Neuro-Otological Manifestations in Patients with a Hemifacial Port-Wine Stain: A Report of 2 Cases.

A port-wine stain (PWS) or nevus flammeus is a congenital capillary malformation that often affects the skin of the head and neck region. Little is known about neuro-otological manifestations associated with this birthmark. We describe 2 patients with a hemifacial PWS and sensorineural hearing loss, caused by involvement of the internal auditory canal (IAC) and inner ear structures. Case one had a history of sudden vestibular hypofunction, followed by high-frequency sensorineural hearing loss 2 years later. In the second case, the exact onset of the high-frequency hearing loss could not be determined. In both patients, magnetic resonance imaging (MRI) showed dural enhancement of the IAC and a loss of T2 signal intensity of the ipsilateral labyrinth. This report shows that a PWS of the head and neck region may be associated with dural thickening within the IAC and secondary inner ear dysfunction. In patients with this capillary malformation suffering from hearing impairment or balance problems, MRI is warranted to detect involvement of the ipsilateral dura and labyrinth.