Inclusive Research Environments for Deaf and Hard of Hearing English Speakers.

In recent years, an increasing number of deaf and hard of hearing (D/HH) undergraduates have chosen to study in STEM fields and pursue careers in research. Yet, very little research has been undertaken on the barriers and inclusive experiences often faced by D/HH undergraduates who prefer to use spoken English in research settings, instead of American Sign Language (ASL). To identify barriers and inclusive strategies, we studied six English speaking D/HH undergraduate students working in research laboratories with their eight hearing mentors, and their three hearing peers sharing their experiences. Three researchers observed the interactions between all three groups and conducted interviews and focus groups, along with utilizing the Communication Assessment Self-Rating Scale (CASS). The main themes identified in the findings were communication and environmental barriers in research laboratories, creating accessible and inclusive laboratory environments, communication strategies, and self-advocating for effective communication. Recommendations for mentors include understanding the key elements of creating an inclusive laboratory environment for English speaking D/HH students and effectively demonstrating cultural competence to engage in inclusive practices.

An Alternative Method of Assessing d/DHH Secondary-School Students' L2 Language Development.

Little information is available on d/Deaf and hard of hearing (d/DHH) learners' L2 development. Their limited auditory access may discourage them from taking standardized tests, highlighting the need for alternative ways of assessing their L2 development and proficiency. Therefore, this study suggests adopting processability theory, which demonstrates a universal order of L2 development. Interviews with d/DHH learners and their teachers were conducted to explore their current difficulties in regard to understanding their L2 development. Also, we conducted brief speaking tasks to suggest alternatives to testing the L2 development of learners who are d/DHH in comparison to typical literacy learners. The result showed d/DHH students' L2 developmental patterns are similar to those of typical hearing peers, suggesting that d/DHH students and hearing learners share difficulties in similar areas when learning English. Teachers highlighted the lack of appropriate English tests to determine the d/DHH students' L2 development.

Examining the Language and Communication Factors of a Deaf Child with Autism Spectrum Disorder from an Immigrant Korean Family.

Extant research on learners who are d/Deaf or hard of hearing with disabilities who come from Asian immigrant families is extremely sparse. The authors conducted an intrinsic case study of a deaf student with autism who comes from a Korean immigrant family. To acquire a comprehensive understanding of language and communication characteristics, they analyzed (a) interview data of three administrators who worked with the student and family and (b) school documents/reports issued to the parents. Themes are reported across the three components of the tri-focus framework (Siegel-Causey & Bashinski, 1997): the learner, partner, and environment. Implications for practitioners who work with these learners and their families are discussed, including (a) compiling an individualized language and communication profile that encompasses the framework; (b) utilizing culturally and linguistically responsive practices with the family; (c) practicing interprofessional collaboration; and (d) modifying physical and social environments to increase accessibility.

Teaching Literacy to Filipino Deaf Students in Multilingual Classrooms Amid a Pandemic.

This article describes the current landscape of teaching literacy to Filipino Deaf students in a multilingual, multi-cultural classroom amid the pandemic. The article highlights the uniqueness of Filipino Deaf students as multilingual learners in a multi-cultural classroom and the lack of literature and research on Deaf multilingualism both locally and globally. Moreover, the article focuses on the role of Deaf teachers in teaching Filipino Deaf students, especially in their literacy development. The steps being done to ensure that the curriculum is inclusive of Deaf learners who use Filipino Sign Language (FSL), teacher preparation and materials development, and the challenges in the shift to distance learning amid the COVID-19 pandemic are also discussed. Future directions and recommendations include review of curriculum and adaptation, enhancement of teacher preparation, promotion of collaborative teaching and research efforts, and the production of more appropriate and accessible instructional materials for Deaf students.

Visualizing a Framework in Teaching Literacy to Filipino Deaf Students in Multimodal Learning Spaces.

In this article, we visualize a framework of the intersectionality of literacy, spatial justice, and multimodality in teaching literacy to Filipino Deaf students. We propose a metaphor-based framework and discuss how it can be used in teaching literacy to Filipino Deaf students through classroom examples as well as suggestions and recommendations for teachers. We do this mainly through redefining the term literacy, allowing students access to different modalities, and restructuring learning spaces. We also explore the relationship between spatial justice and the concept of Deaf Space and how this applies in the "new normal" of online learning due to the pandemic. We also address the issue of how classroom and education structure may inadvertently produce spatial injustice, especially for Deaf students. Implications and additional questions in teaching Filipino Deaf students are also discussed.

