Identification of Hearing Loss in Pediatric Patients with Down Syndrome

Park, Albert H.; Wilson, Matt; Stevens, Paul T.; Harward, R.; Hohler, Nancy

doi:10.1177/0194599811425156

Cited by 79 publications

(68 citation statements)

References 15 publications

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“…A longitudinal study of 79 children with DS reported that OME was diagnosed in 93 % at age one, falling to 68 % by age 5 (Barr et al 2011). Similar findings have been reported in other series (Brooks et al 1972; Balkany et al 1979; Maurizi et al 1985; Dahle and McCollister 1986; Hassmann et al 1998; Kattan et al 2000; Mitchell et al 2003; Park et al 2011). COME may persist, and at least 10–20 % of adults with DS have a conductive hearing loss (Evenhuis et al 1992; Van Buggenhout et al 1999).…”

Section: Introductionsupporting

confidence: 87%

Surveying the Down syndrome mouse model resource identifies critical regions responsible for chronic otitis media

et al. 2013

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Chronic otitis media (OM) is common in Down syndrome (DS), but underlying aetiology is unclear. We analysed the entire available mouse resource of partial trisomy models of DS looking for histological evidence of chronic middle-ear inflammation. We found a highly penetrant OM in the Dp(16)1Yey mouse, which carries a complete trisomy of MMU16. No OM was found in the Dp(17)1Yey mouse or the Dp(10)1Yey mouse, suggesting disease loci are located only on MMU16. The Ts1Cje, Ts1RhR, Ts2Yah, and Ts65Dn trisomies and the transchomosomic Tc1 mouse did not develop OM. On the basis of these findings, we propose a two-locus model for chronic middle-ear inflammation in DS, based upon epistasis of the regions of HSA21 not in trisomy in the Tc1 mouse. We also conclude that environmental factors likely play an important role in disease onset.

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Section: Introductionsupporting

confidence: 87%

Surveying the Down syndrome mouse model resource identifies critical regions responsible for chronic otitis media

et al. 2013

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“…Abnormal newborn hearing screen results in 22.8% is consistent with Utah DS neonate data [2]. The high rate of audiologic and hearing anomalies in our patients reinforces the DS Guideline recommendations for testing every 6 months until three years of age and then annually [29]. Our highest noncompliant rate of all guideline recommendations is audiology at 74%, which is disconcerting.…”

Section: Comorbiditiessupporting

confidence: 76%

Pediatric Comorbidities and Medical Complications Identified in Children with Down Syndrome

Hickey¹,

Wolter‐Warmerdam²,

Hickey³

et al. 2017

J Down Syndr Chr Abnorm

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Objective: Children with Down syndrome (DS) have an increased risk of neonatal complications and comorbidities compared to the general population; however, the incidence, optimal screening intervals and outcomes in this population are uncertain. The purpose of this study is to phenotypically define this population of children with DS using a large multi-age and ethnic sample and current American Academy of Pediatrics (AAP) Guidelines testing practices.Method: This is a retrospective review of a large cohort of 1,108 children with DS (male=602; mean age at initial contact: 5.72 years, SD ± 5.51) who received care at the Sie Center for Down Syndrome (SCDS) at The Children's Hospital Colorado from 2011 and 2016. Clinical data were collected from a prospective patient clinic database. Clinical details were collected, which included demographics, prenatal and birth history and complications, comorbidities, procedures, treatment plans and outcomes.Results: Medical complications requiring admission to the neonatal intensive care unit were identified in 70.6% of children with DS. Frequent causes for these neonatal admissions included: Required oxygen (60.7%), feeding problem (48.2%), Respiratory Distress Syndrome (21.4%) and pulmonary hypertension (14.7%). Incidence of medical comorbidities in our population with DS included cardiac defects (64.3%), abnormal sleep study (71.1%), abnormal thyroid study (29.1%), pulmonary aspiration (12.2%), celiac disease (5.0%) and pulmonary arterial hypertension (28.3%). Conclusion:Clinical data provides results from one of the largest investigations at a single pediatric hospital for children and young adults with DS in the US. This study describes the comorbidities affecting individuals with DS more accurately by applying AAP guidelines in studying a larger population than previously defined. This result improves our understanding of the incidence and identification of medical conditions in children with DS and reinforces recommendations on medical care screening for individuals with DS.

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“…Reports on the prevalence of hearing loss in DS vary considerably, but some degree of hearing loss has been noted in audiometric studies of children (Balkany, Downs, Jafek, & Krajicek, 1979; Park, Wilson, Stevens, Harward & Hohler, 2011; Roizen, Wolters, Nicol & Blondis, 1993; Shott, Joseph & Heithaus, 2001) and adults (Buchanan, 1990; Evenhuis, Van Zanten, Brocaar & Roerdinkholder, 1992). Survey studies show moderate prevalence of hearing impairment (Kumin, 2006; Schreve et al, 2009).…”

Section: Speech Sound Disorders (Table 2)mentioning

confidence: 99%

Speech Impairment in Down Syndrome: A Review

Kent

Vorperian

2013

J Speech Lang Hear Res

185

145

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Purpose This review summarizes research on disorders of speech production in Down Syndrome (DS) for the purposes of informing clinical services and guiding future research. Method Review of the literature was based on searches using Medline, Google Scholar, Psychinfo, and HighWire Press, as well as consideration of reference lists in retrieved documents (including online sources). Search terms emphasized functions related to voice, articulation, phonology, prosody, fluency and intelligibility. Conclusions The following conclusions pertain to four major areas of review: (a) Voice. Although a number of studies have been reported on vocal abnormalities in DS, major questions remain about the nature and frequency of the phonatory disorder. Results of perceptual and acoustic studies have been mixed, making it difficult to draw firm conclusions or even to identify sensitive measures for future study. (b) Speech sounds. Articulatory and phonological studies show that speech patterns in DS are a combination of delayed development and errors not seen in typical development. Delayed (i.e., developmental) and disordered (i.e., nondevelopmental) patterns are evident by the age of about 3 years, although DS-related abnormalities possibly appear earlier, even in infant babbling. (c) Fluency and prosody. Stuttering and/or cluttering occur in DS at rates of 10 to 45%, compared to about 1% in the general population. Research also points to significant disturbances in prosody. (d) Intelligibility. Studies consistently show marked limitations in this area but it is only recently that research goes beyond simple rating scales.

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Identification of Hearing Loss in Pediatric Patients with Down Syndrome

Abstract: Patients with DS present with a relatively high incidence of conductive hearing loss, MHL, and SNHL and a higher lost to follow-up rate compared to patients without DS. The authors were not able to diagnose SNHL within the 90-day period recommended by the Joint Committee on Infant Hearing.

Cited by 79 publications

References 15 publications

Surveying the Down syndrome mouse model resource identifies critical regions responsible for chronic otitis media

Surveying the Down syndrome mouse model resource identifies critical regions responsible for chronic otitis media

Pediatric Comorbidities and Medical Complications Identified in Children with Down Syndrome

Speech Impairment in Down Syndrome: A Review

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