An approach designed to fail deaf children and their parents and how to change it.

The matter of raising and educating deaf children has been caught up in percepts of development that are persistently inaccurate and at odds with scientific research. These percepts have negatively impacted the health and quality of life of deaf children and deaf people in general. The all too prevalent advice is to raise the child strictly orally and wait to see what happens. Only when the child is seriously behind is a completely accessible language - a sign language - introduced, and that is far too late for protecting cognitive health. The medical profession, along with others, needs to offer parents better advice and better supports so that neither the children nor their parents wait and watch as the oral-only method fails. All must take responsible action to assure an approach that succeeds.

Crisis interpreting and Deaf community understanding during the COVID-19 pandemic: results from a South Korea-based survey.

This study explores the South Korean Deaf community's response to sign language interpreting during the COVID-19 (coronavirus disease 2019) health crisis, focusing on individual factors affecting the signers' comprehension. The data were collected from a mobile-based questionnaire survey conducted among 401 Deaf adults; binary probit modelling was adopted to analyse the data. The major findings are: (i) 59.9 per cent of the respondents understood less than 70 per cent of the interpreting; (ii) males and urban residents tend to understand better; (iii) younger people (less than 50 years) and signers with a Bachelor's degree or higher are likely to have lower comprehension; and (iv) Deaf adults who visited a doctor after the COVID-19 outbreak tended to have lower comprehension. The findings demonstrate that individual characteristics, including age, impact significantly on the extent to which Deaf individuals understand the sign language interpreting of COVID-19 information, indicating that steps are needed to achieve a Deaf-inclusive society during a health disaster.

Linguistic network in early deaf individuals: A neuroimaging meta-analysis.

This meta-analysis summarizes evidence from 44 neuroimaging experiments and characterizes the general linguistic network in early deaf individuals. Meta-analytic comparisons with hearing individuals found that a specific set of regions (in particular the left inferior frontal gyrus and posterior middle temporal gyrus) participates in supramodal language processing. In addition to previously described modality-specific differences, the present study showed that the left calcarine gyrus and the right caudate were additionally recruited in deaf compared with hearing individuals. In addition, this study showed that the bilateral posterior superior temporal gyrus is shaped by cross-modal plasticity, whereas the left frontotemporal areas are shaped by early language experience. Although an overall left-lateralized pattern for language processing was observed in the early deaf individuals, regional lateralization was altered in the inferior temporal gyrus and anterior temporal lobe. These findings indicate that the core language network functions in a modality-independent manner, and provide a foundation for determining the contributions of sensory and linguistic experiences in shaping the neural bases of language processing.

The time course of Cantonese and Hong Kong Sign Language phonological activation: An ERP study of deaf bimodal bilingual readers of Chinese.

This study investigated Cantonese and Hong Kong Sign Language (HKSL) phonological activation patterns in Hong Kong deaf readers using the ERP technique. Two experiments employing the error disruption paradigm were conducted while recording participants' EEGs. Experiment 1 focused on orthographic and speech-based phonological processing, while Experiment 2 examined sign-phonological processing. ERP analyses focused on the P200 (180-220 ms) and N400 (300-500 ms) components. The results of Experiment 1 showed that hearing readers exhibited both orthographic and phonological effects in the P200 and N400 windows, consistent with previous studies on Chinese reading. In deaf readers, significant speech-based phonological effects were observed in the P200 window, and orthographic effects spanned both the P200 and N400 windows. Comparative analysis between the two groups revealed distinct spatial distributions for orthographic and speech-based phonological ERP effects, which may indicate the engagement of different neural networks during early processing stages. Experiment 2 found evidence of sign-phonological activation in both the P200 and N400 windows among deaf readers, which may reflect the involvement of sign-phonological representations in early lexical access and later semantic integration. Furthermore, exploratory analysis revealed that higher reading fluency in deaf readers correlated with stronger orthographic effects in the P200 window and diminished effects in the N400 window, indicating that efficient orthographic processing during early lexical access is a distinguishing feature of proficient deaf readers.

