Hearing Families Research Articles

A recessive Mendelian model to predict carrier probabilities of DFNB1 for nonsyndromic deafness

Mutations in the DFNB1 locus, where two connexin genes are located (GJB2 and GJB6), account for half of congenital cases of nonsyndromic autosomal recessive deafness. Because of the high frequency of DFNB1 gene mutations and the availability of genetic diagnostic tests involving these genes, they are the best candidates to develop a risk prediction model of being hearing impaired. People undergoing genetic counseling are normally interested in knowing the probability of having a hearing impaired child given his/her family history. To address this, a Mendelian model that predicts the probability of being a carrier of DFNB1 mutations, using family history of deafness, has been developed. This probability will be useful as additional information to decide whether or not a genetic test should be performed. This model incorporates Mendelian mode of inheritance, the age of onset of the disease, and the current age of hearing family members. The carrier probabilities are obtained using Bayes' theorem, in which mutation prevalence is used as the prior distribution. We have validated our model by using information from 305 families affected with congenital or progressive nonsyndromic deafness, in which genetic analysis of GJB2 and GJB6 had already been performed. This model works well, especially in homozygous carriers, showing a high discriminative power. This indicates that our proposed model can be useful in the context of clinical counseling of autosomal recessive disorders.

Evidence from the Wider Use of the BSL Receptive Skills Test

Following the development and standardization of the British Sign Language (BSL) Receptive Skills Test (Herman et al., 1999), the test was made widely available to professionals working with deaf children. Test users were asked to return completed score-sheets on individual children they had tested in order to compare a selection of children from the wider population of deaf children with those from the sample upon whom the test was standardized. The analysis of almost 200 score sheets is presented. Overall, children from the wider population achieved lower standard scores than those from the standardization sample, with the exception of native signers, whose scores were equivalent to the native signers' scores in the original sample. The findings raise important questions about the adequacy of BSL provision for deaf children in hearing families. Data on tester ratings and children's reading scores provide an opportunity for a preliminary investigation of the psychometric properties of the test. Finally, tester feedback on the test itself, the training offered and the overall contribution of the test to assessing deaf children's BSL development are reviewed. Copyright © 2006 John Wiley & Sons, Ltd.

Evidence from the wider use of the BSL Receptive Skills Test

AbstractFollowing the development and standardization of the British Sign Language (BSL) Receptive Skills Test (Herman et al., 1999), the test was made widely available to professionals working with deaf children. Test users were asked to return completed score-sheets on individual children they had tested in order to compare a selection of children from the wider population of deaf children with those from the sample upon whom the test was standardized. The analysis of almost 200 score sheets is presented. Overall, children from the wider population achieved lower standard scores than those from the standardization sample, with the exception of native signers, whose scores were equivalent to the native signers' scores in the original sample. The findings raise important questions about the adequacy of BSL provision for deaf children in hearing families. Data on tester ratings and children's reading scores provide an opportunity for a preliminary investigation of the psychometric properties of the test. F...

Grammatical Subjects in home sign: Abstract linguistic structure in adult primary gesture systems without linguistic input.

Language ordinarily emerges in young children as a consequence of both linguistic experience (for example, exposure to a spoken or signed language) and innate abilities (for example, the ability to acquire certain types of language patterns). One way to discern which aspects of language acquisition are controlled by experience and which arise from innate factors is to remove or manipulate linguistic input. However, experimental manipulations that involve depriving a child of language input are impossible. The present work examines the communication systems resulting from natural situations of language deprivation and thus explores the inherent tendency of humans to build communication systems of particular kinds, without any conventional linguistic input. We examined the gesture systems that three isolated deaf Nicaraguans (ages 14-23 years) have developed for use with their hearing families. These deaf individuals have had no contact with any conventional language, spoken or signed. To communicate with their families, they have each developed a gestural communication system within the home called "home sign." Our analysis focused on whether these systems show evidence of the grammatical category of Subject. Subjects are widely considered to be universal to human languages. Using specially designed elicitation tasks, we show that home signers also demonstrate the universal characteristics of Subjects in their gesture productions, despite the fact that their communicative systems have developed without exposure to a conventional language. These findings indicate that abstract linguistic structure, particularly the grammatical category of Subject, can emerge in the gestural modality without linguistic input.

