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.

The Reading of Deaf Arabic Children in Israel.

Studies on the reading acquisition of deaf children investigate the similarities and differences in the reading process between these readers and typical hearing readers. There is no consensus on the nature of the reading process among deaf readers, whether they use the same reading processing strategies as typical readers or depend on other strategies to close the gap. The present study aimed to test the types of strategies used to process written words by deaf Arabic readers with prelingual deafness, compared to their hearing peers, and to test the effectiveness of deaf readers' use of these strategies. Three experimental paradigms were tested. The findings indicated that deaf Arabic readers rely on essentially similar processing strategies to those used by hearing readers. However, deaf Arabic readers employ these strategies with significantly less effectiveness. The results are discussed in light of international data.

How to Improve Grit Among Deaf or Hard of Hearing Students: The Effect of Thinking Style.

The researchers examined the associations between thinking styles and grit. A cross-sectional design was adopted, with two weeks of data collection. The Thinking Styles Inventory-Revised II and the Grit Scale were administered to 365 signing deaf or hard-of-hearing (DHH) Arts and Design students and 443 hearing university students in mainland China. CFA, MANOVA, hierarchical multiple regression analyses, and a multi-group analysis were executed for data analysis. DHH and hearing students with Type I styles (i.e., more creativity-generating, less structured, and cognitively more complex) had higher grit levels, with large effect sizes for the identified relationships. There were no differences in the relations for either group. The associations between thinking styles and grit may protect against psychological pressure and rehabilitation problems and enable university/school administrators, counselors, social workers, teachers, parents, and students to enhance the grit of students who are deaf or hard of hearing.

"Where I Didn't Have to Hear Anything, and I Could Just Run": The Lived Sport Experiences of Deaf or Hard of Hearing Collegiate Athletes.

Accessible and inclusive participation in sport can provide significant physical, psychological, and social benefits to Deaf or hard of hearing (D/HH) athletes. To understand how to facilitate these benefits, the researchers explored the lived physical education and sport experiences of D/HH collegiate athletes. Six athletes representing six sports were recruited and interviewed. Utilizing an interpretative phenomenological analysis approach to guide data collection, analysis, and interpretation, the researchers found five major themes: Self-Advocating for Awareness, Finding Meaningful Conversations, Overcoming Challenges, Seeking Community Connection, and Escaping Through Physical Activity. These themes illustrate the influence of accessibility and inclusion on the participants' sport experiences as well as the impact of the disability awareness of their coaches and peers. D/HH athletes and their coaches and teammates should work to overcome barriers to accessibility and inclusion to ensure the maximum benefit of being on a college sports team.

Mental Health, Parenting Stress, and Parenting Practices of Parents of Deaf or Hard of Hearing Children During the Pandemic.

The authors investigated parent mental health during the COVID-19 pandemic and its association with parenting behaviors of parents of children who are deaf or hard of hearing. An electronic survey was distributed to parents (N = 103). The results showed that they were experiencing elevated anxiety, depression, and symptoms of post-traumatic stress disorder. A combined model demonstrated that parental distress was significantly associated with depression and with parental reports of symptoms indicating significantly higher distress. Parental distress was also significantly associated with parenting strategies: Parents who endorsed positive strategies reported significantly lower levels of distress, while parents who endorsed negative strategies reporting significantly higher levels. It was found that screening protocols to identify parents in need of support are crucial, particularly among the parent population considered in the present study. Additionally, access to mental health services and evidence-based positive parenting programs is essential.

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.

Auditory and Visual Hallucinations in a Congenitally Deaf Patient with Schizophrenia: A Case Report and Brief Literature Review.

This report discusses the case of a 54-year-old woman with a complex psychiatric history including schizophrenia, tardive dyskinesia, borderline intellectual function, and congenital deafness that reported auditory and visual hallucinations during an acute exacerbation of schizophrenia. After resuming a previous lithium regimen and introducing olanzapine, the patient improved and was discharged without hallucinations. In our report we explore some of the challenges we faced, discuss similar cases, and examine the unresolved debate about whether congenitally deaf patients can experience auditory hallucinations.

A novel variant in GAS2 is associated with autosomal dominant nonsyndromic hearing impairment in a Chinese family.

