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Congenital and progressive hearing impairment is a common distressing disease.The progressive dominant hearing loss DFNA28 in human is associated with a frameshift mutation of Grainyhead-like 2 (GRHL2) but its etiology and mechanism remain unknown.Here we report a zebrafish grhl2bT086 mutant line, in which grhl2b expression is interrupted by an insertion of a Tol2 transposon element.The mutants exhibit enlarged otocysts, smaller or eliminated otoliths, malformed semicircular canals, insensitiveness to sound stimulation, and imbalanced swimming motion.Since Grainyhead-like family members can regulate epithelial adhesion, we examined the expression of some genes encoding junction proteins in mutants.