Technologies of Language and the Embodied History of the Deaf

Looking back, it appears that linguistics was made possible by the invention of writing. Looking ahead, it appears that a science of language and communication, both optic and acoustic, will be enabled . . . not by refinements in rotational systems, but by increasing sophistication in techniques of recording, analyzing, and manipulating visible and auditory events electronically. (Armstrong, Stokoe, and Wilcox 1995, 13-14) MUCH OF the of deaf people has been written by hearing people, and the that exists is, in large part, a of the institutions and concepts created by the hearing for and about the deaf.1 This situation is not unlike that of other minorities who have been kept at the margin of and whose stories have been told by others and from the point of view of others. Anthropologists and historians have made efforts to correct this distortion through the recording of the stories of marginalized groups, who traditionally have had access neither to socially prestigious forms of expression nor to their supporting technologies of production, dissemination, and preservation. For groups without writing, efforts have also been made to teach them both a script for writing their language and the computer technologies necessary to physically produce and circulate their own stories.2 Deaf people, however, differ from other groups because of their unique experience of language acquisition. The linguistic situation of people who are deaf has two major consequences that make the telling of their stories a particular challenge for historians. The first is the intrinsic linguistic diversity of the deaf community, and the second is the specific nature of the language that, for many deaf people, is their identifying achievement: sign language. Because of the nature of deafness and the variety of circumstances that may impinge on the language development of a deaf person, many linguistic outcomes are possible, from exclusive use of the spoken language of the hearing community to the exclusive use of the sign language of the deaf community, and-in still far too many cases-to minimal language use, in which no language becomes fully developed. Only a very few of those who are born deaf or who become deaf at an early age achieve fluency in the spoken language of the hearing community and then only with great effort. For deaf people, the only route to full language mastery is through a sign language, but access to sign language is not always guaranteed because the majority of those born deaf are born into hearing families. On the other hand, the possibility of achieving fluency and expressiveness in sign language (but not language) is what, above all else, motivates the formation of deaf communities. Within these communities, deaf individuals develop a sense of self as a whole person, different from the image they confront in the hearing world and even within their own families, where they are commonly seen, with rare exceptions, as deficient (Padden 1989).3 For the historian, it is significant that the sign languages of deaf communities have no historically developed writing system. This means that sign languages represent a form of primary orality, in the terminology of Ong (1982,16), that is, an little influenced by writing. In addition, the fact that the of the deaf is signed, and not oral, complicates its relation to a possible written form and presents a challenge to the usual techniques and practices of recording, documentation, and elaboration of history. In this article I intend first to discuss briefly the linguistic situation of the deaf; I then discuss the shift in linguistic ideology from graphocentrism to orocentrism, which forms the scenario in which deaf people are struggling to legitimize their natural form of expression, sign language; I then question both graphocentrism and orocentrism and propose neutral terms and a neutral perspective from which one can view orality and oral history as the embodiment of language and experience. …

BILINGUALISM AND IDENTITY IN DEAF COMMUNITIES. Melanie Metzger (Ed.). Washington, DC: Gallaudet University Press, 2000. Pp. xii + 317. $55.00 cloth.

In this volume of the Sociolinguistics in Deaf Communities series, Metzger has edited 11 diverse topics addressing two themes: the perception of Deaf people and Deaf communities, and bilingualism. Deaf people's perception of themselves and their community is explored by authors who discuss an excellent array of topics, ranging from “miracle cures” for Deaf children in Mexico to the nature of name signs in the New Zealand Deaf community; from the linguistic rights of Deaf people in the European Union to a search for the roots of the Nicaraguan Deaf community; from a semiotic analysis of Argentine Sign Language to an analysis of how a Deaf child (American Sign Language) and his hearing family (English) make sense of each other's world views.

