Granulomatosis with polyangiitis is a rare disease, with small vessel vasculitis leading to progressive damage of vital organs. The mainstay of treatment is immunosupressive therapy. Exacerbations of the disease are prevalent and damages of tissues may result in organs failures. In our paper we present a case of a patient, who was qualified to biologic therapy with rituximab due to ongoing ear-nose-throat (ENT) symptoms worsening. Remission of rhinolaryngologic complaints was not met despite rituximab treatment, however significant improvement in hearing occurred.