Updated systematic-narrative review on communication intervention in Rett Syndrome: 2010-2022.

Due to loss of spoken language and resulting complex communication needs, people with Rett syndrome are obvious candidates for communication intervention. To advance evidence-based practice and guide future research efforts, we identified and summarized 16 communication intervention studies published since a previous 2009 review on this topic. Studies were summarized in terms of (a) participants, (b) dependent variables related to communication, (c) intervention characteristics, (d) outcomes, and (e) certainty of evidence. Across the 16 studies, intervention was provided to a total of 100 participants from 3 to 47 years of age. Half of the studies used systematic instruction to teach aided AAC. Other interventions and associated technologies included music therapy, eye tracking technology, and transcranial stimulation. Positive outcomes (e.g., using AAC devices to make requests and/or initiate social-communication interactions) were reported in 13 of the studies. These 16 new studies provide additional guidance on how to enhance the communicative functioning of people with Rett syndrome. Future research directions are highlighted.

A Behavioral Approach to Treatment and Assessment of People with Disorders of Consciousness: The Response-contingent Stimulation Strategy.

Response-contingent stimulation is a behavioral strategy used to improve the situation of patients with disorders of consciousness. Such strategy involves the presentation of brief periods (e.g., 10 to 15 s) of stimulation considered preferred by the patients, contingent on (immediately after) the emission of specific patients' responses. The aim is to help the patients learn the link between their responding and the preferred stimulation and thus learn to use their responding to access the stimulation in a self-determined/independent manner. Achieving these goals is considered important for the patients' recovery process and thus the response-contingent stimulation strategy that promotes such an achievement can be considered a valuable treatment approach. The same strategy combined with the use of periods of non-contingent stimulation (i.e., stimulation delivered independent of responding) may also serve as an assessment supplement with patients with apparent unresponsive wakefulness. The patients' increase in responding during the response-contingent stimulation and decline in responding during the non-contingent stimulation could be taken as a sign of discrimination between conditions, and possibly a sign of awareness of the immediate environmental situation, compatible with a diagnosis of minimally conscious state. This paper analyzes a number of studies aimed at using the response-contingent stimulation as a treatment strategy and a number of studies aimed at combining response-contingent stimulation with non-contingent stimulation for treatment and assessment purposes. The results of the studies are discussed in terms of the effectiveness, accessibility and affordability of the strategy. The need for new research (i.e., replication studies) is also pointed out.

Mainstream technology to support basic communication and leisure in people with neurological disorders, motor impairment and lack of speech.

OBJECTIVE: To assess a simple technology solution to support basic communication and leisure in people with neurological disorders, extensive motor impairment, and absence of speech. DESIGN: The design was a non-concurrent multiple baseline across participants. METHODS: The study included eight participants and assessed a technology setup including a Samsung Galaxy Tab S2 LTE tablet and a Samsung Galaxy A3 smartphone. The smartphone, automated via MacroDroid, presented the participant with leisure, messages, and caregiver options. Choosing leisure or messages (by activating the smartphone's proximity sensor) led the smartphone to present the alternatives available for that option and eventually verbalize the alternative selected. This verbalization triggered the tablet's Google Assistant and led the tablet to present a leisure event or start a message exchange. Choosing the caregiver led the smartphone to invite the caregiver to interact with the participant. RESULTS: During baseline (i.e., when a standard smartphone was available), the participants did not activate any of the options. During intervention and post-intervention (i.e., with the technology described above), participants activated all options and spent most of the session time positively engaged with them. CONCLUSIONS: The aforementioned technology seems to be a useful tool for individuals like those involved in this study.

Promoting physical activity in people with intellectual and multiple disabilities through a basic technology-aided program.

This study assessed a technology-aided program (monitoring responding, and ensuring preferred stimulation and encouragements) for promoting physical activity with 11 participants with severe/profound intellectual and multiple disabilities. Each participant was provided with an exercise device (e.g. a static bicycle and a stepper) and exposed to the program according to an ABAB design, in which A and B represented baseline and intervention phases, respectively. Data recording concerned (a) the participants' responses with the exercise device (e.g. pedaling) during baseline and intervention phases and (b) their heart rates during the last intervention phase. The results showed that all participants had significant increases in responding with the exercise devices during the intervention phases. Heart-rate values during the intervention sessions indicated that the participants' responding during those sessions mostly amounted to moderate-intensity physical activity, with potential benefits for their overall physical condition. Implications of the findings and questions for future research in the area were discussed.