FOXO-regulated DEAF1 controls muscle regeneration through autophagy.

The commonality between various muscle diseases is the loss of muscle mass, function, and regeneration, which severely restricts mobility and impairs the quality of life. With muscle stem cells (MuSCs) playing a key role in facilitating muscle repair, targeting regulators of muscle regeneration has been shown to be a promising therapeutic approach to repair muscles. However, the underlying molecular mechanisms driving muscle regeneration are complex and poorly understood. Here, we identified a new regulator of muscle regeneration, Deaf1 (Deformed epidermal autoregulatory factor-1) - a transcriptional factor downstream of foxo signaling. We showed that Deaf1 is transcriptionally repressed by FOXOs and that DEAF1 targets to Pik3c3 and Atg16l1 promoter regions and suppresses their expression. Deaf1 depletion therefore induces macroautophagy/autophagy, which in turn blocks MuSC survival and differentiation. In contrast, Deaf1 overexpression inactivates autophagy in MuSCs, leading to increased protein aggregation and cell death. The fact that Deaf1 depletion and its overexpression both lead to defects in muscle regeneration highlights the importance of fine tuning DEAF1-regulated autophagy during muscle regeneration. We further showed that Deaf1 expression is altered in aging and cachectic MuSCs. Manipulation of Deaf1 expression can attenuate muscle atrophy and restore muscle regeneration in aged mice or mice with cachectic cancers. Together, our findings unveil an evolutionarily conserved role for DEAF1 in muscle regeneration, providing insights into the development of new therapeutic strategies against muscle atrophy.Abbreviations: DEAF1: Deformed epidermal autoregulatory factor-1; FOXO: Forkhead box O; MuSC: Muscle Stem Cell; PAX7: Paired box 7; PIK3C3: Phosphatidylinositol 3-kinase catalytic subunit type 3.

The Use of a Behavior Chain Interruption Strategy to Teach Mands for Help with an Adult with Intellectual Disability and Deaf-Blindness.

Individuals with deaf-blindness and co-occurring diagnoses, such as intellectual and developmental disabilities, may experience difficulty with independence, specifically with communication. One behavior-analytic procedure that may be useful for increasing independence and teaching communication to this population is the behavior-chain interruption strategy (BCIS). The current study examined the use of the BCIS to teach a 65-year-old deaf-blind participant with severe intellectual disability to use a SadoTech Elderly Monitoring Pager to notify others in the environment when help was needed. The researcher alternated between establishing operation (EO; help needed, items missing, or inoperable) and abolishing operation (AO; help not needed, items present, and operable) trials for three previously mastered daily living routines. The results demonstrated that following intervention, the participant used the device independently during EO trials and never used it during AO trials across behavior chains, and similar results were obtained during a treatment-extension phase. Limitations and implications for applied practice are discussed. Supplementary Information: The online version contains supplementary material available at 10.1007/s40616-024-00204-8.

Understanding the impacts of the COVID-19 response measures on Deaf adults in Cape Town.

Background: International literature has evidenced that Deaf people have been disadvantaged during the COVID-19 pandemic; however, there is currently little research published within the South African context. Objectives: This study investigated the ways in which the COVID-19 pandemic and its consequent response measures impacted Deaf adults in Cape Town. Method: Using a descriptive approach, semi-structured, qualitative interviews were held with 15 Deaf adults in Cape Town, South Africa. Participants were purposively selected through a local Deaf organisation. Data were analysed using thematic analysis. Results: Data revealed the challenges experienced when accessing information, the impact of communication barriers on daily life, and how the response measures impacted access to healthcare. Conclusion: The findings of this study demonstrate how the needs of the Deaf community were overlooked and their voices disregarded during the planning of the national pandemic response, ultimately having detrimental consequences. Therefore, the authors argue for greater inclusion of Deaf representatives to ensure equal access to information and resources, especially during a crisis. Contribution: This study contributes to the growing body of knowledge on the consequences of the COVID-19 pandemic in the field of disability and insights can inform both future research and interventions to promote equity and inclusion for Deaf people.

