Translating birdsong: songbirds as a model for basic and applied medical research.

Songbirds, long of interest to basic neuroscience, have great potential as a model system for translational neuroscience. Songbirds learn their complex vocal behavior in a manner that exemplifies general processes of perceptual and motor skill learning and, more specifically, resembles human speech learning. Song is subserved by circuitry that is specialized for vocal learning and production but that has strong similarities to mammalian brain pathways. The combination of highly quantifiable behavior and discrete neural substrates facilitates understanding links between brain and behavior, both in normal states and in disease. Here we highlight (a) behavioral and mechanistic parallels between birdsong and aspects of speech and social communication, including insights into mirror neurons, the function of auditory feedback, and genes underlying social communication disorders, and (b) contributions of songbirds to understanding cortical-basal ganglia circuit function and dysfunction, including the possibility of harnessing adult neurogenesis for brain repair.

Communicative abilities in young children with a significant cognitive and motor developmental delay.

BACKGROUND: Children with a significant cognitive and motor developmental delay are pre-symbolic communicators. The primary aim of this study was to reveal the variability within the communicative functioning of this group of children in terms of communication level, the reasons to communicate and behavioural expressions. METHODS: Twenty-six children between 14 and 58 months with a significant cognitive and motor developmental delay were recruited. The Communication Matrix of Rowland (2011, Communication Disorders Quarterly, 32, 190) was used to integrate different sources of information on the children's communicative functioning. RESULTS: These children primarily communicated at the level of pre-intentional and intentional behaviour, aimed at refusing, obtaining and, to a lesser extent, social purposes. CONCLUSIONS: To develop or adapt early intervention strategies, and to monitor progress in communicative development, an even more nuanced view on these children's communicative utterances in terms of frequency, duration, idiosyncrasy and context relatedness is needed.

Development of a tool to assess visual attention in Rett syndrome: a pilot study.

This was a two-phase study that aimed to (a) develop a tool for assessing visual attention in individuals with Rett syndrome using AAC with a communication partner during naturalistic interactions in clinical settings; and (b) explore aspects of the tool's reliability, validity, and utility. The Assessment of Visual Attention in Interaction (AVAI) tool was developed to assess visual attention operationalized as focused gazes (1 s or longer) at the communication partner, an object, and a symbol set. For the study, six video-recorded interactions with nine female participants diagnosed with Rett syndrome (range: 15-52-years-old) were used to calculate intra- and inter-rater agreement, and 18 recorded interactions were analyzed to examine sensitivity to change and acceptability. There was a significant difference in the AVAI results between two conditions (with and without aided-language modeling). Inter-rater agreement ranged from moderate and strong. There was a range in scores, indicating that the AVAI could differentiate between participants. The AVAI was found to be reliable, able to detect change, and acceptable to the participants. This tool could potentially be used for evaluating interventions that utilize aided AAC.

Adapted Picture Exchange Communication System using tangible symbols for young learners with significant multiple disabilities.

Practitioners need validated strategies for teaching children with significant multiple disabilities (e.g., cognitive, motor, and sensory disability) to use tangible symbols for expressive communication. This single-case experimental design study replicated the positive effect of an adapted protocol for teaching Phase 1 of the Picture Exchange Communication System (PECS) using tangible symbols and extended it to a younger group (4-7 years old) of learners with multiple disabilities. It also tested the effect of an adapted protocol for Phase 2 of PECS to incorporate use of a single switch speech-generating device to gain the attention of an adult communication partner. Two of three students who reached mastery in Phase 1 also reached mastery in Phase 2 and may have generalized requesting behavior from the interventionist (i.e., researchers) to their classroom teacher. Results add to the growing evidence base that shows that the adapted PECS Phase 1 procedures are a promising practice for learners with multiple disabilities, including sensory impairment, and provide preliminary evidence for a more efficient and effective approach to adapting PECS Phase 2 than previously studied for this group of learners. Directions for future research and recommendations for practice are provided.

Visual processing patterns of adults with traumatic brain injury when viewing image-based grids and visual scenes.

