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PubMed2001

Gilles de la Tourette syndrome in a child with congenital deafness.

We present the case of a 10-year-old boy, Sam, with congenital deafness and Gilles de la Tourette Syndrome (GTS). GTS is characterised by multiple motor tics and one or more vocal tics that wax and wane. Due to his deafness Sam never developed vocal language but instead used sign language from the age of four. His tic disorder rapidly accelerated from the age of seven over a six-month period and soon sign language was incorporated into tics as complex "vocal" tics. Bursting out "words" in sign language would also occur in front of people unfamiliar with sign language and often with an obscene content although this was not evident to someone not trained in sign language. To our knowledge this is the first reported case of a congenital deaf child with GTS. The case presented here supports previously published work that the intentional share of the tics in GTS is very small. This case also questions former theories on which regions and circuits of the brain are involved in GTS.

Anti-Dyskinesia Agents↗
PubMed1984

Family therapy with deaf persons: the systemic utilization of an interpreter.

This paper discusses the theory and practice of providing family therapy to families in which there are hearing parents and at least one Deaf child, particularly regarding the optimal utilization of an interpreter. The therapist must be knowledgeable about the psychosocial effects of deafness, the cultural aspects of deafness, and preferably be able to use American Sign Language and Signed English. The therapeutic benefit of utilizing an interpreter extends far beyond simply facilitating communication between each family member whose primary-language is either spoken English or Sign Language. The presence of an interpreter helps the therapist to modify family rules that deny the implications of deafness and prohibit the use of Sign Language, to modify the balance of power in the family, and to encourage participants to exhibit the ego defense mechanisms of projection and transference. The family therapist can utilize those subtle yet profound influences to therapeutic advantage.

Adaptation, Psychological↗
PubMed1989

Language, modality and the brain.

Studies of the signed languages of deaf people have shown that fully expressive languages can arise, outside of the mainstream of spoken languages, that exhibit the complexities of linguistic organization found in all spoken languages. Thus, the human capacity for language is not linked to some privileged cognitive-auditory connection. However, the formal properties of languages (spoken or signed) appear to be highly conditioned by the modalities involved in their perception and production. Multi-layering of linguistic elements and the use of space in the service of syntax appear to be modality-determined aspects of signed languages. Analyses of patterns of breakdown of signed languages provide new perspectives on the nature of cerebral organization for language. The studies reviewed in this article show that the left cerebral hemisphere in man is specialized for signed as well as spoken languages, and thus may have an innate predisposition for language, independent of language modality.

Adult↗
PubMedDate not supplied

Signed and spoken language: a unique underlying system?

Sign language has only recently become a topic of investigation in cognitive neuroscience and psycholinguistics. In this paper, we review research from these two fields; in particular, we compare spoken and signed language by looking at data concerning either cortical representations or early acquisition. As to cognitive neuroscience, we show that clinical neuropsychological data regarding sign language is partially inconsistent with imaging data. Indeed, whereas both clinical neuropsychology and imagery show the involvement of the left hemisphere in sign language processing, only the latter highlights the importance of the right hemisphere. We discuss several possible interpretations of these contrasting findings. As to psycholinguistics, we survey research on the earliest stages of the acquisition of spoken language, and consider these stages in the acquisition of sign language. We conjecture that under favorable circumstances, deaf children exploit sign input to gain entry into the language system with the same facility as hearing children do with spoken input. More data, however, are needed in order to gain a fuller understanding of the relation of different kinds of natural languages to both the underlying anatomical representations and their early acquisition.

Brain↗
PubMed1998

Interactions of language and memory in deaf children and adults.

This article reviews theoretical and empirical issues concerning the relations of language and memory in deaf children and adults. An integration of previous studies, together with the presentation of new findings, suggests that there is an intimate relation between spoken language and memory. Either spoken language or sign language can serve as a natural mode of communication for young children (deaf or hearing), leading to normal language, social, and cognitive development. Nevertheless, variation in spoken language abilities can be shown to have a direct impact on memory span. Although the ways in which memory span can effect other cognitive processes and academic achievement are not considered in depth here, several variables that can have direct impact on the language-memory interaction are considered. These findings have clear implications for the education of deaf children.

Adult↗
PubMed2000

Preventive attitudes and beliefs of deaf and hard-of-hearing individuals.

