High variability phonetic training facilitates perception-to-production transfer in Mandarin-speaking children with cochlear implants: An acoustic investigation.

This study primarily aimed to evaluate the effectiveness of high variability phonetic training (HVPT) for children with cochlear implants (CIs) via the cross-modal transfer of perceptual learning to lexical tone production, a scope that has been largely neglected by previous training research. Sixteen CI participants received a five-session HVPT within a period of three weeks, whereas another 16 CI children were recruited without receiving any formal training. Lexical tone production was assessed with a picture naming task before the provision (pretest) and immediately after (posttest) and ten weeks after (follow-up test) the completion of the training protocol. The production samples were coded and analyzed acoustically. Despite considerable distinctions from the typical baselines of normal-hearing peers, the trained CI children exhibited significant improvements in Mandarin tone production from pretest to posttest in pitch height of T1, pitch slope of T2, and pitch curvature of T3. Moreover, the training-induced acoustic changes in the concave characteristic of the T3 contour was retained ten weeks after training termination. This study represents an initial acoustic investigation on HVPT-induced benefits in lexical tone production for the pediatric CI population, which provides valuable insights into applying this perceptual training technique as a viable tool in clinical practices.

The role of perceptual and word identification spans in reading efficiency: Evidence from hearing and deaf readers.

Theories of reading posit that decisions about "where" and "when" to move the eyes are driven by visual and linguistic factors, extracted from the perceptual span and word identification span, respectively. We tested this hypothesized dissociation by masking, outside of a visible window, either the spaces between the words (to assess the perceptual span, Experiment 1) or the letters within the words (to assess the word identification span, Experiment 2). We also investigated whether deaf readers' previously reported larger reading span was specifically linked to one of these spans. We analyzed reading rate to test overall reading efficiency, as well as average saccade length to test "where" decisions and average fixation duration to test "when" decisions. Both hearing and deaf readers' perceptual spans extended between 10 and 14 characters, and their word identification spans extended to eight characters to the right of fixation. Despite similar sized rightward spans, deaf readers read more efficiently overall and showed a larger increase in reading rate when leftward text was available, suggesting they attend more to leftward information. Neither rightward span was specifically related to where or when decisions for either group. Our results challenge the assumed dissociation between type of reading span and type of saccade decision and indicate that reading efficiency requires access to both perceptual and linguistic information in the parafovea. (PsycInfo Database Record (c) 2024 APA, all rights reserved).

Differences in biological motion perception associated with hearing status and age of signed language exposure.

This study investigates how American Sign Language (ASL) fluency and hearing status influence the perception of biological motion, using three point-light display (PLD) tasks. Prior research indicates that early exposure to ASL among deaf signers results in more rapid and effortless recognition of biological motion than hearing nonsigners, potentially due to the expertise in deciphering complex human movements or possibly due to neuroplasticity in deaf brains. However, it remains uncertain whether this advantage stems from signed language proficiency or the experience of being deaf. To explore this, we designed three PLD tasks involving viewing randomly moving dots, identifying a person from biological motion PLDs, and determining whether right-side up and inverted PLDs depict actions involving a ball. A diverse cohort of participants (N = 224) with varying ASL fluencies and hearing statuses completed the tasks online, providing us with reaction time and accuracy data. Our results demonstrate that earlier ASL exposure is associated with accuracy, especially on complex action identification tasks. Furthermore, we discovered robust evidence for a speed-accuracy trade-off in deaf participants, in which they performed more quickly but less accurately. The speed-accuracy trade-off was evident in the most difficult task, the action identification task. Further analysis of this deaf group revealed that earlier signed language acquisition led to higher accuracy in action identification task. We conclude that age of ASL exposure and hearing status both significantly contribute to variations in biological motion perception, with implications for understanding visual expertise and cognitive processing in both deaf and signing populations. (PsycInfo Database Record (c) 2024 APA, all rights reserved).

Understanding the Health Care Needs of the Deaf Community Through Medical Interpreters.