Many individuals with traumatic brain injury (TBI) rely on augmentative and alternative communication (AAC) supports to meet functional communication needs. The use of images to display message content may be advantageous for this population, given deficits in literacy, visual processing, and cognition. Grid and visual scene displays are two options for presenting images within AAC systems. Eye-tracking technology was utilized to evaluate the visual processing patterns of 13 adults with a history of TBI and 13 adults without TBI when interpreting themes depicted in grid and visual scene displays. Results indicated that differential processing and effort was required when interpreting the two display types. Both groups of participants required significantly more visual fixation time and exhibited patterns consistent with greater cognitive effort when identifying themes depicted in grids compared to visual scenes (i.e., greater numbers of short fixations). The participants with TBI were overall less efficient than those without TBI when identifying themes presented in both AAC display types. These results add to the growing body of evidence indicating that visual scenes may more effectively represent complex concepts than decontextualized, grid-based images for individuals with TBI. Additional clinical implications are discussed.

Examining bidirectional effects between the autism spectrum disorder (ASD) core symptom domains and anxiety in children with ASD.

BACKGROUND: Although a bidirectional relationship between autism spectrum disorder (ASD) and anxiety symptoms is assumed, few studies have investigated this. Moreover, little is known about potential differential relationships of the two core symptom domains of ASD - social communication impairment and restricted, repetitive behavior - with anxiety over time. METHOD: Participants were 130 children with an ASD (M age 6.7 years, 81.5% boys) of whom 79 participated in a follow-up assessment 2 years later. We used cross-lagged models to test whether social communication impairment and restricted, repetitive behavior at T0 predicted anxiety at T2 and vice versa. RESULTS: Crossed-lagged models showed that anxiety symptoms predicted social communication impairment over time (ß = .22, p = .008), but not vice versa (ß = -.07, p = .49). There were no significant paths from anxiety symptoms to later restricted, repetitive behavior (ß = .11, p = .34) or vice versa (ß = -.11, p = .27). CONCLUSIONS: Our results do not support a bidirectional relationship between the ASD core symptom domains and anxiety, but suggest that higher levels of anxiety symptoms increase the risk of more social communication impairment over time in children with ASD. This underlines the importance of treating anxiety symptoms to improve both social and emotional functioning.

Reliability and validity of the Korean-language version of the Communication Function Classification System in children with cerebral palsy.

PURPOSE: The Communication Function Classification System (CFCS) and Viking Speech Scale (VSS) are useful systems for describing the broad communication function and speech intelligibility, respectively, of children with cerebral palsy (CP). The aims of this study were to determine the reliability and validity of the Korean version of the CFCS and also to investigate the association between the CFCS and the VSS and other functional classifications for children with CP. MATERIALS AND METHODS: Participants were 50 children with CP (33 males, 17 females; mean age 7.2 years, range 4-16 years) recruited from a rehabilitation hospital. We analysed the interrater and intrarater reliabilities of the Korean version of the CFCS and VSS between parents, a physiatrist, and a speech-language pathologist (SLP). The social function domain of the Paediatric Evaluation of Disability Inventory was assessed to examine the concurrent validity of the CFCS and VSS. RESULTS: The intrarater reliabilities of the CFCS and VSS were excellent in a physiatrist (ƙ = 0.92, ƙ = 0.94, respectively) and an SLP (ƙ = 0.98, ƙ = 0.98) and very good in parents (ƙ = 0.87, ƙ = 0.89). The interrater reliability of the CFCS and VSS was very good between the physiatrist and SLP (ƙ = 0.87, ƙ = 0.89) and good between parents and the SLP (ƙ = 0.63, ƙ = 0.78) and between parents and the physiatrist (ƙ = 0.61, ƙ = 0.76). The CFCS and VSS were strongly related with the social function domain of Paediatric Evaluation of Disability Inventory. In addition, we found very strong associations between the VSS and CFCS. CONCLUSIONS: The Korean version of the CFCS is a valid and reliable tool to classify communication ability and is strongly associated with the VSS, a reliable tool to classify speech intelligibility.

Visual-spatial cognition in children using aided communication.

