Hearing loss in PHACE syndrome: clinical and radiologic findings

Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery
Mark D MamloukKristina W Rosbe

Abstract

To characterize the types of hearing loss, auditory-related imaging findings, and hemangioma characteristics in patients with Posterior fossa malformations, Hemangiomas, Arterial anomalies, Cardiac defects, and abnormalities of the Eye (PHACE) syndrome. Retrospective medical records, audiologic data, and imaging review of all patients presenting to a tertiary care children's hospital with a proven diagnosis of PHACE syndrome from 2005 to 2016. Twelve patients were identified with hearing and imaging data. 5/12 had hearing loss, 1 had unilateral severe sensorineural loss with minor conductive component, 1 had unilateral moderate sensorineural loss with minor conductive component, 1 had mild bilateral conductive loss, 1 had bilateral hearing loss (left severe mixed and right severe sensorineural), and 1 had moderate bilateral conductive loss. All patients passed their newborn hearing screening. Of the 5 patients with hearing loss, 3 had IAC hemangiomas (1 bilateral), 3 had enlarged IACs with prominent posterior petrous bones (1 bilateral), 2 had dysgenesis of the cerebellar vermis and hemispheres, there was 1 patient each with a deformed pinna and middle ear and mastoid effusions, and 1 patient had no abnormal auditory-related im...Continue Reading

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Citations

Jun 22, 2019·Pediatric Dermatology·Nicole S StefankoDawn H Siegel
Dec 24, 2018·American Journal of Medical Genetics. Part C, Seminars in Medical Genetics·Dawn H Siegel
Nov 21, 2020·AJNR. American Journal of Neuroradiology·M D MamloukC P Hess
Jun 22, 2021·Pediatric Blood & Cancer·Jennifer L R MayerFrancine Blei
Jun 22, 2021·Journal of the American Academy of Dermatology·Lamiaa HamieMazen Kurban
Aug 23, 2021·Journal of the American Academy of Dermatology·Ana I Rodríguez BanderaLi-Chuen F Wong
Jan 10, 2022·Journal of the European Academy of Dermatology and Venereology : JEADV·O LetertreC Léauté-Labrèze

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