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Friday, April 12, 2019

Study Uncovers 38 New Genes Related to Hearing Loss

​Thirty-eight genes previously unsuspected to be involved in hearing are actually linked to hearing loss, and they could be useful targets for the development of hearing restoration drugs, according to a new study (PLoS Biol. 2019 Apr 11;17(4):e3000194). A team of researchers from King's College London and the Wellcome Sanger Institute conducted a large-scale screen of new targeted mouse mutant lines using the auditory brainstem response. Of the 1,211 genes tested, 38 genes with no prior association with deafness had raised thresholds for detecting a response to sounds—five showed severe or profound deafness, 10 had raised thresholds at high frequencies only, two showed raised thresholds at low frequencies only, seven had moderately raised thresholds across frequencies, and 14 showed a mild hearing impairment. The 38 genes are involved in a broad range of functions, including transcriptional and translational regulation, chromatin modification, splicing factors, cytoskeletal proteins, membrane trafficking, and more, and some of them have links to existing pathways involved in deafness. The authors therefore posited it is likely that therapies will need to be directed at common molecular pathways involved in deafness rather than individual genes or mutations. The authors added that over 600 more genes required for normal auditory thresholds have yet to be found. Added to the 362 already known and 38 reported in this study, this indicates that there may be as many as 1,000 genes involved in deafness, a very high level of genetic heterogeneity.