CMP-Neu5Ac hydroxylase (Cmah) disruption caused several abnormalities and diseases including hearing loss in old age. However, underling molecular mechanisms that give rise to age-related hearing loss
Gene expression was analyzed in endolymphatic sacs of two groups of mice: Slc26a4Δ/Δ and Slc26a4Δ/+ mice. Slc26a4Δ/Δ mice fail to develop hearing and are a model for Enlarged Vestibular Aqueduct and
Mutations in the gene for Norrie disease protein (Ndp) cause syndromic deafness and blindness. We show that cochlear function in an Ndp knockout (KO) mouse deteriorated with age: at P3-P4, hair cells