Based on our experience with this case and review of the literature, patients presenting with bilateral SSNHL of unknown etiology should have the following imaging and laboratory studies to exclude autoimmune disease: CBC, ESR, CRP, ANA, anticardiolipin antibodies, lupus anticoagulant, rheumatoid factor, anti-neutrophil cytoplasmic antibodies, clotting factors, and urgent MRI of the brain and internal acoustic meatus. Here, ESR1 is linked to autoimmune disease.