This patient with profound SNHL had a diagnosis of enlarged vestibular aqueducts (EVA) and biallelic pathogenic variants in SLC26A4 (NM_000441.2 c.626G > T (p.Gly209Val), and c.707T > C (p.Leu236Pro)). The gene discussed is SLC26A4; the disease is sensorineural hearing loss disorder.