New Classification of Cochlear Hypoplasia Type Malformation: Relevance in Cochlear Implantation

J Int Adv Otol. 2020 Aug;16(2):153-157. doi: 10.5152/iao.2020.7690.


Objectives: This paper attempts to create a new classification type of cochlear hypoplasia (CH)-type malformation taking into consideration of vestibular section and internal auditory canal (IAC).

Materials and methods: Preoperative computed-tomography (CT) scans of cochlear implant (CI) candidates (N=31) from various clinics across the world with CH type malformation were taken for analysis. CT dataset were loaded into 3D-slicer freeware for three-dimensional (3D) segmentation of the inner-ear by capturing complete inner-ear structures from the entire dataset. Cochlear size in terms of diameter of available cochlear basal turn and length of cochlear lumen was measured from the dataset. In addition, structural connection between IAC and cochlear portions was scrutinized, which is highly relevant to the proposed CH classification in this study.

Results: CH group-I has the normal presence of IAC leading to cochlear and vestibular portions, whereas CH group-II is like CH group-I but with some degree of disruption in vestibular portion. In CH group-III, a disconnection between IAC and the cochlear portion irrespective of other features. Within all these three CH groups, the basal turn diameter varied between 3.1 mm and 9.6 mm, and the corresponding cochlear lumen length varied between 3 mm and 21 mm for the CI electrode array placement.

Conclusion: A new classification of CH mainly based on the IAC connecting the cochlear and vestibular portions is presented in this study. CI electrode array length could be selected based on the length of the cochlear lumen, which can be observed from the 3D image.

MeSH terms

  • Cochlea / abnormalities*
  • Cochlea / diagnostic imaging*
  • Cochlea / surgery
  • Cochlear Diseases / classification*
  • Cochlear Diseases / congenital
  • Cochlear Diseases / surgery
  • Cochlear Implantation*
  • Humans
  • Preoperative Period
  • Semicircular Canals / abnormalities
  • Semicircular Canals / diagnostic imaging
  • Semicircular Canals / surgery
  • Tomography, X-Ray Computed / classification*
  • Vestibule, Labyrinth / abnormalities
  • Vestibule, Labyrinth / diagnostic imaging
  • Vestibule, Labyrinth / surgery