In one previously unresolved family, we found a homozygous frameshift PLP variant in GRXCR2 (OMIM: 615762), GRXCR2‐c.251delC (p.Arg84 frameshift), in two affected siblings; and additionally, in 1/80 unrelated individuals affected with non‐syndromic hearing impairment (NSHI). Here, GRXCR2 is linked to hearing loss disorder.