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Correspondence to Kwee Yen Goh; [email protected]
Background
Granulomatosis with polyangiitis (GPA)—previously known as Wegener’s granulomatosis—is an idiopathic autoimmune disease characterised by necrotising granulomatous vasculitis.1 2 It affects the ears, upper and lower respiratory tract and the kidneys.1 It is not uncommon for the disease to present solely with ear, nose and throat (ENT) symptoms.3 4
ENT manifestations are usually the first to arise,1 with involvement of the nose and paranasal sinuses in 85% of cases and ear involvement in approximately 35% of all cases.5 However, these symptoms are often non-specific, leading to a delay in diagnosis. Otologic involvement during GPA can manifest as chronic serous otitis media, sensorineural hearing loss, vertigo and facial palsy.3 Facial nerve palsy is rare, being reported in only 5% of cases.3 5 The most common rhinological symptoms are crusting and epistaxis. Other features include mucopurulent discharge associated with fever (10%) and septal perforation (24%), which can progress to saddle deformity of the nose.6 Subglottic stenosis is another recognised complication and is present in approximately 23% of cases, which can present with dyspnoea on exertion and stridor.6
Given the high mortality rate, prompt diagnosis and treatment of GPA is essential.2 Otolaryngologists must be equipped to recognise this disease in the face of common otolaryngological symptoms.
We describe the case of a patient who presented with intractable serous otitis media, rhinosinusitis and unilateral facial palsy, with subsequent investigations revealing extensive lung and renal disease.
Case presentation
An 18-year-old man with learning difficulties was referred to the ENT department with a 6-week history of bilateral otalgia with discharge, which had failed to respond to both oral and topical antibiotics. There was associated anosmia, purulent nasal discharge, intermittent epistaxis, generalised myalgia, increasing lethargy and fatigue. No lower respiratory symptoms such as cough was reported. He was initially treated for otitis externa in the community, however, a left ear swab sent by his general practitioner revealed a profuse growth of Moraxella catarrhalis, consistent with otitis media.
On anterior rhinoscopy, there was bilateral acute inflammation with exposed septal cartilage in the left nasal cavity (figure 1). Otoscopy revealed small inferior perforation of both tympanic membranes with offensive purulent discharge.
He was initially diagnosed with acute...