OBJECTIVE: To investigate the unique health care issues of deaf and hard-of-hearing (D&HH) persons by studying their attitudes, beliefs, and behaviors toward preventive medicine. DESIGN: A self-administered, cross-sectional survey, written in a format comprehensible to persons whose primary language is American Sign Language. POPULATION: One hundred forty D&HH persons recruited from southeastern Michigan, Chicago, Ill, and Rochester, NY, and 76 hearing subjects from southeastern Michigan and Rochester. RESULTS: No significant differences existed between D&HH or hearing persons from different states. However, numerous differences existed between D&HH and hearing persons. Deaf and hard-of-hearing persons were less likely to report receiving preventive information from physicians or the media, and more likely to report receiving it from a Deaf club. They rated the following physician-initiated procedures as less important than hearing persons: discussion of alcohol consumption, smoking, depression, and diet, plus screening for hypertension, hearing loss, and cancer. Deaf and hard-of-hearing persons often considered a preventive procedure important if it was reported performed at their last health maintenance examination. They were less likely to report being asked about alcohol consumption and smoking, or to having been examined for hypertension, cancer, height, and weight. They were more likely to report receiving a hearing examination, mammogram, and Papanicolaou smear. Deaf and hard-of-hearing persons were less likely to report believing that smoking less, exercising regularly, maintaining ideal weight, and regular physical examinations improve health. Differences existed within the D&HH cohort depending on the respondent's preferred language (oral English vs American Sign Language); our sample size was too small for a complete assessment of these differences. CONCLUSIONS: Deaf and hard-of-hearing persons appear to have unique knowledge, attitudes, and behaviors regarding preventive medicine, and their attitudes are influenced by their personal experiences with physicians. Preventive practices addressed during health visits may differ between D&HH and hearing patients. Further research is needed to clarify the reasons for these differences, including within D&HH subgroups, and to develop effective mechanisms to improve the health care of all D&HH persons.

Adult↗
PubMed2001

Visual contrast sensitivity in deaf versus hearing populations: exploring the perceptual consequences of auditory deprivation and experience with a visual language.

Early deafness in humans provides a unique opportunity to examine the perceptual consequences of altered sensory experience. In particular, visual perception in the deaf may be altered as a result of their auditory deprivation and/or because the deaf rely heavily upon a visual language (American Sign Language, or ASL, in the US). Recently, we found that deaf, but not hearing, subjects exhibit a right visual field/left hemisphere advantage on a low-level direction of motion task, a finding that has been attributed to the deaf's experience with ASL [Psychol. Sci. 10 (1999) 256; Brain Res. 405 (1987) 268]. In order to determine whether this visual field asymmetry generalizes to other low-level visual functions, in this study we measured contrast sensitivity in deaf and hearing subjects to moving stimuli over a range of speeds (0.125-64 degrees /s). We hypothesized that if ASL use drives differences between hearing and deaf subjects, such differences may occur over a restricted range of speeds most commonly found in ASL. In addition, we tested a third group, hearing native signers who learned ASL early from their deaf parents, to further assess whether potential differences between groups results from ASL use. These experiments reveal no overall differences in contrast sensitivity, nor differences in visual field asymmetries, across subject groups at any speed tested. Thus, differences previously observed between deaf and hearing subjects for discriminating the direction of moving stimuli do not generalize to contrast sensitivity for moving stimuli, a result that has implications for the neural level at which plastic changes occur in the visual system of deaf subjects.

Adult↗
PubMed1984

Cerebral asymmetry in congenitally deaf subjects.

Congenitally deaf subjects exposed to tachistoscopic presentation of English words, British Sign Language (BSL) signs, manual letters stimuli and a non-verbal task showed a left hemisphere advantage for English words and signs, a right hemisphere advantage for manual letters and no field differences for the non-verbal task.

Deafness↗
PubMed1993

Symbolic gesture versus word: is there a modality advantage for onset of symbol use?

Researchers have hypothesized in the past that children learning sign languages develop signs at an earlier age than is typically expected for vocal words. This assumption, however, has recently been questioned on the grounds that researchers have not always guaranteed that words and gestures are being used in a comparable fashion. The present study was designed to shed light on this controversy by comparing the onset of symbolic use of signs and words in a group of 22 hearing children exposed to symbolic gestures from 11 months onward. Bimonthly interviews emphasizing contexts of use of gestures and vocal words indicated a smaller modality difference than early research had predicted, thus providing support for the hypothesis that strides in cognitive abilities such as memory, categorization, and symbolization underlie this milestone in both modalities. At the same time, however, the data also indicated that the small difference in onset time was reliable, thus providing support for the notion that the gestural modality is, in fact, easier for many infants to master once the requisite cognitive skills are in place.