Communication breakdown repair skills: Comparison between deaf and hard-of-hearing, and normal-hearing adolescents.

OBJECTIVES: Communication breakdowns and their repair by deaf and hard-of-hearing (DHH) and hearing adolescents were examined in conversation with an unfamiliar communication partner. METHOD: This study compared the number and type of clarification requests and responses to those requests of 16 DHH and 16 normal-hearing adolescents aged 11-16 years, in a 10-minute conversation with an unfamiliar adult. It also analyzed the relationship between speech intelligibility, communication breakdowns, and clarification requests by an unfamiliar adult. the Children's Communication Checklist (CCC) was completed by parents. RESULTS: DHH adolescents demonstrated significantly higher usage of nonverbal clarification requests and verbal and nonverbal responses to clarification requests compared to normal-hearing adolescents in conversations with an unfamiliar adult. Furthermore, the subscale scores of the CCC and the speech intelligibility of DHH adolescents were significantly lower than those of normal-hearing adolescents. There were correlations between speech intelligibility and the speech subscale score of the CCC, as well as correlations between the pragmatic composite score of the CCC, the number of communication breakdowns, and the number of clarification requests by an unfamiliar adult. DISCUSSION: The adolescents with DHH experienced more communication breakdowns in conversation with an unfamiliar adult and the number of clarification requests made by adults was higher.

When It Rains It Pours...: Wernicke Encephalopathy with Sudden Blindness after Bariatric Surgery in a Patient with Congenital Deafness.

BACKGROUND: Malabsorption syndromes are known chronic complications of bariatric surgery. Therefore, it is recommended to take oral supplementation with multivitamins. Wernicke's encephalopathy represents an acute neuropsychiatric syndrome associated with alcoholism or severe malnutrition; sporadic cases of this potential complication related to bariatric surgery are described in the literature. We present a case of Wernicke's encephalopathy due to severe vitamin B1 deficiency after bariatric surgery. CASE REPORT: A 31-year-old woman with deaf-mutism from the age of 3 years old, operated 3 months before with a mini-gastric bypass for severe obesity, was transferred to our unit after accessing the emergency room. In the immediate medical history, there was the sudden and rapid decline in vision, leading to complete loss of vision, marked asthenia, and paresthesia in the four limbs. Considering the previous bariatric surgery, the diagnosis of non-alcoholic Wernicke's syndrome was suspected, for which IV therapy with Vitamin B1 was started at a dosage of 5 vials of 200 mg in 100 cc of saline solution (three times a day for the first 72 hours, subsequently 1 once/day). After 12 hours, there was an improvement in visual acuity, and the symptoms completely resolved within 48 hours. She was discharged with complete resolution of all symptoms after 1 month. CONCLUSION: Initial vision loss without confusion or encephalopathy is one atypical presentation of Wernicke syndrome. Clinical suspicion must be high in case of alcoholism or post-bariatric surgery. Early recognition of atypical symptoms, including vision loss, and timely administration of therapy improves the prognosis of this potentially reversible but time-dependent neurological emergency.

Outcomes of Aural Rehabilitation Provided in Person or by Telehealth Among Deaf/Hard of Hearing Young Children with Cochlear Implants or Hearing Aids.

