Primary paraganglioma of the external auditory canal is a rare otological finding. To date, only three cases have been reported in the world-wide literature. Such a tumour is now described in a 42-year-old female. The clinical and histopathological features of the case are discussed. The role of immunocytochemistry in defining diagnosis, from a large list of differentials, is illustrated. We present the first magnetic resonance images of this rare tumour and highlight the benefit of intra-operative frozen section in limiting surgery to canal excision.
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