Deaf on the Prairie: Combining Traditional and Contemporary Learning Theories.

This single case study concerns an 11-year-old girl, Agata, who recently moved to a rural community in the United States from the Philippines. Agata is profoundly deaf, has had no access to amplification, and has had very limited access to language and formal school. The journey through the next year, including the COVID-19 pandemic, saw Agata's language and literacy skills blossom at an unexpected rate. The study examines the how and why of Agata's progress by using multiple sources of both quantitative and qualitative data. A conceptual framework of both direct instruction theory (Engelmann & Carnine, 1982) and resilience theory (Garmezy, 1993) was used to systematically analyze the factors that contributed to Agata's growth and offer a more thorough understanding of the complex challenges and potential successes in supporting students from Asian communities who are d/Deaf or hard of hearing (d/DHH).

Changes in visually and auditory attended audiovisual speech processing in cochlear implant users: A longitudinal ERP study.

Limited auditory input, whether caused by hearing loss or by electrical stimulation through a cochlear implant (CI), can be compensated by the remaining senses. Specifically for CI users, previous studies reported not only improved visual skills, but also altered cortical processing of unisensory visual and auditory stimuli. However, in multisensory scenarios, it is still unclear how auditory deprivation (before implantation) and electrical hearing experience (after implantation) affect cortical audiovisual speech processing. Here, we present a prospective longitudinal electroencephalography (EEG) study which systematically examined the deprivation- and CI-induced alterations of cortical processing of audiovisual words by comparing event-related potentials (ERPs) in postlingually deafened CI users before and after implantation (five weeks and six months of CI use). A group of matched normal-hearing (NH) listeners served as controls. The participants performed a word-identification task with congruent and incongruent audiovisual words, focusing their attention on either the visual (lip movement) or the auditory speech signal. This allowed us to study the (top-down) attention effect on the (bottom-up) sensory cortical processing of audiovisual speech. When compared to the NH listeners, the CI candidates (before implantation) and the CI users (after implantation) exhibited enhanced lipreading abilities and an altered cortical response at the N1 latency range (90-150 ms) that was characterized by a decreased theta oscillation power (4-8 Hz) and a smaller amplitude in the auditory cortex. After implantation, however, the auditory-cortex response gradually increased and developed a stronger intra-modal connectivity. Nevertheless, task efficiency and activation in the visual cortex was significantly modulated in both groups by focusing attention on the visual as compared to the auditory speech signal, with the NH listeners additionally showing an attention-dependent decrease in beta oscillation power (13-30 Hz). In sum, these results suggest remarkable deprivation effects on audiovisual speech processing in the auditory cortex, which partially reverse after implantation. Although even experienced CI users still show distinct audiovisual speech processing compared to NH listeners, pronounced effects of (top-down) direction of attention on (bottom-up) audiovisual processing can be observed in both groups. However, NH listeners but not CI users appear to show enhanced allocation of cognitive resources in visually as compared to auditory attended audiovisual speech conditions, which supports our behavioural observations of poorer lipreading abilities and reduced visual influence on audition in NH listeners as compared to CI users.

Exploring the relationship between auditory late latency response and language age in children using cochlear implant.

AIM & OBJECTIVES: The study aimed to compare P1 latency and P1-N1 amplitude with receptive and expressive language ages in children using cochlear implant (CI) in one ear and a hearing aid (HA) in non-implanted ear. METHODS: The study included 30 children, consisting of 18 males and 12 females, aged between 48 and 96 months. The age at which the children received CI ranged from 42 to 69 months. A within-subject research design was utilized and participants were selected through purposive sampling. Auditory late latency responses (ALLR) were assessed using the Intelligent hearing system to measure P1 latency and P1-N1 amplitude. The assessment checklist for speech-language skills (ACSLS) was employed to evaluate receptive and expressive language age. Both assessments were conducted after cochlear implantation. RESULTS: A total of 30 children participated in the study, with a mean implant age of 20.03 months (SD: 8.14 months). The mean P1 latency and P1-N1 amplitude was 129.50 ms (SD: 15.05 ms) and 6.93 µV (SD: 2.24 µV) respectively. Correlation analysis revealed no significant association between ALLR measures and receptive or expressive language ages. However, there was significant negative correlation between the P1 latency and implant age (Spearman's rho = -0.371, p = 0.043). CONCLUSIONS: The study suggests that P1 latency which is an indicative of auditory maturation, may not be a reliable marker for predicting language outcomes. It can be concluded that language development is likely to be influenced by other factors beyond auditory maturation alone.

