Mondini malformation

Case contributed by Mohammad Taghi Niknejad
Diagnosis certain


Bilateral sensorineural hearing loss.

Patient Data

Age: 4 years
Gender: Female

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 :

  1. 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
  2. enlarged vestibule with normal semicircular canals
  3. enlarged vestibular aqueduct containing a dilated endolymphatic sac

A case of Dr. Masood Gholamian, Tabriz university.

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