FIG 4. Cochlear dysplasia with LEDS.

A, Axial T2-weighted FSE MR image of the right inner ear in a patient with LEDS shows mild dysplasia. The cochlear outline is distorted, and the normal notch between the middle and apical turns laterally (white arrow) is blunted. Note that the modiolus is deficient (black arrow).

B, Axial T2-weighted FSE MR image in another patient shows severe dysplasia. The cochlea (C) appears as a common cavity, the internal architecture is lost, and the modiolus is absent. The vestibule also shows severe dysplastic changes, including gross vestibular enlargement (V) and hypoplasia of the lateral semicircular canal (arrowhead). A portion of the enlarged endolymphatic duct is also apparent (asterisk).

(Reprinted from [22] with permission.)