Wegener's granulomatosis presenting with bilateral facial nerve palsy

Abstract

Primary manifestation of Wegener's granulomatosis in the mucosa of the middle ear is rather rare, and has been reported as presenting with serous otitis media, chronic otitis media, sensorineural hearing loss, and, in rare instances, unilateral facial palsy. Bilateral facial palsy has never been reported. This last fact constitutes the interest in our report of a 23-year-old female patient who presented with symptoms of recurrent bilateral otitis media, eventually developing sensorineural hearing loss and bilateral facial palsy. Soon thereafter neurological symptoms appeared and lung extension was noted. Histological examination of repeated biopsies taken from the nasal and middle ear mucosa was not conclusive for the suspected disease, and c-ANCA titers were also initially repeatedly negative. Eventually, positive lung biopsy and elevated c-ANCA titers when the patient had developed pulmonary granulomas confirmed the diagnosis of Wegener's granulomatosis. Mastoid surgery with facial nerve decompression of the most severely afflicted side did not result in the recovery of facial nerve function. Medical therapy with corticosteroids and cyclophosphamide improved the clinical picture but were ineffective in improving the bilateral sensorineural hearing loss and the facial paralysis on the operated side. We would contribute to the literature a unique case of bilateral facial nerve palsy due to Wegener's granulomatosis.

Bilateral Facial Paralysis and Otitis Media as the First Presentations of Wegener’s Granulomatosis: A Case Report

“…In a literature review, only 1 case occurring at a young age was found, and that patient was a 23-year-old woman with bilateral recurrent otitis media, hearing loss, bilateral facial paralysis, and lung involvement. 10 In the patient presented herein, nose and middle ear biopsies were normal, but the lung biopsy was positive. 10 The CANCA test was also positive in the current case.…”

“…10 In the patient presented herein, nose and middle ear biopsies were normal, but the lung biopsy was positive. 10 The CANCA test was also positive in the current case.…”

Wegener's Granulomatosis Presenting with Bilateral Facial Nerve Palsy and Limited to the Head and Neck

“…The serological ANCA test can be useful as a marker of disease activity and remission following treatment. Although biopsies of suspicious lesions are able to confirm the diagnosis, they are often unrewarding and show only non-specific granulomatous disease, particularly when taken from the middle ear, as occurred in this case with the nasal mucosal biopsies returning normal results [4,9,12].…”

“…Since the differential diagnosis of this presentation of WG includes tuberculosis, sarcoidosis, polyarteritis nodosa, and malignancy of the temporal bone, nasopharynx or parotid gland, a more aggressive investigative course should be undertaken [4]. Additionally, the likely failure of an antibiotic course should lead the treating team to investigations including chest X-rays, mantoux tests, p-and c-ANCA titers, and biopsies of the middle ear mucosa [4,9]. Importantly, though elevated c-ANCA levels are well reported indicators of WG, the sensitivity has been shown to be only 66%, rising to 90% in active cases [9].…”

“…Confirming the diagnosis of WG can be problematic in many cases, as it tends to rely on laboratory and histological findings [4,9,12]. An elevated cytoplasmic pattern antineutrophil cytoplasmic antibody (c-ANCA) level is highly specific for active WG, with a specificity of 98% [4,12].…”