Research note: attitudes of teachers and undergraduate students regarding three augmentative and alternative communication modalities.

The social validity of different communication modalities is a potentially important variable to consider when designing augmentative and alternative communication (AAC) interventions. To assess the social validity of three AAC modes (i.e., manual signing, picture exchange, and an iPad®-based speech-generating device), we asked 59 undergraduate students (pre-service teachers) and 43 teachers to watch a video explaining each mode. They were then asked to nominate the mode they perceived to be easiest to learn as well as the most intelligible, effective, and preferred. Participants were also asked to list the main reasons for their nominations and report on their experience with each modality. Most participants (68-86%) nominated the iPad-based speech-generating device (SGD) as easiest to learn, as well as the most intelligible, effective, and preferred. This device was perceived to be easy to understand and use and to have familiar and socially acceptable technology. Results suggest that iPad-based SGDs were perceived as more socially valid among this sample of teachers and undergraduate students. Information of this type may have some relevance to designing AAC supports for people who use AAC and their current and future potential communication partners.

Microswitch Technology for Enabling Self-Determined Responding in Children with Profound and Multiple Disabilities: A Systematic Review.

We reviewed 18 studies reporting on the use of microswitch technology to enable self-determined responding in children with profound and multiple disabilities. Identified studies that met pre-determined inclusion criteria were summarized in terms of (a) participants, (b) experimental design, (c) microswitches and procedures used, and (d) main results. The 18 studies formed three groups based on whether the microswitch technology was primarily intended to enable the child to (a) access preferred stimuli (7 studies), (b) choose between stimuli (6 studies), or (c) recruit attention/initiate social interaction (5 studies). The results of these studies were consistently positive and support the use of microswitch technology in educational programs for children with profound and multiple disabilities as a means to impact their environment and interact with others. Implications for delivery of augmentative and alternative communication intervention to children with profound and multiple disabilities are discussed.

EXTENDING THE ASSESSMENT OF TECHNOLOGY-AIDED PROGRAMS TO SUPPORT LEISURE AND COMMUNICATION IN PEOPLE WITH ACQUIRED BRAIN INJURY AND EXTENSIVE MULTIPLE DISABILITIES.

Intervention programs for people with acquired brain injury and extensive motor and communication impairment need to be diversified according to their characteristics and environment. These two studies assessed two technology-aided programs for supporting leisure (i.e., access to songs and videos) and communication (i.e., expressing needs and feelings and making requests) in six of those people. The three people participating in Study 1 did not possess speech but were able to understand spoken and written sentences. Their program presented leisure and communication options through written phrases appearing on the computer screen. The three people participating in Study 2 did not possess any speech and were unable to understand spoken or written language. Their program presented leisure and communication options through pictorial images. All participants relied on a simple microswitch response to enter the options and activate songs, videos, and communication messages. The data showed that the participants of both studies learned to use the program available to them and to engage in leisure and communication independently. The importance of using programs adapted to the participants and their environment was discussed.

Tangible Symbols as an AAC Option for Individuals with Developmental Disabilities: A Systematic Review of Intervention Studies.

We reviewed nine studies evaluating the use of tangible symbols in AAC interventions for 129 individuals with developmental disabilities. Studies were summarized in terms of participants, tangible symbols used, communication functions/skills targeted for intervention, intervention procedures, evaluation designs, and main findings. Tangible symbols mainly consisted of three-dimensional whole objects or partial objects. Symbols were taught as requests for preferred objects/activities in five studies with additional communication functions (e.g., naming, choice making, protesting) also taught in three studies. One study focused on naming activities. With intervention, 54% (n = 70) of the participants, who ranged from 3 to 20 years of age, learned to use tangible symbols to communicate. However, these findings must be interpreted with caution due to pre-experimental or quasi-experimental designs in five of the nine studies. Overall, tangible symbols appear promising, but additional studies are needed to establish their relative merits as a communication mode for people with developmental disabilities.

Two men with advanced amyotrophic lateral sclerosis operate a computer-aided television system through mouth or throat microswitches.

