title: Circling behavior in the Ecl mouse is caused by lateral semicircular canal defects.
abstract: The epistatic circler mouse (Ecl mouse) is a preexisting mutant, which displays a circling phenotype and hyperactivity. It has been shown that the circling phenotype in this mutant results from a complex inheritance pattern, but the vestibular pathology has not been analyzed. The present study deals with the morphological and functional basis responsible for the circling behavior in the Ecl mouse. Morphological examination of the inner ears revealed a bilateral malformation of the horizontal (lateral) semicircular canal and duct. No cochlear abnormalities were detected, and auditory brainstem response (ABR) measurements indicated that the auditory system is not affected. Investigation of the vestibuloocular reflex (VOR) in Ecl mice showed that their horizontal VOR on stimulation is virtually absent, which correlates with the morphological findings.
authors.affiliation: Department of Medical Genetics, University of Antwerp, 2610 Antwerp, Belgium.
authors.forename: Kim
authors.initials: K
authors.lastname: Cryns
authors.forename: Arjan M
authors.initials: AM
authors.lastname: van Alphen
authors.forename: Michiel P
authors.initials: MP
authors.lastname: van Spaendonck
authors.forename: Paul H
authors.initials: PH
authors.lastname: van de Heyning
authors.forename: Jean-Pierre
authors.initials: JP
authors.lastname: Timmermans
authors.forename: Chris I
authors.initials: CI
authors.lastname: de Zeeuw
authors.forename: Guy
authors.initials: G
authors.lastname: van Camp
mesh_terms: D000818:Animals; D001306:Auditory Pathways / Q000503:physiopathology; D003051:Cochlea / Q000002:abnormalities / Q000503:physiopathology; D018069:Hair Cells, Vestibular / Q000002:abnormalities / Q000648:ultrastructure; D051379:Mice; D008818:Mice, Neurologic Mutants / Q000002:abnormalities* / Q000235:genetics; D008854:Microscopy, Electron; D009069:Movement Disorders / Q000235:genetics* / Q000503:physiopathology; D010641:Phenotype; D012027:Reflex, Vestibulo-Ocular / Q000502:physiology; D012665:Semicircular Canals / Q000002:abnormalities* / Q000503:physiopathology / Q000648:ultrastructure; D015837:Vestibular Diseases / Q000235:genetics* / Q000503:physiopathology; D014725:Vestibular Nerve / Q000503:physiopathology*