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1. Bony atresia of the external auditory canal

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Figure 33-1

Bony atresia of the external auditory canal.

A. The right ear is normal; B. There is a thin bony atresia plate (arrow) on the left.

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2. Dilated vestibular aqueduct

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Figure 33-2

Dilated vestibular aqueduct.

An axial CT image of a 3-year-old child with left-sided hearing loss shows a markedly dilated, funnel-shaped vestibular aqueduct (arrow).

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3. Large vestibular aqueduct with large endolymphatic duct and sac

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Figure 33-3

Large vestibular aqueduct with large endolymphatic duct and sac.

This 5-year-old child was evaluated for sensorineural hearing loss. A. An axial 3D T2-weighted steady-state free-precession (TRUFI) MR image shows dilation of the endolymphatic duct and sac (arrows). B. This image is 9mm inferior to (A). The dilated endolymphatic sac (arrow) is adjacent to the surface of the cerebellum and medial to the sigmoid sinus. (Images courtesy of Brian Lundeen, M.D., Pediatric Diagnostic Imaging, Milwaukee, WI)

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4. Complete labyrinthine aplasia

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Figure 33-4

Complete labyrinthine aplasia.

A, B, C. Axial and coronal images of a 12-year-old boy show complete lack of inner ear structures bilaterally. The ossicles are present. This child also has hypoplastic right mastoid air cells. There is fluid in the right middle ear cavity.

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5. Common cavity deformity

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Figure 33-5

Common cavity deformity.

The dysplastic cochlea and enlarged vestibule form a large cyst (arrow) that lacks differentiation.

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6. Cystic cochleovestibular malformation

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Figure 33-6

Cystic cochleovestibular malformation.

A, B. Anterior-to-superior axial CT images of a 2-year-old boy show marked hypoplasia of the cochlea (arrows). C. There is mild enlargement of the vestibule. D. The cochlea (arrow) has the appearance of a small cyst on this coronal image.

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7. Mondini dysplasia

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Figure 33-7

Mondini dysplasia.

A, B, C. Contiguous coronal reformatted CT images show a wide basal turn (arrow in A). The remainder of the cochlea is a common cavity (arrow in C) that lacks an interscalar septum.

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8. Lateral semicircular canal dysplasia

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Figure 33-8

Lateral semicircular canal ...

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