This study assessed a simple technology to enable two men affected by amyotrophic lateral sclerosis, in an advanced stage (i.e., with pervasive motor disabilities and lack of speech), to operate a computer-aided television system. The technology included microswitches, a portable computer, an interface connecting the microswitches to the computer, a commercial software package to allow the possibility of watching television via computer, and specific software to allow microswitch activations to be recorded as forward commands for channel change. The participants (a) gained control over the television programs to watch or not to watch (i.e., through channel changes) and (b) showed increased attention to the programs (i.e., an increase in their watching time). The practical relevance of the findings, the integration of the technology used in this study within the participants' wider recreation and communication program, and ways of extending the research were discussed.

Case studies of technology for adults with multiple disabilities to make telephone calls independently.

Recent literature has shown the possibility of enabling individuals with multiple disabilities to make telephone calls independently via computer-aided telephone technology. These two case studies assessed a modified version of such technology and a commercial alternative to it for a woman and a man with multiple disabilities, respectively. The modified version used in Study 1 (a) presented the names of the persons available for a call and (b) reminded the participant of the response she needed to perform (i.e., pressing a microswitch) if she wanted to call any of those names/persons. The commercial device used in Study 2 was a Galaxy S3 (Samsung) equipped with the S-voice module, which allowed the participant to activate phone calls by uttering the word "Call" followed by the name of the persons he wanted to call. The results of the studies showed that the participants learned to make phone calls independently using the technology/device available. Implications of the results are discussed.

Enabling People With Intellectual and Sensory Disabilities to Trigger a Tablet's Delivery of Task Instructions by Walking to the Tablet: Proof-of-Concept Study.

BACKGROUND: People with intellectual and sensory or sensory-motor disabilities tend to have problems performing multistep tasks. To alleviate their problems, technological solutions have been developed that provide task-step instructions. Instructions are generally delivered at people's request (eg, as they touch an area of a computer or tablet screen) or automatically, at preset intervals. OBJECTIVE: This study carried out a preliminary assessment of a new tablet-based technology system that presented task-step instructions when participants with intellectual and sensory disabilities walked close to the tablet (ie, did not require participants to perform fine motor responses on the tablet screen). METHODS: The system entailed a tablet and a wireless camera and was programmed to present instructions when participants approached the tablet, that is, when the camera positioned in front of the tablet detected them. Two instructions were available for each task step. One instruction concerned the object(s) that the participants were to collect, and the other instruction concerned the "where" and "how" the object(s) collected would need to be used. For 3 of the six participants, the two instructions were presented in succession, with the second instruction presented once the required object(s) had been collected. For the other 3 participants, the two instructions were presented simultaneously. Instructions consisted of pictorial representations combined with brief verbal phrases. The impact of the system was assessed for each of the 2 groups of participants using a nonconcurrent multiple baseline design across individuals. RESULTS: All participants were successful in using the system. Their mean frequency of correct task steps was close to or above 11.5 for tasks including 12 steps. Their level of correct performance tended to be much lower during the baseline phase when they were to receive the task-step instructions from a regular tablet through scrolling responses. CONCLUSIONS: The findings, which need to be interpreted with caution given the preliminary nature of the study, suggest that the new tablet-based technology system might be useful for helping people with intellectual and sensory disabilities perform multistep tasks.

Teaching multi-step requesting and social communication to two children with autism spectrum disorders with three AAC options.

The present study involved comparing the acquisition of multi-step requesting and social communication across three AAC options: manual signing (MS), picture exchange (PE), and speech-generating devices (SGDs). Preference for each option was also assessed. The participants were two children with autism spectrum disorders (ASD) who had previously been taught to use each option to request preferred items. Intervention was implemented in an alternating-treatments design. During baseline, participants demonstrated low levels of correct communicative responding. With intervention, both participants learned the target responses (two- and three-step requesting responses, greetings, answering questions, and social etiquette responses) to varying levels of proficiency with each communication option. One participant demonstrated a preference for using the SGD and the other preferred PE. The importance of examining preferences for using one AAC option over others is discussed.

Enabling people with intellectual and other disabilities to make verbal requests using cardboard chips with mini objects or pictures and a smartphone.

