Granulomatosis with polyangiitis presenting as isolated ear involvement: a case series and literature review

Abstract

Objective: To describe the clinical characteristics and outcomes of patients affected by granulomatosis with polyangiitis (GPA) presenting with isolated ear involvement.

Methods: A retrospective review of patients affected by GPA and treated at the University of Brescia, Italy, from 2002 to 2023 was conducted. Only patients with exclusive otologic manifestation as first presentation were included.

Results: Among 610 patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV) diagnosed and followed at our Institution, 6 (0.8%) presented with exclusive ear involvement as first presentation, all affected by GPA. Most frequently patients presented with otitis media with effusion, sensorineural or mixed hearing loss, and dizziness. Two patients developed systemic symptoms. All patients experienced at least a partial recovery of middle ear function after starting immunosuppressive therapy.

Conclusions: AAVs rarely show initial presentation as isolated ear involvement, and more commonly present as otitis media with hearing loss that is unresponsive to conventional therapy. Once an AAV is suspected, surgery should be avoided since further damage can be caused by local iatrogenic inflammation sustained by the underlying condition. Local improvement is generally seen after the start of immunosuppressive therapy.

Keywords: anti-neutrophil cytoplasmic antibody-associated vasculitis; granulomatosis with polyangiitis; hearing loss; otitis media; vasculitis.

Cover figure.

Imaging of a patient affected by granulomatosis with polyangiitis with involvement of the right middle and inner ear. CT images (A: axial plane; B: coronal plane) show right middle ear and mastoid opacification with osteolysis of the bony walls of labyrinth and semicircular canals, along with calcification of the cochlea. T1-weighted MR of the same patient (C), with respect to the left side, shows enhancement of the right middle and inner ear, consistent with chronic inflammation.