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Updated: Aug 26, 2025
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In Vivo Morphometric Analysis of Human Cranial Nerves Using Magnetic Resonance Imaging in Menière's Disease Ears and Normal Hearing Ears
Published on: February 21, 2018
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Vestibular Imaging and Function in Patients With Inner Ear Malformation Presenting With Profound Hearing Loss
Alexander Edward S Dy1,2, Akinori Kashio1, Chisato Fujimoto1
1Department of Otolaryngology and Head and Neck Surgery, Faculty of Medicine, The University of Tokyo, Tokyo, Japan.
OTO Open
|October 3, 2022
Summary
Children with inner ear malformations often have abnormal vestibular function. Abnormal vestibule volume correlates with nonreactive cervical vestibular evoked myogenic potential (cVEMP) tests, suggesting a link between anatomy and function.
Area of Science:
- Otolaryngology
- Pediatric Audiology
- Neuroscience
Background:
- Vestibular impairment is noted in congenital hearing loss.
- Limited understanding of vestibular anatomy and function in children with inner ear malformations.
Purpose of the Study:
- Investigate the association between vestibulocochlear anatomy and vestibular function test results in children with inner ear malformations.
Main Methods:
- Retrospective chart review of pediatric patients with inner ear malformations and profound hearing loss.
- Classification of ears based on CT imaging and analysis of vestibular function tests (cVEMP, rotatory chair, caloric).
Main Results:
- Incomplete partition type II was the most common malformation (29.3%).
- Isolated vestibular organ anomaly (20.7%) was frequent and not a recognized category.
- Most ears showed abnormal vestibular function; abnormal vestibule volume linked to nonreactive cVEMP (P < .001).
- Abnormal lateral semicircular canals correlated with abnormal caloric and rotatory chair results (P < .001).
Conclusions:
- Suggest adding isolated vestibular organ anomaly as a distinct category of inner ear malformations.
- Abnormal vestibule volume is significantly associated with nonreactive cVEMP.
- Most children with hearing loss due to inner ear malformations exhibit abnormal vestibular function.
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