In the present study, we identified pathogenic missense variants of LMX1A (MIM: 600298), a gene associated with a complex phenotype in mice, including recessive deafness and vestibular defects (Bergstrom et al. 1999; Chizhikov et al. 2006; Steffes et al. 2012), as a cause of dominant NSHI and vestibular dysfunction in humans. The gene discussed is LMX1A; the disease is deafness.