Deaf Families Research Articles

Applying relational autonomy in US special education: Educators’ role in supporting parents’ decisions for deaf or hard-of-hearing children

ABSTRACT The concept of relational autonomy is widely applied in bioethics research to elucidate how patients’ abilities in agentively making medical decisions are constrained within a complex structural system and set of relations; thus, rather than being individualistic in nature, autonomy is relationally and interdependently situated. While this perspective is thought-provoking, it is seldom explored in settings outside of healthcare. An equally critical site of decision-making processes is found in special education, where parents of children with disabilities must contend with information from both teachers and physicians to make choices that best support their child’s learning and development. Families of deaf or hard-of-hearing (D/HH) children in particular are uniquely positioned to encounter decisions that intersect educational, medical, and sociocultural levels – that is, in the earliest years of a D/HH child’s life, parents must make decisions about the child’s medical care and whether they will receive hearing technology or treatments, which in turn shapes the inherently cultural decision of linguistic choice, which in turn shapes educational decisions about the type of schooling environment for the child. This study broadly asks: In the field of special education, how do parents make decisions for their D/HH child? Data is drawn from semi-structured, ethnographic interviews with 16 educators of the deaf or hard-of-hearing (EODs) across public, private, and state levels in California. IMPACT STATEMENT The findings can help contribute academic research into conversation with social justice-oriented work to improve language and educational access for D/HH children.

A Case Study of Suspected Childhood Apraxia of Sign.

We provide a case report of "Zoe," a 4-year-old deaf child from a deaf signing family, who presented with a possible case of Childhood Apraxia of Sign (CASign). The description is based on reports from the child's speech-language pathologist, her Individualized Education Program report, and a clinician-created sign imitation task. Zoe's sign articulation errors in American Sign Language differed from those reported for typically developing deaf children and were parallel to the types of errors observed for hearing children with childhood apraxia of speech. Specifically, Zoe produced inconsistent errors across signs, substituted more complex handshapes for simple handshapes, made errors on both unmarked (common) and marked (less common) forms, produced articulatory distortions (rather than substitutions), and exhibited "groping" behaviors (a sequence of attempts to move her hands into the correct position). In addition, Zoe sometimes self-corrected her errors by manipulating her own hands, for example, using her left hand to move the thumb of her right hand into the correct position. Zoe's pattern of sign errors is consistent with an underlying deficit in motor planning and/or programming and may constitute the first reported case of CASign.

Unveiling a novel GJB2 dominant K22T mutation in a Chinese family with hearing loss.

Hearing loss constitutes one of the most prevalent conditions within the field of otolaryngology. Recent investigations have revealed that mutations in deafness-associated genes, including point mutations and variations in DNA sequences, can cause hearing impairments. With the ethology of deafness remaining unclear for a substantial portion of the affected population, further screenings for pathogenic mutations are imperative to unveil the underlying mechanisms. On this study, by using next-generation sequencing, we examine 129 commonly implicated deafness-related genes in a Chinese family with hearing loss, revealing a novel heterozygous dominant mutation in the GJB2 gene (GJB2: c.65T>G: p. Lys22Thr). This mutation consistently occurs in affected family members but is not detected in unaffected individuals, strongly suggesting its causative role in hearing loss. Structural analysis indicates potential disruption to the Cx26 gap junction channel's hydrogen bond and electrostatic interactions, aligning with predictions from the PolyPhen and SIFT algorithms. In conclusion, our study provides conclusive evidence that the identified heterozygous GJB2 mutation (GJB2: c.65T>G: p. Lys22Thr), specifically the K22T alteration, is the primary determinant of the family's deafness. This contribution enhances our understanding of the interplay between common deafness-associated genes and hearing loss, offering valuable insights for diagnostic guidance and the formulation of therapeutic strategies for this condition.

Australian families of deaf and hard of hearing children: Are they using sign?

