Case report: A 20 years old patient with chronic mastoiditis and facial nerve palsy within a major-recidive of a granulomatosis with polyangiits

Introduction:

Granulomatosis with polyangiitis is an ANCA-associated vasculitis of the small to medium vessels which, as a systemic disease, can affect the whole body, but mostly shows manifestation in the ENT tract. Following the nose, the ear is the second most affected organ in the ENT region.

Case:

We report the case of a 20 years old woman with a major recidive of a biopsy proven GPA, who presented a subacute hearing loss, recidivating otitis mediae and otorrhea on the right side. During the stationary stay she developed a right-sided facial nerve palsy. No immunomodulatory systemic therapy had been started.

Results:

The patient presented a pressure-sensitive swollen right mastoid with redness and hyperthermia and a peripheric facial palsy on the right side. The BVAS was 15 points, the VDI was 4 points, the ENTAS 2 showed a high-grade GPA activity. The c-ANCA was 108.3 U/ml. The audiometry presented a sensoneurinal hearing loss pantonal to a maximum of 50dB at 1 kHz. CT-morphological image of a mastoiditis on the right with arrosion of the bordering osseous structures.

Discussion:

In the context of our case report, we discuss the operative and systemic-medicamentous therapy options of this GPA manifestation.

Publication History

Publication Date:
23 April 2019 (online)