NLRP3 and hearing loss disorder: DFN34 is a recently described NLRP3-related autoinflammatory disease (NLRP3-AID), clinically characterized by middle-age onset of bilateral, symmetric, and progressive sensorineural hearing loss (14); Nakashini and colleagues, first genetically characterized this syndrome in two unrelated families, showing a missense pathogenic mutation in the LRR domain of NLRP3 gene (exon 7), NM_001243133.2(NLRP3): c.2753G > A; p.Arg918Gln (14).