For approximately 500,000 Deaf people in the United States who use American Sign Language (ASL), accessing quality health care is challenging.1 Deaf people are more likely to have been admitted to a hospital in the past 12 months and have a history of chronic illness (e.g., cardiovascular disease, arthritis, HIV, obesity) compared with those without hearing impairment.2,3 Contributing factors include lack of cultural competency, few appropriately trained ASL interpreters, insufficient clinician education, and low health literacy levels.4 Deaf is defined here as people who are culturally Deaf (values, traditions, and norms5,6) and use ASL as a primary mode of communication.

[Auditory characteristics and disease progression trends of patients with common recessive deafness genes GJB2 and SLC26A4].

Patients who aged≥6 years and had hearing loss due to GJB2 or SLC26A4 mutation from the Chinese Deafness Genome Project between January 2020 and December 2023 were included. A total of 43 patients with GJB2 mutation were detected, including 25 males and 18 females, with a mean age of (20.4±11.4) years. There were 20 patients with SLC26A4 mutation, including 9 males and 11 females, with a mean age of (15.7±9.1) years. The mean hearing threshold of GJB2 and SLC26A4 mutation patients was 54.1 (95%CI: 49.1-59.1) decibel hearing level (dB HL) and 66.1 (95%CI: 58.6-73.6) dB HL, respectively. The hearing threshold of GJB2 and SLC26A4 mutation patients increased at a rate of 0.21 (95%CI:-0.15-0.57) dB HL/year and 2.22 (95%CI: 1.46-2.99) dB HL/year, respectively. The current study indicates that patients with SLC26A4 mutation show a progressive trend of hearing decline with the increase of age.

Exome variant prioritization in a large cohort of hearing-impaired individuals indicates IKZF2 to be associated with non-syndromic hearing loss and guides future research of unsolved cases.

Although more than 140 genes have been associated with non-syndromic hereditary hearing loss (HL), at least half of the cases remain unexplained in medical genetic testing. One reason is that pathogenic variants are located in 'novel' deafness genes. A variant prioritization approach was used to identify novel (candidate) genes for HL. Exome-wide sequencing data were assessed for subjects with presumed hereditary HL that remained unexplained in medical genetic testing by gene-panel analysis. Cases in group AD had presumed autosomal dominantly inherited HL (n = 124), and in group AR, presumed autosomal recessive HL (n = 337). Variants in known and candidate deafness genes were prioritized based on allele frequencies and predicted effects. Selected variants were tested for their co-segregation with HL. Two cases were solved by variants in recently identified deafness genes (ABHD12, TRRAP). Variant prioritization also revealed potentially causative variants in candidate genes associated with recessive and X-linked HL. Importantly, missense variants in IKZF2 were found to co-segregate with dominantly inherited non-syndromic HL in three families. These variants specifically affected Zn2+-coordinating cysteine or histidine residues of the zinc finger motifs 2 and 3 of the encoded protein Helios. This finding indicates a complex genotype-phenotype correlation for IKZF2 defects, as this gene was previously associated with non-syndromic dysfunction of the immune system and ICHAD syndrome, including HL. The designed strategy for variant prioritization revealed that IKZF2 variants can underlie non-syndromic HL. The large number of candidate genes for HL and variants therein stress the importance of inclusion of family members for variant prioritization.

Hearing restoration by gene replacement therapy for a multisite-expressed gene in a mouse model of human DFNB111 deafness.

