Therefore, the aims of study were: (1) to describe a longitudinal cohort of consecutive anti-IL1-treated paediatric and adult CAPS patients with sensorineural hearing loss, (2) to examine risk factors associated with a high WHO grade of hearing impairment and (3) to identify real-life barriers to optimal long-term management of hearing loss in CAPS. The gene discussed is IL1B; the disease is cryopyrin-associated periodic syndrome.