The most prevalent is Usher syndrome (sensorineural hearing loss and RP) which can be further subclassified to Usher type I (USH1 [MIM: 276900]), type II (USH2 [MIM: 276901]) and type III (USH3 [MIM: 276902]), and other syndromes such as Bardet-Biedl (BBS [MIM: 209900]) or Alström (ALMS [MIM: 203800])3. Here, USH2A is linked to Bardet-Biedl syndrome.