Background: Cochlear implants and hearing aids may facilitate the development of listening and spoken language (LSL) in deaf/hard of hearing young children, but they require aural rehabilitation therapy-often unavailable outside urban areas-for optimal outcomes. This trial assessed the relative effectiveness of LSL therapy delivered either in person or by interactive video. The hypothesis was that telehealth service delivery would be noninferior to in-person therapy. Methods: Most parents refused randomization of their children to telehealth or in-person conditions; therefore, randomization was impossible. In consultation with the funder (NIDCD), the study design was modified. Parents were allowed to select their preferred study condition, and the study team was blinded to group membership. Forty-two families were in the in-person group and 35 in telehealth (40 and 30, respectively, after attrition). Primary endpoints were total score, auditory comprehension, and expressive communication on the Preschool Language Scale, 5th edition. There were several secondary speech, hearing, and language outcome measures. Assessments occurred at baseline and at follow-up after 6 months of LSL therapy. Results: Propensity scores were used to create two matched groups. At baseline, groups did not differ on PLS-5 scores. Change from baseline to F/U on age-equivalents for all three scores was nearly identical for both groups, although the telehealth group was younger, on average, than the in-person group. Discussion: Telehealth was noninferior to in-person services for all primary endpoints. For secondary outcomes, neither group demonstrated a significant advantage. Magnitudes of estimated group differences were small, suggesting nonsignificant differences not predominantly because of sample size. The telehealth group showed greater improvement on 15/24 of secondary language outcome measures. The findings provide evidence that telehealth is equivalent to in-person care for providing LSL therapy to young children with cochlear implants and hearing aids.

Investigating the crowding effect on letters and symbols in deaf adults.

Reading requires the transformation of a complex array of visual features into sounds and meaning. For deaf signers who experience changes in visual attention and have little or no access to the sounds of the language they read, understanding the visual constraints underlying reading is crucial. This study aims to explore a fundamental aspect of visual perception intertwined with reading: the crowding effect. This effect manifests as the struggle to distinguish a target letter when surrounded by flanker letters. Through a two-alternative forced choice task, we assessed the recognition of letters and symbols presented in isolation or flanked by two or four characters, positioned either to the left or right of fixation. Our findings reveal that while deaf individuals exhibit higher accuracy in processing letters compared to symbols, their performance falls short of that of their hearing counterparts. Interestingly, despite their proficiency with letters, deaf individuals didn't demonstrate quicker letter identification, particularly in the most challenging scenario where letters were flanked by four characters. These outcomes imply the development of a specialized letter processing system among deaf individuals, albeit one that may subtly diverge from that of their hearing counterparts.

Neural underpinnings of sentence reading in deaf, native sign language users.

The goal of this study was to investigate sentence-level reading circuits in deaf native signers, a unique group of deaf people who are immersed in a fully accessible linguistic environment from birth, and hearing readers. Task-based fMRI, functional connectivity and lateralization analyses were conducted. Both groups exhibited overlapping brain activity in the left-hemispheric perisylvian regions in response to a semantic sentence task. We found increased activity in left occipitotemporal and right frontal and temporal regions in deaf readers. Lateralization analyses did not confirm more rightward asymmetry in deaf individuals. Deaf readers exhibited weaker functional connectivity between inferior frontal and middle temporal gyri and enhanced coupling between temporal and insular cortex. In conclusion, despite the shared functional activity within the semantic reading network across both groups, our results suggest greater reliance on cognitive control processes for deaf readers, possibly resulting in greater effort required to perform the task in this group.

Exploring the Interplay of Diabetes, Deaf Patient Reported Outcomes, and Cancer Screening in Deaf and Hard of Hearing Women.

Purpose: Some deaf and hard-of-hearing (DHH) individuals face health information barriers, increasing their risk of diabetes mellitus (DM) and subsequent cancer development. This study examines if health-related quality of life (HRQoL) and deaf patient-reported outcomes (DHH-QoL) mediate the relationship between DM diagnosis and cancer screening adherence among DHH individuals. Patients and Methods: In a cross-sectional study, US DHH adults assigned female at birth answered questions on cervical and breast cancer screenings from the ASL-English bilingual Health Information National Trends Survey (HINTS-ASL) and the PROMIS (Patient Reported Outcome Measurement Information System) Deaf Profile measure's Communication Health and Global Health domains. Odds ratios (OR) and 95% confidence intervals (CI) were obtained from multivariable logistic and linear regression models, examining the association between DM, DHH-QoL, and cancer screening adherence, adjusting for other covariates and HRQoL. A Baron and Kenny causal mediation analysis was used. A two-sided p < 0.05 indicated significance. Results: Most respondents were White (66.4%), heterosexual (66.2%), did not have DM (83.9%), had health insurance (95.5%), and adhered to pap smears (75.7%) and mammograms (76.9%). The average (standard deviation) DHH-QoL score was 50.9 (8.6). Those with DM had lower HRQoL scores (46.2 (9.5) vs 50.2 (8.8); p < 0.0001) than those without. Non-significant multivariable models indicate that those with DM were more adherent to pap testing (OR: 1.48; 95% CI: 0.72, 3.03; p = 0.285) and mammograms (2.18; 95% CI: 0.81, 5.88; p = 0.122), with DHH-QoL scores slightly increasing them to 1.53 (0.74, 3.16; p = 0.250) for pap testing and 2.55 (0.91, 7.13; p = 0.076) for mammograms. DHH-QoL was significantly associated with mammograms (p = 0.027), with 6% increased adherence per unit increase in the score. Overall, HRQoL and DHH-QoL were not significant mediators. Conclusion: While HRQoL/DHH-QoL in DHH individuals with DM does not mediate cancer screening adherence, higher DHH-QoL scores are associated with it. DHH-focused health literacy and communication training can improve cancer-related outcomes.

