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Volume 5, Issue 2, December 2019, Page: 44-47
Clinical Presentation of Congenital Cholesteatoma of the External Auditory Canal in Goldenhar Syndrome: A Case Series
Sarah Elizabeth Hodge, Department of Otolaryngology/Head and Neck Surgery, The University of North Carolina School of Medicine, Chapel Hill, USA; Craniofacial Center, The University of North Carolina School of Dentistry, Chapel Hill, USA
April Amber Peterson, Department of Otolaryngology/Head and Neck Surgery, The University of North Carolina School of Medicine, Chapel Hill, USA; Craniofacial Center, The University of North Carolina School of Dentistry, Chapel Hill, USA
Brendan Powers O’Connell, Department of Otolaryngology/Head and Neck Surgery, The University of North Carolina School of Medicine, Chapel Hill, USA; Craniofacial Center, The University of North Carolina School of Dentistry, Chapel Hill, USA
Amelia Fischer Drake, Department of Otolaryngology/Head and Neck Surgery, The University of North Carolina School of Medicine, Chapel Hill, USA; Craniofacial Center, The University of North Carolina School of Dentistry, Chapel Hill, USA
Received: Sep. 27, 2019;       Accepted: Nov. 25, 2019;       Published: Dec. 6, 2019
DOI: 10.11648/j.ijo.20190502.13      View  277      Downloads  52
Abstract
Background: Goldenhar Syndrome, also called Oculoauricular Vertebral Dysplasia, includes abnormalities of the first and second branchial arches. Ear abnormalities are present in approximately 92% of those with Goldenhar Syndrome [1]. There is a wide spectrum for ear anomalies; they can range from preauricular tags and pits, microtia, external auditory canal (EAC) atresia, ossicular malformations, and facial nerve hypoplasia, which can lead to an array of clinical findings [5]. Hearing loss is an unfortunate consequence of these clinical findings, and often results in subsequent speech and language delays [4]. Despite the widely reported auricular and audiologic manifestations of this disease, a review of the literature revealed no detailed description or analysis of congenital canal cholesteatoma specifically in this patient population. Objective: This study aims to characterize congenital external auditory canal cholesteatoma as a feature of Goldenhar Syndrome. Method: A retrospective case series was conducted with patients seen at the UNC Craniofacial Center in Chapel Hill, NC. Inclusion criteria included patients with a diagnosis of Goldenhar Syndrome also found to have congenital cholesteatoma necessitating surgery. A total of three patients were identified who met inclusion criteria. Results: Three patients with Goldenhar Syndrome were identified who also had congenital external auditory canal cholesteatoma. All three patients underwent surgical management of their disease. Conclusion: Congenital external auditory canal cholesteatoma appears to be a feature of Goldenhar Syndrome not widely described in current literature. Though rare, it is a relevant disease process with significant clinical implications for both hearing as well as speech and language development. Awareness of this condition can thus help guide practitioners in the care of these patients.
Keywords
Goldenhar Syndrome, Congenital External Auditory Canal Cholesteatoma, Oculauriculovertbral Spectrum, Hemifacial Microsomia, Craniofacial Microsomia
To cite this article
Sarah Elizabeth Hodge, April Amber Peterson, Brendan Powers O’Connell, Amelia Fischer Drake, Clinical Presentation of Congenital Cholesteatoma of the External Auditory Canal in Goldenhar Syndrome: A Case Series, International Journal of Otorhinolaryngology. Vol. 5, No. 2, 2019, pp. 44-47. doi: 10.11648/j.ijo.20190502.13
Copyright
Copyright © 2019 Authors retain the copyright of this article.
This article is an open access article distributed under the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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