Demographics:
57 years old, Female
Indication:
Congenital bilateral sensorineural hearing loss
Findings
- External ears:
- Normal appearance bilaterally
- Middle ears:
- Normal appearance bilaterally
- Internal ears:
- The cochlea has a cystic appearance bilaterally with incompletely formed middle and apical turns and absent modiolus
- On the right, the lateral semicircular has a dysplastic appearance with absent central bone. Additionally, there is bulbous enlargement of the common crus of the posterior and superior semicircular canals
- Mild bilateral internal auditory canal narrowing
- Additional:
- Atlanto-occipital assimilation with platybasia
Diagnosis
Cochlear hypoplasia (type II)
Key Imaging Features
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Differential Diagnosis
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Pearls
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Discussion