Mondini malformation

Case contributed by A.Prof Frank Gaillard

Presentation

Hearing loss bilaterally; Sensorineural loss on audiometry. No discharge.

Patient Data

Age: 6
Gender: Male
CT

Cochlea has 1.5 turns instead of usual 2.5 turns bilaterally; Dilated vestibular aqueduct as well as horizontal semicircular canals.

Case Discussion

Mondini malformation (bilateral) as seen in this case, is a common cause of congenital sensorineural hearing loss. Since basal turns are intact, hearing would not be however completely lost, and it would be purely sensorineural.

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Case information

rID: 5590
Case created: 13th Feb 2009
Last edited: 9th Dec 2015
System: Head & Neck
Inclusion in quiz mode: Included

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