Nine-year-old child with profound bilateral SNHL and no other relevant clinical features (#P92) in whom a previously not described heterozygous nonsense variant in the endothelin-B receptor gene, EDNRB (NM_000115.5), was identified and classified as likely pathogenic: c.65C>A p. (Ser22*). The gene discussed is EDNRB; the disease is sensorineural hearing loss disorder.