- MR Imaging in Menière Disease: Is the Contact between the Vestibular Endolymphatic Space and the Oval Window a Reliable Biomarker?
The authors retrospectively enrolled 49 patients, 24 affected by unilateral sudden hearing loss and 25 affected by definite Meniére disease, who had undergone a 4-hour delayed 3D-FLAIR sequence. Two readers analyzed the MR images investigating whether the vestibular endolymphatic space bulged in the third inferior portion of the vestibule contacting the stapes footplate. The vestibular endolymphatic space contacting the oval window has high specificity and positive predictive value in differentiating Meniére disease ears from other ears, thus resulting in a valid tool for ruling in Meniére disease in patients with mimicking symptoms.
- Expanding the Distinctive Neuroimaging Phenotype of ACTA2 Mutations
Patients with the ACTA2 mutation have distinctive clinical and angiographic features—specifically, a combination of ectasia and stenosis, a straight arterial course, absence of basal collaterals, and more widespread cerebrovascular involvement in comparison with Moyamoya disease. Neuroimaging studies from 13 patients with heterozygous Arg179His mutations in ACTA2 and 1 patient with pathognomonic clinicoradiologic findings for ACTA2 mutation were retrospectively reviewed. Characteristic bending and hypoplasia of the anterior corpus callosum, apparent absence of the anterior gyrus cinguli, and radial frontal gyration were present in 100% of the patients; flattening of the pons on the midline and multiple indentations in the lateral surface of the pons were demonstrated in 93% of the patients.
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