Presentation
Bilateral sensorineural hearing loss.
Patient Data
Age: 4 years
Gender: Female
From the case:
Mondini malformation
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Abnormal cochlea (only with basal turn) and enlarged vestibule are seen bilaterally.
Case Discussion
A Mondini malformation is an inner ear abnormality that consists a triad :
- abnormal cochlea
- only 1.5 turns (instead of the normal 2.5 turns)
- normal basal turn with a cystic apex in place of the distal 1.5 turns
- enlarged vestibule with normal semicircular canals
- enlarged vestibular aqueduct containing a dilated endolymphatic sac
A case of Dr. Masood Gholamian, Tabriz university.