We report a case of an arteriovenous malformation within the internal auditory meatus and cerebellopontine angle. A 46-year-old woman presented with a five-year history of unilateral hearing loss and recent aggravation. Initially she misdiagnosed as sudden hearing loss and treated with systemic steroid. 6 months later, her hearing worsened and vertigo was developed. Magnetic resonance imaging and magnetic resonance angiography demonstrated a lesion consisting of multiple high flow vessels within the left internal auditory canal and cerebellopontine angle. Patient was recommended to receive transarterial embolisation, but refused and was just followed up. Arteriovenous malformations of the internal auditory canal and cerebellopontine angle are exceedingly rare. It is important that a pre-treatment diagnosis is made, as the management of arteriovenous malformation differs from that of other, more common lesions at this site.
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