GJB2 and Tinnitus: While the precise mechanisms underlying the emergence of tinnitus remain unclear and are likely multifactorial [90,92,93], cochlear implants have been shown to reduce tinnitus in individuals with severe hearing loss, suggesting that restoration of peripheral input may reverse these homeostatic changes [94,95] and that early intervention to restore hearing may improve functional outcomes in forms of congenital deafness arising from variants in Gjb2.