Vestibular Function in Children With the CHARGE Association

Wiener-Vacher, Sylvette; Amanou, Laurent; Denise, Pierre; Narcy, P; Manac'h, Y

doi:10.1001/archotol.125.3.342

Cited by 62 publications

(48 citation statements)

References 23 publications

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“…13,16 In addition, congenital and earlyonset complete vestibular loss are known to induce severe delays in the acquisition of the first posturomotor milestones, such as the ability to hold the head stably and to sit and walk independently. 23,24 The main objective of this study was to determine the prevalence, severity, and changes over time of vestibular deficits in pediatric CMV cases and their correlation with other sensorineural disorders induced by CMV infection.…”

Section: What This Study Addsmentioning

confidence: 99%

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Vestibular Disorders in Children With Congenital Cytomegalovirus Infection

Bernard¹,

Wiener-Vacher

Abbeele

et al. 2015

Pediatrics

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BACKGROUND: Congenital cytomegalovirus (CMV) infection is the leading infectious cause of neurologic disabilities and sensorineural hearing loss in children. Sensorineural hearing loss prevalence in CMV suggests a viral tropism for the inner ear. Vestibular disorders induced by CMV infection are underestimated. This is the largest and most thorough study to assess the incidence of vestibular disorders and their correlation with hearing thresholds in children with CMV.

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Section: What This Study Addsmentioning

confidence: 99%

“…Data were compared with an age-matched non-CMVinfected pediatric population (n = 58) (data previously published [23][24][25][26][27][28] and determined from our database).…”

Section: Clinical Evaluation Of Postural Development and Behaviormentioning

confidence: 99%

Vestibular Disorders in Children With Congenital Cytomegalovirus Infection

Bernard¹,

Wiener-Vacher

Abbeele

et al. 2015

Pediatrics

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“…Vestibulo-ocular responses to earth-vertical axis rotation are abolished, but otolith vestibulo-ocular responses to the offvertical axis rotation test are preserved. 34 Deafness affects 60-90% of cases. The commonest hearing defects are severe conductive or mixed loss, and usually affects more the high frequencies than the low ones.…”

Section: Charge Syndrome D Sanlaville and A Verloesmentioning

confidence: 99%

CHARGE syndrome: an update

2007

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In association with CHARGE syndrome: an update CHARGE syndrome is a rare, usually sporadic autosomal dominant disorder due in 2/3 of cases to mutations within the CHD7 gene. The clinical definition has evolved with time. The 3C triad (ColobomaChoanal atresia-abnormal semicircular Canals), arhinencephaly and rhombencephalic dysfunctions are now considered the most important and constant clues to the diagnosis. We will discuss here recent aspects of the phenotypic delineation of CHARGE syndrome and highlight the role of CHD7 in its pathogeny. We review available data on its molecular pathology as well as cytogenetic and molecular evidences for genetic heterogeneity within CHARGE syndrome.

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“…Gross motor delay in children with CHARGE can be attributed in large part to vestibular dysfunction since the Mondini defect and the associated vestibular anomalies are a frequent finding [Tellier et al, 1998;Wiener-Vacher et al, 1999;Satar et al, 2003]. However, the delay can also be affected by lack of upper visual fields secondary to ocular…”

Section: Emerging Featuresmentioning

confidence: 99%

Behavior in CHARGE syndrome: Introduction to the special topic

Hartshorne

Hefner

Davenport

2005

American J of Med Genetics Pt A

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Challenging behavior in children with CHARGE syndrome has been increasingly a concern of parents, educators, and health professionals. This article introduces the special topic in the American Journal of Medical Genetics on behavior in individuals with CHARGE syndrome. It provides background on CHARGE syndrome, diagnostic criteria, and the relationship of sensory and other physical deficits with both development and behavior. Four themes related to our developing understanding of behavior in CHARGE are described: children with CHARGE have behaviors different from those seen in other syndromes with or without deafblindness. The behavior they display is often very adaptive to their environment and to their own disabilities. These behaviors may be partially related to problems with arousal and self‐regulation. And, finally, all papers point to behavior as communication, especially within relationships, where it is essential for maximizing intellectual and social outcomes. © 2005 Wiley‐Liss, Inc.

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Vestibular Function in Children With the CHARGE Association

Cited by 62 publications

References 23 publications

Vestibular Disorders in Children With Congenital Cytomegalovirus Infection

Vestibular Disorders in Children With Congenital Cytomegalovirus Infection

CHARGE syndrome: an update

Behavior in CHARGE syndrome: Introduction to the special topic

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