BackgroundDeaf and hard of hearing (DHH) children may experience communication delays, irrespective of early intervention and technology. Australian Sign Language (Auslan) is one approach in early intervention to address language delays. Current prevalence of Auslan use among Australian families with DHH children is unknown. AimsThe first aim was to determine the proportion of families enrolled in an Australian statewide hearing loss databank who use Auslan with their DHH child. The second aim was to explore the relationships between indicators of child hearing loss (bilateral or unilateral hearing loss, degree of hearing loss, and device use: hearing aids and cochlear implants), family factors (maternal education, attendance at early intervention, family history of deafness, and socio-economic disadvantage) and the family's reported use of Auslan. MethodsWe analysed the enrolment data from 997 families who participated in an Australian statewide hearing loss databank between 2012 and 2021. We described the proportion of families who used Auslan with their DHH child at home. The association between indicators of child hearing loss and family factors, and the parental reports of communication approach were examined using correlation analyses. ResultsEighty-seven of 997 parents (8.7%) reported using Auslan with their DHH child. Of these, 26 (2.6%) used Auslan as their primary language. The use of Auslan at home was associated with the following indicators of child hearing loss: bilateral hearing loss, profound compared to mild hearing loss, and cochlear implant and hearing aid use compared to no device use. The family factors associated with the use of Auslan were: referral or attendance at early intervention compared to those who did not attend, and a family history of deafness compared to those with none. No association was found between maternal education and socio-economic disadvantage and the use of Auslan. ConclusionThis Australian study found a low proportion (8.7%) of families with a DHH child who reported using Auslan. Seven child hearing loss and family factors were considered, and five were significantly associated with using Auslan at home. Children with a greater degree of hearing loss, attendance at early intervention and family history of deafness tended to use Auslan.

Sian Heder’s CODA and the Struggle for Artistic Freedom

This study analyzed the relationship between the main character’s artistic freedom and her complex family responsibilities as reflected in Sian Heder’s CODA. The writers focused on analyzing how Ruby, a young musician from a deaf family, handles her inner conflict between gaining her creative ambition and her social expectations and familial responsibilities. The writers analyzed how Ruby resolves the tension she faces and makes decisions and resolutions to pursue her artistic ambition. This study was expected to deepen our understanding of the complexities of balancing creative expression, family, and self-discovery. The writers got the main data from Sian Heder’s CODA, while the supporting data were derived from English literature journals, e-books, and other sources on the internet. The result shows that Ruby manages to handle her inner conflict, make decisions and resolutions to pursue her dream, and make her family believe that what she decides will bring betterment for her and her family.

A helping hand

In this article, I examine responses to the taking of food items during snack time in an early childhood education classroom with deaf toddlers (18 months to three years old) who are native signers of American Sign Language (ASL). These children have grown up with exposure to ASL from deaf family members and are attending a classroom where all individuals use ASL. Through the combined analysis of ethnographic and interactional data, I argue that the teachers’ corporeal socialization of the deaf toddlers into a visual orientation leads to their development of a social and moral understanding of ownership rights within the classroom which is displayed in the children’s social awareness, social responsiveness and self-reliance in responding to food takings.

OBSTACLES TO RECEIVING MEDICAL AND DENTAL CARE IN PATIENTS WITH HEARING LOSS (LITERATURE REVIEW) BARRIERS TO ACCESSING MEDICAL AND DENTAL CARE AMONG INDIVIDUALS WITH HEARING LOSS

The article presents current problems that arise in the communication between hearing- impaired patients and doctors, in particular, dentists.
 Every year, the number of hearing-impaired people in the world is increasing. In Ukraine, there is no accurate statistical information about people with hearing loss.
 Hearing-impaired patients, trying to access medical and dental hearing care, face many obstacles related to the language barrier. This review of the literature highlights the main reasons and obstacles that arise for people with hearing impairments on the way to receiving medical and dental care and medical professionals in providing such care. This article aims to make medical professionals more aware of the features and needs of deaf patients, which must be taken into account when providing medical and dental care to patients with hearing impairments.
 The quality of medical care depends on the interaction between the patient and the doctor. Deaf people mostly use sign language to communicate, which is why they face significant barriers when trying to get medical, especially dental, services.
 The health of the oral cavity of children and adults with hearing impairment is a rather sensitive issue that few have investigated. People with hearing impairments are more likely to need dental care, but rarely seek it because of communication barriers. Training doctors, particularly dentists, to work with deaf patients and families who communicate through sign language opens up many opportunities to improve communication during appointments and provide quality medical care.

Patterns of identity development in ethnic minority/multicultural deaf families

Although the existence of internal ethnic diversity within Deaf communities is recognized by several authors, there has been less analysis of the impact of the ethnicity of marriage partners on the development and transmission of identity within Deaf families. This paper addresses language use and crossgenerational identity transmission in ethnic minority or ethnically mixed families set up by Deaf spouses living in Oradea (Romania). The research conducted in the Deaf community of Oradea is based on a survey of ethnic Hungarian Deaf community members (including ethnic Romanian spouses), lifepath interviews with selected Deaf individuals, and family case studies. The research results denote that in the case of Deaf people living in ethnically homogenous marriages who have graduated from a special school in Hungarian language, the transmission of Hungarian identity, knowledge of Hungarian sign language/oral language tends to be a natural, undisturbed process. However, if one of the spouses is of Romanian ethnicity, the language of communication within the family will probably be Romanian sign language/oral language. On the other hand, in families where both minority and majority ethnic family members, Deaf and hearing, are present, complex, multifaceted patterns of linguistic communication may operate, favoring specific multicultural ways of developing and transmitting identity.

