Characteristics and Progression of Hearing Loss in Children with Down Syndrome

Kreicher, Kathryn L.; Weir, Forest W.; Nguyen, Shaun A.; Meyer, Ted A.

doi:10.1016/j.jpeds.2017.09.053

Cited by 61 publications

(40 citation statements)

References 26 publications

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“…Children with DS often have impaired hearing caused by otitis media, an inflammation of the middle ear [33][34][35] . To examine if this phenotype was present in Dp1Tyb mice, we evaluated hearing using the auditory brainstem response.…”

Section: Dp1tyb Mice Have Impaired Hearingmentioning

confidence: 99%

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Comprehensive phenotypic analysis of the Dp1Tyb mouse strain reveals a broad range of Down Syndrome-related phenotypes

Lana-Elola

Cater

Watson-Scales

et al. 2021

Preprint

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Down syndrome (DS), trisomy 21, results in many complex phenotypes including cognitive deficits, heart defects and craniofacial alterations. Phenotypes arise from an extra copy of human chromosome 21 (Hsa21) genes. However, causative genes remain mostly unknown. Animal models enable identification of these genes and pathological mechanisms. The Dp1Tyb mouse model of DS has an extra copy of 63% of Hsa21-orthologous mouse genes. Here, we comprehensively phenotype Dp1Tyb mice and find wide-ranging DS-like phenotypes including aberrant megakaryopoiesis, reduced bone density, and deficits in memory, locomotion, hearing and sleep. Thus, Dp1Tyb mice are an excellent model for studies of many complex DS phenotypes.

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Section: Dp1tyb Mice Have Impaired Hearingmentioning

confidence: 99%

“…Sensory deficits are often seen in DS, including in hearing and vision 53 . Dp1Tyb mice have defective hearing, which is most likely due to otitis media, a common condition in DS [33][34][35] . We found no eye defects in the mice, but further studies would be needed to evaluate visual acuity.…”

mentioning

confidence: 99%

Comprehensive phenotypic analysis of the Dp1Tyb mouse strain reveals a broad range of Down Syndrome-related phenotypes

Lana-Elola

Cater

Watson-Scales

et al. 2021

Preprint

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“…In Williams syndrome, a propensity to show hyperacusis seems tied to fears of sirens, thunder, and other loud noises (Dykens, 2003; Gothelf, Farber, Raveh, Apter, & Attias, 2006). In Down syndrome, the propensity for individuals to have large and thick tongues may relate to their articulation problems (Bunton & Leddy, 2011), while hearing problems relate to difficulties in language functioning, during both childhood (Kreicher, Weir, Nguyen, & Meyer, 2018) and adulthood (Lin et al, 2015). As we document ties between physical–health concerns and development, we require the knowledge, methods, and perspectives of geneticists, biomedical workers, and behavioral researchers all working together.…”

Section: Future Directionsmentioning

confidence: 99%

Ed Zigler's developmental approach to intellectual disabilities: Past, present, and future contributions

Hodapp

2021

Dev Psychopathol

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Comprising two parts, Ed Zigler's developmental approach has greatly influenced how one conceptualizes children with intellectual disabilities (ID). In part one, Zigler championed a “two-group approach” concerning the cause of children's ID. He distinguished persons with a clear, organic cause of their ID from those displaying no clear cause. Members of this “organic” group often displayed IQs below 50 and co-occurring physical–medical conditions. The second, “cultural–familial” group, mostly showed IQs of 50–70, did not possess co-occurring physical or health problems, and often came from families of lower IQs and lower socioeconomic status. While the presence of these two groups has been supported, recent advances have also further differentiated the organic group, mostly in relation to behavioral phenotypes of persons with several genetic etiologies. In part two, Zigler championed the child with ID as a “whole person.” Originally focused on the child's reactions to social deprivation and failure, recent studies directly examine parent–child, within-family, and wider system interactions throughout the life span. For decades a force within the ID field, Zigler's developmental approach to children with ID continues to influence researchers, interventionists, and policymakers.

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“…Conductive hearing loss (CHL) is the most common type of hearing issue in Down syndrome, seen in 53-88 %of patients, frequently due to chronic otitis media and eustachian tube dysfunction with decreased cartilage density [65]. In addition, external and middle ear anomalies are described and include external ear canal stenosis and ossicular anomalies.…”

Section: Petrous Bone Imaging Findingsmentioning

confidence: 99%

Temporal bone and intracranial abnormalities in syndromic causes of hearing loss: an updated guide

D’Arco

Youssef

Ioannidou

et al. 2020

European Journal of Radiology

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To describe in detail the temporal bone and brain findings in both common and rare syndromic causes of hearing loss, with the purpose of broadening among radiologists and enhance the current understanding of distinct imaging features in paediatric patients with syndromic hearing loss. Methods: A detailed search of electronic databases has been conducted, including PubMed, Ovid Medline, Scopus, Cochrane Library, Google Scholar, National Institute for Health and Care Excellence (NICE), Embase, and PsycINFO. Results: Syndromic causes of hearing loss are characterised by different and sometimes specific abnormalities in the temporal bone. Conclusion: A complete knowledge of the image findings in the temporal bones, brain, skull and other body regions is critical for the optimal assessment and management of these patients. 2.1. Inner ear The inner ear development starts late in the 3rd week of gestation, with the formation of the otic placode (an ectodermal thickening on both sides of the developing head).The otic placode later invaginates to form otic pits which then fuse to form the otic vesicle. By day 26, the ventral region of otic vesicle differentiates into the saccule, the lower pole of which gives rise to the cochlear duct, which will form 2.5 turns by the 8th week. During the 7th week, cochlear duct epithelial cells differentiate to form the Organ of Corti. The mesenchyme surrounding the cochlear

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Characteristics and Progression of Hearing Loss in Children with Down Syndrome

Cited by 61 publications

References 26 publications

Comprehensive phenotypic analysis of the Dp1Tyb mouse strain reveals a broad range of Down Syndrome-related phenotypes

Comprehensive phenotypic analysis of the Dp1Tyb mouse strain reveals a broad range of Down Syndrome-related phenotypes

Ed Zigler's developmental approach to intellectual disabilities: Past, present, and future contributions

Temporal bone and intracranial abnormalities in syndromic causes of hearing loss: an updated guide

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