Head and Neck ImagingMondini's malformation
(C. Mondini, 1729-1803, Italian physician),
congenital malformation of the
inner ear
. The original description by Mondini refers to a vestibule larger than normal in size and a
cochlea
with only 1.5 turns. The term has become ambiguous as it has been used to describe a wide variety of cochlear anomalies. It should be reserved for a cochlea showing an incomplete partition: the absence of the interscalar septum results in confluence of the apical and middle turn (
Fig. 1). Mondini's malformation is associated in 20% of cases with anomalies of the vestibule, semicircular canals and endolymphatic duct/sac.
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Already registered? Enter your e-mail in the window below.Re-registerFig. 01a
Axial CT image through right temporal bone. Absent interscalar septum, confluence of apical (upper arrowhead) and middle cochlear turn (oblique arrowhead) (courtesy by Marc Lemmerling, MD, PhD, Gent, Belgium).
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Mondini's malformation, Fig. 01a | |