Children with severe motor impairments are restricted in their manipulation and exploration of objects, but little is known about how such limitations influence cognitive development. This study investigated visual-constructional abilities in 75 children and adolescents, aged 5;0-15;11 (years;months), with severe speech impairments and no intellectual disabilities (aided group) and in 56 children and adolescents with typical development (reference group). Verbal comprehension, non-verbal reasoning, and visual-spatial perception were assessed with standardized tests. The task of the participants was to verbally instruct communication partners to make physical constructions identical to models that the partner could not see. In the aided group, 55.7% of the constructions were identical to the models participants described, compared to 91.3% in the reference group. In the aided group, test results explained 51.4% of the variance in construction errors. The results indicate that the participants' language skills were decisive for construction success. Visual-perceptual challenges were common among the aided communicators, and their instructions included little information about size and spatial relations. This may reflect less experience with object manipulation and construction than children with typical development, and using aided communication to instruct others to make three-dimensional constructions. The results imply a need for interventions that compensate for the lack of relevant experience.

Assessment of aided language comprehension and use in children and adolescents with severe speech and motor impairments.

There is limited knowledge about aided language comprehension and use in children who use aided communication and who are considered to have a relatively good comprehension of spoken language. This study's purpose was to assess their aided language skills. The participants were 96 children and adolescents who used communication aids (aided group) and 73 children and adolescents with natural speech (reference group), aged 5 to 15 years. All of the participants who used aided communication were regarded by their teachers or professionals as having age-appropriate language comprehension. All of the participants completed (a) standardized tests of visual perception, non-verbal reasoning, and comprehension of spoken language, and (b) tasks designed for this study that measured comprehension and production of graphic utterances through communicative problem solving. Using their own communication systems, the participants achieved an average of 72% correct on the graphic symbol comprehension task items, and 63% on the expressive tasks. The participants with natural speech achieved an average of 88% correct on comprehension items, and 93-96% accuracy on production items. The differences between groups were significant on all the tasks and standardized tests. There was considerable variation within the group of participants who used aided communication, and the results reveal a need to develop instruments with norms for aided language competence that can inform the implementation of interventions to support aided language development.

Postoperative pain assessment in children: a pilot study of the usefulness of the analgesia nociception index.

BACKGROUND: The ability to perform objective pain assessment is very important in paediatric patients. The goal of this study was to investigate the relationship between the analgesia nociception index (ANI), which is based on the heart rate variability, and objective measurements of pain intensity in young or cognitively impaired children, after surgical or imaging procedures (control group) under general anaesthesia. METHODS: On arrival in the recovery room and subsequently at 5-10 min intervals, the level of pain was rated using the FLACC pain scale (0-10). The ANI values (0-100; 0 indicating the worst pain) were recorded simultaneously. The area under the receiver operating characteristic curve (AUC) and grey zone approach were used to evaluate the performance of the ANI to detect patients with FLACC >4. Instantaneous ANI values were compared with ANI values averaged over 256 s periods of time. RESULTS: All children in the surgical group (n=32) developed moderate-to-severe pain (FLACC >4). Children in the control group (n=30) exhibited minimal pain. Instantaneous ANI values were lower in children of the surgical group than in the control group [52 (sd16) vs 69 (16), P<0.001]. The AUC for the 256 s ANI recording period [0.94 (95% confidence interval 0.85-0.99)] was significantly higher than for instantaneous ANI (P<0.05). When measured for a period of 256 s, an ANI cut-off value of 56 (grey zone [58-60]) was most predictive of a FLACC ≥4. CONCLUSIONS: The ANI may provide an objective measurement of acute postoperative pain, which is correlated with that measured on a FLACC scale in young or cognitively impaired children.

The effect of hypocalcemia in early childhood on autism-related social and communication skills in patients with 22q11 deletion syndrome.

