Long-Term Cochlear Implant Outcomes in Children with GJB2 and SLC26A4 Mutations

Objectives

To investigate speech and language outcomes in children with cochlear implants (CIs) who had mutations in common deafness genes and to compare their performances with those without mutations.

Study Design

Prospective study.

Methods

Patients who received CIs before 18 years of age and had used CIs for more than 3 years were enrolled in this study. All patients underwent mutation screening of three common deafness genes: GJB2, SLC26A4 and the mitochondrial 12S rRNA gene. The outcomes with CIs were assessed at post-implant years 3 and 5 using the Categories of Auditory Performance (CAP) scale, Speech Intelligibility Rating (SIR) scale, speech perception tests and language skill tests.

Results

Forty-eight patients were found to have confirmative mutations in GJB2 or SLC26A4, and 123 without detected mutations were ascertained for comparison. Among children who received CIs before 3.5 years of age, patients with GJB2 or SLC26A4 mutations showed significantly higher CAP/SIR scores than those without mutations at post-implant year 3 (p = 0.001 for CAP; p = 0.004 for SIR) and year 5 (p = 0.035 for CAP; p = 0.038 for SIR). By contrast, among children who received CIs after age 3.5, no significant differences were noted in post-implant outcomes between patients with and without mutations (all p > 0.05).

Conclusion

GJB2 and SLC26A4 mutations are associated with good post-implant outcomes. However, their effects on CI outcomes may be modulated by the age at implantation: the association between mutations and CI outcomes is observed in young recipients who received CIs before age 3.5 years but not in older recipients.     

Pediatric Health-Related Quality of Life: A Structural Equation Modeling Approach

Objectives

One of the most referenced theoretical frameworks to measure Health Related Quality of Life (HRQoL) is the Wilson and Cleary framework. With some adaptions this framework has been validated in the adult population, but has not been tested in pediatric populations. Our goal was to empirically investigate it in children.

Methods

The contributory factors to Health Related Quality of Life that we included were symptom status (presence of chronic disease or hospitalizations), functional status (developmental status), developmental aspects of the individual (social-emotional) behavior, and characteristics of the social environment (socioeconomic status and area of education). Structural equation modeling was used to assess the measurement structure of the model in 214 German children (3–5 years old) participating in a follow-up study that investigates pediatric health outcomes.

Results

Model fit was χ2 = 5.5; df = 6; p = 0.48; SRMR  = 0.01. The variance explained of Health Related Quality of Life was 15%. Health Related Quality of Life was affected by the area education (i.e. where kindergartens were located) and development status. Developmental status was affected by the area of education, socioeconomic status and individual behavior. Symptoms did not affect the model.

Conclusions

The goodness of fit and the overall variance explained were good. However, the results between children'' and adults'' tests differed and denote a conceptual gap between adult and children measures. Indeed, there is a lot of variety in pediatric Health Related Quality of Life measures, which represents a lack of a common definition of pediatric Health Related Quality of Life. We recommend that researchers invest time in the development of pediatric Health Related Quality of Life theory and theory based evaluations.     

Analysis of Causal Relationships by Structural Equation Modeling to Determine the Factors Influencing Cognitive Function in Elderly People in Japan

The purpose of this study is to identify a potentiality factor that is a preventive factor for decline in cognitive function. Additionally, this study pursues to clarify the causal relationship between the each potential factor and its influence on cognitive function. Subjects were 366 elderly community residents (mean age 73.7 ± 6.4, male 51, female 315) who participated in the Taketoyo Project from 2007 to 2011. Factor analysis was conducted to identify groupings within mental, social, life, physical and cognitive functions. In order to detect clusters of 14 variables, the item scores were subjected to confirmatory factor analysis. We performed Structural Equation Modeling analysis to calculate the standardization coefficient and correlation coefficient for every factor. The cause and effect hypothesis model was used to gather two intervention theory hypotheses for dementia prevention (direct effect, indirect effect) in one system. Finally, we performed another Structural Equation Modeling analysis to calculate the standardization of the cause and effect hypothesis model. Social participation was found to be activated by the improvement of four factors, and in turn, activated “Social participation” acted on cognitive function.     

