Neuro-Otological Manifestations in Patients with a Hemifacial Port-Wine Stain: A Report of 2 Cases
Year: 2025
Type: article
Study design: Case Report
Abstract: : A port-wine stain (PWS) or nevus flammeus is a congenital capillary malformation that often affects the skin of the head and neck region. Littleis 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 ofT2 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.Cite this article as: Delrue S, Van Oosterwyck R, Cammaert T, et al. Neuro-otological manifestations in patients with a hemifacial port-wine stain: a report of 2 cases. J Int Adv Otol. 2025, 21(2), 1684, doi: 10.5152/iao.2025.241684.
Institutions: AZ Sint-Lucas, AZ Delta, Ghent University, Antwerp University Hospital
Language: English
Subfield: Neurology
Field: Neuroscience
Domain: Life Sciences
Open Access status: diamond
OpenAlex ID: W4408908953