22q11 deletion syndrome (22qDS), also known as DiGeorge syndrome, is a copy number variant disorder that has a diverse clinical presentation including hypocalcaemia, learning disabilities, and psychiatric disorders. Many patients with 22q11DS present with signs that overlap with autism spectrum disorder (ASD) yet the possible physiological mechanisms that link 22q11DS with ASD are unknown. We hypothesized that early childhood hypocalcemia influences the neurobehavioral phenotype of 22q11DS. Drawing on a longitudinal cohort of 22q11DS patients, we abstracted albumin-adjusted serum calcium levels from 151 participants ranging in age from newborn to 19.5 years (mean 2.5 years). We then examined a subset of 20 infants and toddlers from this group for the association between the lowest calcium level on record and scores on the Communication and Symbolic Behavior Scales-Developmental Profile Infant-Toddler Checklist (CSBS-DP ITC). The mean (SD) age at calcium testing was 6.2 (8.5) months, whereas the mean (SD) age at the CSBS-DP ITC assessment was 14.7 (3.8) months. Lower calcium was associated with significantly greater impairment in the CSBS-DP ITC Social (p < 0.05), Speech (p < 0.01), and Symbolic domains (p < 0.05), in regression models adjusted for sex, age at blood draw, and age at the psychological assessment. Nevertheless, these findings are limited by the small sample size of children with combined data on calcium and CSBS-DP ITC, and hence will require replication in a larger cohort with longitudinal assessments. Considering the role of calcium regulation in neurodevelopment and neuroplasticity, low calcium during early brain development could be a risk factor for adverse neurobehavioral outcomes.

Comprehension of spoken language in non-speaking children with severe cerebral palsy: an explorative study on associations with motor type and disabilities.

AIM: To assess spoken language comprehension in non-speaking children with severe cerebral palsy (CP) and to explore possible associations with motor type and disability. METHOD: Eighty-seven non-speaking children (44 males, 43 females, mean age 6y 8mo, SD 2y 1mo) with spastic (54%) or dyskinetic (46%) CP (Gross Motor Function Classification System [GMFCS] levels IV [39%] and V [61%]) underwent spoken language comprehension assessment with the computer-based instrument for low motor language testing (C-BiLLT), a new and validated diagnostic instrument. A multiple linear regression model was used to investigate which variables explained the variation in C-BiLLT scores. Associations between spoken language comprehension abilities (expressed in z-score or age-equivalent score) and motor type of CP, GMFCS and Manual Ability Classification System (MACS) levels, gestational age, and epilepsy were analysed with Fisher's exact test. A p-value <0.05 was considered statistically significant. RESULTS: Chronological age, motor type, and GMFCS classification explained 33% (R=0.577, R(2) =0.33) of the variance in spoken language comprehension. Of the children aged younger than 6 years 6 months, 52.4% of the children with dyskinetic CP attained comprehension scores within the average range (z-score ≥-1.6) as opposed to none of the children with spastic CP. Of the children aged older than 6 years 6 months, 32% of the children with dyskinetic CP reached the highest achievable age-equivalent score compared to 4% of the children with spastic CP. No significant difference in disability was found between CP-related variables (MACS levels, gestational age, epilepsy), with the exception of GMFCS which showed a significant difference in children aged younger than 6 years 6 months (p=0.043). INTERPRETATION: Despite communication disabilities in children with severe CP, particularly in dyskinetic CP, spoken language comprehension may show no or only moderate delay. These findings emphasize the importance of introducing alternative and/or augmentative communication devices from early childhood.

Does metacognitive strategy instruction improve impaired receptive cognitive-communication skills following acquired brain injury?

AIMS: To investigate if metacognitive strategy instruction (MSI) improves the receptive language skills of adults with cognitive-communication disorders secondary to acquired brain injury (ABI). RESEARCH DESIGN: An ABA intervention programme was implemented with eight adults with ABI, aged 25-70 years. The Measure of Cognitive-Linguistic Abilities (MCLA) was administered at baseline and following treatment. The treatment employed in this study involved three components: individual goal-based therapy, group remediation therapy using self-instruction and home practice. RESULTS: No receptive language sub-tests of the MCLA reached statistical significance. However, participants' raw score improvements in receptive language sub-tests indicated that MSI may be effective at remediating CCDs following ABI. CONCLUSIONS: Preliminary findings indicate that MSI may be effective in improving receptive language skills in adults with CCDs following ABI. Further research involving a more rigorous study, a larger sample size and a more reliable outcome measure is necessary and may provide statistically significant evidence for the effectiveness of MSI for remediating receptive language disorders.

