Presentation
Hearing loss bilaterally; Sensorineural loss on audiometry. No discharge.
Patient Data
Age: 6 years
Gender: Male
From the case:
Mondini malformation
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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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