Neurocognition,Insight and Medication Nonadherence in Schizophrenia: A Structural Equation Modeling Approach

Objective

The aim of this study was to examine the complex relationships among neurocognition, insight and nonadherence in patients with schizophrenia.

Methods

Design: Cross-sectional study. Inclusion criteria: Diagnosis of schizophrenia according to the DSM-IV-TR criteria. Data collection: Neurocognition was assessed using a global approach that addressed memory, attention, and executive functions; insight was analyzed using the multidimensional ‘Scale to assess Unawareness of Mental Disorder;’ and nonadherence was measured using the multidimensional ‘Medication Adherence Rating Scale.’ Analysis: Structural equation modeling (SEM) was applied to examine the non-straightforward relationships among the following latent variables: neurocognition, ‘awareness of positive symptoms’ and ‘negative symptoms’, ‘awareness of mental disorder’ and nonadherence.

Results

One hundred and sixty-nine patients were enrolled. The final testing model showed good fit, with normed χ² = 1.67, RMSEA = 0.063, CFI = 0.94, and SRMR = 0.092. The SEM revealed significant associations between (1) neurocognition and ‘awareness of symptoms,’ (2) ‘awareness of symptoms’ and ‘awareness of mental disorder’ and (3) ‘awareness of mental disorder’ and nonadherence, mainly in the ‘attitude toward taking medication’ dimension. In contrast, there were no significant links between neurocognition and nonadherence, neurocognition and ‘awareness of mental disorder,’ and ‘awareness of symptoms’ and nonadherence.

Conclusions

Our findings support the hypothesis that neurocognition influences ‘awareness of symptoms,’ which must be integrated into a higher level of insight (i.e., the ‘awareness of mental disorder’) to have an impact on nonadherence. These findings have important implications for the development of effective strategies to enhance medication adherence.     

Audiovisual Speech Perception at Various Presentation Levels in Mandarin-Speaking Adults with Cochlear Implants

Objectives

(1) To evaluate the recognition of words, phonemes and lexical tones in audiovisual (AV) and auditory-only (AO) modes in Mandarin-speaking adults with cochlear implants (CIs); (2) to understand the effect of presentation levels on AV speech perception; (3) to learn the effect of hearing experience on AV speech perception.

Methods

Thirteen deaf adults (age = 29.1±13.5 years; 8 male, 5 female) who had used CIs for >6 months and 10 normal-hearing (NH) adults participated in this study. Seven of them were prelingually deaf, and 6 postlingually deaf. The Mandarin Monosyllablic Word Recognition Test was used to assess recognition of words, phonemes and lexical tones in AV and AO conditions at 3 presentation levels: speech detection threshold (SDT), speech recognition threshold (SRT) and 10 dB SL (re:SRT).

Results

The prelingual group had better phoneme recognition in the AV mode than in the AO mode at SDT and SRT (both p = 0.016), and so did the NH group at SDT (p = 0.004). Mode difference was not noted in the postlingual group. None of the groups had significantly different tone recognition in the 2 modes. The prelingual and postlingual groups had significantly better phoneme and tone recognition than the NH one at SDT in the AO mode (p = 0.016 and p = 0.002 for phonemes; p = 0.001 and p<0.001 for tones) but were outperformed by the NH group at 10 dB SL (re:SRT) in both modes (both p<0.001 for phonemes; p<0.001 and p = 0.002 for tones). The recognition scores had a significant correlation with group with age and sex controlled (p<0.001).

Conclusions

Visual input may help prelingually deaf implantees to recognize phonemes but may not augment Mandarin tone recognition. The effect of presentation level seems minimal on CI users'' AV perception. This indicates special considerations in developing audiological assessment protocols and rehabilitation strategies for implantees who speak tonal languages.     