Efhamni: A Deep Learning-Based Saudi Sign Language Recognition Application.

Deaf and hard-of-hearing people mainly communicate using sign language, which is a set of signs made using hand gestures combined with facial expressions to make meaningful and complete sentences. The problem that faces deaf and hard-of-hearing people is the lack of automatic tools that translate sign languages into written or spoken text, which has led to a communication gap between them and their communities. Most state-of-the-art vision-based sign language recognition approaches focus on translating non-Arabic sign languages, with few targeting the Arabic Sign Language (ArSL) and even fewer targeting the Saudi Sign Language (SSL). This paper proposes a mobile application that helps deaf and hard-of-hearing people in Saudi Arabia to communicate efficiently with their communities. The prototype is an Android-based mobile application that applies deep learning techniques to translate isolated SSL to text and audio and includes unique features that are not available in other related applications targeting ArSL. The proposed approach, when evaluated on a comprehensive dataset, has demonstrated its effectiveness by outperforming several state-of-the-art approaches and producing results that are comparable to these approaches. Moreover, testing the prototype on several deaf and hard-of-hearing users, in addition to hearing users, proved its usefulness. In the future, we aim to improve the accuracy of the model and enrich the application with more features.

The Segregation of p.Arg68Ter-CLDN14 Mutation in a Syrian Deaf Family, Phenotypic Variations, and Comparative Analysis with the GJB2 Gene.

Hearing impairment, a rare inherited condition, is notably prevalent in populations with high rates of consanguinity. The most common form observed globally is autosomal recessive non-syndromic hearing loss. Despite its prevalence, this genetic disorder is characterized by a substantial genetic diversity, making diagnosis and screening challenging. The emergence of advanced next-generation sequencing (NGS) technologies has significantly advanced the discovery of genes and variants linked to various conditions, such as hearing loss. In this study, our objective was to identify the specific variant causing hearing loss in a family from Syria using clinical exome sequencing. The proband in the family exhibited profound deafness as shown by pure-tone audiometry results. The analysis of the different variants obtained by NGS revealed the presence of a nonsense mutation within the CLDN14 gene. Through Sanger sequencing, we verified that this variant segregates with the disease and was not present in the control population. Moreover, we conducted a comprehensive review of all reported deafness-related CLDN14 mutations and their associated phenotypes. Furthermore, we endeavored to carry out a comparative analysis between the CLDN14 and GJB2 genes, with the objective of identifying potential factors that could explain the notable discrepancy in mutation frequency between these two genes.

OSBPL2 compound heterozygous variants cause dyschromatosis, ichthyosis, deafness and atopic disease syndrome.

PURPOSE: In this study, we identified and diagnosed a novel inherited condition called Dyschromatosis, Ichthyosis, Deafness, and Atopic Disease (DIDA) syndrome. We present a series of studies to clarify the pathogenic variants and specific mechanism. METHODS: Exome sequencing and Sanger sequencing was conducted in affected and unaffected family members. A variety of human and cell studies were performed to explore the pathogenic process of keratosis. RESULTS: Our finding indicated that DIDA syndrome was caused by compound heterozygous variants in the oxysterol-binding protein-related protein 2 (OSBPL2) gene. Furthermore, our findings revealed a direct interaction between OSBPL2 and Phosphoinositide phospholipase C-beta-3 (PLCB3), a key player in hyperkeratosis. OSBPL2 effectively inhibits the ubiquitylation of PLCB3, thereby stabilizing PLCB3. Conversely, OSBPL2 variants lead to enhanced ubiquitination and subsequent degradation of PLCB3, leading to epidermal hyperkeratosis, characterized by aberrant proliferation and delayed terminal differentiation of keratinocytes. CONCLUSIONS: Our study not only unveiled the association between OSBPL2 variants and the newly identified DIDA syndrome but also shed light on the underlying mechanism.

Novel likely pathogenic variant in the EYA1 gene causing Branchio oto renal syndrome and the exploration of pathogenic mechanisms.

