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Pediatric Radiology
Published in: Pediatric Radiology 7/2015

01-07-2015 | Case Report

Ectopic external auditory canal and ossicular formation in the oculo-auriculo-vertebral spectrum

Authors: Nucharin Supakul, Stephen F. Kralik, Chang Y. Ho

Published in: Pediatric Radiology | Issue 7/2015

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Abstract

Ear abnormalities in oculo-auricular-vertebral spectrum commonly present with varying degrees of external and middle ear atresias, usually in the expected locations of the temporal bone and associated soft tissues, without ectopia of the external auditory canal. We present the unique imaging of a 4-year-old girl with right hemifacial microsomia and ectopic location of an atretic external auditory canal, terminating in a hypoplastic temporomandibular joint containing bony structures with the appearance of auditory ossicles. This finding suggests an early embryological dysfunction involving Meckel’s cartilage of the first branchial arch.
Literature
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Rosa R, Silva A, Goetze T et al (2011) Ear abnormalities in patients with oculo-auriculo-vertebral spectrum (Goldenhar syndrome). Braz J Otorhinolaryngol 77:455–460PubMedCrossRef
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Rollnick B, Kaye C, Nagatoshi K et al (1987) Oculoauriculovertebral dysplasia and variants: phenotypic characteristics of 294 patients. Am J Med Genet 26:361–375PubMedCrossRef
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Hartsfield J (2007) Review of the etiologic heterogeneity of the oculo-auriculo-vertebral spectrum (Hemifacial Microsomia). Orthod Craniofac Res 10:121–128
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Barisic I, Odak L, Loane M et al (2014) Prevalence, prenatal diagnosis and clinical features of oculo-auriculo-vertebral spectrum: a registry-based study in Europe. Eur J Hum Genet 22:1026–1033PubMedCrossRef
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Dos Santos PA, de Oliveira SF, Freitas EL et al (2014) Non-overlapping 22q11.2 microdeletions in patients with oculo-auriculo-vertebral spectrum. Am J Med Genet A 164A:551–553PubMedCrossRef
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Bisdas S, Lenarz M, Lenarz T et al (2005) Inner ear abnormalities in patients with Goldenhar syndrome. Otol Neurotol 26:398–404PubMedCrossRef
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Metadata
Title
Ectopic external auditory canal and ossicular formation in the oculo-auriculo-vertebral spectrum
Authors
Nucharin Supakul
Stephen F. Kralik
Chang Y. Ho
Publication date
01-07-2015
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Radiology / Issue 7/2015
Print ISSN: 0301-0449
Electronic ISSN: 1432-1998
DOI
https://doi.org/10.1007/s00247-014-3237-0

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