A Resonance Approach to Cochlear Mechanics

Background

How does the cochlea analyse sound into its component frequencies? In the 1850s Helmholtz thought it occurred by resonance, whereas a century later Békésy''s work indicated a travelling wave. The latter answer seemed to settle the question, but with the discovery in 1978 that the cochlea emits sound, the mechanics of the cochlea was back on the drawing board. Recent studies have raised questions about whether the travelling wave, as currently understood, is adequate to explain observations.

Approach

Applying basic resonance principles, this paper revisits the question. A graded bank of harmonic oscillators with cochlear-like frequencies and quality factors is simultaneously excited, and it is found that resonance gives rise to similar frequency responses, group delays, and travelling wave velocities as observed by experiment. The overall effect of the group delay gradient is to produce a decelerating wave of peak displacement moving from base to apex at characteristic travelling wave speeds. The extensive literature on chains of coupled oscillators is considered, and the occurrence of travelling waves, pseudowaves, phase plateaus, and forced resonance in such systems is noted.

Conclusion and significance

This alternative approach to cochlear mechanics shows that a travelling wave can simply arise as an apparently moving amplitude peak which passes along a bank of resonators without carrying energy. This highlights the possible role of the fast pressure wave and indicates how phase delays and group delays of a set of driven harmonic oscillators can generate an apparent travelling wave. It is possible to view the cochlea as a chain of globally forced coupled oscillators, and this model incorporates fundamental aspects of both the resonance and travelling wave theories.     

基于结构方程模型的用药安全影响因素研究

目的 通过分析用药安全的影响因素和发生机制,从而提高用药安全性,预防用药差错的发生。方法 随机抽取三级、二级医院各6家,分析2010—2016年12家医院的423例用药差错事件发生的原因,同时结合个人访谈的研究方式,访谈用药差错事件发生的相关医护人员及用药安全管理部门领导共计126人,最终通过访谈及文献研究的方法确定了26个用药安全的影响因素。通过运用因子分析结合结构方程模型的方式,分析各个影响因素之间的相互作用及对用药安全的影响程度。结果 通过结构方程模型分析可知：药品因素、员工素质、患者因素、社会因素、医院管理都是高度影响用药安全的主要因素。结论 通过对关键指标的管理,提出科学、有效的防范措施。     

High Risk Sexual Behaviors for HIV among the In-School Youth in Swaziland: A Structural Equation Modeling Approach

Background

Global efforts in response to the increased prevalence of the human immunodeficiency virus (HIV) are mainly aimed at reducing high risk sexual behaviors among young people. However, knowledge regarding intentions of young people to engage in protective sexual behaviors is still lacking in many countries around the world, especially in Sub-Saharan Africa where prevalence of human immunodeficiency virus is the highest. The objective of this study was to test the theory of planned behavior (TPB) for predicting factors associated with protective sexual behaviors, including sexual abstinence and condom use, among in-school youths aged between 15 and 19 years in Swaziland.

Methods

This cross-sectional survey was conducted using a anonymous questionnaire. A two-stage stratified and cluster random sampling method was used. Approximately one hundred pupils from each of four schools agreed to participate in the study, providing a total sample size of 403 pupils of which 369 were ultimately included for data analysis. The response rate was 98%. Structural equation modeling was used to analyse hypothesized paths.

Results

The TPB model used in this study was effective in predicting protective sexual behavior among Swazi in-school youths, as shown by model fit indices. All hypothesized constructs significantly predicted intentions for abstinence and condom use, except perceived abstinence controls. Subjective norms were the strongest predictors of intention for premarital sexual abstinence; however, perceived controls for condom use were the strongest predictors of intention for condom use.

Conclusions

Our findings support application of the model in predicting determinants of condom use and abstinence intentions among Swazi in-school youths.     

