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OtherHEAD AND NECK

Osteogenesis Imperfecta of the Temporal Bone: CT and MR Imaging in Van der Hoeve-de Kleyn Syndrome

Hatem Alkadhi, Diana Rissmann and Spyros S. Kollias
American Journal of Neuroradiology June 2004, 25 (6) 1106-1109;
Hatem Alkadhi
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Diana Rissmann
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Spyros S. Kollias
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    Fig 1.

    Initial axial (A) and direct coronal (B) high-resolution CT scans of the temporal bones through the level of the labyrinthine segment of the facial nerve (axial) and the cochlea (coronal) processed with a bone algorithm (2-mm section thickness; 512 × 512 matrix). The facial nerve canal showed slight irregularities along the labyrinthine segment (arrowhead) but otherwise appeared normal. Bandlike, undermineralized areas around the cochlea can be seen (arrowheads).

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    Fig 2.

    Further axial (A) and direct coronal (B) high-resolution CT scans through the level of the labyrinthine segment of the facial nerve (axial) and the cochlea (coronal) obtained 2 years after the initial scans (1-mm section thickness; 512 × 512 matrix). The labyrinthine segment, the geniculate ganglion (arrowheads), and the proximal tympanic segment of the facial nerve canal are severely involved and have indistinct, irregular margins. Progression of demineralization is also demonstrated in pericochlear areas (arrowheads).

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    Fig 3.

    Axial (A) and coronal (B) T1-weighted gadolinium-enhanced images (TR/TE/NEX, 500/20/3; section thickness, 3 mm) through the level of the distal internal auditory canal (axial) on the left, and the cochlea (coronal) on the right side. Enhancement is depicted in the fundus of the internal auditory canal, along the labyrinthine and proximal tympanic segments of the facial nerve, and in the geniculate ganglion (arrows). Further bandlike, pericochlear contrast enhancement is demonstrated, corresponding to the areas of CT hypoattenuation (arrowheads).

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American Journal of Neuroradiology: 25 (6)
American Journal of Neuroradiology
Vol. 25, Issue 6
1 Jun 2004
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Hatem Alkadhi, Diana Rissmann, Spyros S. Kollias
Osteogenesis Imperfecta of the Temporal Bone: CT and MR Imaging in Van der Hoeve-de Kleyn Syndrome
American Journal of Neuroradiology Jun 2004, 25 (6) 1106-1109;

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Osteogenesis Imperfecta of the Temporal Bone: CT and MR Imaging in Van der Hoeve-de Kleyn Syndrome
Hatem Alkadhi, Diana Rissmann, Spyros S. Kollias
American Journal of Neuroradiology Jun 2004, 25 (6) 1106-1109;
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