Appearance of ocular vestibular evoked myogenic potential elicited by bone-conducted vibration in a patient with CHARGE syndrome with aplasia of all semicircular canals

Qing Zhang; Kimitaka Kaga; Hideki Takegoshi; Takeshi Matsuda

doi:10.1016/j.ijporl.2013.12.002

Appearance of ocular vestibular evoked myogenic potential elicited by bone-conducted vibration in a patient with CHARGE syndrome with aplasia of all semicircular canals

Int J Pediatr Otorhinolaryngol. 2014 Mar;78(3):554-7. doi: 10.1016/j.ijporl.2013.12.002. Epub 2014 Jan 10.

Authors

Qing Zhang¹, Kimitaka Kaga², Hideki Takegoshi³, Takeshi Matsuda⁴

Affiliations

¹ National Tokyo Medical Center, National Institute of Sensory Organs, Tokyo, Japan; Department of Otolaryngology, Head and Neck Surgery and Ear Institute, The 2nd Affiliated Hospital, Xi'an Jiaotong University School of Medicine, Xi'an, PR China.
² National Tokyo Medical Center, National Institute of Sensory Organs, Tokyo, Japan. Electronic address: kimikaga-tky@umin.ac.jp.
³ National Tokyo Medical Center, National Institute of Sensory Organs, Tokyo, Japan; Department of Otolaryngology, Head and Neck Surgery, Mita Hospital, International University of Health and Welfare, Tokyo, Japan.
⁴ National Tokyo Medical Center, National Institute of Sensory Organs, Tokyo, Japan; Department of Otolaryngology, Head and Neck Surgery, Nihon University School of Medicine, Tokyo, Japan.

PMID: 24480121
DOI: 10.1016/j.ijporl.2013.12.002

Abstract

We report VEMP results in a patient with aplasia of bilateral semicircular canals and a small vestibular cavity. The patient was a 27-year-old male. The computed tomograph showed absolutely no formation of his semicircular canals, together with hypoplasia of his vestibular cavity and cochlea in both ears. His oVEMP was recorded near the extraocular muscles on the left side when elicited by BCV in the Fz. The clinical profile of this patient suggested that oVEMP elicited by BCV recorded near the extraocular muscles originated from otolithic end organs, and not from semicircular canal afferents.

Keywords: Aplasia; Ocular vestibular evoked myogenic potential; Otolithic end organs; Semicircular canal; Vestibular evoked myogenic potential.

Publication types

Case Reports

MeSH terms

Adult
Bone Conduction / physiology*
CHARGE Syndrome / diagnosis*
Humans
Male
Rare Diseases
Semicircular Canals / abnormalities*
Severity of Illness Index
Vestibular Evoked Myogenic Potentials / physiology*
Vestibular Function Tests
Vibration