Circling behavior in the <i>Ecl</i> mouse is caused by lateral semicircular canal defects

Cryns, Kim; Alphen, Arjan M. van; Spaendonck, Michiel P. Van; Heyning, Paul Van de; Timmermans, Jean‐Pierre; Zeeuw, Chris I. De; Camp, Guy Van

doi:10.1002/cne.10975

Cited by 29 publications

(23 citation statements)

References 37 publications

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“…Correlations between circling mice and an inner ear phenotype have been examined in other mouse mutants. All circling Ecl mice display bilateral lateral semicircular canal malformations, which vary in severity among animals and between ears of the same animal (Cryns et al, 2004). This is consistent with what we have observed with Chd7…”

Section: In Chd7supporting

confidence: 91%

Defects in vestibular sensory epithelia and innervation in mice with loss of Chd7 function: Implications for human CHARGE syndrome

Adams

Hurd

Beyer

et al. 2007

J of Comparative Neurology

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CHD7 is a chromodomain gene mutated in CHARGE syndrome, a multiple anomaly condition characterized by ocular coloboma, heart defects, atresia of the choanae, retarded growth and development, genital hypoplasia, and ear defects including deafness and semicircular canal dysgenesis. Mice with heterozygous Chd7 deficiency have circling behavior and semicircular canal defects and are an excellent animal model for exploring the pathogenesis of CHARGE features. Inner ear vestibular defects have been characterized in heterozygous Chd7-deficient embryos and early postnatal mice, but it is not known whether vestibular defects persist throughout adulthood in Chd7-deficient mice or whether the vestibular sensory epithelia and their associated innervation and function are intact. Here we describe a detailed analysis of inner ear vestibular structures in mature mice that are heterozygous for a Chd7-deficient, gene-trapped allele (Chd7(Gt/+)). Chd7(Gt/+) mice display variable asymmetric lateral and posterior semicircular canal malformations, as well as defects in vestibular sensory epithelial innervation despite the presence of intact hair cells in the target organs. These observations have important functional implications for understanding the clinical manifestations of CHD7 mutations in humans and for designing therapies to treat inner ear vestibular dysfunction.

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Section: In Chd7supporting

confidence: 91%

Defects in vestibular sensory epithelia and innervation in mice with loss of Chd7 function: Implications for human CHARGE syndrome

Adams

Hurd

Beyer

et al. 2007

J of Comparative Neurology

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“…1E, I). Such circular pattern displayed by the Oc90 nulls is different than the hyperactive, incessant circling behavior observed in mice with labyrinthine dysfunction affecting the semicircular canals (Wenngren and Anniko, 1989;Holme et al, 2002;Cryns et al, 2004) and/or central deficits (Shima and Hassler, 1982). In fact, the homozygous mutants in our study were usually less active.…”

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confidence: 86%

Otoconin-90 deletion leads to imbalance but normal hearing: A comparison with other otoconia mutants

et al. 2008

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Our sense of gravitation and linear acceleration is mediated by stimulation of vestibular hair cells through displacement of otoconia in the utricle and saccule (the gravity receptor organ). We recently showed that otoconin-90 (Oc90) deletion led to formation of giant otoconia. In the present study, we determined the extent to which the giant otoconia affected balance and gravity receptor sensory input and compared the findings with other otoconia mutants. We employed a wide spectrum of balance behavioral tests, including reaching and air-righting reflexes, gait, swimming, beam-crossing, rotorod latencies, and a direct measure of gravity receptor input, vestibular evoked potentials (VsEPs). All tests on homozygous adult mutants consistently ranked the order of imbalance as (from worst to best) Nox3 het

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“…Anatomic studies have revealed that some mouse mutants, such as Otx1 (Acampora et al 1996), Ecl (Cryns et al 2004), Whl (Alavizadeh et al 2001), Obt, Todo, Dz, Cyn, Edy, Mt, and Lda (Kiernan et al 2002), and Crsl and Whi (Hawker et al 2005), show gross changes in the bony semicircular canals. In contrast, others, such as stargazer (Khan et al 2004), het (Paffenholz et al 2004), isk (Vetter et al 1996), tlt and mlh (Hurle et al 2003), Pmca2 (Kozel et al 1998), shaker/waltzer (Sun 2001), and hscy (Longo-Guess et al 2005), have not been reported as having significant bony abnormalities.…”

Section: Introductionmentioning

confidence: 99%

Planar Relationships of the Semicircular Canals in Two Strains of Mice

Calabrese

Hullar

2006

JARO

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The mouse is increasingly important as a subject of vestibular research. Although many studies have focused on the vestibular responses of mice to angular rotation, the geometry of their semicircular canals has not been described. High-voltage X-ray computed tomography was used to measure the anatomy of the semicircular canals of two strains of mice, C57Bl/6J and CBA/CaJ. The horizontal plane of a stereotaxic coordinate system was defined by the midpoints of the external auditory meati and the point where the incisors emerge from the maxilla. The centroids of the lumens of the bony canals were calculated, and planes that describe the canals were fit using a least-squares regression analysis to the resulting points. Vectors normal to each regressed plane were used to represent the corresponding canal's axis of rotation, and angles of these vectors relative to skull landmarks as well as to each other were calculated. The horizontal canal of the mouse was found to be angled anteriorly upward 17.8-for CBA/CaJ and 32.6-for C57Bl/6J from the reference horizontal plane. Angles between ipsilateral canals deviated up to 12.3-from orthogonal, and angles between contralateral synergistic canals (left anterior-right posterior, right anterior-left posterior, and horizontal-horizontal) deviated from parallel by up to 14.8-. The orientations of the canals within the head as well as the orientations of the canals relative to each other were significantly different between the two strains, suggesting that care must be taken in the design and interpretation of developmental and physiologic studies involving different mouse strains.

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Circling behavior in the Ecl mouse is caused by lateral semicircular canal defects

Cited by 29 publications

References 37 publications

Defects in vestibular sensory epithelia and innervation in mice with loss of Chd7 function: Implications for human CHARGE syndrome

Defects in vestibular sensory epithelia and innervation in mice with loss of Chd7 function: Implications for human CHARGE syndrome

Otoconin-90 deletion leads to imbalance but normal hearing: A comparison with other otoconia mutants

Planar Relationships of the Semicircular Canals in Two Strains of Mice

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