Otolaryngology: Open Access
Open Access

Abstract

Genetic factors are among the main etiologies of severe to profound hearing loss and play an important role in cochlear implantation (CI) outcomes. Genomic technologies facilitate the discovery of new deafness genes at an unprecedented rate, thereby translating to improved patient care through more rapid diagnosis while also advancing our understanding of the molecular physiology of hearing and deafness. While genes for common forms of deafness have been cloned, efforts to correlate the functional outcome of CIs with a genetic form of deafness carried by the patient have been largely anecdotal to date. It has been suggested that the differences in auditory performance may be explained by differences in the number of surviving spiral ganglion cells, etiology of hearing loss, and other factors. Knowledge of the specific loci and mutations involved in patients who receive cochlear implants may elucidate other factors related to CI performance. In this presentation, current advances of genetic deafness and their impacts on CI will be discussed. There are few reasons not to offer cochlear implantation to children, such as advanced age or nonverbal language. Rather, many individuals implanted with genetic forms of hearing loss, especially those implanted at a young age, do remarkably well due to preservation of the spiral ganglion and upper CNS pathways. Future studies of larger cohorts of patients are necessary to support very positive preliminary findings. Additionally, greater focus should be placed on appropriate screening and counseling of individuals with hearing loss.

Biography

Xue Zhong Liu is a Professor of Otolaryngology, Human Genetics, Biochemistry, & Pediatrics and Vice Chair and Director of Research of the Department of Otolaryngology at the University of Miami. He completed general surgery and otolaryngology residency at University of Miami and obtained his PhD in the University of Manchester in England. He is board certified in Otolaryngology in USA. He is the author of over 140 papers in top journals and is the most successfully NIH funded Otolaryngologist in the world. He has received national and international recognition for his clinical activities and research in the field of otology.