Hearing level in children with Down syndrome at the age of eight

Austeng, Marit Erna; Akre, Harriet; Falkenberg, Eva-Signe; Øverland, Britt; Abdelnoor, Michael; Kværner, Kari J.

doi:10.1016/j.ridd.2013.04.006

Cited by 35 publications

(33 citation statements)

References 19 publications

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“…Thus, having a PET placed at an older age may be due to an unresolved, long-standing middle ear disease in children with DS. This finding of hearing loss in some children with DS, despite having patent PETs, has been reported in other studies (Austeng et al, 2013; Paulson et al, 2014; Manickam et al, 2016). Some DS cases had apparent sensorineural components by bone conduction at 2–4 kHz, but these could also represent “Carhart notching” (altered bone conduction transmission due to a middle ear effect) that can occur in the presence of CHL (Stenfelt, 2015).…”

Section: Discussionsupporting

confidence: 89%

“…In the first year of life, the incidence of conductive hearing loss is 34%–38%, while the incidence of sensorineural hearing loss (SNHL) is 6% and mixed hearing loss (MHL) is 3% (Park et al, 2012; Raut et al, 2011). A population-based study in Norway (Austeng et al, 2013) reported that 35% of eight-year-old children with DS had hearing levels greater than 25 dB HL in the better ear, with a higher incidence of SNHL (18%), compared to CHL (16%) and MHL (6%). Paulson et al (2014) reported that 28% of children with DS had mild to profound hearing loss in the worst ear after the insertion of pressure equalizing tubes (PETs).…”

Section: Introductionmentioning

confidence: 99%

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Wideband acoustic immittance in children with Down syndrome: prediction of middle-ear dysfunction, conductive hearing loss and patent PE tubes

Hunter

Keefe

Feeney

et al. 2017

International Journal of Audiology

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Objective The purpose of this study was to evaluate pressurized wideband acoustic immittance (WAI) tests in children with Down syndrome (DS) and in typically developing children (TD) for prediction of conductive hearing loss (CHL) and patency of pressure equalizing tubes (PETs). Design Audiologic diagnosis was determined by audiometry in combination with distortion-product otoacoustic emissions, 226-Hz tympanometry and otoscopy. WAI results were compared for ears within diagnostic categories (Normal, CHL and PET) and between groups (TD and DS). Study Sample Children with DS (n=40; mean age 6.4 yrs.), and TD children (n=48; mean age 5.1 yrs.) were included. Results Wideband absorbance was significantly lower at 1–4 kHz in ears with CHL compared to NH for both TD and DS groups. In ears with patent PETs, wideband absorbance and group delay (GD) were larger than in ears without PETs between 0.25–1.5 kHz. Wideband absorbance tests performed similarly for prediction of CHL and patent PETs in TD and DS groups. Conclusions Wideband absorbance and group delay revealed specific patterns in both TD children and those with DS that can assist in detection of the presence of significant CHL, assess the patency of PETs, and provide frequency-specific information in the audiometric range.

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Section: Discussionsupporting

confidence: 89%

Section: Introductionmentioning

confidence: 99%

Wideband acoustic immittance in children with Down syndrome: prediction of middle-ear dysfunction, conductive hearing loss and patent PE tubes

Hunter

Keefe

Feeney

et al. 2017

International Journal of Audiology

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“…Its diagnosis is confirmed by cytogenetic investigation considering a series of signs and symptoms, with the following most commonly found characteristics: intellectual impairment (100%), muscular hypotonia (99%), oblique palpebral fissure (90%), increased vascularity (90%), microcephaly (85%), flat occiput (80%), and joint hypermobility (80%) (3) . 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) .…”

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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“…An increased risk of hearing loss in DS due to altered anatomy and related otolaryngology diseases and disorders is known [19]. However, previous prevalence studies performed in children with DS used varying definitions of hearing loss and reported impairment ranging from 34% to 81% [19][20][21]. The children with DS examined in this study were underdiagnosed.…”

Section: Discussionmentioning

confidence: 88%

Cytogenetic Profiles of 1213 Children with Down Syndrome in South Region of Turkey

Demırhan

TanrÄ±verdi²,

Süleymanova³

et al. 2014

J Mol Genet Med

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Down syndrome (DS) is a complex disorder characterized by well-defined and distinctive phenotypic features. Different karyotypes are associated with varying phenotypic expression of DS. The type of karyotypes in the children with DS plays an important role in genetic diagnosis and family counselling. This study describes the characteristics of karyotypes leading to phenotypic Down syndrome in 1213 cases diagnosed between 1992 and 2009 in South Region of Turkey. The frequency of occurrence of the different karyotypes was analyzed. The karyotype results were normal in 9.1% of all cases. However, chromosomal abnormalities (CAs) were detected in nearly 91% of all cases. The free trisomy 21 was the most common karyotype (nearly 93% of all cases). The ration of mosaic trisomy 21 was 2.5%. The CAs in addition to trisomy 21 was present in 1.5% of cases. The ratio of Robertsonian and reciprocal translocations in these variants were 2.3% and 0.3%, respectively. The ration of other variants was nearly 1%. This study showing the frequency and distribution of karyotypes causing DS, are the great value to be gleaned from studies of DS patients in furthering our understanding of the atypical clinical features associated with DS. These cytogenetic investigations carried out greatly helped in the management of these children and for counseling the affected families.

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Hearing level in children with Down syndrome at the age of eight

Cited by 35 publications

References 19 publications

Wideband acoustic immittance in children with Down syndrome: prediction of middle-ear dysfunction, conductive hearing loss and patent PE tubes

Wideband acoustic immittance in children with Down syndrome: prediction of middle-ear dysfunction, conductive hearing loss and patent PE tubes

Caracterização eletrofisiológica da audição em indivíduos com Síndrome de Down

Cytogenetic Profiles of 1213 Children with Down Syndrome in South Region of Turkey

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