Gene therapy has made significant progress in the treatment of hereditary hearing loss. However, most research has focused on deafness-related genes that are primarily expressed in hair cells with less attention given to multisite-expressed deafness genes. MPZL2, the second leading cause of mild-to-moderate hereditary deafness, is widely expressed in different inner ear cells. We generated a mouse model with a deletion in the Mpzl2 gene, which displayed moderate and slowly progressive hearing loss, mimicking the phenotype of individuals with DFNB111. We developed a gene replacement therapy system mediated by AAV-ie for efficient transduction in various types of cochlear cells. AAV-ie-Mpzl2 administration significantly lowered the auditory brainstem response and distortion product otoacoustic emission thresholds of Mpzl2-/- mice for at least seven months. AAV-ie-Mpzl2 delivery restored the structural integrity in both outer hair cells and Deiters cells. This study suggests the potential of gene therapy for MPZL2-related deafness and provides a proof of concept for gene therapy targeting other deafness-related genes that are expressed in different cell populations in the cochlea.

Preliminary Validation of Measures of Experienced, Perceived, and Internalized Stigma Among Adults Who Are d/Deaf or Hard of Hearing in the United States and Ghana.

OBJECTIVES: In this article, we examine the psychometric performance of 3 scales measuring experienced, perceived, and internalized d/Deaf or hard of hearing (d/DHH) stigma among adult (18 and older) populations of individuals who are d/DHH, including those who have been d/DHH since before they developed language (lifelong) and those who became d/DHH after they developed language (acquired) in the United States and Ghana. DESIGN: The preliminary validation study took place in the Greater Accra and Eastern regions of Ghana and across the United States. In the United States, all data were collected online via self-administered surveys in English. In Ghana, trained interviewers who are d/DHH and fluent in Ghanaian Sign Language conducted interviews with participants who are lifelong d/DHH using a video survey. Ghanaian participants with acquired d/DHH status were surveyed by trained hearing interviewers. We calculated polychoric correlation matrices between the measures to remove redundant and unrelated items and used exploratory factor analysis to create the final scales. We also tested the association between the factor scores and a simple summing method for calculating the scale. RESULTS: The study sample included people who have been d/DHH since before they developed language (Ghana: n = 171, United States n = 100) and people who became d/DHH after they developed language (Ghana: n = 174, United States: n = 219). The final experienced, perceived, and internalized scales included six, seven, and five items, respectively. All three scales performed well as unidimensional measures across all four samples. Across the four samples, the experienced, perceived, and internalized stigma scales yielded ordinal αs ranging from 0.725 to 0.947, 0.856 to 0.935, and 0.856 to 0.935, respectively. It would be acceptable to operationalize all stigma scales as sum-of-item scores. CONCLUSIONS: The scales performed well and appear to provide a valid means of measuring different types of stigma among diverse groups of people who are d/DHH. Future work should refine and validate these scales in additional contexts.

Development and Preliminary Validation of Stigma Measures for Care Partners of Persons Who Are d/Deaf or Hard of Hearing.

OBJECTIVES: A great deal of literature documents the significant demands, both physical and psychosocial, that care partners experience when providing care to persons with a range of health conditions. There is, however, far less research available on care partners of adult persons who are d/Deaf or hard of hearing (d/DHH). In response to this gap, the authors developed measures of d/DHH stigma among care partners as part of the work of the Lancet Commission on Hearing Loss' Measures, Models, and Stigma Reduction Subgroup. The measures examined in this article are part of a larger set of parallel measures to enable comparison of stigma measurement across groups. DESIGN: The present study describes the preliminary validation of five stigma scales specifically tailored for use to assess the prevalence and effect of stigma on the care partners of adult people who are d/DHH in the United States. Care partners in this context are defined broadly and can include spouses, partners, adult children, siblings, and friends of persons who are d/DHH. The scales describe the care partner's own assessment of how the person who is d/DHH experiences and perceives stigma as well as the care partner's own internalized, experienced, and perceived stigma as a result of their association with a person who is d/DHH. This latter set of three scales describes secondary stigma, or the stigma a person experiences related to their association with a member of a stigmatized group. Measures were developed through a process that included a literature review, Delphi groups with people who became d/DHH after they developed spoken language, cognitive interviews, and a pretest. An online, self-administered preliminary validation survey was conducted with 151 care partners. RESULTS: Results support the internal reliability of each of the five stigma scales (ordinal α's all greater than 0.9) and that each scale is assessing a single factor. CONCLUSIONS: Additional testing is needed to confirm the validity of these measures. After further validation, they can be used to assess the prevalence and effect of stigma on care partners of persons who are d/DHH and to evaluate the success of interventions developed to address stigma and its effects both on the care partner and the person who is d/DHH and receiving the care.