Age Factors↗
PubMed1992

[The effects of early manual instruction on the oral language development of two deaf children].

The speech and language training for deaf children at our clinic is performed using a multisensory method, which consists of reception and expression training for sign language and fingerspelling as well as auditory training, lip reading, and written language training (the Kanazawa Method). We have already reported that acquisition of written language is not dependent on oral language, and that written language is easier to learn than oral language for deaf children. In the present investigation, we analyzed the acquisition of comprehensible and expressive vocabulary in sign language and fingerspelling. The subjects were two children congenitally deaf at levels higher than 105dB. Recorded language samples by the age of 48 months were analyzed. Acquisition of sign language was found to be significantly easier than acquisition of oral language. The development of expressive noun words, function words, and Wh-question words in sign language at the early period was almost equivalent to that of hearing peers, and then the sign language appeared transfer to the oral language. These results suggest that early presentation of sign language with written and oral language is effective in the acquisition of communicative attitudes, function words and interrogative sentences which are most difficult for the hearing-impaired. It was shown that early presentation of sign language with written and oral language serves to promote acquisition of oral language.

Child↗
PubMed2002

Inequalities in access to healthcare faced by women who are deaf.

The Cheshire Deaf Women's Health Project undertook a research study to assess the access to healthcare of women who are deaf in Cheshire, UK. Group discussions took place with 13 women who were hard of hearing and 14 women who were Deaf Sign Language users. Questionnaires were distributed to a stratified random sample of 103 women taken from the social services register, 38 of which were returned. In order to reach more women whose first language was British Sign Language, 129 questionnaires were distributed to the leaders of various clubs and organizations for people who are deaf, and 100 of these were returned. The data revealed inequities in access to healthcare. For example, women who are deaf face a lack of awareness by health staff of how to communicate with them. The survey confirmed that these problems are of major importance to the majority of women who are deaf. For example, fewer than one in 10 deaf women said that they usually fully understand what the doctor says to them when they visit the doctor on their own. There are many other difficulties faced by women who are deaf, leading to inequalities when they are compared with hearing people. Almost half the respondents said that they would be more likely to use health services if help and/or services for deaf women were available. The introduction of various relatively simple measures would greatly help to reduce the inequalities of access to healthcare faced by deaf women. Under the terms of the Disability Discrimination Act 1995, such action is essential if providers are to avoid facing possible legal action.

Adult↗
PubMed1995

Communication with deaf patients. Knowledge, beliefs, and practices of physicians.

OBJECTIVE: To assess physicians' knowledge and beliefs regarding communication with deaf people and compare their knowledge and beliefs with their methods of communicating with deaf patients in their practices. DESIGN: Survey. SETTING: University medical center. SUBJECTS: Attending physicians in an internal medicine department. INTERVENTIONS: Physicians were surveyed regarding prior contacts with deaf patients and with deaf people outside the medical setting, and regarding their knowledge and beliefs concerning methods of communicating with deaf people. Physicians were asked to estimate the fraction of encounters in which they communicated with deaf patients by lipreading, writing, translation by a relative or friend, a sign language interpreter, or other methods. RESULTS: Writing was the method used most frequently in communicating with deaf patients. Although 63% of physicians knew that signing should be the initial method of communicating with deaf patients who sign, only 22% used sign language interpreters more frequently than other methods in their practices. Past contact with deaf people (P = .05), belief that communication by signing was the best means of communication (P = .04), and knowledge of the inefficiency of lipreading (P = .04) were predictors of the use of sign language interpreters for deaf patients. Physicians who used sign language interpreters more frequently than other methods believed that much more time and effort were involved in caring for deaf than for hearing patients compared with those who used interpreters less frequently (P = .08). CONCLUSION: Although most physicians believed that use of sign language interpreters was preferable, only a minority used them in their practices. Greater recognition of the advantages of signing over other methods and greater availability of sign language interpreters should lead to more effective communication between deaf patients and physicians.

Adult↗
PubMed1988

Training social skills to severely mentally retarded multiply handicapped adolescents.

