4 Y/O female with bilateral hearing loss.
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Abnormal cochlea (only with basal turn) and enlarged vestibule are seen bilaterally.
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.
- 1. Gorlin RJ, Toriello HV, Cohen MM. Hereditary hearing loss and its syndromes. Oxford University Press, USA. (1995) ISBN:0195065522. Read it at Google Books - Find it at Amazon
- 2. Lo WW. What is a 'Mondini' and what difference does a name make? AJNR Am J Neuroradiol. 1999;20 (8): 1442-4. AJNR Am J Neuroradiol (full text) - Pubmed citation