Linguistic identity in multigenerational ethnic minority/ethnically heterogeneous deaf families.

This paper discusses language use and identity patterns in ethnic minority/ethnically heterogeneous multigenerational deaf families in Romania, where at least one of the family members belongs to the ethnic Hungarian minority. Early childhood and school linguistic socialization, language use within the family, and cross-generational transmission of identity to children are explored. The research is based on five ethnic minority/ethnically heterogeneous multigenerational deaf family case studies. The research results show that within ethnic-national minority/ethnically heterogeneous multigenerational deaf families, complex identity patterns may develop and multifaceted linguistic communication models may prevail, including the use of national oral languages and national sign languages of the parties involved. At the same time, a kind of special linguistic and cultural enrichment, a striving for cultural-linguistic balance, may occur.

Enablers and barriers for hearing parents with deaf children: Experiences of parents and workers in Wales, UK.

More than 90% of deaf children are born to hearing families who know little about deafness. Benefits from hearing screening at birth are often lost, as families find little information about pathways for deaf children but are key to ensuring deaf children receive relevant language and communication support. Systems surrounding deaf children and family members are crucial for children's health and social development. Experiences of hearing parents raising deaf children and understanding factors that influence families'experience of navigating pathways for deaf children through health and education servicesare currently underreported. An exploratory study was conducted in Wales, UK. Twenty participants were interviewed, including 10 hearing parents of deaf children and 10 people who work with deaf children using semistructured interviews. Bronfenbrenner's ecological systems theory was used as a lens to explore the micro-, meso-, exo-, macro- and chronosystems that surround children and families. This study explores potential supports and barriers in those systems. Findings are reported under two broad headings: enablers and barriers. Under enablers, it was found that provision of resources, supporting people and knowledge were key factors. Under barriers, a lack of knowledge, lack of provision and battling services and attitudes were key issues that need addressing. Hearing parents of deaf children in Wales, UK reported experiencing a range of enablers and barriers that impact upon their experiences of raising a deaf child. Further provision is needed by policymakers and governments to recognise support needs to improve the outcomes for deaf children. This project was developed from initial discussions with the stakeholder reference groupand progressed with the group's deaf paneland hearing parents with deaf children. The project's steering group was involved in study design, recruitment and continuous feedback on all stages of the research process.

Retrospective assessment of the impact of an education specialist on the care of children with hearing loss

ObjectiveTo assess the impact of an education specialist in a multidisciplinary pediatric hearing loss clinic. Study designRetrospective review and cross-sectional survey. SettingSingle tertiary care center. MethodsConsultations held between an education specialist and families of pediatric deaf or hard of hearing (DHH) children within a two-year period were reviewed. Reasons for referral and services provided to each patient and family who subsequently worked with the educational specialist were assessed. Parents of patients who had previously worked with the education specialist were invited to complete a survey evaluating their experience. Results102 patients were referred to the educational specialist in a two-year period. Most common reasons for referral included need for special education plans to accommodate their hearing deficit (32) or family request to support for revisions to such plans (37). 14 patient families completed our survey. 76.9 % of respondents confirmed that the education specialist recommended resources they had not been introduced to before. Given a scale of 1 (“completely dissatisfied”) and 10 being “completely satisfied,” the average rating of the 14 respondents was 9.0. ConclusionThe role of an education specialist in a pediatric hearing loss clinic is to optimize patient and family access to resources that could benefit their DHH child's academic development over time. Future studies should prospectively investigate the impact of education specialist services on the educational progress of DHH patients compared to outcomes without these supports.