Knockout of GAS2 (growth arrest-specific protein 2), causes disorganization and destabilization of microtubule bundles in supporting cells of the cochlear duct, leading to hearing loss in vivo. However, the molecular mechanism through which GAS2 variant results in hearing loss remains unknown. By Whole-exome sequencing, we identified a novel heterozygous splicing variant in GAS2 (c.616-2 A > G) as the only candidate mutation segregating with late-onset and progressive nonsyndromic hearing loss (NSHL) in a large dominant family. This splicing mutation causes an intron retention and produces a C-terminal truncated protein (named GAS2mu). Mechanistically, the degradation of GAS2mu via the ubiquitin-proteasome pathway is enhanced, and cells expressing GAS2mu exhibit disorganized microtubule bundles. Additionally, GAS2mu further promotes apoptosis by increasing the Bcl-xS/Bcl-xL ratio instead of through the p53-dependent pathway as wild-type GAS2 does, indicating that GAS2mu acts as a toxic molecule to exacerbate apoptosis. Our findings demonstrate that this novel variant of GAS2 promotes its own protein degradation, microtubule disorganization and cellular apoptosis, leading to hearing loss in carriers. This study expands the spectrum of GAS2 variants and elucidates the underlying pathogenic mechanisms, providing a foundation for future investigations of new therapeutic strategies to prevent GAS2-associated progressive hearing loss.

[Analysis of one-year outcomes after cochlear implantation in adults with single-sided deafness].

Objectives: To investigate the outcomes of cochlear implantation in Mandarin-speaking cochlear implant (CI) users with single-sided deafness (SSD). Methods: This study was a single-center prospective cohort study. Eleven Mandarin-speaking adult SSD patients who underwent CI implantation at Capital Medical University Beijing Tongren Hospital from August 2020 to October 2021 were recruited, including 6 males and 5 females, with the age ranging from 24 to 50 years old. In a sound field with 7 loudspeakers distributed at 180°, we measured root-mean-square error(RMSE)in SSD patients at the preoperative, 1-month, 3-month, 6-month, and 12-month after switch-on to assess the improvement of sound source localization. The Mandarin Speech Perception (MSP) was used in the sound field to test the speech reception threshold (SRT) of SSD patients under different signal-to-noise locations in a steady-state noise under conditions of CI off and CI on, to reflect the head shadow effect(SSSDNNH), binaural summation effect(S0N0) and squelch effect(S0NSSD). The Tinnitus Handicap Inventory (THI) and the Visual Analogue Scale (VAS) were used to assess changes in tinnitus severity and tinnitus loudness in SSD patients at each time point. The Speech, Spatial and Qualities of Hearing Scale(SSQ) and the Nijmegen Cochlear Implantation Scale (NCIQ) were used to assess the subjective benefits of spatial speech perception and quality of life in SSD patients after cochlear implantation. SPSS 19.0 software was used for statistical analysis. Results: SSD patients showed a significant improvement in the poorer ear in hearing thresholds with CI-on compared with CI-off; The ability to localize the sound source was significantly improved, with statistically significant differences in RMSE at each follow-up time compared with the preoperative period (P<0.05). In the SSSDNNH condition, which reflects the head shadow effect, the SRT in binaural hearing was significantly improved by 6.5 dB compared with unaided condition, and the difference was statistically significant (t=6.25, P=0.001). However, there was no significant improvement in SRT between the binaural hearing condition and unaided conditions in the S0N0 and S0NSSD conditions (P>0.05). The total score of THI and three dimensions were significant decreased (P<0.05). Tinnitus VAS scores were significantly lower in binaural hearing compared to the unaided condition (P<0.001). The total score of SSQ, and the scores of speech and spatial dimensions were significant improved in binaural hearing compared to the unaided condition (P<0.001). There was no statistical difference in NCIQ questionnaire scores between preoperative and postoperative (P>0.05), and only the self-efficacy subscore showed a significant increase(Z=-2.497,P=0.013). Conclusion: CI could help Mandarin-speaking SSD patients restore binaural hearing to some extent, improve sound localization and speech recognition in noise. In addition, CI in SSD patients could suppress tinnitus, reduce the loudness of tinnitus, and improve subjective perceptions of spatial hearing and quality of life.

Lexical tone recognition in multi-talker babbles and steady-state noise by Mandarin-speaking children with unilateral cochlear implants or bimodal hearing.