Inner lives of deaf children: interviews and analysis

By conducting interviews with seven deaf children, ages seven to ten, Martha Sheridan offers a fresh look at their private thoughts and feelings in this watershed book. Each child possesses a unique cultural background, and Sheridan communicated with each child in his or her preferred method of communication. Her procedure remained consistent with each: in addition to standard questions, Sheridan asked each child to draw a picture based on his or her life, then tell a story about it. Next, she showed them magazine pictures and asked them to describe what they saw. The results proved to be as varied as they were engaging. Angie, an adopted deaf girl who communicates in Signed English, expressed a desire to attend a hearing college when she grows up while also stating she hoped her own children will be deaf. Joe, an African-American, hard of hearing boy, drew pictures of deaf people who are teased in public school, reflecting his own difficult experiences. Sheridan calls upon her tenure as a social worker as well as her own experience as a deaf child growing up in a hearing family in analyzing her study's results. She writes, These children have strengths, they have positive experiences, and they enjoy positive relationships. Inner Lives of Deaf Children will prove to be an enlightening read for parents and scholars alike.

Moving beyond a Deficit Perspective with Qualitative Research Methods

Four researchers argue the merits of qualitative methodology and its particular relevance to those in special education who seek to move beyond a deficit perspective. Their studies examine the experiences of a young deaf child in a hearing family, Portuguese-speaking parents of special needs students with severe disabilities, a class in basic reading-writing in an urban university, and fifth- and sixth-grade students in a literature discussion group. Unconstrained by the defined variables and decontextualized settings characteristic of much research in special education, qualitative methods allowed the authors to extend the scope of their studies beyond originally stated research questions when unanticipated findings emerged from descriptive data. In doing so, each researcher discovered unexpected strengths and abilities in her participants.

"A Good Future for Deaf Children": A Five-Year Sign Language Intervention Project

Deaf preschoolers and hearing family members learned sign language in a 5-year intervention project. Once weekly, each child met with a teacher who was deaf. Parents, siblings, and other relatives met about once monthly to study sign language, and all families in the project signed together about twice yearly. The present study addressed four questions asked of parents about the project: (a) How did the children learn to sign? (b) Did both the parents and the children benefit from the project? (c) What was the position of sign language in the family? (d) Did the project have some impact on the family's social network? The families indicated satisfaction with the project; they learned to sign and their social networks expanded. Parents favored bilingual education: Sign language was the main language but learning Finnish was also important. Learning sign language was not easy, especially for the fathers. The families that were most actively involved in the lessons learned the most.

Year 2000 Position Statement: Principles and Guidelines for Early Hearing Detection and Intervention Programs.

Year 2000 Position Statement: Principles and Guidelines for Early Hearing Detection and Intervention Programs.

Insights into Theory of Mind from Deafness and Autism

This paper summarizes the results of 11 separate studies of deaf children’s performance on standard tests of false belief understanding, the results of which combine to show that deaf children from hearing families are likely to be delayed in acquiring a theory of mind. Indeed, these children generally perform no better than autistic individuals of similar mental age. Conversational and neurological explanations for deficits in mental state understanding are considered in relation to recent evidence from studies of deaf, autistic, and normally developing children with varied levels of access to talk about mental states at home with family members during the preschool years.

Representing Inner Worlds: Theory of Mind in Autistic, Deaf, and Normal Hearing Children

The purpose of the study reported here was to examine the degree to which delays or deficits in developing a theory of mind are specific to children with autism or extend to other groups of atypical children with varying conversational experience and awareness. The performance of deaf children from a variety of conversational backgrounds was compared with that of autistic and normal hearing children on a range of tasks requiring representation of others' mental states. Native signers, oral deaf children, and normal hearing children scored similarly, and their performance exceeded that shown by signing deaf children from hearing families and children with autism. The latter two groups did not differ significantly from each other. These results point to an interplay among biology, conversation, and culture in the development of a theory of mind.

Practitioner Review: The Assessment and Treatment of Deaf Children with Psychiatric Disorders

The assessment and treatment of deaf children with psychiatric disorder is intimately related to the individual child's communication, which in turn is affected by a number of factors, medical, social, and cultural. The deafness can be aetiologically related to the psychiatric disorder or can be incidental. Treatment strategies should be adapted to meet the individual child and family's needs. Deaf professionals have a vital role in mental health services for this population. The use of an interpreter can clarify communication and cultural issues for deaf and hearing children, families, and professionals.

