Despite its wide expression, defects in usherin have only been linked with Usher syndrome type 2 (USH2; MIM: 276901) [11], [12], [13], [14], nonsyndromic RP [15], and nonsyndromic hearing loss (NSHL) [16], implying its special role in maintaining the function of the capillary and structural basement membranes of the two ciliated sensory neurons, namely photoreceptor cells and cochlear hair cells. Here, USH2A is linked to Usher syndrome type 2.