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- W4319458853 abstract "Gap junction gene GJB2 (Cx26) mutations cause >50% of nonsyndromic hearing loss. Its recessive hetero-mutation carriers, who have no deafness, occupy ~10 to 20% of the general population. Here, we report an unexpected finding that these heterozygote carriers have hearing oversensitivity, and active cochlear amplification increased. Mouse models show that Cx26 hetero-deletion reduced endocochlear potential generation in the cochlear lateral wall and caused outer hair cell electromotor protein prestin compensatively up-regulated to increase active cochlear amplification and hearing sensitivity. The increase of active cochlear amplification also increased sensitivity to noise; exposure to daily-level noise could cause Cx26+/- mice permanent hearing threshold shift, leading to hearing loss. This study demonstrates that Cx26 recessive heterozygous mutations are not harmless for hearing as previously considered and can cause hyperacusis-like hearing oversensitivity. The data also indicate that GJB2 hetero-mutation carriers are vulnerable to noise and should avoid noise exposure in daily life." @default.
- W4319458853 created "2023-02-09" @default.
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- W4319458853 date "2023-02-10" @default.
- W4319458853 modified "2023-09-26" @default.
- W4319458853 title "Cx26 heterozygous mutations cause hyperacusis-like hearing oversensitivity and increase susceptibility to noise" @default.
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- W4319458853 doi "https://doi.org/10.1126/sciadv.adf4144" @default.
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