Objective: This study aimed to help six participants with intellectual disability combined with sensory and motor impairments to make verbal requests through the use of a technology system involving cardboard chips and a smartphone. Method: The participants were divided into two groups of three based on whether they did or did not have visual skills. Each group was exposed to the intervention with the technology system according to a non-concurrent multiple baseline across participants design. During the 20 min intervention sessions, the participants were provided with a smartphone and nine cardboard chips each of which had a picture or object (i.e., a mini object replica or raised object contour) and several radio frequency identification tags attached to it. To make a request, the participants were to bring a cardboard chip in contact with the smartphone. This read the tags attached to the cardboard and verbalized the request related to that cardboard. Results: During the baseline (without cardboard chips and smartphone), the participants' mean frequency of independent requests (all non-verbal requests) varied between zero and near 1.5 per session. During the intervention (with cardboard chips and smartphone), the participants' mean frequency of independent requests (all verbal requests) varied between over 4.5 and about 10 per session. Conclusion: The results suggest that the system might be useful to help participants like the ones included in this study to make verbal requests with simple responses.

Use of technology to sustain mobility in older people with cognitive impairment and dementia: a scoping review.

PURPOSE: This paper provides a review of studies that evaluated technology devices to sustain various forms of mobility in older people with cognitive impairment or dementia and mobility problems (e.g., inability to ambulate or difficulties to orient with consequent failures to reach relevant destinations). METHODS: A literature search was performed for English language articles indexed in PubMed, PsycINFO, and Web of Science. This search was then supplemented with an ancestral and forward search. The search covered the period ranging between January 2010 and October 2020. RESULTS: Twenty-seven articles were included in the review. They were divided into four groups according to whether they aimed to promote (a) supported ambulation, (b) use of safe power wheelchair, (c) ambulation guided by orientation cues, and (d) ambulation with GPS-based assistance. The studies were coded in terms of the objective pursued, technology used, participants involved, strategies applied to assess the technology, and outcome reported. CONCLUSION: A variety of technology devices are available to address the mobility problems of older people with cognitive impairment or dementia. The devices' accessibility and usability differ widely. Data on their impact are still largely preliminary and new systematic research is needed.IMPLICATIONS FOR REHABILITATIONA variety of mobility problems may afflict older people with cognitive impairment or dementia and curtail their opportunities to freely move indoor or outdoor.Technology devices have been developed to (a) promote forms of supported ambulation, (b) facilitate safe use of power wheelchairs, (c) deliver orientation/navigation cues, and (d) ensure tracking and possible assistance.The technology devices differ in terms of accessibility and affordability, with some of them readily available and others at a developmental stage.Data on the effectiveness of the various technology devices in reducing the impact of the mobility problems are still largely preliminary and new systematic research is needed.

Low-Cost Technology-Aided Programs for Supporting People With Motor, Visual, and Intellectual Disabilities in Functional Forms of Occupation and Communication: Proof-of-Concept Study.

BACKGROUND: People with motor, visual, and intellectual disabilities may have serious problems in independently accessing various forms of functional daily occupation and communication. OBJECTIVE: The study was aimed at developing and assessing new, low-cost technology-aided programs to help people with motor or visual-motor and intellectual disabilities independently engage in functional forms of occupation and communication with distant partners. METHODS: Two programs were set up using a smartphone interfaced with a 2-switch device and a tablet interfaced with 2 pressure sensors, respectively. Single-subject research designs were used to assess (1) the first program with 2 participants who were blind, had moderate hand control, and were interested in communicating with distant partners through voice messages; and (2) the second program with 2 participants who possessed functional vision, had no or poor hand control, and were interested in communicating with their partners through video calls. Both programs also supported 2 forms of occupational engagement, that is, choosing and accessing preferred leisure events consisting of songs and music videos, and listening to brief stories about relevant daily topics and answering questions related to those stories. RESULTS: During the baseline phase (when only a conventional smartphone or tablet was available), 2 participants managed sporadic access to leisure or leisure and communication events. The other 2 participants did not show any independent leisure or communication engagement. During the intervention (when the technology-aided programs were used), all participants managed to independently engage in multiple leisure and communication events throughout the sessions and to listen to stories and answer story-related questions. CONCLUSIONS: The findings, which need to be interpreted with caution given the nature of the study and the small number of participants, seem to suggest that the new programs may be viable tools for helping people with motor or visual-motor and intellectual disabilities independently access leisure, communication, and other forms of functional engagement.

Technology-based intervention to help persons with minimally conscious state and pervasive motor disabilities perform environmentally relevant adaptive behavior.

