In humans, DIAPH1 and DIAPH3 have been associated with different types of hearing loss (HL) [3]: heterozygous mutations in DIAPH1 are responsible for autosomal dominant NSHL with or without thrombocytopenia (locus DFNA1; Mendelian Inheritance in Man, MIM #124900) [11, 12], whereas overexpression of DIAPH3 –resulting from a mutation in the 5’ untranslated region (UTR) of the gene—causes auditory neuropathy, autosomal dominant, 1 (AUNA1; MIM #609129) [10]. This evidence concerns the gene DIAPH1 and auditory neuropathy.