Profound Deafness in Childhood

Kral, Andrej; O’Donoghue, Gerard M.

doi:10.1056/nejmra0911225

Cited by 355 publications

(272 citation statements)

References 85 publications

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“…Although comprehensive population studies for Africa do not exist, available reports suggest that the prevalence of profound hearing loss is higher than the estimated 20-30% of children with permanent childhood hearing loss in the developed world [3,9,10,11].…”

Section: Introductionmentioning

confidence: 99%

“…These include the lack of development of spoken language which results in restricted learning, literacy and educational achievements, as well as later employment opportunities [10,16]. Profound hearing loss also results in a considerable cost for both the child and society [16] with the costs expected to be even higher in developing countries [11].…”

Section: Introductionmentioning

confidence: 99%

“…Profound hearing loss also results in a considerable cost for both the child and society [16] with the costs expected to be even higher in developing countries [11]. Early auditory stimulation during periods of maximal receptiveness is therefore critical for this population, since congenital/early onset profound hearing loss alters the functional properties of the auditory system and impairs cortical development [10,[16][17][18].…”

Section: Introductionmentioning

confidence: 99%

“…Profound childhood hearing loss is more than just a sensory loss, since central nervous system consequences of congenital deafness are aggravated with an increase in degree of hearing loss [10]. Also, approximately 30% of children with a profound hearing loss are reported to have an additional disability, with cognitive impairment and neurodevelopmental disabilities being the most common [36,37].…”

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Profound childhood hearing loss in a South Africa cohort: Risk profile, diagnosis and age of intervention

Roux

Swanepoel

Louw

et al. 2015

International Journal of Pediatric Otorhinolaryngology

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Profound childhood hearing loss in a South Africa cohort: Risk profile, diagnosis and age of intervention

Roux

Swanepoel

Louw

et al. 2015

International Journal of Pediatric Otorhinolaryngology

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“…Postoperative results in terms of speech perception tend to be much poorer in prelingually deaf adults when they received their cochlear implant after a long duration of deafness (Teoh et al 2004;Klop et al 2007). These poor performances might be explained by a hampered development of the auditory pathway and cross-modal changes during the period of auditory deprivation (Doucet et al 2006;Lee et al 2007;Kral and O'Donoghue 2010;Kral and Sharma 2012).…”

Section: Introductionmentioning

confidence: 99%

Altered Cortical Activity in Prelingually Deafened Cochlear Implant Users Following Long Periods of Auditory Deprivation

Lammers

Versnel

Zanten

et al. 2014

JARO

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Auditory stimulation during childhood is critical for the development of the auditory cortex in humans and with that for hearing in adulthood. Age-related changes in morphology and peak latencies of the cortical auditory evoked potential (CAEP) have led to the use of this cortical response as a biomarker of auditory cortical maturation including studies of cortical development after deafness and subsequent cochlear implantation. To date, it is unknown whether prelingually deaf adults, with early onset deafness (before the age of 2 years) and who received a cochlear implant (CI) only during adulthood, would display absent or aberrant CAEP waveforms as predicted from CAEP studies in late implanted prelingually deaf children. In the current study, CAEP waveforms were recorded in response to electric stimuli in prelingually deaf adults, who received their CI after the age of 21 years. Waveform morphology and peak latencies were compared to the CAEP responses obtained in postlingually deaf adults, who became deaf after the age of 16. Unexpectedly, typical CAEP waveforms with adult-like P1-N1-P2 morphology could be recorded in the prelingually deaf adult CI users. On visual inspection, waveform morphology was comparable to the CAEP waveforms recorded in the postlingually deaf CI users. Interestingly, however, latencies of the N1 peak were significantly shorter and amplitudes were significantly larger in the prelingual group than in the postlingual group. The presence of the CAEP together with an early and large N1 peak might represent activation of the more innate and less complex components of the auditory cortex of the prelingually deaf CI user, whereas the CAEP in postlingually deaf CI users might reflect activation of the mature neural network still present in these patients. The CAEPs may therefore be helpful in the assessment of developmental state of the auditory cortex.

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Neurocognitive Risk in Children With Cochlear Implants

Kronenberger

Beer

Castellanos

et al. 2014

JAMA Otolaryngol Head Neck Surg

111

117

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IMPORTANCE Children who receive a cochlear implant (CI) for early severe to profound sensorineural hearing loss may achieve age-appropriate spoken language skills not possible before implantation. Despite these advances, reduced access to auditory experience may have downstream effects on fundamental neurocognitive processes for some children with CIs. OBJECTIVE To determine the relative risk (RR) of clinically significant executive functioning deficits in children with CIs compared with children with normal hearing (NH). DESIGN, SETTING, AND PARTICIPANTS In this prospective, cross-sectional study, 73 children at a hospital-based clinic who received their CIs before 7 years of age and 78 children with NH, with average to above average mean nonverbal IQ scores, were recruited in 2 age groups: preschool age (age range, 3–5 years) and school age (age range, 7–17 years). No children presented with other developmental, cognitive, or neurologic diagnoses. INTERVENTIONS Parent-reported checklist measures of executive functioning were completed during psychological testing sessions. MAIN OUTCOMES AND MEASURES Estimates of the RR of clinically significant deficits in executive functioning (≥ 1 SDs above the mean) for children with CIs compared with children with NH were obtained based on 2 parent-reported child behavior checklists of everyday problems with executive functioning. RESULTS In most domains of executive functioning, children with CIs were at 2 to 5 times greater risk of clinically significant deficits compared with children with NH. The RRs for preschoolers and school-aged children, respectively, were greatest in the areas of comprehension and conceptual learning (RR [95% CI], 3.56 [1.71–7.43] and 6.25 [2.64–14.77]), factual memory ( 4.88 [1.58–15.07] and 5.47 [2.03–14.77]), attention (3.38 [1.03–11.04] and 3.13 [1.56–6.26]), sequential processing (11.25 [1.55–81.54] and 2.44 [1.24–4.76]), working memory (4.13 [1.30–13.06] and 3.64 [1.61–8.25] for one checklist and 1.77 [0.82–3.83] and 2.78 [1.18–6.51] for another checklist), and novel problem-solving (3.93 [1.50–10.34] and 3.13 [1.46–6.67]). No difference between the CI and NH samples was found for visual-spatial organization (2.63 [0.76–9.03] and 1.04 [0.45–2.40] on one checklist and 2.86 [0.98–8.39] for school-aged children on the other checklist). CONCLUSIONS AND RELEVANCE A large proportion of children with CIs are at risk for clinically significant deficits across multiple domains of executive functioning, a rate averaging 2 to 5 times that of children with NH for most domains. Screening for risk of executive functioning deficits should be a routine part of the clinical evaluation of all children with deafness and CIs.

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Profound Deafness in Childhood

Cited by 355 publications

References 85 publications

Profound childhood hearing loss in a South Africa cohort: Risk profile, diagnosis and age of intervention

Profound childhood hearing loss in a South Africa cohort: Risk profile, diagnosis and age of intervention

Altered Cortical Activity in Prelingually Deafened Cochlear Implant Users Following Long Periods of Auditory Deprivation

Neurocognitive Risk in Children With Cochlear Implants

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