SEM: Structural Equation Modeling in Molecular Biology

Structural equation modeling (SEM) is a second-generation multivariate method to estimate the causal interactions in a set of variables and includes, as special cases, several statistical methods (regression analysis, path analysis, and confirmatory factor analysis). This review focuses on all of the main SEM models and various methods used to optimize the model parameters. Representative examples are discussed to illustrate SEM application in molecular biology, including modeling of biochemical processes, relationships between genetic markers and diseases, and interactions within gene networks.     

Neurological Soft Signs and Their Relationships to Neurocognitive Functions: A Re-Visit with the Structural Equation Modeling Design

Background

Neurological soft signs and neurocognitive impairments have long been considered important features of schizophrenia. Previous correlational studies have suggested that there is a significant relationship between neurological soft signs and neurocognitive functions. The purpose of the current study was to examine the underlying relationships between these two distinct constructs with structural equation modeling (SEM).

Methods

118 patients with schizophrenia and 160 healthy controls were recruited for the current study. The abridged version of the Cambridge Neurological Inventory (CNI) and a set of neurocognitive function tests were administered to all participants. SEM was then conducted independently in these two samples to examine the relationships between neurological soft signs and neurocognitive functions.

Results

Both the measurement and structural models showed that the models fit well to the data in both patients and healthy controls. The structural equations also showed that there were modest to moderate associations among neurological soft signs, executive attention, verbal memory, and visual memory, while the healthy controls showed more limited associations.

Conclusions

The current findings indicate that motor coordination, sensory integration, and disinhibition contribute to the latent construct of neurological soft signs, whereas the subset of neurocognitive function tests contribute to the latent constructs of executive attention, verbal memory, and visual memory in the present sample. Greater evidence of neurological soft signs is associated with more severe impairment of executive attention and memory functions. Clinical and theoretical implications of the model findings are discussed.     

Visual Cross-Modal Re-Organization in Children with Cochlear Implants

Background

Visual cross-modal re-organization is a neurophysiological process that occurs in deafness. The intact sensory modality of vision recruits cortical areas from the deprived sensory modality of audition. Such compensatory plasticity is documented in deaf adults and animals, and is related to deficits in speech perception performance in cochlear-implanted adults. However, it is unclear whether visual cross-modal re-organization takes place in cochlear-implanted children and whether it may be a source of variability contributing to speech and language outcomes. Thus, the aim of this study was to determine if visual cross-modal re-organization occurs in cochlear-implanted children, and whether it is related to deficits in speech perception performance.

Methods

Visual evoked potentials (VEPs) were recorded via high-density EEG in 41 normal hearing children and 14 cochlear-implanted children, aged 5–15 years, in response to apparent motion and form change. Comparisons of VEP amplitude and latency, as well as source localization results, were conducted between the groups in order to view evidence of visual cross-modal re-organization. Finally, speech perception in background noise performance was correlated to the visual response in the implanted children.

Results

Distinct VEP morphological patterns were observed in both the normal hearing and cochlear-implanted children. However, the cochlear-implanted children demonstrated larger VEP amplitudes and earlier latency, concurrent with activation of right temporal cortex including auditory regions, suggestive of visual cross-modal re-organization. The VEP N1 latency was negatively related to speech perception in background noise for children with cochlear implants.

Conclusion

Our results are among the first to describe cross modal re-organization of auditory cortex by the visual modality in deaf children fitted with cochlear implants. Our findings suggest that, as a group, children with cochlear implants show evidence of visual cross-modal recruitment, which may be a contributing source of variability in speech perception outcomes with their implant.     

