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.
| Published in | International Journal of Otorhinolaryngology (Volume 5, Issue 2) |
| DOI | 10.11648/j.ijo.20190502.13 |
| Page(s) | 44-47 |
| Creative Commons |
This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited. |
| Copyright |
Copyright © The Author(s), 2019. Published by Science Publishing Group |
Goldenhar Syndrome, Congenital External Auditory Canal Cholesteatoma, Oculauriculovertbral Spectrum, Hemifacial Microsomia, Craniofacial Microsomia
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APA Style
Sarah Elizabeth Hodge, April Amber Peterson, Brendan Powers O’Connell, Amelia Fischer Drake. (2019). Clinical Presentation of Congenital Cholesteatoma of the External Auditory Canal in Goldenhar Syndrome: A Case Series. International Journal of Otorhinolaryngology, 5(2), 44-47. https://doi.org/10.11648/j.ijo.20190502.13
ACS Style
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. Int. J. Otorhinolaryngol. 2019, 5(2), 44-47. doi: 10.11648/j.ijo.20190502.13
AMA Style
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. Int J Otorhinolaryngol. 2019;5(2):44-47. doi: 10.11648/j.ijo.20190502.13
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Download@article{10.11648/j.ijo.20190502.13,
author = {Sarah Elizabeth Hodge and April Amber Peterson and Brendan Powers O’Connell and Amelia Fischer Drake},
title = {Clinical Presentation of Congenital Cholesteatoma of the External Auditory Canal in Goldenhar Syndrome: A Case Series},
journal = {International Journal of Otorhinolaryngology},
volume = {5},
number = {2},
pages = {44-47},
doi = {10.11648/j.ijo.20190502.13},
url = {https://doi.org/10.11648/j.ijo.20190502.13},
eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ijo.20190502.13},
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.},
year = {2019}
}
TY - JOUR T1 - Clinical Presentation of Congenital Cholesteatoma of the External Auditory Canal in Goldenhar Syndrome: A Case Series AU - Sarah Elizabeth Hodge AU - April Amber Peterson AU - Brendan Powers O’Connell AU - Amelia Fischer Drake Y1 - 2019/12/06 PY - 2019 N1 - https://doi.org/10.11648/j.ijo.20190502.13 DO - 10.11648/j.ijo.20190502.13 T2 - International Journal of Otorhinolaryngology JF - International Journal of Otorhinolaryngology JO - International Journal of Otorhinolaryngology SP - 44 EP - 47 PB - Science Publishing Group SN - 2472-2413 UR - https://doi.org/10.11648/j.ijo.20190502.13 AB - 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. VL - 5 IS - 2 ER -
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