Down syndrome: An electrophysiological and radiological profile

Saliba, Issam; Sbeity, Samer; El-Zir, Elie; Yammine, Fady G.; Noun, Carla T; Haddad, Antoine

doi:10.1002/lary.24375

Cited by 20 publications

(23 citation statements)

References 27 publications

(111 reference statements)

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“…There is a growing recognition about the role of subtle inner ear malformation and temporal bone dysplasia in people with DS discernible by radiologic imaging (Intrapiromkul, Aygun, Tunkel, Carone, & Yousem, ; Saliba et al, ). The role of risk factors such as childhood history of otitis media or cholesteatoma are also not well investigated (Bacciu et al, ).…”

Section: Resultsmentioning

confidence: 99%

Co‐occurring medical conditions in adults with Down syndrome: A systematic review toward the development of health care guidelines

Capone

Chicoine

Bulova

et al. 2017

American J of Med Genetics Pt A

132

136

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Adults with Down syndrome (DS) represent a unique population who are in need of clinical guidelines to address their medical care. The United States Preventive Service Task Force (USPSTF) has developed criteria for prioritizing conditions of public health importance with the potential for providing screening recommendations to improve clinical care. The quality of existing evidence needed to inform clinical guidelines has not been previously reviewed. Using the National Library of Medicine (NLM) database PubMed, we first identified 18 peer reviewed articles that addressed co-occurring medical conditions in adults with DS. Those conditions discussed in over half of the articles were prioritized for further review. Second, we performed detailed literature searches on these specific conditions. To inform the search strategy and review process a series of key questions were formulated a priori. The quality of available evidence was then graded and knowledge gaps were identified. The number of participating adults and the design of clinical studies varied by condition and were often inadequate for answering all of our key questions. We provide data on thyroid disease, cervical spine disease, hearing impairment, overweight-obesity, sleep apnea, congenital heart disease, and osteopenia-osteoporosis. Minimal evidence demonstrates massive gaps in our clinical knowledge that compromises clinical decision-making and management of these medically complex individuals. The development of evidence-based clinical guidance will require an expanded clinical knowledge-base in order to move forward.

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Section: Resultsmentioning

confidence: 99%

Co‐occurring medical conditions in adults with Down syndrome: A systematic review toward the development of health care guidelines

Capone

Chicoine

Bulova

et al. 2017

American J of Med Genetics Pt A

132

136

View full text Add to dashboard Cite

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“…Auditory impairment in individuals with DS has been widely reported in the specific literature (4)(5)(6) . Hearing loss -conductive, sensorineural, or mixed -occurs in approximately two thirds of children with DS (7,8) , with highest incidence for conductive hearing losses (around 80%) owing to the presence of otitis caused by constant respiratory tract infections (4,9,10) . In addition, studies have shown that the cochlea of individuals with DS is anatomically smaller, but apparently the abnormalities of the inner ear are not frequent (11) .…”

Section: Introductionmentioning

confidence: 99%

Caracterização eletrofisiológica da audição em indivíduos com Síndrome de Down

Kazan

Samelli

Neves-Lobo

et al. 2016

CoDAS

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RESUMO Introdução Poucos estudos realizaram, concomitantemente, o potencial evocado auditivo de tronco encefálico (PEATE) e o P300 na Síndrome de Down (SD), em indivíduos audiologicamente normais, para a avaliação da via auditiva central, principalmente pela dificuldade de realizar estes procedimentos nesta população. Estudos anteriores sugeriram que indivíduos com SD podem apresentar padrões de respostas diferentes das encontradas em indivíduos com desenvolvimento típico, sendo que a identificação destes seria fundamental para o estabelecimento de um diagnóstico audiológico preciso. Objetivo Caracterizar o PEATE e o P300 em indivíduos com SD audiologicamente normais. Método Foram analisados o PEATE e o P300 de 17 indivíduos com SD e 21 com desenvolvimento típico de sete a 15 anos. A análise foi quantitativa e qualitativa, utilizando medidas descritivas e os testes de hipótese. Resultados Os valores de latência foram menores no PEATE para o grupo SD, com diferença estatisticamente significante para a onda V, interpicos III-V e I-V; não foram encontradas diferenças significantes nos valores de latência do P300. Observou-se maior número de indivíduos com valores precoces para as latências do PEATE e com latências atrasadas para o P300 no grupo SD; ambas as comparações mostraram diferenças significantes. Conclusão Crianças e adolescentes com SD podem apresentar respostas precoces para os componentes do PEATE, sugerindo que a via auditiva destes necessita de menor tempo para a transmissão neural do estímulo acústico até o tronco encefálico. Quanto ao P300, indivíduos com SD podem apresentar latências aumentadas, sugerindo comprometimento na via auditiva central quanto ao processamento cortical da informação auditiva.

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“…As for sensorineural hearing loss (SNHL), which is thought to be associated with inner ear structural abnormalities in this population, holds a comparatively smaller proportion in DS patients suffering from hearing impairment, and the incidence of SNHL in this population differs between studies [4]. For instance, Saliba et al [5] reported the rate of SNHL in this population is around 41% while Lau et al [6] reported the percentage is about 28%. However, Austeng et al [7] and Park et al [8] unveiled much lower incidences of SNHL in DS patients-18% and 6% respectively.…”

Section: Discussionmentioning

confidence: 94%

Two Down Syndrome Patients with Bilateral Profound Hearing Loss: Case Report and Literature Review

Zheng

Yang

Zhao

et al. 2018

JOHBM

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Hearing loss is not uncommon among patients with Down syndrome (DS). It has been reported in 38–78% of the Down syndrome population. However, profound hearing loss in DS patients is rarely noticed due to its low incidence. In this article, we reported two Down syndrome patients with bilateral profound hearing loss in two cases. The first case involved an eight-year-old DS child experiencing extremely severe defects in terms of language and severe defects in terms of gross motor function, adaptability, and sociability. The second case revolved around another DS child with bilateral cochlear nerve absence. We review literature on the DS patients with hearing loss and conclude that profound sensorineural hearing loss in those patients has not received enough attention so far. We also recommend that cochlear implantation (CI) suitability assessment and timely intervention via cochlear implantation are necessary in DS patients. Besides, benefits from CI would be limited and hearing rehabilitation process could be much slower when compared with children without additional inabilities.

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Down syndrome: An electrophysiological and radiological profile

Abstract: 3b.

Cited by 20 publications

References 27 publications

Co‐occurring medical conditions in adults with Down syndrome: A systematic review toward the development of health care guidelines

Co‐occurring medical conditions in adults with Down syndrome: A systematic review toward the development of health care guidelines

Caracterização eletrofisiológica da audição em indivíduos com Síndrome de Down

Two Down Syndrome Patients with Bilateral Profound Hearing Loss: Case Report and Literature Review

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