Direct brain recordings reveal impaired neural function in infants with single-suture craniosynostosis: a future modality for guiding management?

BACKGROUND: Patients with single-suture craniosynostosis (SSC) are at an elevated risk for long-term learning disabilities. Such adverse outcomes indicate that the early development of neural processing in SSC may be abnormal. At present, however, the precise functional derangements of the developing brain remain largely unknown. Event-related potentials (ERPs) are a form of noninvasive neuroimaging that provide direct measurements of cortical activity and have shown value in predicting long-term cognitive functioning. The current study used ERPs to examine auditory processing in infants with SSC to help clarify the developmental onset of delays in this population. METHODS: Fifteen infants with untreated SSC and 23 typically developing controls were evaluated. ERPs were recorded during the presentation of speech sounds. Analyses focused on the P150 and N450 components of auditory processing. RESULTS: Infants with SSC demonstrated attenuated P150 amplitudes relative to typically developing controls. No differences in the N450 component were identified between untreated SSC and controls. CONCLUSIONS: Infants with untreated SSC demonstrate abnormal speech sound processing. Atypicalities are detectable as early as 6 months of age and may represent precursors to long-term language delay. Electrophysiological assessments provide a precise examination of neural processing in SSC and hold potential as a future modality to examine the effects of surgical treatment on brain development.

Do communication disorders extend to musical messages? An answer from children with hearing loss or autism spectrum disorders.

BACKGROUND: Effective musical communication requires conveyance of the intended message in a manner perceptible to the receiver. Communication disorders that impair transmitting or decoding of structural features of music (e.g., pitch, timbre) and/or symbolic representation may result in atypical musical communication, which can have a negative impact on music therapy interventions. OBJECTIVE: This study compared recognition of symbolic representation of emotions or movements in music by two groups of children with different communicative characteristics: severe to profound hearing loss (using cochlear implants [CI]) and autism spectrum disorder (ASD). Their responses were compared to those of children with typical-development and normal hearing (TD-NH). Accuracy was examined as a function of communicative status, emotional or movement category, and individual characteristics. METHODS: Participants listened to recorded musical excerpts conveying emotions or movements and matched them with labels. Measures relevant to auditory and/or language function were also gathered. RESULTS: There was no significant difference between the ASD and TD-NH groups in identification of musical emotions or movements. However, the CI group was significantly less accurate than the other two groups in identification of both emotions and movements. Mixed effects logistic regression revealed different patterns of accuracy for specific emotions as a function of group. CONCLUSION: Conveyance of emotions or movements through music may be decoded differently by persons with different types of communication disorders. Because music is the primary therapeutic tool in music therapy sessions, clinicians should consider these differential abilities when selecting music for clinical interventions focusing on emotions or movement.

Affective communication deficits associated with cerebellar degeneration.

The cerebellum has extensive connections with the frontal lobes. Cerebellar injury has been reported to induce frontal-executive cognitive dysfunction and blunting of affect. We examined a patient with idiopathic cerebellar degeneration with impaired family relationships attributed to an "emotional disconnection." Examination revealed ataxia, dysmetria, and adiadochokinesia more severe on the left and frontal-executive dysfunction; memory and cognitive functions were otherwise normal. Testing of emotional communication included assessments of emotional semantic knowledge, emotional prosody, and emotional facial expressions. Comprehension was normal but expression was severely impaired. Cerebellar dysfunction can cause a defect in facial and prosodic emotional communication.

Traumatic brain injury in veterans of the wars in Iraq and Afghanistan: communication disorders stratified by severity of brain injury.

