Superficial siderosis causing retrolabyrinthine involvement in both cochlear and vestibular branches of the eighth cranial nerve.
Although superficial siderosis (SS) has been clinically characterized as a combination of sensorineural hearing impairment, cerebellar ataxia and pyramidal signs, precise evaluation of the function of the eighth cranial nerve has rarely been reported. The purpose of this study was to evaluate the audiological and vestibular function. We present a patient with complaints of progressive bilateral hearing loss and gait difficulty. We evaluated the audiological and vestibular functions with auditory brainstem responses and vestibular evoked myogenic potentials (VEMPs) by clicks and galvanic stimuli. The patient showed linear hypointensities surrounding the brainstem, cerebellum and the eighth cranial nerve on T2-weighted MRI images, which is characteristic of SS. Auditory brainstem response showed only wave I in the right ear and no response in the left ear. Click VEMPs and galvanic VEMPs showed no response on either side. The results of a neuro-otological examination suggested that both audiological and vestibular dysfunction in the patient with SS is of retrolabyrinthine origin.
Ushio
Munetaka
M
Department of Otolaryngology, Faculty of Medicine, University of Tokyo, Japan. IZT01356@nifty.ne.jp
Iwasaki
Shinichi
S
Sugasawa
Keiko
K
Murofushi
Toshihisa
T
D001259:Ataxia / Q000209:etiology / Q000503:physiopathology; D016057:Evoked Potentials, Auditory, Brain Stem / Q000502:physiology; D005684:Gait / Q000502:physiology; D006312:Hearing Loss, Bilateral / Q000209:etiology; D006319:Hearing Loss, Sensorineural / Q000209:etiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D012806:Siderosis / Q000150:complications* / Q000503:physiopathology*; D014724:Vestibular Function Tests; D000159:Vestibulocochlear Nerve; D000160:Vestibulocochlear Nerve Diseases / Q000209:etiology* / Q000503:physiopathology*