患者背景:
3 歳, 男性
適応:
Bilateral sensorineural hearing loss
所見
- 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
診断
Cochlear incomplete partition type III (IP-III)
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鑑別診断
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臨床のポイント
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考察