OBJECTIVE: Branchio-oto-renal syndrome (BOR, OMIM#113,650) is a rare autosomal dominant disorder that presents with a variety of symptoms, including hearing loss (sensorineural, conductive, or mixed), structural abnormalities affecting the outer, middle, and inner ear, branchial fistulas or cysts, as well as renal abnormalities.This study aims to identify the pathogenic variants by performing genetic testing on a family with Branchio-oto-renal /Branchio-otic (BO, OMIM#602,588) syndrome using whole-exome sequencing, and to explore possible pathogenic mechanisms. METHODS: The family spans 4 generations and consists of 9 individuals, including 4 affected by the BOR/BO syndrome. Phenotypic information, including ear malformation and branchial cleft, was collected from family members. Audiological, temporal bone imaging, and renal ultrasound examinations were also performed. Whole-exome sequencing was conducted to identify candidate pathogenic variants and explore the underlying molecular etiology of BOR/BO syndrome by minigene experiments. RESULTS: Intra-familial variability was observed in the clinical phenotypes of BOR/BO syndrome in this family. The severity and nature of hearing loss varied in family members, with mixed or sensorineural hearing loss. The proband, in particular, had profound sensorineural hearing loss on the left and moderate conductive hearing loss on the right. Additionally, the proband exhibited developmental delay, and her mother experienced renal failure during pregnancy and terminated the pregnancy prematurely. Genetic testing revealed a novel heterozygous variant NM_000503.6: c.639 + 3 A > C in the EYA1 gene in affected family members. In vitro minigene experiments demonstrated its effect on splicing. According to the American College of Medical Genetics (ACMG) guidelines, this variant was classified as likely pathogenic. CONCLUSION: This study highlights the phenotypic heterogeneity within the same family, reports the occurrence of renal failure and adverse pregnancy outcomes in a female patient at reproductive age with BOR syndrome, and enriches the mutational spectrum of pathogenic variants in the EYA1 gene.

On the Difficulty Predicting Word Recognition Performance After Cochlear Implantation.

HYPOTHESIS: Preimplantation word scores cannot reliably predict postimplantation outcomes. BACKGROUND: To date, there is no model based on preoperative data that can reliably predict the postoperative outcomes of cochlear implantation in the postlingually deafened adult patient. METHODS: In a group of 228 patients who received a cochlear implant between 2002 and 2021, we tested the predictive power of nine variables (age, etiology, sex, laterality of implantation, preimplantation thresholds and word scores, as well as the design, insertion approach, and angular insertion depth of the electrode array) on postimplantation outcomes. Results of multivariable linear regression analyses were then interpreted in light of data obtained from histopathological analyses of human temporal bones. RESULTS: Age and etiology were the only significant predictors of postimplantation outcomes. In agreement with many investigations, preimplantation word scores failed to significantly predict postimplantation outcomes. Analysis of temporal bone histopathology suggests that neuronal survival must fall below 40% before word scores in quiet begin to drop. Scores fall steeply with further neurodegeneration, such that only 20% survival can support acoustically driven word scores of 50%. Because almost all cochlear implant implantees have at least 20% of their spiral ganglion neurons (SGNs) surviving, it is expected that most cochlear implant users on average should improve to at least 50% word recognition score, as we observed, even if their preimplantation score was near zero as a result of widespread hair cell damage and the fact that ~50% of their SGNs have likely lost their peripheral axons. These "disconnected" SGNs would not contribute to acoustic hearing but likely remain electrically excitable. CONCLUSION: The relationship between preimplantation word scores and data describing the survival of SGNs in humans can explain why preimplantation word scores obtained in unaided conditions fail to predict postimplantation outcomes.

The (in)visibility of deafness: Identity, stigma, quality of life and the potential role of totally implantable cochlear implants.

