title: Enlargement of the Internal Auditory Canal and Associated Posterior Fossa Anomalies in PHACES Association.
abstract: We noted enlargement of the internal auditory canal in several of our patients with posterior fossa malformations, hemangiomas, arterial anomalies, cardiac defects, eye abnormalities, and sternal or supraumbilical defects (PHACES) association and hence evaluated children with PHACES for the presence of an enlarged internal auditory canal and potential associated findings, including infantile hemangioma within the internal auditory canal, to understand the genesis of this enlargement. We reviewed our records to identify children with PHACES association who had been evaluated with MR imaging at our institutions. Imaging was reviewed for abnormal enhancement in the internal auditory canal, internal auditory canal enlargement, cerebellar hypoplasia, prominence of the petrous ridge, and deformity of the calvarium. We raise the possibility of an association between enlargement of the internal auditory canal in PHACES and a generalized malformation of the posterior fossa with cerebellar and calvarial hypoplasia.
authors.affiliation: From the Department of Radiology (D.E.M., F.B.), St. Luke's-Roosevelt Hospital Center, New York, New York dmeltzer@chpnet.org.
authors.forename: D E
authors.initials: DE
authors.lastname: Meltzer
authors.affiliation: Department of Radiology (C.D.R.), Boston Children's Hospital/Harvard Medical School, Boston, Massachusetts.
authors.forename: C D
authors.identifier: http://orcid.org/0000-0001-5592-249X
authors.initials: CD
authors.lastname: Robson
authors.affiliation: From the Department of Radiology (D.E.M., F.B.), St. Luke's-Roosevelt Hospital Center, New York, New York Vascular Birthmark Institute of New York (F.B.), New York, New York.
authors.forename: F
authors.initials: F
authors.lastname: Blei
authors.affiliation: New York Eye and Ear Infirmary (R.A.H.), New York, New York.
authors.forename: R A
authors.initials: RA
authors.lastname: Holliday
mesh_terms: D001017:Aortic Coarctation / Q000473:pathology*; D002531:Cerebellum / Q000002:abnormalities / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D002658:Developmental Disabilities / Q000209:etiology / Q000473:pathology; D007758:Ear, Inner / Q000473:pathology*; D005124:Eye Abnormalities / Q000473:pathology*; D005260:Female; D006801:Humans; D008297:Male; D009421:Nervous System Malformations / Q000209:etiology / Q000473:pathology; D020752:Neurocutaneous Syndromes / Q000473:pathology*; D012886:Skull / Q000473:pathology