Environmental Sounds Recognition in Children with Cochlear Implants

The aims of this study were (1) to document the recognition performance of environmental sounds (ESs) in Mandarin-speaking children with cochlear implants (CIs) and to analyze the possible associated factors with the ESs recognition; (2) to examine the relationship between perception of ESs and receptive vocabulary level; and (3) to explore the acoustic factors relevant to perceptual outcomes of daily ESs in pediatric CI users. Forty-seven prelingually deafened children between ages 4 to 10 years participated in this study. They were divided into pre-school (group A: age 4–6) and school-age (group B: age 7 to 10) groups. Sound Effects Recognition Test (SERT) and the Chinese version of the revised Peabody Picture Vocabulary Test (PPVT-R) were used to assess the auditory perception ability. The average correct percentage of SERT was 61.2% in the preschool group and 72.3% in the older group. There was no significant difference between the two groups. The ESs recognition performance of children with CIs was poorer than that of their hearing peers (90% in average). No correlation existed between ESs recognition and receptive vocabulary comprehension. Two predictive factors: pre-implantation residual hearing and duration of CI usage were found to be associated with recognition performance of daily-encountered ESs. Acoustically, sounds with distinct temporal patterning were easier to identify for children with CIs. In conclusion, we have demonstrated that ESs recognition is not easy for children with CIs and a low correlation existed between linguistic sounds and ESs recognition in these subjects. Recognition ability of ESs in children with CIs can only be achieved by natural exposure to daily-encountered auditory stimuli if sounds other than speech stimuli were less emphasized in routine verbal/oral habilitation program. Therefore, task-specific measures other than speech materials can be helpful to capture the full profile of auditory perceptual progress after implantation.     

基于结构方程的公共卫生服务提供效果影响因素研究

为了分析公立医院提供公共卫生服务效果的影响因素,通过问卷调查,收集了黑龙江省102家公立医院公共卫生服务相关数据。运用软件SPSS18.0和AMOS7.0对数据进行统计分析,建立公立医院提供公共卫生服务效果影响因素结构方程模型,探讨公共卫生服务提供效果的影响因素。研究发现,财政补偿与政府监管、医院管理、医院投入对公共卫生服务提供效果具有促进作用。     

Timing of Surgical Intervention with Cochlear Implant in Patients with Large Vestibular Aqueduct Syndrome

Objectives

(1) To report the speech perception and intelligibility results of Mandarin-speaking patients with large vestibular aqueduct syndrome (LVAS) after cochlear implantation (CI); (2) to compare their performance with a group of CI users without LVAS; (3) to understand the effects of age at implantation and duration of implant use on the CI outcomes. The obtained data may be used to guide decisions about CI candidacy and surgical timing.

Methods

Forty-two patients with LVAS participating in this study were divided into two groups: the early group received CI before 5 years of age and the late group after 5. Open-set speech perception tests (on Mandarin tones, words and sentences) were administered one year after implantation and at the most recent follow-up visit. Categories of auditory perception (CAP) and Speech Intelligibility Rating (SIR) scale scores were also obtained.

Results

The patients with LVAS with more than 5 years of implant use (18 cases) achieved a mean score higher than 80% on the most recent speech perception tests and reached the highest level on the CAP/SIR scales. The early group developed speech perception and intelligibility steadily over time, while the late group had a rapid improvement during the first year after implantation. The two groups, regardless of their age at implantation, reached a similar performance level at the most recent follow-up visit.

Conclusion

High levels of speech performance are reached after 5 years of implant use in patients with LVAS. These patients do not necessarily need to wait until their hearing thresholds are higher than 90 dB HL or PB word score lower than 40% to receive CI. They can do it “earlier” when their speech perception and/or speech intelligibility do not reach the performance level suggested in this study.     

Investigating the Relationships among Stressors,Stress Level,and Mental Symptoms for Infertile Patients: A Structural Equation Modeling Approach

Objective

Patients with infertility are a high risk group in depression and anxiety. However, an existing theoretically and empirically validated model of stressors, stress, and mental symptoms specific for infertile patients is still a void. This study aimed to determine the related factors and their relational structures that affect the level of depressive and anxiety symptoms among infertile patients.

Methods

A cross-sectional sample of 400 infertility outpatients seeking reproduction treatments in three teaching hospitals across Taiwan participated in the structured questionnaire survey in 2011. The hypothesized model comprising 10 latent variables was tested by Structural Equation Modeling using AMOS 17.

