Autoimmune sensorineural hearing loss (ASNHL) is a clinical syndrome that typically produces a bilateral rapidly progressive hearing loss. Autoantibodies and autoreactive T cells have been implicated in the etiopathogenesis of ASNHL. However, the identity of a specific or highly relevant inner-ear self-antigen is still required. In recent years, a role for antiphospholipid antibodies has been also suggested, assuming sudden/progressive hearing loss to be part of the primary antiphospholipid syndrome (APS). In this review, we address the need for specific diagnostic tools in order to establish an autoimmune origin for hearing loss. Moreover, the decision of whether anti-inflammatory drugs or anti-coagulation should be given is also discussed.