Autoimmune Inner Ear Disease (AIED): This condition is characterized by hearing loss, often bilateral and progressive, associated with autoimmune disorders. The presence of a positive ANA (antinuclear antibody) supports an autoimmune etiology, making AIED a strong consideration.

Systemic Lupus Erythematosus (SLE): SLE is a systemic autoimmune disease that can affect multiple organs, including the ears, leading to hearing loss. A positive ANA is a hallmark of SLE, although not all patients with positive ANA have SLE.
Rheumatoid Arthritis (RA): While primarily known for its effects on joints, RA can also involve other systems and has been associated with hearing loss. The presence of a positive ANA can be seen in some patients with RA.
Relapsing Polychondritis: This rare autoimmune disorder affects cartilage and can lead to hearing loss among other symptoms. Although less common, its autoimmune nature and potential for hearing loss make it a consideration.

Wegener's Granulomatosis (Granulomatosis with Polyangiitis, GPA): This is a life-threatening autoimmune disorder that can affect various parts of the body, including the ears, leading to hearing loss. Early diagnosis is crucial due to its potential severity and the availability of effective treatments.
Sjögren's Syndrome: An autoimmune disorder that primarily affects the glands that produce tears and saliva but can also cause hearing loss. It is often associated with a positive ANA and can be life-threatening if not properly managed.

Muckle-Wells Syndrome: A rare genetic disorder characterized by periodic episodes of fever, rash, and joint pain, which can also lead to hearing loss. It's part of the cryopyrin-associated periodic syndrome (CAPS) group.
Nonsyndromic X-linked hearing loss: Although not typically associated with a positive ANA, some forms of X-linked hearing loss could be considered in the differential diagnosis of familial hearing loss, especially if other autoimmune causes are ruled out.