Article Figures & Data
Figures
- FIG 1.
Assessment of TAR of the cochlea on a patient without BOR (A–C) and a patient with EYA1-BOR (D–F). Standardized reformatted axial images are utilized, in which the planes are parallel to the plane of the lateral semicircular canal (A and D). Line a (green) is drawn parallel to the long axis of the basal turn (B and E), which is propagated across all axial images, including those where the apical or uppermost developed turn is visible (C and F). The midpoint of the uppermost turn is identified on the image that best displays it (black arrows in C and F); this point (point b) can also be propagated across all axial images. Line c (blue) is then drawn through point b (black arrow), perpendicular to line a (green). Distance d (between the anterior round window and the point of intersection, in yellow) and distance e (between medial bend of basal turn and the point of intersection, in red) are measured. TAR is e/d. As can be seen on these images, TAR in the patient with EYA1-BOR (F) is smaller than in the patient without BOR (C).
- FIG 2.
A, Fifths of a cochlea, as demonstrated on a 3D reconstruction of the inner ear from a heavily T2-weighted sequence (3D DRIVE). The first fifth is in red (including the hook region), the second fifth in orange, the third fifth in blue, the fourth fifth in green, and the fifth fifth in black. (B, C, and D). CT of the temporal bone in a bone algorithm in the Stenvers view (B) and axial (C and D) planes shows the fifths of the cochlea in the same color scheme as depicted on the 3D model in A.
- FIG 3.
Cochlear TAR among cochleae-deemed offset and not offset on visual assessment. EYA1-BOR: black round dots; SIX1-BOR: black square dots; unknown genotype: gray rhomboid dots; controls: gray triangle dots. The dashed line indicates the TAR cutoff (0.476) as determined by ROC curve analysis.
- FIG 4.
ROC curve.
- FIG 5.
Cochlear TAR among individuals with EYA1-BOR, SIX1-BOR, BOR of unknown genotype, and controls without BOR or sensorineural hearing loss. The TAR cutoff of 0.476 was determined by ROC curve analysis. All except one of the EYA1-BOR cochleae have TAR below the cutoff value. All except one of the SIX1-BOR cochleae have TAR above the cutoff value. All individuals with BOR of unknown genotype have TAR below the cutoff value. None of the controls have TAR below the cutoff value.
- FIG 6.
Axial CT images of 3 different patients with EYA1-BOR showing the anteriorly offset unwound cochlea, an imaging feature characteristic of EYA1-BOR. Notice that the middle and apical turns are anteriorly located relative to the basal turn (red arrows) and slightly tilted away and separated from the basal turn (yellow arrowheads).
- FIG 7.
CT image of the patient with SIX1-BOR in whom the right cochlea does not demonstrate any offset (long arrow), while the left cochlea has an offset but with an appearance akin to cochlear hypoplasia type 4 rather than the typical unwound and offset cochlea of EYA1-BOR. Notice the normal size and morphology of the basal turn first half (short arrow), while the distal basal, middle, and apical turns are hypoplastic (arrowhead).
- FIG 8.
A thorny apical turn in a patient with SIX1-BOR. The apical turn of the cochlea appears as a short, protuberant, thorny tip, as seen on CT (A) and MR imaging (B).
Tables
- Table 1:
Qualitative (columns 3 and 4) and quantitative assessment (TAR, column 5) of cochlear offset
Total No. of Cochleae No. of Cochleae with Offset No. of Cochleae without Offset Mean TAR EYA1-BOR 32 32 0 0.338 (SD, 0.071) SIX1-BOR 8 1 7 0.577 (SD, 0.107) Unknown genotype BOR 40 40 0 0.339 (SD, 0.057) Controls 80 0 80 0.598 (SD, 0.051) Total No. of Cochleae No. of Cochleae with Thorny Tips EYA1-BOR 32 0 SIX1-BOR 8 7 Unknown genotype BOR 40 0 Controls 80 0
Jump to section
Related Articles
Cited By...
- No citing articles found.