BACKGROUND AND OBJECTIVES: Lexical tone presents challenges to cochlear implant (CI) users especially in noise conditions. Bimodal hearing utilizes residual acoustic hearing in the contralateral side and may offer benefits for tone recognition in noise. The purpose of the present study was to evaluate tone recognition in both steady-state noise and multi-talker babbles by the prelingually-deafened, Mandarin-speaking children with unilateral CIs or bimodal hearing. METHODS: Fifty-three prelingually-deafened, Mandarin-speaking children who received CIs participated in this study. Twenty-two of them were unilateral CI users and 31 wore a hearing aid (HA) in the contralateral ear (i.e., bimodal hearing). All subjects were tested for Mandarin tone recognition in quiet and in two types of maskers: speech-spectrum-shaped noise (SSN) and two-talker babbles (TTB) at four signal-to-noise ratios (-6, 0, +6, and +12 dB). RESULTS: While no differences existed in tone recognition in quiet between the two groups, the Bimodal group outperformed the Unilateral CI group under noise conditions. The differences between the two groups were significant at SNRs of 0, +6, and +12 dB in the SSN conditions (all p < 0.05), and at SNRs of +6 and +12 dB of TTB conditions (both p < 0.01), but not significant at other conditions (p > 0.05). The TTB exerted a greater masking effect than the SSN for tone recognition in the Unilateral CI group as well as in the Bimodal group at all SNRs tested (all p < 0.05). Among demographic or audiometric variables, only age at implantation showed a weak but significant correlation with the mean tone recognition performance under the SSN conditions (r = -0.276, p = 0.045). However, when Bonferroni correction was applied to the correlation analysis results, the weak correlation became not significant. CONCLUSION: Prelingually-deafened children with CIs face challenges in tone perception in noisy environments, especially when the noise is fluctuating in amplitude such as the multi-talker babbles. Wearing a HA on the contralateral side when residual hearing permits is beneficial for tone recognition in noise.

Assessment of refractive errors, amblyopia, strabismus, and low vision among hearing-impaired and deaf students in Kermanshah.

BACKGROUND AND AIM: Refractive errors, amblyopia, strabismus, and low vision are more common among children with hearing impairments in comparison with their hearing peers. Neglecting visual disorders can pose educational and social problems for these children. The present study aimed to assess the prevalence of refractive errors, amblyopia, strabismus, and low vision among hearing-impaired and deaf students in Kermanshah. MATERIALS AND METHODS: A total of 79 deaf and hearing impaired students within the age range of 7-20 years (mean age of 15.01 ± 2.72) underwent optometric examinations, including autorefractometry, retinoscopy, ophthalmoscopy, slit lamp, visual acuity measurement, and cover-uncover test. Those who needed further evaluation were referred to the Ophthalmology Clinic of Imam Khomeini Hospital. RESULTS: Regarding the prevalence of refractive errors, 32 (40.5%) subjects had one or a combination of refractive errors, the most common of which was astigmatism (36.7%), followed by amblyopia (15.1%). The most common type of strabismus was latent strabismus (heterophoria) (88.6%), followed by exophoria (81%). Moreover, 3 (3.7%) cases had nystagmus. A significant difference was observed between the prevalence of amblyopia and the degree of hearing loss (P = 0.026), and no significant difference was detected in other cases. CONCLUSION: As evidenced by the obtained results, refractive errors, amblyopia, strabismus, and low vision are more prevalent among deaf and hearing-impaired children compared to normal children because deaf and hearing-impaired children are not able to convey their vision problems and need to compensate for their poor hearing with an enhanced sense of sight, inattention to these disorders can present these children with serious educational and social problems. Therefore, eye screening examinations are of paramount importance in deaf and hearing-impaired children.

Spectrum of genetic variants in 306 patients with non-syndromic hearing loss from Croatia.

AIM: To determine the spectrum and frequency of disease-causing variants in patients with non-syndromic hearing loss (NSHL) and to investigate the diagnostic yield of the applied genetic methods. METHODS: The study enrolled 306 unrelated patients with childhood-onset, mild-to-profound NSHL referred to Children's Hospital Zagreb for genetic testing between March 2006 and October 2023. The GJB2 variants were analyzed with the multiplex ligation-dependent probe amplification method and Sanger sequencing of the coding region of the GJB2 gene. In 21 patients negative for GJB2 biallelic variants, clinical exome sequencing (CES) was performed. RESULTS: Among 234 disease-associated GJB2 alleles detected, 19 were clinically relevant, of which 18 were reported as pathogenic/likely pathogenic. The c.35delG variant accounted for 73.5% of the mutated alleles. More than half of the patients with biallelic GJB2 variants (64/110, 58.2%) were 35delG homozygotes. Seventeen non-GJB2 variants were found in 10 genes (TECTA, NOG, SLC26A4, PCDH15, TMPRSS3, USH2A, GATA3, MYO15A, SOX10, COL2A1) in 11 participants, and 5 variants (in TECTA, NOG, PCDH15, and SOX10) were novel (29.4%). CONCLUSION: We were able to elucidate the genetic cause of hearing loss in 121 patients, with an overall diagnostic rate of 39.5%. The c.35delG was the most common variant. CES allowed us to diagnose almost half of the patients with HL; to distinguish NSHL from the syndromic form of HL in cases where the phenotype was unclear or where symptoms were absent from an early age; and to discover novel variants.

