...Morag A. Lewis; Francesca Di Domenico; Neil J. Ingham; Haydn M. Prosser; Karen P. Steel ABSTRACT The microRNA miR-96 is important for hearing, as point mutations in humans and mice result in dominant progressive hearing loss. Mir96 is expressed in sensory cells along with Mir182 and Mir183...

... be modified by L-type voltage-gated calcium channel agonists. Zebrafish Hair cell Ribbon synapse myo7aa Hearing loss Up to 11% of deaf/hard of hearing children and 1 in 6000 people in the United States have Usher syndrome (USH) ( Kimberling et al., 2010 ). USH is characterized...

... for Ak2 deficiencies. One of the clinical features of RD is hearing loss, but its pathophysiology and causes have not been determined. In adult mammals, sensory hair cells of the inner ear do not regenerate; however, their regeneration has been observed in several non-mammalian vertebrates, including...

...Sen Chen; Le Xie; Kai Xu; Hai-Yan Cao; Xia Wu; Xiao-Xiang Xu; Yu Sun; Wei-Jia Kong ABSTRACT Mutations in the GJB2 gene [which encodes connexin 26 (Cx26)] are the most common causes of hereditary hearing loss in humans, and previous studies showed postnatal development arrest of the organ of Corti...

... and p38. In humans, mutant alleles of MAP3K1 are associated with 46,XY sex reversal. Until recently, the only phenotype observed in Map3k1 tm1Yxia mutant mice was open eyelids at birth. Here, we report that homozygous Map3k1 tm1Yxia mice have early-onset profound hearing loss accompanied...

... mutant, goya , which exhibits the eyes-open-at-birth and microphthalmia phenotypes. In addition, these mice also have hearing loss. The goya mice carry a splice site mutation in the Map3k1 gene. We show that goya and kinase-deficient Map3k1 homozygotes initially develop supernumerary cochlear outer hair...