A woman with progressive hearing loss and recurrent episodes of fever and urticaria may be diagnosed with:

The correct answer is Muckle-Wells syndrome, which is a genetic autoinflammatory condition associated with sensorineural hearing loss, intermittent fever, and skin manifestations such as urticaria. The episodes of urticaria and fever are characteristic of the periodic inflammatory attacks that occur in this syndrome. Additionally, the progressive hearing loss observed in the patient aligns with the known auditory complications associated with Muckle-Wells syndrome.

Muckle-Wells syndrome is caused by mutations in the NLRP3 gene, which plays a crucial role in the inflammatory response. The combination of symptoms in this context makes Muckle-Wells the most fitting diagnosis since it specifically encompasses hearing loss and recurrent fever with urticarial-type rash.

Other conditions listed may feature some overlapping symptoms but do not present with this specific combination to the same extent or context. For instance, Still's disease primarily involves arthritis, fever, and rash but is not characterized by progressive hearing loss. Behçet's disease involves systemic vasculitis and can cause oral and genital ulcers and eye inflammation, but it does not usually lead to hearing impairment. Systemic lupus erythematosus also presents with a wide variety of systemic symptoms, including skin rashes and fever, but tends to have different