Compound heterozygous mutation in LOXHD1 c.4690C > T (p.Leu1564Phe) c.2054G > A (p.Arg685His) and c.5813G > A (p.Arg1938His), c.1191G > T (p.Trp397Cys) was observed in two patients (P2, P4) with hearing impairment. This evidence concerns the gene LOXHD1 and hearing loss disorder.