Abstract

Introduction

Wegener’s granulomatosis (Granulomatosis with Polyangiitis, GPA) is a systemic vasculitis that primarily involves the lungs, kidneys, and respiratory tract. It is characterized by necrotizing granulomatous inflammation of the upper and lower airways. In more than 70% of cases, the upper respiratory tract including the nose, paranasal sinuses, and ears is affected. Here, we aim to present a rare case of isolated ear involvement of GPA, manifesting as mixed hearing loss and facial paralysis.

Case Report

45-year-old female patient presented to our clinic with a two-week history of hearing loss and otorrhea in the left ear. Panendoscopic examination revealed no remarkable findings. Two years ago, the patient had been diagnosed with bilateral acute otitis media and left peripheral facial paralysis (Figure 1), and subsequently underwent left canal wall down mastoidectomy with left ventilation tube insertion at our institution. Preoperative audiometry showed hearing thresholds of R: 66/43 dB and L: 91/66 dB (Figure 2). During surgery, dense granulation tissue was observed in the middle ear and mastoid cavity; the long process of the incus was found to be eroded, and the stapes and malleus could not be visualized. These granulation tissues were sent for the pathological examination. Postoperative audiometry demonstrated hearing thresholds of R: 29/16 dB and L: 68/49 dB, and facial paralysis resolved after several months of follow-up.

Figure 1A Left-sided House–Brackmann Grade IV peripheral facial paralysis in the patient

Figure 1B Left-sided House–Brackmann Grade IV peripheral facial paralysis in the patient

Histopathological examination of the patient’s granulation tissue suggested GPA, and the patient was referred to the rheumatology department for consultation. Following the detection of c-ANCA positivity in the blood tests (Table 1), the patient was placed under rheumatologic follow-up and treatment was initiated.

Table 1 Laboratory findings of the patient

Figure 2 The pure-tone audiometry test from two years ago

Figure 3 The most recent pure-tone audiometry test

At the patient’s most recent visit after irregular follow-ups mixed hearing loss and otorrhea had developed (audiometry: R: 55/23 dB, L: 116/65+ dB) (Figure 3). Based on the recommendations of the rheumatology department, 1 g pulse methylprednisolone therapy was initiated. In addition, temporal bone CT (Figure 4) revealed marked density increases in both the middle ear and mastoid cavities. Following right paracentesis, serous fluid was obtained, and a Paparella type II ventilation tube was inserted into the right tympanic membrane. Diffusion MRI (Figure 4-5) demonstrated increased effusion extending to the left petrous apex.

Figure 4 Temporal bone CT images of the patient

Figure 5 Temporal diffusion MRI images of the patient

Based on rheumatology recommendations, the patient was started on methotrexate 20 mg and deltacortril 7.5 mg. Due to the development of hepatotoxicity during follow-up, methotrexate was discontinued and replaced with leflunomide 20 mg. During subsequent follow-up, the patient remained asymptomatic.

Discussion

Granulomatosis with polyangiitis (GPA) was first described in 1936. It is a rare systemic disease characterized by necrosis, granuloma formation, and vasculitis; predominantly affecting the upper and lower respiratory tracts and the kidneys [1]. The mean age of onset is between 20 and 40 years, and it is observed 1,5 times more frequently in males than females [2]. According to the 2022 European Alliance of Associations for Rheumatology (EULAR) guidelines, diagnostic criteria for GPA include bloody nasal discharge, nasal crusting, cartilage involvement, conductive or sensorineural hearing loss, c-ANCA or anti-PR3 ANCA positivity, pulmonary nodules, masses or cavitations on thoracic imaging, granulomas or giant cells on biopsy, and pauci-immune glomerulonephritis [3].

Otologic involvement has been reported in 20–60% of GPA cases. Common manifestations include otitis media with effusion, chronic otitis media, sensorineural hearing loss, vertigo, tinnitus, and peripheral facial paralysis. Patients may also present with nasal crusting, septal perforation, and subglottic stenosis [4]. Differential diagnoses should include other ANCA-associated vasculitides, such as Churg-Strauss syndrome, drug-induced vasculitis, polyarteritis nodosa, and Goodpasture syndrome.

In the literature, Safavi et al. (2017) reported that 36% of 55 patients with GPA had otologic symptoms, most commonly hearing loss and otalgia [5]. A 2024 review reported that 86% of GPA patients developed hearing loss, with 39% experiencing otalgia as the second most frequent symptom [6]. Regarding initial presentation, a 2025 case series of 610 patients reported that isolated ear involvement was the first symptom in only 6 cases (0.8%), most commonly presenting as otitis media with effusion, mixed hearing loss, and imbalance. Middle ear function partially recovered with immunosuppressive therapy [7].

The mechanism of facial paralysis in GPA is hypothesized to involve either microvascular occlusion of the vessels supplying the facial nerve or granulomatous lesions exerting pressure on the nerve [8]. To date, 28 cases of facial paralysis in GPA have been reported, with 10 demonstrating bilateral involvement. Sixteen patients underwent surgery (e.g., tympanomastoidectomy, exploratory tympanotomy) for facial paralysis, but surgical intervention generally failed to correct the paralysis, whereas immunosuppressive therapy was effective [6], consistent with our case.

Treatment of GPA primarily involves immunosuppressive agents, which is divided into induction and maintenance phases. Commonly used medications include cyclophosphamide, glucocorticoids, rituximab, azathioprine, and methotrexate [9]. Chen et al. (2025) demonstrated that rituximab may prevent potential hearing loss in GPA patients [10]. Unexplained vertigo, mixed hearing loss, or acute otitis media with effusion without nasopharyngeal pathology should raise suspicion for GPA [11].

Conclusion

Our case demonstrates several otologic manifestations of GPA. The presence of facial paralysis and hearing loss as initial symptoms represents a rare and noteworthy presentation, contributing to the literature. In complex cases, clinicians should consider that existing otologic findings may reflect a multisystemic disease.

Informed Consent

References

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