Mouse models confirmed that Cx26 and Cx30 are essential for auditory function [13] and have helped establishing a link between inherited deafness, connexin expression, endolymphatic K+ concentration, endocochlear potential [14], [15], [16], transfer of nutrients within the sensory epithelium of the inner ear [17] and cellular degeneration in the cochlea [18]. Here, GJB2 is linked to deafness.