NF2 and hearing loss disorder: Activation of NLRP3 triggers the production of IL-1β, a potent proinflammatory cytokine; treatment with a recombinant human IL-1 receptor antagonist, reversed the hearing loss seen in a family with sensorineural hearing loss and NLRP3 mutations.39 In VS, genes associated with NLRP3 were significantly upregulated in patients with poor hearing.40 Future work is needed to ascertain the therapeutic role of IL-1β blockade in patients with hearing loss secondary to sporadic or NF2-associated VS.