emotional, linguistic and intellectual development. Because children experience a sequence of critical developmental milestones in the first few years of life, early intervention through hearing technologies and rehabilitation has an enormous impact on a child’s long-term development.

The center’s comprehensive programs integrate research, clinical services and education to help its young patients academically, developmentally and socially. Each child is evaluated and tested to determine the degree and type of hearing loss and whether they would benefit from assistive devices such as hearing aids, bone conduction devices and cochlear implants. Specialists also provide comprehensive follow-up support and training needed for deaf children hearing sound for the first time. The rehabilitation team works closely with each patient’s family to ensure continuity of the training interpreting the signals transmitted by implants into the daily life of the child.

In addition to its clinical services, the center aims to serve as a major research and training facility and is already taking part in a number of National Institutes of Health (NIH)-funded research projects that focus on hearing and speech development. Seven NIH-funded scientists at Keck Medicine of USC are currently conducting vital research, bringing the latest developments directly to the center’s patients.

Acoustic Neuroma

Keck Medicine of USC is also home to the top surgeons treating acoustic neuroma, a benign tumor of the nerve that connects the inner ear to the brain. The region of the brain where this type of tumor grows is located near the back of the skull and controls hearing and balance. Patients come from around the world seeking the expert care available at Keck Medical Center of USC for this type of tumor.

“We have great harmony in the operating room and truly define a comprehensive treatment center,” says Rick A. Friedman, MD, PhD, director of the USC Acoustic Neuroma Center and division director of Otology, Neurotology and Skull Base Surgery. “We have an incredible team that helps us manage each patient following surgery.”

Friedman and his team are recognized worldwide as some of the leading experts in acoustic neuroma, offering each patient an individualized care plan created by a multi-disciplinary group of medical experts in neurotology, neurosurgery, radiation, internal medicine, nursing, neuroradiology, anesthesia, ophthalmology, otolaryngology, plastic and reconstructive surgery, audiology and physical therapy.

Expert surgeons at the USC Acoustic Neuroma Center are often able to identify the nerve where smaller tumors originate and use state-of-the-art imaging and laser surgery tools for precise treatment and greater preservation of hearing.

While these tumors do not spread to other parts of the body, they’re located deep inside the skull and are adjacent to areas that control vital functions. The most common early symptoms are loss of hearing, usually on one side, ringing in ears, dizziness and balance problems. The tumor can eventually cause numbness or paralysis of the face, headaches and mental confusion. If it continues to grow, it can press against the brain, causing life-threatening complications.

The best treatment option will depend on the size and location of the tumor, the patient’s age and health condition. Because this type of tumor is located critically close to vital brain structures, the goal of treatment is to eliminate the tumor without jeopardizing neurological function.

Age-Related Hearing Loss

Friedman, professor of otolaryngology and neurosurgery at the Keck School of Medicine of USC, is also working on the first ever and largest genome-wide association study for age-related hearing loss. Working with scientists at the Translational Genomics Research Institute and from the University of Antwerp in Belgium, Friedman says the team has identified a gene that could help explain why some people lose their hearing as they age.

Finding the genetic causes of age-related hearing loss could provide insight into the development of the disease and lead to treatment options for the millions of people worldwide, says Friedman. “This is an exciting time in the lab,” he says. “Once we understand the genetic pathways, we can begin precisely targeting treatment.”

Brainstem Surgery

The field of hearing loss management has seen tremendous innovation in the last two decades. The advancement in science is perhaps most evident in 3-year-old Auguste Majkowski, deaf since birth, who was recently able to detect sound for the first time inside the USC otolaryngology clinic. Scientists and surgeons from Keck Medicine of USC, Children’s Hospital Los Angeles and Huntington Medical Research Institutes were able to activate a hearing device that had been surgically implanted in Auguste’s brainstem.

Auguste was the first child in the United States to undergo an auditory brainstem implant (ABI) surgery in a U.S. Food and Drug Administration-approved trial supported by a National Institutes of Health clinical trial grant. The device uses an external processor on the ear to send sound to a surgically implanted electrode. Since Auguste’s procedure in May, two other children have also had ABI surgery. All three children had bilateral cochlear implants initially. But those cochlear implants proved to be unsuccessful because the children were born without an auditory nerve, which transmits sound from the inner ear to the brain.

The ABI surgery, device activation and future behavioral study are part of a five-year clinical trial in which 10 devices will be implanted in deaf children under the age of 5 and studied over the course of three years. The Los Angeles study, co-led by audiologist Laurie Eisenberg, PhD, is the only one in the United States to be supported by the NIH.

The study’s goal is to establish safety and efficacy protocols for the ABI surgery and to allow researchers to study how the brain learns to hear sound and develop speech over time. Eisenberg and her team are writing the manuals for all the procedures of this technology.

“When I first started practicing in the 1970s, there was not much we could do for patients identified as deaf. It was a very isolating experience that affected their work, family, overall quality of life,” says Eisenberg, professor of research otolaryngology at the Keck School of Medicine of USC. “The evolution is truly remarkable.”

Niparko sums up the feeling of momentum that fills the Department of Otolaryngology ­– Head and Neck Surgery. “This is an exciting time at USC.”

By Elena F. Epstein

Editor’s Note: In memoriam of John Niparko, former chair of the The Caruso Department of Otolaryngology – Head and Neck Surgery. Click here to honor his legacy of transformative research and care.