PT  - JOURNAL ARTICLE
AU  - R. Hong
AU  - Q. Du
AU  - Y. Pan
TI  - New Imaging Findings of Incomplete Partition Type III Inner Ear Malformation and Literature Review
AID  - 10.3174/ajnr.A6576
DP  - 2020 Jun 01
TA  - American Journal of Neuroradiology
PG  - 1076--1080
VI  - 41
IP  - 6
4099  - http://www.ajnr.org/content/41/6/1076.short
4100  - http://www.ajnr.org/content/41/6/1076.full
SO  - Am. J. Neuroradiol.2020 Jun 01; 41
AB  - SUMMARY: 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.CNcochlear nerveDFNX2X-linked deafness type 2IACinternal auditory canalIP-IIIincomplete partition type IIISCCsemicircular canalVAvestibular aqueductVNvestibular nerve