Newswise — The University of Miami Miller School of Medicine Department of Otolaryngology has received a new $3.2 million, five-year grant from the National Institutes of Health (NIH) to support their multidisciplinary translational research program on human genetic hearing loss (HL), a common sensory disorder affecting more than 28 million Americans.
Led by PI Xue Zhong Liu, M.D., Ph.D., professor of otolaryngology, human genetics, biochemistry and pediatrics, the longstanding program, continuously funded since 2001, includes investigators from the Dr. John T. MacDonald Department of Human Genetics, the Hussman Institute for Human Genomics and the Department of Biology.
HL affects 15-26% of the world’s population and is the most common neurological disability among the elderly. Given the high prevalence and debilitating consequences associated with HL, it constitutes a major health and socioeconomic burden on all populations. With an estimated 115% increase in the geriatric population over 65 years of age by the year 2050, the number of hearing/balance-disordered patients with a declining sensory system is certain to reach near-epidemic proportions, according to the National Institute of Deafness and Other Communication Disorders of the NIH.
Cognitive decline such as Alzheimer’s disease and other forms of dementia are associated independently with hearing loss in the elderly population.
“The health care burden and human/economic costs of hearing loss and associated conditions such as cognitive decline are ever increasing,” according to Fred Telischi, M.D., M.E.E., James R. Chandler Chair and professor of otolaryngology, neurosurgery and biomedical engineering. He added, “Dr. Liu’s team and the UM co-investigators are leaders in understanding the genetic and molecular bases for many forms of hearing loss leading to more comprehensive management paradigms for the tsunami of hearing disordered patients expected in the very near future.”
The grant will allow Dr. Liu, who is the vice chair of Department of Otolaryngology and the Marian and Walter Hotchkiss Chair in Otolaryngology and one of the top NIH-funded surgeon-scientists in the specialty, to continue the longstanding research program identifying novel hearing loss genetic mutations and possible treatments. Having also received another NIH $3.5 million grant to initiate gene therapy research for hearing loss in 2022, Dr. Liu is currently the “most successfully funded genetic hearing loss researcher in the U.S.,” according to the announcement.
“We’re pleased that the NIH and scientific community have continued to validate the importance of the University of Miami’s Genetic Deafness Program and the research we are conducting,” said Dr. Liu. Within this long-standing grant funded program, Dr. Liu’s team will expand both the search for underlying coding and non-coding variants in patients/families with NSHL using state-of-the-art genomic/epigenetic tools and the initiation of preclinical studies of gene- and cell-based therapy approaches of CRISPR/Cas9-mediated genome editing to treat hearing loss.
The grant will also allow expansion of UM’s study into minority focused populations affected by hearing loss and enhance the investigation of novel disease mechanisms through cellular/tissue assays and animal models of rare and common human deafness genes/variants.
Even with current therapeutic advances, such as hearing aids and cochlear implants, deafness exerts a significant negative effect on the quality of social, family, and professional lives of the deaf and hearing impaired. This new grant will enable scientists to translate basic research findings into clinical tools and the development of novel treatments for hereditary deafness.
“Recent breakthroughs in genetic screening and gene-based therapeutics using novel gene editing for the inner ear can lead to novel therapies for multiple classes of hereditary hearing loss,” said Dr. Liu. “Together with our program for genetic hearing loss, these tools and strategies will create a clear path to clinical treatment and accelerate the advent of a new era of personalized medicine for hearing loss.”
The Genetic Hearing Loss Clinic at the University of Miami Ear Institute is a multi-disciplinary program that provides diagnostic, molecular testing, genetic counseling and intervention options for patients and families who are dealing with various types of hearing impairment.
Co-investigators of the grant are Susan Blanton, Ph.D., Stephan Züchner, M.D., Ph.D., and Anthony Griswold, Ph.D. of the Dr. John T. MacDonald Department of Human Genetics and the John P. Hussman Institute for Human Genomics, and Dr. John Lu of the University of Miami Department of Biology.