| Summary: | Plexiform neurofibromas (PNs) are slow-growing, vascularised, limited, non-capsular benign tumours. They may cause functional impairments, cosmetic problems, and issues with pain or a sense of pressure. Involvement of the external ear canal, which can trigger conductive-type hearing loss and cosmetic problems, is rare. A 14-year-old male diagnosed with, and under follow-up for, type 1 neurofibromatosis presented with hearing loss and an auricle deformity caused by a mass on the postauricular region of the mastoid bone. This mass had grown gradually over the past 5 years and at the time of consultation completely filled the external ear canal, pushing the auricle forward. It was surgically removed. Postoperative histological examination allowed diagnosis of a plexiform neurofibroma. The external ear canal was successfully cleared, affording a good cosmetic outcome. Audiometric tests revealed that the air-bone gap had closed.
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