Currently, ~ 90 mutations in OTOF (see the most complete list in review [58]) have been reported to cause a nonsyndromic severe-to-profound prelingual HL and autosomal recessive auditory neuropathy-1 (DFNB9, deafness, autosomal recessive 9, AUNB1, MIM 601071) characterized by disruption of auditory nerve activity with preservation of outer hair cell function. This evidence concerns the gene OTOF and deafness.