ENT manifestations of Wegeners granulomatosis

Abstract

Wegeners granulomatosis is a necrotizing granulomatous vasculitis with multisystemic involvement. We present two cases of Wegener's presenting with otological manifestations as the first symptom. These symptoms are subtle and diagnosis may be easily overlooked. Hence a high index of suspicion is required. Early diagnosis and treatment goes a long way in improving the outcomes and in preventing further complications.

Granulomatosis with Polyangiitis with Bilateral Facial Palsy and Severe Mixed Hearing Loss

“…Chronic otitis media is associated with the presence of granulation tissue in the middle ear and the mastoid, which occurs in approximately 24% of cases [16, 17]. Destructive granulomatous masses may cause erosion of the ossicles or spread through the mastoid towards the petrous apex [18]. This process may be accompanied by effusion, mastoiditis, and/or facial palsy [14].…”

“…Introduction of tests determining serum levels of specific markers such as antineutrophil cytoplasmic antibodies (ANCA) and PR3 revolutionized the diagnosis of GPA, since they allow early diagnosis [16, 18]. The specificity of positive c-ANCA testing in GPA is greater than 95% [20].…”

“…The specificity of positive c-ANCA testing in GPA is greater than 95% [20]. The levels of c-ANCA tests correlate well with the disease activity, falling in patients during proper treatment and rising if a relapse after treatment occurs [18, 20]. The sensitivity of c-ANCA tests in the generalized form of the disease is 93%–97%, whereas in the regional form it is approximately 60% [16, 18].…”

“…The levels of c-ANCA tests correlate well with the disease activity, falling in patients during proper treatment and rising if a relapse after treatment occurs [18, 20]. The sensitivity of c-ANCA tests in the generalized form of the disease is 93%–97%, whereas in the regional form it is approximately 60% [16, 18]. Positive ANCA tests may help in setting the correct diagnosis in histologically dubious cases, which was the case in our patient.…”

Manifestaciones otorrinolaringológicas de la granulomatosis con poliangitis (Wegener)

“…Algunos pacientes también pueden presentar otitis media purulenta, (manifestada como otorrea y engrosamiento hipervascularizado o perforación de la membrana timpánica), otros una franca granulomatosis (tejido de granulación presente) que comprometa la oreja media y la mastoides, causando otorrea y dolor crónico (5,6). El proceso puede extenderse y comprometer el nervio facial (parálisis facial, sin dehiscencia del canal de Falopio en la TC) y la oreja interna, lo cual está manifestado como una pérdida auditiva neurosensorial rápidamente progresiva (55% de los casos revisados), tinnitus y pérdida de la función vestibular, estas últimas secundarias al depósito de complejos inmunitarios en el neuroepitelio del oído interno (1,3,7,8). La enfermedad otológica puede ser la presentación inicial en muchos pacientes con GPA.…”