In the last 10 years (from 2006 to 2016), the genetic analysis of patients with hearing loss in our tertiary hospital was restricted to detect the most frequent pathogenic variants responsible for hereditary sensorineural hearing loss in Spain, specifically the complete coding sequence of the GJB2 gene, the deletions D13S1830 and delD13S1854 in the GJB6 gene and the OTOF p.Q829X variants. The gene discussed is GJB2; the disease is sensorineural hearing loss disorder.