Vol.98 No.2 contentsPRACTICA OTO-RHINO-LARYNGOLOGICA
Vol. 98 No. 2 February 2005
Two Cases of Large Vestibular Aqueduct Syndrome
Takashi Shimizu, Gunji Nagatani, Takeyuki Fujimura,
Kyoya Goto and Hideaki Suzuki
(University of Occupational and Environmental Health, School of Medicine)
We report two cases of large vestibular aqueduct syndrome, a rare congenital malformation of the inner ear originally described by Valvassori and Clemis in 1978.
The first case was a 38-year-old woman who had complained of progressive hearing loss since childhood and periodic episodes of recurrent vertigo since 30 years of age. She had been misdiagnosed as having Meniere's disease by previous doctors. The second case was a 9-year-old boy with acute hearing loss several hours after minor head collision. Psychogenic hearing loss was initially suspected, but was eventually ruled out based on diminished auditory brainstem response. When these patients were initially seen in our clinic, both patients showed severe bilateral combined hearing loss on pure tone audiogram. Definitive diagnosis was established by magnetic resonance imaging, which demonstrated expanded endolymphatic ducts and sacs.
Clinical characteristics and possible pathogenesis of large vestibular aqueduct syndrome is discussed with a review of the literature.
Key words : large vestibular aqueduct syndrome, magnetic resonance imaging, endolymphatic duct, endolymphatic sac