Granulomatosis with polyangiitis (GPA) is an etiologically unknown systemic disease characterized by necrotizing granulomatous inflammation. Additionally, it is accompanied by vasculitis of small and medium-sized blood vessels. It manifests clinically as a triad involving the lungs, upper airways, and kidneys. It is estimated that 90% of patients will exhibit upper or lower airway symptoms and around 80% develops the renal disease. In this article, we describe three case scenarios with varying presentations. GPA should be considered among the possible etiologies of cavitary pulmonary lesions with ear manifestations including hearing loss with poor response to unusual treatment.