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Ectopic external auditory canal and ossicular formation in the oculo-auriculo-vertebral spectrum
Nucharin Supakul1, Stephen F Kralik, Chang Y Ho
1Department of Radiology, Indiana University School of Medicine, Indianapolis, IN, USA.
Pediatric Radiology
|December 7, 2014
Summary
This study details a rare case of an ectopic external auditory canal in a child with hemifacial microsomia. The unique imaging suggests early embryonic issues related to the first branchial arch.
Area of Science:
- Medical Imaging
- Embryology
- Genetics
Background:
- Oculo-auricular-vertebral spectrum (OAVS) typically involves ear abnormalities with atresias in normal locations.
- External auditory canal ectopia is uncommon in OAVS.
Observation:
- A 4-year-old girl presented with right hemifacial microsomia.
- Unique imaging revealed an atretic external auditory canal in an ectopic location.
- The canal terminated in a hypoplastic temporomandibular joint with ossicle-like structures.
Findings:
- This case presents a unique manifestation of ear abnormalities within the oculo-auricular-vertebral spectrum.
- The ectopic location of the atretic external auditory canal is a significant deviation from typical presentations.
- The presence of structures resembling auditory ossicles within the temporomandibular joint is a novel observation.
Implications:
- This finding points to a potential early embryological dysfunction.
- Meckel's cartilage of the first branchial arch is implicated in this unique presentation.
- Further research into first branchial arch development may elucidate mechanisms behind such rare conditions.
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