Unilateral Semicircular Canal Aplasia in Goldenhar's Syndrome
Marc M. Lemmerling
,a,
Bart D. Vanzieleghema,
Geert R. Mortiera,
Ingeborg J. Dhoogea and
Marc F. Kunnena
a From the Departments of Radiology (M.M.L., B.D.V., M.F.K.), Medical Genetics (G.R.M.), and Otorhinolaryngology (I.J.D.), University Hospital Gent, Gent, Belgium.
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FIG 1. Three-month-old boy with Goldenhar's syndrome.
A, Axial 1-mm CT section through the left temporal bone shows a normal cochlea (large arrow), with normal appearance of the modiolus and osseous spiral lamina, as well as normal posterior (small arrow) and lateral (black arrowhead) semicircular canals. Note also the normal incudomalleal articulation (white arrowhead) and the opacification of the middle ear.
B, Axial 1-mm CT section through the right middle ear shows fixation of the short process of the incus to the tympanic wall (arrow). The middle ear cavity is opacified.
C, Axial 1-mm CT section through the right inner ear shows deficiency of the cochlear modiolus (black arrow). Note the enlargement of the vestibular aqueduct (arrowheads). Because of hemifacial microsomia, extreme skull base asymmetry is present, with rotation of the clivus (white arrow). Note also the absence of semicircular canals.
D, Axial 1-mm 3D FT-CISS MR image through both temporal bones shows normal inner ear structures on the left side. Note the presence of normal lateral (arrowhead) and posterior (small arrows) semicircular canals on the left side, whereas these structures are absent on the right side. The vestibular aqueduct on the right side is abnormally enlarged (large arrow).
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AJNR - American Journal of Neuroradiology