Anti-neutrophil cytoplasmic antibody (ANCA) -associated vasculitides (AAVs) include microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic granulomatosis with polyangiitis (EGPA), the incidences of which are reported to be increasing in Japan. We reviewed the clinical records of 20 cases with systemic AAVs (five cases with MPA, nine cases with GPA, and six cases with EGPA), who visited our otolaryngology department with otological symptoms from 2004 to 2014, and compared the otological characteristics among the diseases. Otologic symptoms appeared as an initial symptom(s) in 40% of MPA cases, 56% of GPA cases, and 83% of EGPA cases. GPA and EGPA cases showed a variety of symptoms such as otalgia, otorrhea, hearing loss, ear fullness, tinnitus and dizziness, while MPA cases showed only hearing loss and ear fullness, but otalgia or otorrhea. AAVs and otitis media associated with ANCA vasculitis (OMAAV) are usually diagnosed shortly after the appearance of otological symptoms in GPA cases, while the final diagnosis is delayed in EGPA cases. Furthermore, the diagnosis of OMAAV was made after the diagnosis of AAV in most cases of EGPA. More than half of MPA cases did not meet the diagnostic. criteria for OMAAV. It is noteworthy that in a significant number of AAV patients with ear disease, otological symptoms are supposed to appear as an initial symptom(s). Therefore, otolaryngologists have a major role to achieve early diagnosis of AAV. The patients with adult-onset inflammation of the middle ear, inner ear or both should undergo careful examinations, and they should be closely followed even if the diagnosis is uncertain.