Preliminary Validation of Hearing Device-Related Stigma Measures in Four United States Populations.

OBJECTIVES: Although hearing devices such as cochlear implants and hearing aids often improve communication, many people who are d/Deaf or hard of hearing (d/DHH) choose not to use them. Hearing device-related stigma, or negative societal beliefs about people who use hearing devices, often drives this decision. Although much research has documented the negative effects of hearing device-related stigma, no widely accepted, validated measure to quantify such stigma across populations currently exists. In this article, we describe the preliminary validation of four distinct but related scales measuring hearing device-related stigma in different populations, including people who use hearing devices and those close to them. DESIGN: We preliminarily validated four measures for quantifying hearing device-related stigma in different populations that were previously developed and refined through a literature review, Delphi interviews, cognitive interviews, and a pretest. We preliminarily validated these measures through self-administered online surveys in a convenience sample in the United States. Among participants who use a hearing device and who either (a) self-identified as being d/DHH before they developed language (lifelong; n = 78) or (b) those who self-identified as having acquired a d/DHH identity after they developed language (acquired n = 71), we validated an anticipated hearing device-related stigma scale (d/DHHS-LE-HDA). We validated three scales that measure perceived hearing device-related stigma observed by parents of children who are d/DHH and who use a hearing device (n = 79) (d/DHHS-P-HDPO), care partners of adults who are d/DHH and use a hearing device (n = 108) (d/DHHS-CP-HDPO), and health care providers (n = 203) (d/DHHS-HCP-HDSH). Exploratory factor analysis assessed the reliability of each measure. RESULTS: Each of the four scales loaded onto one factor. Factor loadings for the eight-item scale measuring anticipated hearing device-related stigma among the two populations with lived experience ranged from 0.635 to 0.910, with an ordinal α of 0.93 in the lifelong d/DHH participants and 0.94 among the acquired d/DHH participants. The six-item scale of perceived stigma observed by parents had item loadings from 0.630 to 0.920 (α = 0.91). The nine-item scale of hearing device-related stigma observed by care partners had item loadings from 0.554 to 0.922 (α = 0.95). The eight-item scale of hearing device-related stigma reported by health care providers had item loadings from 0.647 to 0.941 (α = 0.89). CONCLUSIONS: Preliminary validation results show that the four stigma measures perform well in their respective populations. The anticipated stigma scale performed similarly well for both lifelong d/DHH and acquired d/DHH, which suggests that it could perform well in different contexts. Future research should further validate the scales described here as well as measure hearing device-related stigma in different populations-including people who live in different geographic regions and people using different kinds of hearing devices-and evaluate the success of interventions developed to reduce hearing device-related stigma.

Research Agenda and Applications for Preliminarily Validated Measures of d/Deaf and Hard of Hearing Stigma.

In this special supplement of Ear and Hearing, we have presented preliminarily validated measures for stigma related to being d/Deaf or hard of hearing (d/DHH) in the United States and Ghana. In this concluding article, we suggest avenues for the future refinement and use of these measures. First, the measures should be further validated. Second, they should be used to assess the current state of d/DHH stigma and the importance of different kinds of stigma in different populations, which should in turn drive the development of interventions to reduce d/DHH stigma. Third, these measures can assist in evaluating the effectiveness and cost-effectiveness of those interventions. The evidence from this work can then inform investment cases and cost-of-condition studies, which will support advocacy efforts and policy development for reducing stigma and improving the lives of people who are d/DHH.

Preliminary Validation of Stigma Measures Among Parents of Children Who Are d/Deaf or Hard of Hearing in the United States and Ghana.

