25. Multiple Imputation for Nonresponse in Surveys. By D. B. Rubin. ISBN 0 471 08705 X. Wiley, Chichester, 1987. 258 pp. £30.25.

Multiple Imputation for Nonresponse in Surveys

Objectives This study examined the language outcomes of children with mild to severe hearing loss during the preschool years. The longitudinal design was leveraged to test whether language growth trajectories were associated with degree of hearing loss and whether aided hearing influenced language growth in a systematic manner. The study also explored the influence of the timing of hearing aid fitting and extent of use on children's language growth. Finally, the study tested the hypothesis that morphosyntax may be at particular risk due to the demands it places on the processing of fine details in the linguistic input. Design The full cohort of children in this study comprised 290 children who were hard of hearing (CHH) and 112 children with normal hearing who participated in the Outcomes of Children with Hearing Loss (OCHL) study between the ages of 2 and 6 years. CHH had a mean better-ear pure-tone average of 47.66 dB HL (SD = 13.35). All children received a comprehensive battery of language measures at annual intervals, including standardized tests, parent-report measures, and spontaneous and elicited language samples. Principal components analysis supported the use of a single composite language score for each of the age levels (2, 3, 4, 5, and 6 years). Measures of unaided (better-ear pure-tone average, speech intelligibility index) and aided (residualized speech intelligibility index) hearing were collected, along with parent-report measures of daily hearing aid use time. Mixed modeling procedures were applied to examine the rate of change (227 CHH; 94 children with normal hearing) in language ability over time in relation to (1) degree of hearing loss, (2) aided hearing, (3) age of hearing aid fit and duration of use, and (4) daily hearing aid use. Principal components analysis was also employed to examine factor loadings from spontaneous language samples and to test their correspondence with standardized measures. Multiple regression analysis was used to test for differential effects of hearing loss on morphosyntax and lexical development. Results Children with mild to severe hearing loss, on average, showed depressed language levels compared with peers with normal hearing who were matched on age and socioeconomic status. The degree to which CHH fell behind increased with greater severity of hearing loss. The amount of improved audibility with hearing aids was associated with differential rates of language growth; better audibility was associated with faster rates of language growth in the preschool years. Children fit early with hearing aids had better early language achievement than children fit later. However, children who were fit after 18 months of age improved in their language abilities as a function of the duration of hearing aid use. These results suggest that the language learning system remains open to experience provided by improved access to linguistic input. Performance in the domain of morphosyntax was found to be more delayed in CHH than their semantic abilities. Conclusion The data obtained in this study largely support the predictions, suggesting that mild to severe hearing loss places children at risk for delays in language development. Risks are moderated by the provision of early and consistent access to well-fit hearing aids that provide optimized audibility.

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Language Outcomes in Young Children with Mild to Severe Hearing Loss.

Sensorineural hearing impairment is the most common form of hearing loss, and encompasses pathologies of the cochlea and the auditory nerve. Hearing impairment caused by abnormal neural encoding of sound stimuli despite preservation of sensory transduction and amplification by outer hair cells is known as 'auditory neuropathy'. This term was originally coined for a specific type of hearing impairment affecting speech comprehension beyond changes in audibility: patients with this condition report that they "can hear but cannot understand". This type of hearing impairment can be caused by damage to the sensory inner hair cells (IHCs), IHC ribbon synapses or spiral ganglion neurons. Human genetic and physiological studies, as well as research on animal models, have recently shown that disrupted IHC ribbon synapse function--resulting from genetic alterations that affect presynaptic glutamate loading of synaptic vesicles, Ca(2+) influx, or synaptic vesicle exocytosis--leads to hearing impairment termed 'auditory synaptopathy'. Moreover, animal studies have demonstrated that sound overexposure causes excitotoxic loss of IHC ribbon synapses. This mechanism probably contributes to hearing disorders caused by noise exposure or age-related hearing loss. This Review provides an update on recently elucidated sensory, synaptic and neural mechanisms of hearing impairment, their corresponding clinical findings, and discusses current rehabilitation strategies as well as future therapies.

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Auditory neuropathy--neural and synaptic mechanisms.