Persons with a diagnosis of minimally conscious state and pervasive motor disabilities tend to be passive and isolated. A way to help them improve their adaptive behavior (relate to their environment) involves the use of intervention packages combining assistive technology with motivational strategies. The types of assistive technology included in those packages may consist of (a) microswitches allowing direct access to environmental stimuli, (b) combinations of microswitches and voice output communication devices (VOCAs) allowing stimulus access and calls for caregivers' attention, respectively, and (c) computer presentations of stimulus options and microswitches allowing choice among those options and access to them.

Microswitch technology and contingent stimulation to promote adaptive engagement in persons with minimally conscious state: a case evaluation.

This study assessed whether a post-coma woman functioning at the lower end of the minimally conscious state would (a) develop adaptive responding through the use of microswitch technology and contingent stimulation, (b) consolidate and maintain her responding over time, and (c) show evidence of response-consequences awareness (learning and discrimination). The study involved an ABABB1CB1 sequence in which the A represented baseline phases, the B and B1 intervention phases, and the C a control phase with continuous stimulation. Results indicated that the woman developed adaptive responding and consolidated it over the intervention phases of the study. The woman also showed evidence of being aware of response-consequences links. Potential implications and limitations of these findings are discussed.

Two persons with multiple disabilities use camera-based microswitch technology to control stimulation with small mouth and eyelid responses.

BACKGROUND: A camera-based microswitch technology was recently developed to monitor small facial responses of persons with multiple disabilities and allow those responses to control environmental stimulation. This study assessed such a technology with 2 new participants using slight variations of previous responses. METHOD: The technology involved a computer with a CPU using a 2GHz clock, a USB video camera with 16-mm lens, and special software. Small colour spots were used under the lower lip of one participant and on the eyelid of the other participant to aid the camera and computer to detect their mouth and eyelid responses. The study involved an ABAB design and included a 3-week post-intervention check. RESULTS: The participants' mouth and eyelid responses increased during the intervention (B) phases and post-intervention check (i.e., when the technology allowed them to control stimulation). CONCLUSIONS: Camera-based microswitch technology can help persons with multiple disabilities control stimulation with small responses.

Access to environmental stimulation via eyelid responses for persons with acquired brain injury and multiple disabilities: a new microswitch arrangement.

This study assessed a new microswitch arrangement for eyelid responses using an optic sensor placed above the cheekbone and a small sticker on the person's eyelid. This new arrangement, which was designed to avoid interference of the microswitch with the person's visual functioning, was tested on three adults with acquired brain injury and multiple (consciousness, communication, and motor) disabilities. The study was carried out according to a non-concurrent multiple baseline design across participants. Data showed the new microswitch arrangement was suitable for all three participants, who increased their responding during the intervention phase of the study when their responses allowed them to access preferred stimulation. Practical implications of the findings are discussed.

Behavioral intervention approaches for people with disorders of consciousness: a scoping review.

PURPOSE: This review was intended to provide an overall picture of work conducted during the last decade to assess the impact of behavioral intervention strategies on people with disorders of consciousness (i.e., comatose state, vegetative state/unresponsive wakefulness, or minimally conscious state). The intervention strategies considered were those not based on music or including music as a component of the intervention package. METHODS: Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses Extension for Scoping Reviews (PRISMA-ScR) checklist, a scoping review was carried out to identify and provide a synthesis of eligible studies published in English during the 2010-2021 period. Three databases (i.e., PubMed, PsycINFO, and Web of Science) were employed for the literature search. RESULTS: Forty studies met the inclusion criteria. Those studies were grouped into three categories based on whether they assessed the effects of: (i) verbal stories/messages, (ii) multiple stimulation, and (iii) response-contingent stimulation. Then, a narrative synthesis of the studies of each of the three categories was provided to specify the types of patients involved, the intervention and assessment conditions implemented, and the outcome attained. CONCLUSIONS: The evidence reported in most of the studies might be considered encouraging and convincing. Even so, it might be very difficult to view the evidence of the various studies cumulatively and make general/conclusive statements due to a number of differences in the intervention conditions applied.Implications for rehabilitationAn informative picture of the studies using behavioral interventions with people with disorders of consciousness is essential to any professional working in the area.Such picture can be highly useful in providing a view of the intervention strategies used for those people, of the variations existing within and across strategies, and of the evidence available.An analysis of the strategies, their implementation and their effects may provide new insights for improving those strategies and eventually increasing their impact.The individuals charged with the implementation process (e.g., family members or nurses) may have a relevant influence on the overall impact of the strategy.