Demographics in the formation of language communities and in the emergence of languages: The early years of ASL in New England

How may the structure of a new linguistic community shape language emergence and change? The 1817 founding of the US’s first enduring school for the deaf, the American School for the Deaf (ASD) in Hartford, Connecticut, heralded profound changes in the lives of deaf North Americans. We report the demographics of the early signing community at ASD through quantitative analyses of the 1,700 students who attended the school during its first fifty years. The majority were adolescents, with adults also well represented. Prior to 1845, children under age eight were absent. We consider two groups of students who may have made important linguistic contributions to this early signing community: students with deaf relatives and students from Martha’s Vineyard. We conclude that adolescents played a crucial role in forming the New England signing community. Young children may have pushed the emergence of ASL, but likely did so at home in deaf families, not at ASD.*

Please Pass the Translanguaging: The Dinner Table Experience in the Lives of Newcomer Canadian Deaf Youth and Their Families

While translanguaging occurs in the homes of deaf people and their hearing family members who do not sign or possess limited signing skills, in this article we argue that translanguaging alone does not explain the complex, domestic-sphere language experiences of three young, newcomer artists in Saskatchewan, Canada. We frame our inquiry around the “dinner table experience” phenomenon, wherein deaf family members receive partial or little access to conversational exchanges. At the dinner table, which is both a literal setting and a metaphor for exclusion experienced by deaf people in audiocentric cultures, many deaf family members report feeling loved yet disconnected. However, translanguaging serves to expand linguistic repertoires among hearing and deaf interlocutors amidst the dinner table experience. We draw from three interviews with deaf youth who describe the dinner table experience through both dialogue and art making, including descriptions of ways in which communication is facilitated or not facilitated, thereby highlighting available and unavailable translanguaging practices in the domestic sphere. The interview data suggest that the dinner table experience is a significant setting for translanguaging, and that promoting accessible and equitable translanguaging practices in the home remains a significant challenge, especially when combined with newcomer lived experience that does not always match current descriptions of translanguaging. We posit that translanguaging is a necessary practice among hearing and deaf persons at the table that can and should be expanded to consider the intersectional experiences of communicators in this literal and metaphorical setting.

Single-cell whole-genome sequencing, haplotype analysis in prenatal diagnosis of monogenic diseases.

Monogenic inherited diseases are common causes of congenital disabilities, leading to severe economic and mental burdens on affected families. In our previous study, we demonstrated the validity of cell-based noninvasive prenatal testing (cbNIPT) in prenatal diagnosis by single-cell targeted sequencing. The present research further explored the feasibility of single-cell whole-genome sequencing (WGS) and haplotype analysis of various monogenic diseases with cbNIPT. Four families were recruited: one with inherited deafness, one with hemophilia, one with large vestibular aqueduct syndrome (LVAS), and one with no disease. Circulating trophoblast cells (cTBs) were obtained from maternal blood and analyzed by single-cell 15X WGS. Haplotype analysis showed that CFC178 (deafness family), CFC616 (hemophilia family), and CFC111 (LVAS family) inherited haplotypes from paternal and/or maternal pathogenic loci. Amniotic fluid or fetal villi samples from the deafness and hemophilia families confirmed these results. WGS performed better than targeted sequencing in genome coverage, allele dropout (ADO), and false-positive (FP) ratios. Our findings suggest that cbNIPT by WGS and haplotype analysis have great potential for use in prenatally diagnosing various monogenic diseases.

Writing Development and Translanguaging in Signing Bilingual Deaf Children of Deaf Parents

Translanguaging allows for a holistic lens on the natural language practices of multilinguals and the strategies they use to communicate and to make meaning, such as writing. Multilinguals have a single, integrated linguistic repertoire and reasoning in which all languages are naturally activated in the brain throughout the writing process. Some deaf children are raised in environments where signed language is first acquired and then used to support the development of a written language. The current literature on translanguaging is scant in capturing signing bilingual deaf writers’ translanguaging practices. This study took a closer look at the written texts produced by three deaf siblings within an American Sign Language/English bilingual deaf family across the course of their childhood between three and ten years old. Their applications of linguistic features in writing over time were qualitatively identified, described, and analyzed. This study’s findings provided rich descriptions and examples of the ways signing bilingual deaf children leverage their whole linguistic repertoire to express ideas in print. Because translanguaging practices were modeled at home and school during natural interactions and direct instruction, these siblings’ language and writing skills continued to flourish over time.

Translanguaging Practices of a Multiethnic and Multilingual Deaf Family in a Raciolinguistic World and Beyond

In this autoethnography, I recount the translanguaging practices of my multiethnic and multigenerational signing deaf family in Manila, Philippines. I examine the impact of a multilingual upbringing on how family members function in various milieus, particularly in education. I discuss how language use throughout my childhood has impacted my experiences with languaging after immigrating to the United States as an adult. Interspersed in this personal narrative are traipses into historical and sociological observations about the Filipino community’s view of the deaf identity and how deaf Filipinos have been and are still being regarded. Finally, I explore the promulgation and implementation of the language policies in my motherland and assimilation efforts of immigrant parents on the translanguaging practices of the Filipino deaf here in the United States. I describe the ways in which home discourse practices affect the educational experiences of deaf Filipino immigrants in the U.S.