IMPORTANCE Hearing loss (HL) in children can be deleterious to their speech and language development. The standard of practice has been early provision of hearing aids (HAs) to moderate these effects; however, there have been few empirical studies evaluating the effectiveness of this practice on speech and language development among children with mild-to-severe HL. OBJECTIVE To investigate the contributions of aided hearing and duration of HA use to speech and language outcomes in children with mild-to-severe HL. DESIGN, SETTING, AND PARTICIPANTS An observational cross-sectional design was used to examine the association of aided hearing levels and length of HA use with levels of speech and language outcomes. One hundred eighty 3- and 5-year-old children with HL were recruited through records of Universal Newborn Hearing Screening and referrals from clinical service providers in the general community in 6 US states. INTERVENTIONS All but 4 children had been fitted with HAs, and measures of aided hearing and the duration of HA use were obtained. MAIN OUTCOMES AND MEASURES Standardized measures of speech and language ability were obtained. RESULTS Measures of the gain in hearing ability for speech provided by the HA were significantly correlated with levels of speech (ρ179 = 0.20; P = .008) and language: ρ155 = 0.21; P = .01) ability. These correlations were indicative of modest levels of association between aided hearing and speech and language outcomes. These benefits were found for children with mild and moderate-to-severe HL. In addition, the amount of benefit from aided hearing interacted with the duration of HA experience (Speech: F4,161 = 4.98; P < .001; Language: F4,138 = 2.91; P < .02). Longer duration of HA experience was most beneficial for children who had the best aided hearing. CONCLUSIONS AND RELEVANCE The degree of improved hearing provided by HAs was associated with better speech and language development in children. In addition, the duration of HA experience interacted with the aided hearing to influence outcomes. These results provide support for the provision of well-fitted HAs to children with HL. In particular, the findings support early HA fitting and HA provision to children with mild HL.

https://jamanetwork.com/journals/jamaotolaryngology/articlepdf/1851413/ooi140016.pdf

The Influence of Hearing Aids on the Speech and Language Development of Children With Hearing Loss

OBJECTIVES: Universal newborn hearing screening has been implemented to detect permanent childhood hearing loss (PCHL) early, with the ultimate goal of improving outcomes through early treatment. However, there is disagreement between studies on the size of this benefit and in some cases whether it is significantly different from 0. There have been no studies of sufficient size in which researchers have determined reliably whether the effect varies with degree of PCHL. We aimed to explore how intervention timing influences 5-year language in children with PCHL. METHODS: Via a prospective study of 350 children, we used standard multiple regression analyses to investigate the effect of age at intervention or hearing screening on language outcomes after allowing for the effects of nonverbal IQ, degree of PCHL, sex, birth weight, maternal education, additional disabilities, and communication mode. RESULTS: The benefit of early intervention for language development increased as hearing loss increased. Children whose amplification started at age 24 months had poorer language than those whose amplification started at 3 months. The difference was larger for 70-dB HL (−11.8 score points; 95% confidence interval [95% CI]: −18.7 to −4.8) than for 50-dB HL (−6.8; 95% CI: −10.8 to −2.8). Children who received cochlear implants at 24 months had poorer language than those implanted at 6 months (−21.4; 95% CI: −33.8 to −9.0). There was no significant effect of screening on outcomes. CONCLUSIONS: Early intervention improves language outcomes, thereby lending support to streamlining clinical pathways to ensure early amplification and cochlear implantation after diagnosis.

https://pediatrics.aappublications.org/content/pediatrics/140/3/e20164274.full.pdf

Age at Intervention for Permanent Hearing Loss and 5-Year Language Outcomes

Objective:To address the question of whether, on a population level, early detection and amplification improve outcomes of children with hearing impairment.Design:All families of children who were born between 2002 and 2007, and who presented for hearing services below 3 years of age at Australian H

/pdf/outcomes-of-early-and-late-identified-children-at-3-years-of-1vmbv19tfj.pdf

Outcomes of early- and late-identified children at 3 years of age: findings from a prospective population-based study.

