Circling behavior in the Ecl mouse is caused by lateral semicircular canal defects.
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.
Cryns
Kim
K
Department of Medical Genetics, University of Antwerp, 2610 Antwerp, Belgium.
van Alphen
Arjan M
AM
van Spaendonck
Michiel P
MP
van de Heyning
Paul H
PH
Timmermans
Jean-Pierre
JP
de Zeeuw
Chris I
CI
van Camp
Guy
G
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*