title: Congenital inner ear malformations without sensorineural hearing loss in children.
abstract: Inner ear malformations are frequently found in patients with congenital hearing loss. It has been reported that normal hearing is rare in patients with severe inner ear vestibular malformations. A 9-year-old boy had had complained of recurrent dizziness and disequilibrium for 2 months. Clinical and neuro-otological examinations showed peripheral involvement of the vestibular system, while audiological investigation was normal. High-resolution magnetic resonance imaging, with three-dimensional reconstruction, showed dysplasia of the bilateral lateral semicircular canals (LSCCs). Isolated vestibular malformation might not be as rare as previously thought, and should be examined by imaging of the temporal bone.
authors.affiliation: Department of Pediatrics, Graduate School of Medicine, Gifu University, 1-1 Yanagido, Gifu 501-1194, Japan. mi_ti_ti_1227@yahoo.co.jp
authors.forename: Michio
authors.initials: M
authors.lastname: Ozeki
authors.forename: Zenichiro
authors.initials: Z
authors.lastname: Kato
authors.forename: Hideo
authors.initials: H
authors.lastname: Sasai
authors.forename: Kazuo
authors.initials: K
authors.lastname: Kubota
authors.forename: Michinori
authors.initials: M
authors.lastname: Funato
authors.forename: Kenji
authors.initials: K
authors.lastname: Orii
authors.forename: Hideo
authors.initials: H
authors.lastname: Kaneko
authors.forename: Toshiyuki
authors.initials: T
authors.lastname: Fukao
authors.forename: Naomi
authors.initials: N
authors.lastname: Kondo
mesh_terms: D001301:Audiometry, Pure-Tone / Q000379:methods; D002648:Child; D000013:Congenital Abnormalities / Q000175:diagnosis*; D004244:Dizziness / Q000175:diagnosis / Q000209:etiology; D007758:Ear, Inner / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000473:pathology; D005072:Evoked Potentials, Auditory; D005500:Follow-Up Studies; D006309:Hearing / Q000502:physiology*; D006319:Hearing Loss, Sensorineural / Q000151:congenital / Q000175:diagnosis; D006801:Humans; D021621:Imaging, Three-Dimensional / Q000379:methods*; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D011857:Radiographic Image Interpretation, Computer-Assisted; D012016:Reference Values; D018570:Risk Assessment; D012665:Semicircular Canals / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000473:pathology; D012678:Sensation Disorders / Q000175:diagnosis / Q000209:etiology; D036542:Tomography, Spiral Computed / Q000379:methods; D014722:Vestibule, Labyrinth / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000473:pathology