Neurocognitive outcomes in young adults with cochlear implants: The role of early language access and crossmodal plasticity.

Many children with profound hearing loss have received cochlear implants (CI) to help restore some sense of hearing. There is, however, limited research on long-term neurocognitive outcomes in young adults who have grown up hearing through a CI. This study compared the cognitive outcomes of early-implanted (n = 20) and late-implanted (n = 21) young adult CI users, and typically hearing (TH) controls (n=56), all of whom were enrolled in college. Cognitive fluidity, nonverbal intelligence, and American Sign Language (ASL) comprehension were assessed, revealing no significant differences in cognition and nonverbal intelligence between the early and late-implanted groups. However, there was a difference in ASL comprehension, with the late-implanted group having significantly higher ASL comprehension. Although young adult CI users showed significantly lower scores in a working memory and processing speed task than TH age-matched controls, there were no significant differences in tasks involving executive function shifting, inhibitory control, and episodic memory between young adult CI and young adult TH participants. In an exploratory analysis of a subset of CI participants (n = 17) in whom we were able to examine crossmodal plasticity, we saw greater evidence of crossmodal recruitment from the visual system in late-implanted compared with early-implanted CI young adults. However, cortical visual evoked potential latency biomarkers of crossmodal plasticity were not correlated with cognitive measures or ASL comprehension. The results suggest that in the late-implanted CI users, early access to sign language may have served as a scaffold for appropriate cognitive development, while in the early-implanted group early access to oral language benefited cognitive development. Furthermore, our results suggest that the persistence of crossmodal neuroplasticity into adulthood does not necessarily impact cognitive development. In conclusion, early access to language - spoken or signed - may be important for cognitive development, with no observable effect of crossmodal plasticity on cognitive outcomes.

Awake surgery for a deaf patient using sign language: A case report.

Background: Although awake surgery is the gold standard for resecting brain tumors in eloquent regions, patients with hearing impairment require special consideration during intraoperative tasks. Case Description: We present a case of awake surgery using sign language in a 45-year-old right-handed native male patient with hearing impairment and a neoplastic lesion in the left frontal lobe, pars triangularis (suspected to be a low-grade glioma). The patient primarily communicated through sign language and writing but was able to speak at a sufficiently audible level through childhood training. Although the patient remained asymptomatic, the tumors gradually grew in size. Awake surgery was performed for tumors resection. After the craniotomy, the patient was awake, and brain function mapping was performed using tasks such as counting, picture naming, and reading. A sign language-proficient nurse facilitated communication using sign language and the patient vocally responded. Intraoperative tasks proceeded smoothly without speech arrest or verbal comprehension difficulties during electrical stimulation of the tumor-adjacent areas. Gross total tumor resection was achieved, and the patient exhibited no apparent complications. Pathological examination revealed a World Health Organization grade II oligodendroglioma with an isocitrate dehydrogenase one mutant and 1p 19q codeletion. Conclusion: Since the patient in this case had no dysphonia due to training from childhood, the task was presented in sign language, and the patient responded vocally, which enabled a safe operation. Regarding awake surgery in patients with hearing impairment, safe tumor resection can be achieved by performing intraoperative tasks depending on the degree of hearing impairment and dysphonia.

