This is a rare disease, generally presenting after 40 years of age. Symptoms typically involve persistant blood tinged ear discharge, deafness and/ or deviation of the face. There could be associated swelling in or around the ear ( infront, below or behind). Diagnosis is confirmed with appropriate tests in the form of a biopsy and imaging.

Surgery encompasses a lateral, subtotal or total temporal bone resection whereby the tumour is excised with adequate margins with/ without a parotidectomy with/ without reconstruction.Decision for further treatment of radiotherapy/ chemoradiotherapy is made based on final histopathology.