Appearance of ocular vestibular evoked myogenic potential elicited by bone-conducted vibration in a patient with CHARGE syndrome with aplasia of all semicircular canals.
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.
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.
Qing
Q
Zhang
National Tokyo Medical Center, National Institute of Sensory Organs, Tokyo, Japan. Electronic address: kimikaga-tky@umin.ac.jp.
Kimitaka
K
Kaga
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.
Hideki
H
Takegoshi
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.
Takeshi
T
Matsuda
D000328:Adult; D001844:Bone Conduction / Q000502:physiology*; D058747:CHARGE Syndrome / Q000175:diagnosis*; D006801:Humans; D008297:Male; D035583:Rare Diseases; D012665:Semicircular Canals / Q000002:abnormalities*; D012720:Severity of Illness Index; D058585:Vestibular Evoked Myogenic Potentials / Q000502:physiology*; D014724:Vestibular Function Tests; D014732:Vibration
Aplasia; Ocular vestibular evoked myogenic potential; Otolithic end organs; Semicircular canal; Vestibular evoked myogenic potential