For instance, in a mouse model of HSCR (a model with a mutation in c-Ret, the major susceptibility gene in Hirschsprung’s disease), the impaired phosphorylation of NF-κB was pointed to as the possible cause of neurodegeneration of the spiral ganglion neurons (SGNs) in the inner ears and subsequent syndromic deafness [55]. Here, NFKB1 is linked to Hirschsprung disease.