New Imaging Findings of Incomplete Partition Type III Inner Ear Malformation and Literature Review

AJNR Am J Neuroradiol. 2020 Jun;41(6):1076-1080. doi: 10.3174/ajnr.A6576. Epub 2020 May 28.


Incomplete partition type III, also referred to as X-linked deafness, is a rare genetic inner ear malformation. Its characteristic CT findings, including bulbous dilation of the internal auditory canal and absence of the modiolus with the interscalar septa present, have been well-recognized. In this series of 19 cases, we report the abnormalities of the vestibule and semicircular canals and provide a comprehensive description of their CT and MR imaging findings. The inner ear malformations in incomplete partition type III were bilateral and basically symmetric, with involvement of the internal auditory canal, nerve canals in the fundus, cochlea, vestibule, semicircular canals, vestibular aqueduct, otic capsule, round window, oval window, and stapes. An irregular vestibule with a cystic appearance is also a distinctive imaging feature, which could be seen in about 90% of our patients, with a cystic appearance of the semicircular canals present in nearly half of the cases.

Publication types

  • Review

MeSH terms

  • Adult
  • Ear, Inner / abnormalities*
  • Ear, Inner / diagnostic imaging*
  • Female
  • Genetic Diseases, X-Linked / diagnostic imaging*
  • Genetic Diseases, X-Linked / pathology*
  • Hearing Loss, Conductive / diagnostic imaging*
  • Hearing Loss, Conductive / pathology*
  • Hearing Loss, Sensorineural / diagnostic imaging*
  • Hearing Loss, Sensorineural / pathology*
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Retrospective Studies
  • Tomography, X-Ray Computed

Supplementary concepts

  • Progressive hearing loss stapes fixation