Fact-sheet: Otic capsule dysplasia
Updated on 04/20/2018 at 5:51 PM
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Definition
Inner ear malformation.
Classification
Sennaroglu classification.
- complete labyrinthine aplasia: Michel aplasia
- cochlear aplasia
- common cavity
- incomplete partition of the cochlea, 3 types: absence of the modiolus and septa, with the cochlea appearing as a coarse cystic structure. The vestibule is most often dilated.
- Mondini malformation: normal basal turn of the cochlea, modiolar hypoplasia with fusion of the middle and apical turns. Enlarged vestibular aqueduct.
- cochlear malformation seen in X-linked deafness. Absent modiolus but preserved septa. Cochlea in direct continuity with the IAC.
- cochlear hypoplasia, 3 types: "cochlear bud" without modiolus or septa. Resembles incomplete partition type I but the cochlea is "small."
- cystic cochlea without modiolus or septa but with preserved external architecture. Wide communication with the IAC (gusher or geyser ear).
- micro-cochlea, < 2 turns.
- LVAS "large vestibular aqueduct syndrome": enlargement of the vestibular aqueduct.
- Narrow IAC.
Always assess the status of the cochlear nerve (essential for cochlear implantation), the course of the facial nerve, and the presence of a gusher/geyser ear (wide cochlear fossette).
