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Mondini deformity

There is a lack of interscalar septum with incomplete partition of middle and apical turn of cochlea with resultant enlarged cystic appearing cavity. Note that the basilar turn is present. (Contributed by Gaurang V Shah.)

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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