INTRODUCTION: The disclosure of deafness is complex, given the historic and on-going stigma associated with being deaf. The aim of this study was to explore how identity, stigma, and quality of life may be impacted when using cochlear implants (CIs) and totally implantable cochlear implants (TICIs). The physical difference between these two assistive listening devices is significant, given many CI users opt to hide their sound processor behind hair or headwear, in contrast to TICIs (an emerging technology) whereby all components are implanted internally and thus invisible. METHODS: This qualitative study involved semistructured interviews and demographic questionnaires with 12 adult participants with more than 1 year of experience using their CI. Participants were recruited Australia-wide through community organisations that support deaf and hard-of-hearing individuals. Interview transcripts were analysed thematically, with the themes generated through an inductive process, with consensus generated through group working with three members from the research team. RESULTS: Four major themes were identified: (1) CI challenges; (2) The importance of social and support networks; (3) Identity and disclosure and (4) Concerns about TICIs. The underlying finding was centred around the construction of deaf identity. Participant attitudes were generally categorised as 'Loud and proud', with the recognition that displaying the CI was an extension of self, something to be proud of, and a means to normalise deafness; or 'Out of sight and out of mind', which sought to minimise the visibility of deafness. While both identities differed in how deafness is disclosed, they are fundamentally related to the same ideas of self-agency and empowerment. CONCLUSION: TICIs present a novel opportunity-the ability for CI users to control the visibility of their deafness and thus control disclosure. This study explored the impact of stigma and categorised two core identities that CI users construct. Future directions include investigating potential CI candidates, to explore if TICIs may be a facilitator to CI uptake. PATIENT OR PUBLIC CONTRIBUTION: The semistructured interview guide was developed in consultation with adults with CIs. Feedback led to adjustments and improvement to the interview guide. In addition, F. R. has a lived experience with hearing loss, and C. Y. L. is an executive committee member for a nonprofit charity organisation that supports families that are D/deaf and hard-of-hearing.

Electrically evoked auditory brainstem responses in deaf children with cochlear nerve canal stenosis.

BACKGROUND: Deaf children with cochlear nerve canal stenosis (CNCs) are always considered poor candidates for cochlear implantation. OBJECTIVES: To investigate the function of the peripheral auditory pathway in deaf children with CNCs, as revealed by the electrically evoked auditory brainstem response (EABR), and postoperative cochlear implants (CIs) outcomes. MATERIALS AND METHODS: Thirteen children with CNCs and 13 children with no inner ear malformations (IEMs) who received CIs were recruited. The EABR evoked by electrical stimulation from the CI electrode was recorded. Postoperative CI outcomes were assessed using Categories of Auditory Performance (CAP) and Speech Intelligibility Rate (SIR). RESULTS: Compared with children with no IEMs, children with CNCs showed lower EABR extraction rates, higher thresholds, a longer wave V (eV) latency and lower CAP and SIR scores. The auditory and speech performance was positively correlated with the diameter of the cochlear nerve canal and the number of channels showing wave III (eIII) and eV in children with CNCs. CONCLUSIONS AND SIGNIFICANCE: The physiological function of the peripheral auditory pathway in children with CNCs is poorer than that in children with no IEMs. Postoperative auditory and speech abilities may depend on the severity of cochlear nerve malformation and auditory conduction function.

Assessing parent-child interaction with deaf and hard of hearing infants aged 0-3 years: An international multi-professional e-Delphi.

INTRODUCTION: Most deaf babies are born to hearing families who do not yet have the skills to communicate effectively with their child. Adaptations to communication are important because the quality of parent-child interaction (PCI) predicts how a deaf child develops language. Teachers of Deaf children and Speech and Language Therapists support families with communication in the home. Currently, there are no assessments that appraise how a parent interacts with their deaf baby. Previous research has identified which parent behaviours and approaches are used in PCI assessments in research and practice. The current paper forms consensus on the core content and best practices of a new PCI tool for deaf children aged 0-3 years. METHODS: An international sample of expert academics and practitioners (n = 83) were recruited to take part in a two-round modified electronic Delphi study. Participants were presented with 69 statements focusing on (i) which parent behaviours were important in assessment (ii) the methods to be used in PCI assessment. Participants rated the extent to which they agreed or disagreed with each statement on a five-point Likert scale and gave comments to support their response. Consensus was defined as >80% of participants rating the statement as a (4) 'highly important' or a (5) 'essential'. If consensus was not reached, participant comments were used to generate new statements which were rated in the second round. This project involved a patient and public involvement (PPI) group of hearing and deaf parents and professionals to design and guide the study. RESULTS: Consensus was achieved on 52 statements and ranged from 80-99%. A further six statements were additionally included. Within the 58 statements included, 36 were parent behaviours which centred on the parent's observation of, and response to, their child's behaviour and/or language. The remaining 22 statements focused on methods used in the assessment such as parents having their PCI filmed, parents having the opportunity to review the video and assess themselves alongside a professional, and parents being involved in subsequent goal setting. CONCLUSIONS: This e-Delphi presented the parent behaviours and methods of assessment to be included in a new PCI tool for deaf children. Future co-production work and acceptability and feasibility testing are discussed.