OBJECTIVES: Parents are integral to the development and overall well-being of their child. Previous research has studied the emotional effects parenting experiences have on parents. However, parents caring for children with disabilities have unique parenting experiences, filled with both victories and challenges. Parenting a child with disabilities can bring additional responsibilities as parents respond to their child's special needs. Specifically, parents of children who are d/Deaf or hard of hearing (d/DHH) are required to make ongoing life-changing decisions about their child's life, including mode of communication, medical care, and education. Across the world, many adults who are d/DHH experience stigma. However, less is known about the stigma faced by children who are d/DHH and their parents. Measuring the nature and magnitude of stigma-affecting parents of children who are d/DHH could offer insights into how to additionally support these parents. Nonetheless, there is a gap in validated scales to measure stigma among parents of children who are d/DHH. In response, we developed and preliminarily validated five measures of stigma among parents of children who are d/DHH. DESIGN: Measures were developed through a mixed-method process: (1) a scoping literature review, (2) a modified Delphi process consisting of two group discussions (n = 3, n = 4) and two individual discussions with parents of children who are d/DHH from high-income countries (HICs) and low- and middle-income countries (LMICs), (3) cognitive interviews with parents of children who are d/DHH in the United States (U.S.) (n = 5) and Ghana (n = 5), and (4) a pretest of the survey in the U.S. (n = 28) and Ghana (n = 30). Modifications to the measures were made after each stage. This article focuses on evaluating the psychometric performance of the developed measures. Parents were recruited in the U.S. (n = 100) and Ghana (n = 173). Convenience sampling was used in both countries. In Ghana, survey administration was in-person with trained interviewers collecting data on tablets. In the U.S. data were collected online through self-administered surveys. RESULTS: The final five scales measured: (1) parental observation of stigma their child experiences (seven items), (2) parental perceptions of stigma toward their child (eight items), (3) parental secondary experienced stigma (eight items), (4) perceived parental secondary stigma (five items), and (5) parental internalized stigma (seven items). All scales performed strongly and similarly across both country samples. The scales had ordinal αs ranging from 0.864 to 0.960, indicating strong reliability. CONCLUSIONS: This study provides a set of preliminarily validated stigma measures to capture the experience of parents of children who are d/DHH. Measuring stigma among parents is critical to understanding parental mental health, as parental well-being affects the health and development of their child. Furthermore, measuring observed stigma by parents can allow the researcher to gain an understanding of the stigma experienced by children who are d/DHH that they may not be able to communicate. Further studies testing these measures across other countries and with more diverse samples are needed.

Auditory areas are recruited for naturalistic visual meaning in early deaf people.

Congenital deafness enhances responses of auditory cortices to non-auditory tasks, yet the nature of the reorganization is not well understood. Here, naturalistic stimuli are used to induce neural synchrony across early deaf and hearing individuals. Participants watch a silent animated film in an intact version and three versions with gradually distorted meaning. Differences between groups are observed in higher-order auditory cortices in all stimuli, with no statistically significant effects in the primary auditory cortex. Comparison between levels of scrambling revealed a heterogeneity of function in secondary auditory areas. Both hemispheres show greater synchrony in the deaf than in the hearing participants for the intact movie and high-level variants. However, only the right hemisphere shows an increased inter-subject synchrony in the deaf people for the low-level movie variants. An event segmentation validates these results: the dynamics of the right secondary auditory cortex in the deaf people consist of shorter-length events with more transitions than the left. Our results reveal how deaf individuals use their auditory cortex to process visual meaning.

Professional Issues in the Interpreted Education of Deaf and Hard of Hearing Students.