The visual cortex in the blind but not the auditory cortex in the deaf becomes multiple-demand regions.

The fate of deprived sensory cortices - visual regions in the blind and auditory regions in the deaf - exemplifies the extent to which experience can change brain regions. These regions are frequently seen to activate during tasks involving other sensory modalities, leading many accounts to infer that these regions have started processing sensory information of other modalities. However, such observations can also imply that these regions are now activating to any task event regardless of the sensory modality. Activating to task events, irrespective of the sensory modality involved, is a feature of the multiple-demands (MD) network. These are a common set of regions within the frontal and parietal cortices that activate in response to any kind of control demand. Thus, demands as diverse as attention, perceptual difficulty, rule-switching, updating working memory, inhibiting responses, decision-making, and difficult arithmetic - all activate these same set of regions that are thought to instantiate domain-general cognitive control and underpin fluid intelligence. We investigated if deprived sensory cortices, or foci within them, become part of the MD network. We tested if the same foci within the visual regions of the blind and auditory regions of the deaf activated to different control demands. We found that control demands related to updating auditory working memory, difficult tactile decisions, time-duration judgments, and sensorimotor-speed - all activated the entire bilateral occipital regions in the blind but not in the sighted. These occipital regions in the blind were the only regions outside the canonical fronto-parietal MD regions to show such activation to multiple control demands. Further, compared to the sighted, these occipital regions in the blind had higher functional connectivity with fronto-parietal MD regions. Early deaf, in contrast, did not activate their auditory regions to different control demands, showing that auditory regions do not become MD regions in the deaf. We suggest that visual regions in the blind do not take a new sensory role but become part of the MD network, and this is not a response of all deprived sensory cortices but a feature unique to the visual regions.

Quality of Life of deaf adolescents in high school: A systematic literature review.

There are few studies that have explored the Quality of Life (QoL) for deaf adolescents in high school (13-18 years). Following the PRISMA guidelines, this systematic literature review examined peer-reviewed research that has explored QoL for deaf adolescents in high school by using databases such as Science Citation Index, Scopus and Social Science Citation Index in addition to some related journals such as American Annals of the Deaf, the Journal of Deaf Studies and Deaf Education, Ear and Hearing, and Deafness and Education International spanning 14 years (2010-2024). By analysing the titles, abstracts, and keywords and reading full manuscripts, only seven were deemed appropriate for inclusion in this systematic review. All seven studies used quantitative research. This systematic review found that there is a discrepancy between the studies included in the use of measures. The results of the studies are different and some are contradictory. The QoL concept also differed amongst the studies. This study concluded that there is a great need to conduct more research into the QoL of deaf adolescents in high school with diverse research methods and the use of qualitative or mixed research, as well as expanding the scope of studies to include more dimensions in the concept of QoL.

"They still phone even though they know I'm deaf": exploring experiences of deaf people in health services in Wales, UK.

BACKGROUND: Deaf and hard of hearing people persistently experience barriers accessing health services, largely due to ineffective communication systems, a lack of flexible booking arrangements, and a lack of Deaf awareness training for health professional staff. METHODS: Face to face focus groups were conducted with 66 Deaf and hard of hearing people in Deaf clubs across Wales, UK. Thematic analysis was undertaken. RESULTS: Responses identified from focus groups are reported as barriers faced using health services, improvements that would make a difference, impact of accessibility of health services, and a potential Sign language badge for healthcare staff. CONCLUSIONS: Deaf people report that health professionals lack training on Deaf awareness and do not know how to communicate effectively with Deaf and hard of hearing people. Further research into Deaf awareness and training resources for health professionals are needed to establish what improves Deaf cultural competencies, and ultimately makes healthcare experiences more positive for people who are Deaf.