Head and Neck ImagingGoldenhar's syndrome
(Maurice Goldenhar, 20th century, Swiss physician), oculo-auriculovertebral dysplasia, a syndrome characterized by epibulbar dermoids, auricular pits and appendages, micrognathia, and vertebral and other anomalies. Associated
temporal bone anomalies are seen in the
external ear
(atresia of the external auditory canal),
middle ear
(small cavity with absent ossicles) and sometimes also in the
inner ear
(
Fig. 1).. Most cases are sporadic, but there have been reports of both autosomal dominant and recessive transmissions. Goldenhar's syndrome can be considered as a variant of
craniofacial microsomia
.
RH
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Two-weeks old child. A. Axial CT image of right temporal bone shows duplicated middle ear cavity (arrows), only the medial compartiment being aerated; in the lateral compartiment rudimentary auditory ossicles are seen. The tympanic segment of the facial nerve has an aberrant course, running in the bony plate separating both middle ear compartiments (arrowheads).
B. Coronal CT image shows absence of external auditory canal (asterisks); labyrinthine (black arrow) and tympanic segment (arrowhead) of facial nerve. Malformed auditory ossicles (white arrow).
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Goldenhar's syndrome, Fig. 01a (a) | | Goldenhar's syndrome, Fig. 01b (b) | |