Molecular etiology study of hearing loss in 13 Chinese Han families.

Hearing loss affecting about 2/1000 newborns is the most common congenital disease. Genetic defects caused approximately 70% of patients who have non-syndromic hearing loss. We recruited 13 Chinese Han deafness families who tested negative for GJB2, SLC26A4, and mitochondrial 12S rRNA. The probands of each family were performed whole-exome sequencing (WES) or targeted next-generation sequencing (NGS) for known deafness genes to study for pathogenic causes. We found four novel mutations of CDH23, one novel mutation of MYO15A, one novel mutation of TMC1, one novel mutation of PAX3, and one novel mutation of ADGRV1, one novel CNV of ADGRV1, and one novel CNV of STRC. Hearing loss is a highly hereditary and heterogeneous disease. The results in the limited samples of this study show that Usher and Waardenburg syndrome-related genes account for a major proportion are strongly associated with Chinese Han hearing loss patients negative for GJB2, SLC26A4, and mitochondrial 12S rRNA, followed by STRC resulting in mild to moderate deafness.

Manajemen Pendidikan pada Keluarga Anak Tuli di Desa Bandung Sruni, Alian, Kebumen

Deaf is a person who has lost his ability to hear due to the malfunction of part or all of his hearing apparatus. Educating deaf children is different from educating normal children. The purpose of the study was to analyze the education management of families of deaf children in Bandungsruni Village, Alian, Kebumen. The type of research used is qualitative research using the ethnographic method of communication. The implementation of research activities lasted for 1 month, namely in March 2022. The data collection techniques used observation, interviews, and documentation techniques. The data analysis technique uses the Miles and Huberman model. The results of the analysis show that deaf families in Bandungsruni Village, Alian in terms of education given by parents to their children are very large. The education management function in the family has also been running well starting from planning, implementation, and evaluation. The parenting pattern in the family adheres to a democratic parenting pattern that has a good impact on the development of deaf children.

A paediatric English linguistics intervention on spoken language for English-speaking parents of deaf children having hearing problems

Background: This research aimed to find internet information about hearing loss, hearing technology, and spoken language acquisition for Pakistani English-speaking families of deaf or hard-of-hearing children (DHH). Methods: The existence or lack of parent education material on various websites was investigated using an exploratory, descriptive methodology. The research looked at the many Internet resources available. Results: Many websites with information for parents of DHH children acquiring spoken communication were found to be international. Each website was examined in fifteen different content categories. These websites featured English content. According to the present research findings, internet resources for Pakistani English-speaking parents with DHH students struggling with spoken dialect are typically less and less and not deeply involved. Conclusion: A Paediatric English Linguistics Mediation on Spoken Language for Parents of Deaf Children with Hearing Problems in Pakistani English internet alternatives for Pakistani English-speaking parents of DHH children who are having difficulty with spoken dialect are becoming fewer and more uninvolved.

Sociolinguistic Variation in Mouthings in British Sign Language: A Corpus-Based Study.

Mouth activity forms a key component of all sign languages. This can be divided into mouthings, which originate from words in the ambient spoken language, and mouth gestures, which do not. This study examines the relationship between the distribution of mouthings co-occurring with verb signs in British Sign Language (BSL) and various linguistic and social factors, using the BSL Corpus. We find considerable variation between participants and a lack of homogeneity in mouth actions with particular signs. This accords with previous theories that mouthings constitute code-blending between spoken and signed languages-similar to code-switching or code-mixing in spoken languages-rather than being a phonologically or lexically compulsory part of the sign. We also find a strong association between production of plain verbs (which are body-anchored and cannot be modified spatially) and increased mouthing. In addition, we observe significant effects of region (signers from the south of the United Kingdom mouth more than those from the north), gender (women mouth more than men), and age (signers aged 16-35 years produce fewer mouthings than older participants). We find no significant effect of language background (deaf vs. hearing family). Based on these findings, we argue that the multimodal, multilingual, and simultaneous nature of code-blending in sign languages fits well within the paradigm of translanguaging. We discuss implications of this for concepts of translanguaging, code-switching, code-mixing, and related phenomena, highlighting the need to consider not just modality and linguistic codes but also sequential versus simultaneous patterning.