Through in-depth interviews, this qualitative study explored the perceptions of seven teachers of students who are d/Deaf and hard of hearing (D/HH) regarding inclusive and interpreted education. The findings revealed central themes of concern including inadequate support in inclusive placements, communication modality mismatches, and insufficient quality of interpreted education. The teachers' experiences underscore the need for more comprehensive educational support systems and the development of robust support mechanisms to effectively navigate the challenges of inclusive placements for students who are D/HH. Specifically, further attention must be given to improving the qualifications, training, support, and supervision of educational interpreters facilitating education in inclusive settings. These findings have implications for multiple stakeholders, including policymakers, educators, and supportive personnel involved in shaping inclusive education practices to ensure equitable access and the creation of supportive spaces where all students can thrive.

Effectiveness of Group-Play Training on Emotion Recognition for Young Children Who Are Deaf or Hard of Hearing.

Deaf and hard of hearing (DHH) children are developmentally delayed in facial emotion recognition (FER). This study aimed to explore the possibility of enhancing the FER ability of DHH preschoolers through a group-play intervention. Nineteen children with a cochlear implant or hearing aid were enrolled in a 4-week intervention; six DHH children were assigned to a control group. The training program included a learning procedure for four basic emotions: happiness, sadness, anger, and fear. A pretest/posttest design was used to measure the DHH children's FER performance. The results indicated that although the two groups performed comparably on the FER task before the training (pretest), the performance of the intervention group was significantly better than that of the control group in the posttest. Moreover, the DHH children in the intervention group showed the greatest improvement in FER of happiness.

Development of Measures for d/Deaf and Hard of Hearing Stigma: Introduction to the Special Supplement on Stigma Measurement Tools.

People who are d/Deaf or hard of hearing (d/DHH) often experience stigma and discrimination in their daily lives. Qualitative research describing their lived experiences has provided useful, in-depth insights into the pervasiveness of stigma. Quantitative measures could facilitate further investigation of the scope of this phenomenon. Thus, under the auspices of the Lancet Commission on Hearing Loss, we developed and preliminarily validated survey measures of different types of stigma related to d/Deafness and hearing loss in the United States (a high-income country) and Ghana (a lower-middle income country). In this introductory article, we first present working definitions of the different types of stigma; an overview of what is known about stigma in the context of hearing loss; and the motivation underlying the development of measures that capture different types of stigma from the perspectives of different key groups. We then describe the mixed-methods exploratory sequential approach used to develop the stigma measures for several key groups: people who are d/DHH, parents of children who are d/DHH, care partners of people who are d/DHH, healthcare providers, and the general population. The subsequent manuscripts in this special supplement of Ear and Hearing describe the psychometric validation of the various stigma scales developed using these methods.

Medical Home Care for Children with Deafness and Hearing Problems.

BACKGROUND AND OBJECTIVES: Since 2001, the American Academy of Pediatrics (AAP) has advocated for improving the effectiveness of early hearing screening, diagnosis, and intervention (EHDI) and reducing hearing health disparities through the medical home model of care (MHMOC). However, the scope of the MHMOC and its components and potential disparities among US children with deafness or hearing problems (DHH) have not been identified. METHODS: Data from the 2016 to 2020 National Survey of Children's Health (N = 174 551) were analyzed, including 12 121 children with DHH, 171 523 children without DHH, and 38 722 with other special health needs (SHCN). Univariable statistics and multivariable logistic regression were used to analyze the association of hearing problems with medical home composite and components and cohort disparities. RESULTS: After adjusting for sociodemographic characteristics, medical home care was less likely for children with DHH (adjusted odds ratio: 0.74; 95% confidence interval: 0.56-0.97) compared with children without DHH. Children with DHH and SHCN were also less likely to receive medical home care compared with other SHCN children (adjusted odds ratio: 0.71; 95% confidence interval: 0.55-0.92). Specific medical home components significantly less prevalent among children with DHH included family-centered care, ease of referrals, and effective care coordination, whereas a usual source for sick care was more prevalent, although not statistically significant. CONCLUSIONS: Despite over two decades of efforts to improve EHDI through the medical home, rates of the MHMOC for children with DHH remain disparately low. Systems-level quality improvement efforts supporting the medical home will be necessary to allow the MHMOC to support early hearing detection and intervention (EHDI) systems.