["Nothing about us without us": deafness in history teaching and historiography, 2015-2022]. / "Nada sobre nós, sem nós": a surdez no ensino de história e na produção historiográfica, 2015-2022.

This text presents the partial results of ongoing research into deafness in history teaching and historiography between 2015 and 2022. The study problematizes the place of disabled people in top-ranking periodicals (the top two categories in Brazil) and in pedagogical projects on degree courses in history (with and without teacher-training certification) at the University of São Paulo and the State University of Campinas. These universities were chosen because they topped the ranking in a survey conducted by Folha de S.Paulo newspaper. The study observes how the Brazilian Inclusion Law (law 13.146, of July 6, 2015) is incorporated into the initial training of these professionals.

O texto aponta resultados parciais de uma pesquisa em andamento sobre a surdez no ensino de história e na produção historiográfica entre 2015 e 2022. O trabalho problematiza o lugar da pessoa com deficiência nos periódicos A1 e A2 e nos projetos pedagógicos de cursos de graduação em história (formação de professores e pesquisadores) da Universidade de São Paulo e da Universidade Estadual de Campinas, por conta de serem indicadas como as mais bem posicionadas no ranking de uma pesquisa realizada pela Folha de S.Paulo, levando-se em conta os critérios de articulação entre a Lei Brasileira de Inclusão, lei 13.146, de 6 de julho de 2015, e a formação inicial desses profissionais.

Unraveling the propensity of various genetic disorders and syndromes in the Koraga, an aboriginal tribe from southern India.

Koragas, recognized as a particularly vulnerable tribal group (PVTG) by the Government of India, are from coastal Karnataka and Kerala. They are experiencing severe socioeconomic and health-related issues and rapid depopulation. The unique genetic makeup of Koragas has been maintained by the practice of endogamy. We aimed to identify genetic factors potentially associated with the predisposition of Koragas towards genetic and multifactorial disorders. We employed genome-wise data of 29 Koraga individuals genotyped on the Infinium Global Screening Array-24 v3.0 BeadChip platform and performed various population genetic analyses including kinship, identity by descent (IBD), and runs of homozygosity (RoH). A high degree of haplotype sharing among the Koraga participants may be indicative of a recent founder event. We identified genetic variants and genes associated with several genetic disorders, higher infant mortality rate, neurological disorders, deafness, and lower fertility rate of this agrarian tribe. Ours is the first genome-wide study on the Koraga tribe that identified genetic factors associated with various genetic disorders. Our findings can provide public healthcare providers with essential genetic information that can be useful in augmenting medical and healthcare services and improving the quality of life of Koragas.

Fatal Presentation of Susac Syndrome in a Congenitally Deaf Patient With a Cochlear Implant.

OBJECTIVE: To report a fatal case of Susac syndrome in a congenitally deaf patient with a cochlear implant and a history of migraines, emphasizing the diagnostic challenges in patients with preexisting conditions. PATIENT: A 33-year-old male with congenital hearing loss, a cochlear implant, and chronic migraines who presented with mild subacute auditory disturbance and headaches that later progressed to severe encephalopathy. INTERVENTION: Explantation of a non-magnetic resonance imaging (MRI) compatible cochlear implant followed by MRI, fundoscopy, and the administration of immunosuppressive medications. MAIN OUTCOME MEASURES: Diagnosis was confirmed by characteristic MRI appearance and the presence of a hemi-retinal artery occlusion. RESULTS: After weeks of immunosuppressive treatment, the patient died of a global cerebral ischemic event of unknown origin. CONCLUSIONS: For patients with preexisting sensorineural hearing loss and cochlear implants, Susac syndrome poses a diagnostic challenge. Auditory disturbances in the absence of cochlear implant failure should prompt further evaluation for visual disturbances and encephalopathy. MRI and fundoscopy should be performed to detect other features of the disease.

Impact of Cochlear Implantation on Quality of Life in Chinese Americans.