The book reading habits of teacher and families to their hearing and hearing impaired kindergarten children

SUMMARY The study was carried out to investigate the book reading habits of families and teachers to hearing impaired and normal hearing kindergarten children. The sample of research was taken from 2 kindergartens. Eleven kindergarten teachers and 36 hearing impaired children and families formed one of the groups. The other group consisted of 11 kindergarten teachers and 40 normal hearing children and families. The questionnaire applied to teachers and families included questions about book choosing and reading habits. The results of this research shows that there is no significant difference in the book reading habits of the 2 groups to their children. But the hearing-impaired children families are more careful in book choosing and reading to their children.

Changing focus on the representational mind: Deaf, autistic and normal children's concepts of false photos, false drawings and false beliefs

We report two experiments designed to explore further the parallels noted previously between deaf and autistic children's mastery of a theory of mind and to compare their emerging concepts of false belief with their understanding of false representation in the photographic and artistic domains. In Expt 1, standard false‐belief and false‐photograph tasks were given to 21 high functioning autistic children, 35 normal preschoolers and 30 signing deaf children from hearing families. The children with deafness performed similarly to those with autism. The vast majority of both groups passed the false‐photograph test, but less than half displayed a correspondingly accurate understanding of false belief. Normal 4‐year‐olds did equally well on photo and belief tasks, significantly surpassing deaf and autistic children's performance on the latter. However, in contrast to some previous research, the normal 3‐year‐olds also found the false‐photograph task easier than the false‐belief task in Expt 1. Experiment 2 was therefore designed to provide a more tightly controlled comparison of the relative difficulty of concepts of false representation in three modalities— photographic, mental and pictorial—using identical procedures in each modality so as to equate the tasks' conceptual, syntactic and pragmatic demands. A total of 24 deaf children, 21 autistic children and 47 normal preschoolers participated in Expt 2. The results replicated those of Expt 1 by showing no difference between deaf and autistic children, both of whom displayed a poorer understanding of false beliefs than of false photographs. But the normal 3‐year‐olds in Expt 2 did no better on the photo than the belief tasks, suggesting procedural factors as an explanation for this age group's Expt 1 performance. The absence of significant differences among the diagnostic groups on the false‐drawing task highlighted the need for further research into the development of representational concepts in the artistic domain. Consideration was given to how impoverished early conversational experience and selective deprivation of talk about intangible mental states might influence deaf and autistic children's development of a theory of mind.

Characteristics of hearing families and their young hearing-impaired children: Early intervention through preschool

Characteristics of hearing families and their young hearing-impaired children: Early intervention through preschool

How Parents Support Home Literacy with Deaf Children

Six case studies of young deaf children from deaf and hearing families are presented. A model home literacy program at the Indiana School for the Deaf is described. Case studies and the home literacy program describe activities which link sign language and finger spelling to early reading and writing at home. Activities include the daily reading of whole storybooks, explaining environmental print, labeling drawings with letters and words, writing lists and notes to family members and playing and experimenting with writing.

Images of Difference: Deaf and Hearing in the United States

Deaf people and hearing people in the United States pose unusually difficult referencing problems for each other despite the fact that most deaf people are born into hearing families. Because the deaf and the hearing do not readily or easily share a language, communication issues are at the center of a larger social debate about whether the deaf are a separate culture, and whether they have a medical disability. This article looks at threads of history that have shaped this cultural debate, including the American egalitarianism that has led to the creation and standardization of separate categories of people such as the Deaf.

The development of visual attention to signed language in deaf children from deaf and hearing families

The development of visual attention to signed language in deaf children from deaf and hearing families

Deaf Life on Isolated Japanese Islands

Thirty-eight deaf persons who had little experience of school were interviewed, as were their hearing families, relatives, and neighbors, in a field survey on some islands of Okinawa, Japan. Findings reported here cover (a) social and language environment during childhood, (b) frequency of contact with other deaf and hearing people, (c) communication methods used, and (d) their past and present social lives. Typical were the use of an indigenous gestural system shared with hearing people that enabled them to be integrated into the local hearing community, and their limited contact with the Deaf community (graduates of the deaf schools who use Japanese Sign Language). Implications of the findings are discussed as they bear on origin and development of Deaf communities and sign languages.