Hearing loss, both sensorineural and conductive, is a well-recognized manifestation of systemic vasculitis, yet has only infrequently been described as its initial presentation. We describe three cases in which hearing loss preceded the diagnosis of systemic vasculitis by a period of a few weeks to more than 6 months. While steroid therapy was initiated immediately in one of the cases, comprehensive immunosuppressive therapy was given only after additional manifestations compatible with vasculitis appeared, allowing for a diagnosis of Wegener's granulomatosis in two of the cases and undifferentiated vasculitis, in the third. Hearing improved in all patients, albeit, residual dysfunction remained. Prompt referral for a complete rheumatologic workup in cases of unexplained hearing loss would allow for a timely diagnosis of a collagen vascular disease and facilitate early initiation of systemic therapy, possibly yielding better audiological results.