Mutations in TBC1D24 are frequently reported in patients with DOOR (Deafness, Onychodystrophy, Osteodystrophy, and mental Retardation) syndrome, myoclonic epilepsy, and epileptic encephalopathy; rarely are such mutations observed in patients with NSHL, including autosomal dominant deafness (DFNA65) and autosomal recessive deafness (DFNB86; Balestrini et al., 2016; Rehman et al., 2014; Zhang et al., 2014). Here, TBC1D24 is linked to autosomal recessive nonsyndromic hearing loss 86.