| Association of Hearing Loss With PHACE Syndrome. | |
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MedLine Citation:
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PMID: 20713775 Owner: NLM Status: In-Data-Review |
Abstract/OtherAbstract:
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BACKGROUND: PHACE syndrome describes a spectrum of anomalies associated with large facial infantile hemangiomas and characterized by posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities. With improved recognition and imaging practices of infants with PHACE syndrome, additional associations have been identified. To our knowledge, the potential association of ipsilateral hearing loss and PHACE syndrome has not been previously emphasized. OBSERVATIONS: We describe 6 patients, 4 with definite and 2 with probable PHACE syndrome, according to the new diagnostic criteria, and associated auditory deficiencies. One patient had isolated conductive hearing loss; 2 patients had isolated sensorineural hearing loss; 1 patient had mixed hearing loss (both conductive and sensorineural components); and 1 patient had hearing loss that was inconclusive at the time. Also, 1 patient had conductive loss and auditory neuropathy and auditory dyssynchrony. Four of the 6 patients had magnetic resonance imaging features of lesions consistent with intracranial hemangiomas involving auditory structures. All 6 patients had facial hemangiomas in a nearly identical distribution ipsilateral to the ear with the hearing loss, with involvement of the proposed facial segments S1 and S3, the affected ear, the periauricular region, and the midoccipital area of the scalp. CONCLUSIONS: There is an underrecognized risk of hearing loss in patients with PHACE syndrome, although the exact nature of such deficiencies can vary. Patients with PHACE syndrome who have cutaneous hemangiomas involving the ear should be evaluated for intracranial hemangiomas and monitored for hearing loss. Early detection and therapy of intracranial hemangiomas may slow or stop tumor growth, resultant hearing loss, and structural damage. |
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Authors:
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Kelly J Duffy; Christina Runge-Samuelson; Michelle L Bayer; David Friedland; Cecille Sulman; Robert Chun; Joseph E Kerschner; Denise Metry; Denise Adams; Beth A Drolet |
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Publication Detail:
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Type: Journal Article Date: 2010-08-16 |
Journal Detail:
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Title: Archives of dermatology Volume: 146 ISSN: 1538-3652 ISO Abbreviation: Arch Dermatol Publication Date: 2010 Dec |
Date Detail:
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Created Date: 2010-12-21 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0372433 Medline TA: Arch Dermatol Country: United States |
Other Details:
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Languages: eng Pagination: 1391-6 Citation Subset: AIM; IM |
Affiliation:
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Department of Dermatology, Medical College of Wisconsin, 9000 W Wisconsin Ave, Milwaukee, WI 53226. drolet@mcw.edu. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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