Three severely mentally retarded, multiply handicapped, adolescents were treated in a classroom setting for social skills deficits. Two of these children exhibited symptoms of autism including periods of echolalia, and fascination with tactile and visual stimulation. One of the pair was deaf. The third child was profoundly mentally retarded and had minimal expressive language skills. All had received sign language training to facilitate communication. Treatment focused on increasing the frequency of eye contact, in seat and response to verbal prompt behaviors, skills deemed necessary to facilitate use of sign language communication and to increase social interaction. Baseline and treatment were evaluated in a multiple baseline, alternating treatment design across children. Baseline was taken on responses to 10 standard questions, asked by the teacher, based on verbal presentation and sign language. This same procedure was then continued during the initial treatment phase following training sessions. During training, the children received social reinforcement, performance feedback and edible reinforcement, in the form of candy, for appropriate performance. Physical and verbal prompts as well as pictorial cues were employed to shape appropriate behavior. In the second treatment phase, training was implemented in the classroom in which baseline data had been collected. Improvement in target behaviors, via training sessions held four days a week, was noted. These data suggest that use of a combination of visual stimuli, operant and social learning methods can remediate social skills deficits in children with multiple psychological and physical deficits. The implications of these findings for current and future research are discussed.

Achievement↗
PubMed1999

Early dissociation of verbal and nonverbal gestural ability in an epileptic deaf child.

Studies of sign language aphasia in deaf adults have provided the evidence for two separable verbal and nonverbal manual gesture systems. We report a congenitally deaf child with a idiopathic focal epilepsy of childhood who showed specific language impairment in French sign language. The child's amazing performances in miming or sketching pictures she was unable to sign support the notion of an early dissociation of the two gestural systems.

Adult↗
PubMed1992

Dissociation between linguistic and nonlinguistic gestural systems: a case for compositionality.

This paper addresses the issue of the separability of disorders of sign language from disorders of gesture and pantomime. The study of a left-lesioned deaf signer presents one of the most striking examples to date of the cleavage between linguistic signs and manual pantomime. The left-hemisphere lesion produced a marked sign language aphasia disrupting both the production and the comprehension of sign language. However, in sharp contrast to the breakdown of sign language, the ability to communicate in nonlinguistic gesture was remarkably spared. This case has important implications for our understanding of the neural mediation of language and gesture. We argue that the differences observed in the fractionation of linguistic versus nonlinguistic gesture reflect differing degrees of compositionality of systems underlying language and gesture. The compositionality hypothesis receives support for the existence of phonemic paraphasias in sign language production, illustrating structural dissolution which is absent in the production of pantomimic gesture. Understanding the neural encoding of compositional motoric systems may lead to a principled anatomical account of the neural separability of language and gesture. This case provides a powerful indication of the left hemisphere's specialization for language-specific functions.

Aphasia↗
PubMed1984

Visual-spatial processing in deaf brain-damaged signers.

Sign language displays all the complex linguistic structure found in spoken languages, but conveys its syntax in large part by manipulating spatial relations. This study investigated whether deaf signers who rely on a visual-spatial language nonetheless show a principled cortical separation for language and nonlanguage visual-spatial functioning. Four unilaterally brain-damaged deaf signers, fluent in American Sign Language (ASL) before their strokes, served as subjects. Three had damage to the left hemisphere and one had damage to the right hemisphere. They were administered selected tests of nonlanguage visual-spatial processing. The pattern of performance of the four patients across this series of tests suggests that deaf signers show hemispheric specialization for nonlanguage visual-spatial processing that is similar to hearing speaking individuals. The patients with damage to the left hemisphere, in general, appropriately processed visual-spatial relationships, whereas, in contrast, the patient with damage to the right hemisphere showed consistent and severe visual-spatial impairment. The language behavior of these patients was much the opposite, however. Indeed, the most striking separation between linguistic and nonlanguage visual-spatial functions occurred in the left-hemisphere patient who was most severely aphasic for sign language. Her signing was grossly impaired, yet her visual-spatial capacities across the series of tests were surprisingly normal. These data suggest that the two cerebral hemispheres of congenitally deaf signers can develop separate functional specialization for nonlanguage visual-spatial processing and for language processing, even though sign language is conveyed in large part via visual-spatial manipulation.

Adult↗
PubMed2003

Space is special in Sign.

Following groundbreaking work by linguists and cognitive scientists over the past thirty years, it is now generally accepted that sign languages of the deaf, such as ASL (American Sign Language) or BSL (British Sign Language), are structured and processed in a similar manner to spoken languages. The one striking difference is that they operate in a wholly non-auditory, visuospatial medium. How does the medium impact on language processing itself?

Journal Article↗