Studies on genes mutated in Usher syndrome (a syndromic form of IRD accompanied by hearing impairment) demonstrated that aminoglycosides and derivatives thereof can mediate TR of different disease-causing PTCs in the PCDH15 and USH1C genes, in in vitro translation assays as well as in cell culture experiments [144,145,146,147,148,149]. This evidence concerns the gene USH1C and Usher syndrome.