We report a case of P-ANCA positive Wegener’s granulomatosis (WG) with progressive hearing loss. A 63-year-old woman consulted a hospital complaining of right hearing loss. While she received therapy under diagnosis of sudden deafness, she developed a right facial nerve palsy and branch retinal artery occlusion. She was diagnosed as having WG based on specific symptoms in the upper respiratory tract (otitis and sinusitis), pathological findings in the nasal mucosa and blood test data including positive findings for P-ANCA. After she achieved remission induction with corticosteroid and immunosuppressant administration, hearing improved markedly and P-ANCA turned negative. Six years after remission, she demonstrated combined hearing loss in her left ear. Five years later, hearing loss in the left ear progressed acutely. Hearing loss was partially improved by medication at each episode, but has generally progressed during the past eleven years. The long-term clinical course showed that the titer of P-ANCA increased several months prior to the exacerbation of the hearing loss and coincidentally became negative with hearing improvement. This case provides a clinical example showing that P-ANCA can be used as an early marker for disease progression in P-ANCA-positive WG.