Results

Goodness-of-fit indexes, including χ²/DF = 1.871, PGFI = 0.746, PNFI = 0.764, and others, confirmed the modified model fit the data well. Marital stressor, importance of children, guilt-and-blame, and social stressor showed a direct effect on perceived stress. Instead of being a factor of stress, social support was directly and positively related to self-esteem. Perceived stress and self-esteem were the two major mediators for the relationships between stressors and mental symptoms. Increase in social support and self-esteem led to decrease in mental symptoms among the infertile patients.

Conclusions

The relational structures were identified and named as the Stressors Stress Symptoms Model, clinically applied to predict anxiety and depression from various stressors. Assessing sources and level of infertility-related stress and implementing culturally-sensitive counseling with an emphasis on positive personal value may assist in preventing the severity of depression and anxiety.     

A Dynamical Low-Rank Approach to the Chemical Master Equation

Stochastic reaction kinetics have increasingly been used to study cellular systems, with applications ranging from viral replication to gene regulatory networks and to signaling pathways. The underlying evolution equation, known as the chemical master equation (CME), can rarely be solved with traditional methods due to the huge number of degrees of freedom. We present a new approach to directly solve the CME by a dynamical low-rank approximation based on the Dirac–Frenkel–McLachlan variational principle. The new approach has the capability to substantially reduce the number of degrees of freedom, and to turn the CME into a computationally tractable problem. We illustrate the accuracy and efficiency of our methods in application to two examples of biological interest.     

Assessing ecological integrity: A multi-scale structural and functional approach using Structural Equation Modeling

Facing increasing levels of ecosystem degradation, scientists and practitioners aim to preserve ecological integrity — to maintain structures and functions expected of ecosystems in a region. This requires an understanding of the relationship between structural components and functional integrity. In this paper we focused on the study forests of the Credit River Watershed (Southern Ontario, Canada). For this ecosystem we consider one of the major contributors to functional integrity: habitat functions which are defined as the capacity of the ecosystem to provide refuge and reproduction habitat to wild species of plants and animals. We define these ‘habitat functions’ as a latent variable in Structural Equation Modeling, which allows us to examine its relationship with a number of candidate indicators. We first determined two community-level structural indicators to represent the latent variable: native plant cover and forest bird abundance. We then found underlying causal relationships between multi-scale structural components of the ecosystem and the provision of habitat functions. Three variables at the local scale explain native plant cover — soil nitrogen, soil organic matter, and soil pH. A significant landscape-level variable, patch area, explained native plant cover. Percent natural land cover in a 500 m radius explained forest bird abundance. From a theoretical point of view, this modeling technique allows us to explore complex and simultaneous interactions between structures and functions of ecosystems. As for its practical applications, it can be used to improve ecological integrity monitoring programs by contributing to the selection of meaningful indicators.     

Contralateral Masking in Bilateral Cochlear Implant Patients: A Model of Medial Olivocochlear Function Loss

Contralateral masking is the phenomenon where a masker presented to one ear affects the ability to detect a signal in the opposite ear. For normal hearing listeners, contralateral masking results in masking patterns that are both sharper and dramatically smaller in magnitude than ipsilateral masking. The goal of this study was to investigate whether medial olivocochlear (MOC) efferents are needed for the sharpness and relatively small magnitude of the contralateral masking function. To do this, bilateral cochlear implant patients were tested because, by directly stimulating the auditory nerve, cochlear implants circumvent the effects of the MOC efferents. The results indicated that, as with normal hearing listeners, the contralateral masking function was sharper than the ipsilateral masking function. However, although there was a reduction in the magnitude of the contralateral masking function compared to the ipsilateral masking function, it was relatively modest. This is in sharp contrast to the results of normal hearing listeners where the magnitude of the contralateral masking function is greatly reduced. These results suggest that MOC function may not play a large role in the sharpness of the contralateral masking function but may play a considerable role in the magnitude of the contralateral masking function.