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ABSTRACT MYO1A is considered the gene underlying autosomal dominant nonsyndromic hearing loss DFNA48, based on six missense variants, one small in-frame insertion, and one nonsense mutation. Results from NGS targeting 66 deafness genes in 109 patients identified three families challenging this assumption: two novel nonsense (p.Tyr740* and p.Arg262*) and a known missense variant were identified heterozygously not only in index patients, but also in unaffected relatives. Deafness in these families clearly resulted from mutations in other genes ( MYO7 A, EYA1, and CIB2). Most of the altogether 10 MYO1 A mutations are annotated in db SNP, and population frequencies (db SNP, 1000 Genomes, Exome Sequencing Project) above 0.1% contradict pathogenicity under a dominant model. One healthy individual was even homozygous for p.Arg262*, compatible with homozygous Myo1a knockout mice lacking any overt pathology. MYO1 A seems dispensable for hearing and overall nonessential. MYO1 A adds to the list of 'erroneous disease genes', which will expand with increasing availability of large-scale sequencing data. [ABSTRACT FROM AUTHOR]