Demographics:
3 years old, Male
Indication:
Bilateral sensorineural hearing loss
Findings
- Absence of the modiolus and lamina cribrosa bilaterally with corkscrew appearance of the cochlea and wide communication between the base of the cochlea and the IAC
- Enlarged, lobulated appearance of the vestibules and posterior aspects of the lateral semicircular canals
- Enlargement of the bilateral labyrinthine facial nerve canals
- Small amount of soft tissue in Prussak’s space on the right without blunting of the scutum or adjacent ossicular erosions
- Abnormal soft tissue in the region of right oval window/stapes footplate could represent abnormal fixation of the stapes
- Normal appearance of the left middle ear
- Normal appearance of the bilateral external ears
Diagnosis
Cochlear incomplete partition type III (IP-III)
Key Imaging Features
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Differential Diagnosis
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Pearls
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Discussion