Fact-sheet: Mondini dysplasia
Updated on 03/26/2020 at 10:07 AM
Note : 8/10
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Definition
Inner ear malformation, with anomalies of the upper turns of the cochlea.
The basal turn of the cochlea remains preserved.
These anomalies are related to modiolus hypoplasia.
Clinical features
Severe sensorineural hearing loss.
CT
Cystic hypodense appearance showing fusion of the second and third turns of the cochlea.
Deformity of the vestibular aqueduct, sometimes short and enlarged.
Enlargement of the vestibule.
MRI
Better assessment of the cochlea and posterior fossa.