Novel gene implicated in deafness

Researchers from King's College London and the Wellcome Trust Sanger Institute have for the first time demonstrated a direct link between the Wbp2 gene and progressive hearing loss. The scientists found that loss of Wbp2 expression led to progressive high-frequency hearing loss in mice as well as in two clinical cases of children with deafness with no other obvious features. This study, published today in EMBO Molecular Medicine , opens up the Wbp2 pathway as a new route to therapeutic approaches that more specifically target the inner ear. These findings also indicate that hearing impairment is linked to hormonal signalling, rather than hair cell degeneration. Wbp2 is known to be a transcriptional co-activator for estrogen receptor Esr1 and progesterone receptor Pgr. The loss of Wbp2 causes not only progressive high frequency hearing loss, but also results in reduced expression of Esr1, Esr2 and Pgr in the cochlea - a part of the inner ear. Understanding the estrogen-sensitive molecular networks specific to hearing offers an unprecedented new target for the control of the estrogen signalling pathway in the auditory system that could prevent or reverse progressive hearing loss.