“…Given that over 50% of gene mutations responsible for hereditary HL affect HCs, gene therapy studies have primarily focused on these sensory cells ( Ahmed et al, 2017 ). Numerous mouse models bearing mutations in genes associated with HCs’ stereocilia, including Myo6 , Tmc1 , Ush1c , Ush1g , Whrn , Lhfpl5 , Pcdh15 , Clrn1 , Klhl18 , and Strc , have shown promising results with the application of AAV-mediated gene therapies ( Askew et al, 2015 ; Chien et al, 2016 ; Shibata et al, 2016 ; Emptoz et al, 2017 ; Geng et al, 2017 ; György et al, 2017 ; Pan et al, 2017 ; Dulon et al, 2018 ; Nist-Lund et al, 2019 ; Yoshimura et al, 2019 ; György et al, 2019a , b ; Yeh et al, 2020 ; Shubina-Oleinik et al, 2021 ; Wu J. et al, 2021 ; Gu et al, 2022 ; Isgrig et al, 2022 ; Liu et al, 2022 ; Xiao et al, 2022 ; Xue et al, 2022 ; Zheng et al, 2022 ). OTOF and VGLUT3 are mainly localized in IHCs and play a significant role in synaptic transmission.…”