A young man presenting with complaints of progressive deafness in both ears with recent onset of facial paralysis was found to have necrotic debris in the nasal cavity. He was diagnosed to have limited form of Wegener's granulomatosis on the basis of nasal biopsy. There was delay in diagnosis as the patient was having atypical and predominantly otological presentation of a potentially serious systemic disorder, Wegener's granulomatosis. In this case report, emphasis was given on the necessity of comprehensive evaluation of the patients to avoid pitfalls in diagnosis.
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