OBJECTIVES: To investigate the quality of life (QOL) of adult Mandarin-speaking Chinese Americans after cochlear implantation (CI) using the cross-culturally adapted Chinese Cochlear Implant Quality of Life-10 (CIQOL-10) Global. STUDY DESIGN: Cross-sectional. SETTING: Tertiary care neurotology practice in New York City. PATIENTS: Thirty adult Mandarin-speaking Chinese Americans (22.8-89.4 yr, mean 48.9 yr) with prelingual (12) or postlingual (18) deafness who underwent CI between 1995 and 2020. All patients were at least 1 year from CI activation. INTERVENTION: CI. MAIN OUTCOMES MEASURES: Chinese CIQOL-10 Global score. RESULTS: There were no detectable differences in mean Chinese CIQOL-10 Global scores between the prelingual (mean 51.9, SD 11.0) and postlingual (mean 44.0, SD 16.4) cohorts (p = 0.1; 95% CI, -2.3 to 18.1; Hedges' g = 0.5). Comparison of the overall cohort (mean 47.1, SD 14.8) with previously published CIQOL-10 Global scores of English-speaking American CI users (mean 51.5, SD 10.4) demonstrated a significant difference (p = 0.02; 95% CI, 0.4-8.4; Hedges' g = 0.4). For the overall cohort, multivariable analysis demonstrated that combined household income (p = 0.007, ß = 7.4; 95% CI, 0.7-14.0) was positively associated with Chinese CIQOL-10 Global scores. CONCLUSIONS: This study is the first to evaluate QOL after CI in Mandarin-speaking Chinese American adults. The CIQOL-10 Global scores of Mandarin-speaking Chinese Americans CI users are significantly worse than those of English-speaking American CI users. Combined household income may be positively associated with QOL in the Mandarin-speaking Chinese American CI population. More resources are needed to assess outcomes and support rehabilitation in this population.

Speech Perception and Quality-of-Life Outcomes in Mandarin-Speaking Postlingual Deaf Adults With Cochlear Implant: Any Difference With the Intonation Language Users?

OBJECTIVE: To evaluate the effects of related factors on long-term speech perception and quality-of-life (QoL) outcomes in postlingual deaf Mandarin-speaking adult cochlear implant (CI) recipients and to assess any differences between tone language and other intonation language CI adult users. STUDY DESIGN: Retrospective cases review. SETTING: Tertiary referral center. PATIENTS: Forty-five adult CI recipients (48 implanted ears). MAIN OUTCOME MEASURES: Post-CI outcomes were evaluated after follow-ups of more than 24 months using speech perception tests and QoL questionnaires. We analyzed the related factors affecting CI outcomes. RESULTS: A shorter duration of profound deafness was predictive factors for better post-CI speech perception. Earlier implantation and better performance of vowel scores were predictive of better subjective improvements in social and emotional life. Post-CI vowel scores of over 56% indicated promising improvements in QoL. CONCLUSION: Shorter duration of deafness could have better post-CI speech perception. Post-CI vowel scores instead of monosyllable words have the more potential to predict the QoL in Mandarin-speaking adult CI recipients.

Dissociated Representation of Binaural Cues in Single-Sided Deafness: Implications for Cochlear Implantation.

Congenital single-sided deafness (SSD) leads to an aural preference syndrome that is characterized by overrepresentation of the hearing ear in the auditory system. Cochlear implantation (CI) of the deaf ear is an effective treatment for SSD. However, the newly introduced auditory input in congenital SSD often does not reach expectations in late-implanted CI recipients with respect to binaural hearing and speech perception. In a previous study, a reduction of the interaural time difference (ITD) sensitivity has been shown in unilaterally congenitally deaf cats (uCDCs). In the present study, we focused on the interaural level difference (ILD) processing in the primary auditory cortex. The uCDC group was compared with hearing cats (HCs) and bilaterally congenitally deaf cats (CDCs). The ILD representation was reorganized, replacing the preference for the contralateral ear with a preference for the hearing ear, regardless of the cortical hemisphere. In accordance with the previous study, uCDCs were less sensitive to interaural time differences than HCs, resulting in unmodulated ITD responses, thus lacking directional information. Such incongruent ITDs and ILDs cannot be integrated for binaural sound source localization. In normal hearing, the predominant effect of each ear is excitation of the auditory cortex in the contralateral cortical hemisphere and inhibition in the ipsilateral hemisphere. In SSD, however, auditory pathways reorganized such that the hearing ear produced greater excitation in both cortical hemispheres and the deaf ear produced weaker excitation and preserved inhibition in both cortical hemispheres.