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Mondini malformation (CT))

High-resolution axial CT scans of the temporal bone in a patient with Mondini malformation, showing (A) a preserved basal turn but absence of the apical turns, the cochlea (black arrow), and a dilated vestibule (white arrow), and (B) an enlarged vestibular aqueduct (white arrow). (Contributed by Creative Commons Attribution 4.0 International License and can be accessed at http://creativecommons.org/licenses/by/4.0/). Note: The figure legend was modified.

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Associated Disorders

  • Cochlear dysplasia
  • DiGeorge syndrome
  • Incomplete partition of cochlea
  • Internal auditory canal dysplasia
  • Klippel-Feil syndrome
  • Labyrinthine dysplasia
  • Large vestibular aqueduct syndrome
  • Pendred syndrome
  • Semicircular canal dysplasia