In the autoinflammatory disorder-related hearing loss, genetic mutations or polymorphisms inherited in an autosomal dominant manner result in a gain-of-function mutation within the gene for nod-like receptor (NLR) family pyrin domain 3 (NLRP3), leading to excessive interleukin (IL)-1β release, sensorineural hearing loss, systemic amyloidosis, and/or transient skin rashes. This evidence concerns the gene NLRP3 and sensorineural hearing loss disorder.