The physical exam showed bilateral serous otitis media, House & Brackmann VI/VI grade left peripheral facial paralysis, and bilateral severe to profound sensorineural hearing loss. Clinical CaseĪ 47-year-old male patient presented with a two-month history of rapid progressive bilateral hearing loss, unilateral facial paralysis, and severe headaches. We report the radiological and histopathology findings of a very aggressive limited GPA, after approval of the ethics committee of the Central Police Hospital, Bogota, Colombia. Although the exact cause still remains unknown, some theories link it to granulomatous compression of the cochlear nerve, deposits of immunocomplexes in the cochlea, and vasculitis of the vasa nervorum and cochlear vessels. When presented ENT is compromised, hearing loss can develop rapidly or gradually over several days or weeks. The localized GPA is not as common as the systemic GPA and affects just a single or a few organs without renal compromise. The systemic type represents the majority of cases of GPA, typically affecting the high and low respiratory tract, and it has been associated with focal segmental glomerulonephritis. Other possible nasosinusal symptoms include septal perforation (4%), chronic dacryocystitis (4%), and tracheal stenosis (1%). The prevalence can vary from 4 to 12% depending on series. Otologic symptoms such as sudden sensorineural hearing loss and facial paralysis as initial manifestations in patient with GPA are extremely rare. Central nervous system (CNS) can be compromised in almost 50% of cases however, most symptoms occur in very advanced disease states, so finding the neurological involvement like the first symptom of the disease tends to be very unusual. Granulomatosis with polyangeitis disease is a systemic entity mediated by autoimmune necrotizing inflammation that has the ability to affect every microsystem of the human body that was related with antineutrophil cytoplasmic antibody (ANCAS) activity. Diffuse dural enhancement of the internal auditory canal in T1-weighted gadolinium-enhanced magnetic resonance imaging could be a helpful and early clinical sign in a very aggressive limited granulomatosis with polyangeitis disease, called previously Wegener Disease (WD).

Intra auditory canal