NFKB1 and hyperinsulinemic hypoglycemia, familial, 4: In humans, the molecular basis of ROR1 missense mutation-caused inner ear anomalies and deafness are linked to NF-κB deficiency (Diaz-Horta et al. 2016), and this pathway also protects cochlear hair cells from aminoglycoside-induced ototoxicity (Jiang et al. 2005).