Facial paralysis in Wegener’s granulomatosis of the middle ear

Abstract

A case of Wegener's granulomatosis, which presented as chronic otitis media with facial nerve palsy, is described. Early diagnosis is vital if unnecessary surgical exploration is to be avoided. A false negative cANCA may delay the diagnosis, especially in cases of locoregional disease, and a policy of repeated titres should be adopted, if clinical suspicion is high.

Bilateral Facial Palsy in Rapidly Progressive Course of Wegener’s Granulomatosis: A Case Report

“…For many authors, it is not recommended. Described in the current literature cases of the facial nerve paralysis in a course of WG, treated surgically, shows that surgical treatment does not improve the prognosis, but could increase the risk of further facial nerve damage [10, 11, 15, 17]. However Magliulo et al and Gottschlich et al emphasized in their publications that in case of purulent otorrhea, not responding to antibiotics, or in case of symptoms of mastoiditis, the surgical treatment should be implemented [5, 17].…”

“…Current treatment regimens based on combination therapy with steroids and immunosuppressive drugs (cyclophosphamide, methotrexate) allow for remission in 70–80% of cases [2, 8, 9]. Lack of treatment might be fatal in 80–82% patients in the first year after diagnosis [9, 10]. Patients with WG have an average survival of 5 months [1, 6].…”

An Unusual Presentation of Wegener’s Granulomatosis with Otologic Symptoms and Unilateral Parotid Gland Enlargement

“…The ear involvement is seen approximately in 35 % of all cases with Wegener's granulomatosis [4]. Otologic manifestations have been reported during the course of the disease but they are rare as initial presenting features [5]. Painless unilateral parotid gland enlargement is even less common [6].…”

Pathology Quiz Case 3: Diagnosis

“…3 Patients with otologic manifestations of Wegener granulomatosis most commonly present with otitis media with effusion, which is thought to occur secondary to Eustachian tube obstruction by a nasopharyngeal lesion. 5 Vertigo, another rare finding, may result from immune complex deposition in the vestibule or from involvement of the central nervous system. Chronic suppurative otitis media is frequently associated with severe postauricular pain, otorrhea, and conductive hearing loss.…”

“…5 Biopsies of tissues from the nasal mucosa have a higher diagnostic yield but still have a significant false-negative rate and a negative predictive value of 74%. The histopathologic findings of vasculitis, necrosis, and granulomatous inflammation are the gold standard for the diagnosis of Wegener granulomatosis.…”