Hearing loss is an etiologically heterogeneous trait with differences in the age of onset, severity and site of lesion. It is caused by a combination of genetic and/or environmental factors. A longitudinal study to examine the efficacy of early intervention for improving child outcomes is ongoing in Australia. To determine the cause of hearing loss in these children we undertook molecular testing of perinatal “Guthrie” blood spots of children whose hearing loss was either detected via newborn hearing screening or detected later in infancy. We analyzed the GJB2 and SLC26A4 genes for the presence of mutations, screened for the mitochondrial DNA (mtDNA) A1555G mutation, and screened for congenital CMV infection in DNA isolated from dried newborn blood spots. Results were obtained from 364 children. We established etiology for 60% of children. One or two known GJB2 mutations were present in 82 children. Twenty-four children had one or two known SLC26A4 mutations. GJB2 or SLC26A4 changes with unknown consequences on hearing were found in 32 children. The A1555G mutation was found in one child, and CMV infection was detected in 28 children. Auditory neuropathy spectrum disorder was confirmed in 26 children whose DNA evaluations were negative. A secondary objective was to investigate the relationship between etiology and audiological outcomes over the first 3 years of life. Regression analysis was used to investigate the relationship between hearing levels and etiology. Data analysis does not support the existence of differential effects of etiology on degree of hearing loss or on progressiveness of hearing loss.

/pdf/etiology-and-audiological-outcomes-at-3-years-for-364-1id8nkjf42.pdf

Etiology and Audiological Outcomes at 3 Years for 364 Children in Australia

Objective: We investigated the factors influencing speech perception in babble for 5-year-old children with hearing loss who were using hearing aids (HAs) or cochlear implants (CIs). Design: Speech reception thresholds (SRTs) for 50% correct identification were measured in two conditions – speech collocated with babble, and speech with spatially separated babble. The difference in SRTs between the two conditions give a measure of binaural unmasking, commonly known as spatial release from masking (SRM). Multiple linear regression analyses were conducted to examine the influence of a range of demographic factors on outcomes. Study sample: Participants were 252 children enrolled in the Longitudinal Outcomes of Children with Hearing Impairment (LOCHI) study. Results: Children using HAs or CIs required a better signal-to-noise ratio to achieve the same level of performance as their normal-hearing peers but demonstrated SRM of a similar magnitude. For children using HAs, speech perception was significan...

/pdf/factors-influencing-speech-perception-in-noise-for-5-year-1zcwvuew2b.pdf

Factors influencing speech perception in noise for 5-year-old children using hearing aids or cochlear implants.

Objective: To determine the influence of the presence of auditory neuropathy spectrum disorder (ANSD) on speech, language, and psycho-social development of children at three years of age. Design: A population-based, longitudinal study was performed on outcomes of children with hearing impairment (LOCHI) in Australia. The demographic characteristics of the children were described, and their developmental outcomes were evaluated at three years of age. Performance of children with ANSD was compared with that of children without ANSD in the LOCHI study. Study sample: There were 47 children with ANSD in the study sample. Results: Sixty-four percent of children with ANSD have hearing sensitivity loss ranging from mild to severe degree, and the remaining have profound hearing loss. At three years, 27 children used hearing aids, 19 used cochlear implants, and one child did not use any hearing device. Thirty percent of children have disabilities in addition to hearing loss. On average, there were no signif...

Impact of the presence of auditory neuropathy spectrum disorder (ANSD) on outcomes of children at three years of age.

ObjectivesThis paper compares language development and speech perception of children with bimodal fitting (a cochlear implant in one ear and a hearing aid in the opposite ear) or bilateral cochlear...

/pdf/language-and-speech-perception-of-young-children-with-3ew3i0bdk8.pdf

Sanna Hou

Papers

Outcomes of early- and late-identified children at 3 years of age: findings from a prospective population-based study.

Etiology and Audiological Outcomes at 3 Years for 364 Children in Australia

Factors influencing speech perception in noise for 5-year-old children using hearing aids or cochlear implants.

Impact of the presence of auditory neuropathy spectrum disorder (ANSD) on outcomes of children at three years of age.

Language and speech perception of young children with bimodal fitting or bilateral cochlear implants