EDNRB and Congenital sensorineural hearing impairment: Interestingly, in congenital sensorineural deafness, the other major EDN3-EDNRB phenotype that with Hirschsprung disease defines Waardenburg-Shah syndrome type IV, the penetrance of hearing impairment in ICR background Edn3 or Ednrb mutant mice is variable (approx. 50%) (Tan et al., 2023).