Demographics:
3 months old, Male
Indication:
Left-sided sensorineural hearing loss
Findings
CT
- LEFT
- Normal appearance of the external and middle ear
- Severe stenosis of the cochlear aperture
- Mildly hypoplastic appearance of the apical turn
- Incidental cochlear cleft
- RIGHT
- Normal appearance of the external ear
- Small amount of soft tissue attenuation material in Prussak’s space, in the sinus tympani, and along the posterior crus of the stapes without bony erosions
- Normal appearance of the inner ear
- Incidental cochlear cleft
MRI
- Normal appearance of the right IAC with all four nerves identified
- Absent left cochlear nerve. The left facial, superior vestibular, and inferior vestibular nerves are identified
Diagnosis
Cochlear nerve aplasia
Key Imaging Features
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Differential Diagnosis
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Pearls
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Discussion
References