Connexin26 (GJB2) deficiency reduces active cochlear amplification leading to late-onset hearing loss.

ABSTRACT: Connexin26 (Cx26, GJB2) mutations account for >50% of nonsyndromic hearing loss. The deafness is not always congenital. A large group of these patients (∼30%) demonstrate a late-onset hearing loss, starting in childhood. They have normal hearing early in life and are therefore good candidates for applying protective and therapeutic interventions. However, the underlying deafness mechanism is unclear. In this study, we used a time-controlled, inducible gene knockout technique to knockout Cx26 expression in the cochlea after birth. We found that deletion of Cx26 after postnatal day 5 (P5) in mice could lead to late-onset hearing loss. Similar to clinical observations, the mice demonstrated progressive, mild to moderate hearing loss. The hearing loss initiated at high frequencies and then ext

SUBMITTER: Zhu Y 

PROVIDER: S-EPMC4268423 | biostudies-literature | 2015 Jan

REPOSITORIES: biostudies-literature