Moreover, we comprehensively summarized all <i>LARS2</i> variants associated with PS via an extensive literature review.<h4>Results</h4>Proband 1 (12-year-old female) harbors compound heterozygous variants <i>LARS2</i> c.235-2A>G (novel) and <i>LARS2</i> c.880G>A, presenting with profound SNHL, primary ovarian insufficiency, and developmental delay. This evidence concerns the gene LARS2 and sensorineural hearing loss disorder.