OBJECTIVE: To describe the prevalence of communication disorders in veterans of the wars in Iraq and Afghanistan with traumatic brain injury (TBI). DESIGN: Retrospective study of the prevalence of aphasia, fluency and voice disorders among veterans with different severity levels of TBI. Data was obtained from the VA National repository for OEF/OIF/OND veterans who received VA care in Fiscal Years 2010 and 2011. RESULTS: Among the 303,716 veterans in this study, 1848 were diagnosed with a communication disorder; 40% of these were also diagnosed with a TBI. Voice disorders were the most prevalent diagnosis (3.5 per 1000) followed by aphasia (1.9 per 1000) and fluency disorder (0.7 per 1000). Individuals with a TBI diagnosis were more likely to have a diagnosis of aphasia, followed by fluency and then voice disorder. The odds ratio (OR) of aphasia with TBI was 11.09-252.75 (95% CI = 8.78-441.52, p < 0.01). OR for fluency disorders with TBI was 3.58-10.41 (95% CI = 2.56-42.40, p < 0.01) and association of voice disorders with TBI was significant for all levels of TBI severity (OR = 1.5-6.61, 95% CI = 1.24-14.05, p < 0.01). CONCLUSIONS: Veterans who sustained a TBI were more likely to have a diagnosis of a communication disorder, regardless of TBI severity. Those with TBI, including mild TBI, should be screened and evaluated for communication disorders.

See me, feel me. Using physiology to validate behavioural observations of emotions of people with severe or profound intellectual disability.

BACKGROUND: Behavioural observations are the most frequently used source of information about emotions of people with severe or profound intellectual disabilities but have not yet been validated against other measures of emotion. In this study we wanted to validate the behavioural observations of emotions using respiration (rib cage contribution, total breath duration, inspiratory time, expiratory time, tidal volume, mean inspiratory flow, minute ventilation) and heart rate variability. METHOD: Twenty-seven participants were presented with four negative and four positive stimuli. During the presentation the participants' respiration and heart rate variability was measured. Each behaviour of the participant was coded as emotive or not. RESULTS: We found the hypothesised higher percentage rib cage contribution, marginal lower mean inspiratory flow and lower heart rate variability when the expressed emotions became more positive. CONCLUSIONS: These results validate the use of behavioural observations to make inferences about emotions.

Advances in the research of melatonin in autism spectrum disorders: literature review and new perspectives.

Abnormalities in melatonin physiology may be involved or closely linked to the pathophysiology and behavioral expression of autistic disorder, given its role in neurodevelopment and reports of sleep-wake rhythm disturbances, decreased nocturnal melatonin production, and beneficial therapeutic effects of melatonin in individuals with autism. In addition, melatonin, as a pineal gland hormone produced from serotonin, is of special interest in autistic disorder given reported alterations in central and peripheral serotonin neurobiology. More specifically, the role of melatonin in the ontogenetic establishment of circadian rhythms and the synchronization of peripheral oscillators opens interesting perspectives to ascertain better the mechanisms underlying the significant relationship found between lower nocturnal melatonin excretion and increased severity of autistic social communication impairments, especially for verbal communication and social imitative play. In this article, first we review the studies on melatonin levels and the treatment studies of melatonin in autistic disorder. Then, we discuss the relationships between melatonin and autistic behavioral impairments with regard to social communication (verbal and non-verbal communication, social interaction), and repetitive behaviors or interests with difficulties adapting to change. In conclusion, we emphasize that randomized clinical trials in autism spectrum disorders are warranted to establish potential therapeutic efficacy of melatonin for social communication impairments and stereotyped behaviors or interests.

[Emotional impact of facial palsy]. / Emotionale Auswirkungen einer Fazialisparese.

Facial palsy is not only a movement disorder but leads also to an emotional and communicative disorder in chronic stage but also in some patients already during the acute phase of the disease. The present review describes the current knowledge of the neurobiological and psychological fundamentals on the relation of facial movement and its emotional context. So far there is not much knowledge on the impact of a facial palsy on the interaction between facial movement, emotional processing and communicative skills of the patient. The emotional contagion seems to be reduced in patients with facial palsy. The ability to express emotions seems also to be reduced. Moreover, the patients feel to be perceived negatively. In fact, most of the expressions of patients with facial palsy are allocated with a negative affect even when the patients are smiling. Patients with facial palsy react with negative stress, anxiety and depression. The patients avoid social contacts. In turn, this reinforces the communicative disorder. The otorhinolaryngologist can use the Facial Disability Index as a simple questionnaire to detect such dysfunctions. Diagnostics that are necessary to develop a therapy program are presented in this review. Standardized therapy concepts that are not only treat the movement disorder but also the emotional context is missing so far. Finally, the review will give an outlook on potential therapy strategies.