Abstract: The patient, a 48-year-old female, was diagnosed with granulomatosis with polyangiitis in September 2020 at RUMG. She suffered a right mild to moderate mixed hearing loss and left profound sensorineural hearing loss. The patient was treated with several rounds of antibiotics, bilateral PE tube insertion, and finally Rituximab infusions. In May 2021, the patient completed a trial with a BiCROS; however, patient did not feel a benefit. In October 2021, the patient underwent cochlear implant surgery for left ear. She is still experiencing fluctuating hearing loss along with middle ear effusion in her right ear, which is currently being observed.
Summary: The learning objectives of this clinical poster are to identify granulomatosis with polyangiitis (GPA), discuss the important of early diagnosis, and discuss the typical otological presentations. Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s Granuloma, is a rare multisystemic autoimmune disease. Its etiology is unknown and is characterized by necrotizing granulomatous lesions in the respiratory tract, vasculitis, and glomerulonephritis. The most common involvements are described as ELK: ENT, lungs, and kidneys. Otological involvement is seen in up to 40% of cases when trying to prevent permanent SNHL and is characterized by: serous otitis media, chronic otitis media, sensorineural hearing loss, vertigo, and facial nerve palsy. Because it is a rare and unpredictable disease, people who are diagnosed with GPA oftentimes relapse. 1⁄4 of patients relapse within two years of diagnosis and 1⁄2 relapse within five years. GPA treatment options happen in two phases: (1) induction phase, lasting 3-6 months with the use of corticosteroids and immunosuppressants and (2) maintenance phase, lasting 12-24 months with the use of oral corticosteroids with infusions. Oftentimes, otologic manifestations are the only presenting symptoms and can aid in the diagnosis. Patients who persist serous otitis media with new onset of SNHL or significant pain should raise suspicion of GPA . Clinicians should maintain a high level of suspicion for patients presenting with mixed or conductive hearing loss with a new onset of OM specifically when previous ETD is absent. There are also varying treatments for amplification due to the varying severity in each patient. It is important to consider every part of their history and how they react to treatment. For these reasons, it is vital to understand the presentation and manifestations of GPA.
Learning Objectives:
Identify granulomatosis with polyangittis (GPA) and discuss the importance of early identification and treatment.
