视觉语言对听觉障碍人群阅读能力的影响及作用机制

听觉障碍人群由于听觉部分或完全受损, 视觉语言——唇读和手语就成为其阅读能力发展的主要途径。唇读有助于听觉障碍人群形成语音表征, 与词汇知识相互影响, 且可以促进字词阅读及阅读理解的水平; 口语或书面语的加工可以激活相应的手语表征, 手语影响着听觉障碍人群各个层次的阅读能力。未来研究应该关注语音意识、词汇知识等技能在视觉语言影响听觉障碍人群阅读能力过程中的作用机制, 并以视觉语言为中心, 发展出适合汉语听觉障碍人群阅读能力习得的理论模型。     

Deaf and hearing adults' recollections of childhood and beyond

Whereas the average age of earliest reportable personal memory among adults is 3 to 3 1/2, there is considerable individual and group variability in the age of earliest autobiographical memory. Some of the variability is thought to be attributable to differential narrative socialisation. In the present research we tested the hypothesis that by virtue of later exposure to language, individuals born deaf to hearing parents will have earliest memories from later in life, relative to hearing individuals. The average age of single earliest identifiable memory for adults who are deaf and adults who are hearing did not differ. Nevertheless, adults who are deaf were found to have less dense representations of early autobiographical memories and to include in their narrative reports fewer categories of information, including visual-spatial information, relative to hearing adults. Participants' ratings of their memories on a number of dimensions were found to have low utility in predicting the content of autobiographical reports from both early and later in life (i.e., after age 10 years).     

Deaf and hearing adults' recollections of childhood and beyond

Whereas the average age of earliest reportable personal memory among adults is 3 to 3½, there is considerable individual and group variability in the age of earliest autobiographical memory. Some of the variability is thought to be attributable to differential narrative socialisation. In the present research we tested the hypothesis that by virtue of later exposure to language, individuals born deaf to hearing parents will have earliest memories from later in life, relative to hearing individuals. The average age of single earliest identifiable memory for adults who are deaf and adults who are hearing did not differ. Nevertheless, adults who are deaf were found to have less dense representations of early autobiographical memories and to include in their narrative reports fewer categories of information, including visual-spatial information, relative to hearing adults. Participants' ratings of their memories on a number of dimensions were found to have low utility in predicting the content of autobiographical reports from both early and later in life (i.e., after age 10 years).     

Prenatal Diagnosis for Inherited Deafness—What is the Potential Demand?

Genetic testing for inherited deafness is now available within some genetics centres. This study used a structured questionnaire to assess the potential uptake of prenatal diagnosis (PND) for inherited deafness, and document the opinions of deaf and hearing individuals toward PND and termination of pregnancy (TOP) for hearing status. Participants were self-selected from the whole of the UK, of whom 644 were deaf, 143 were hard of hearing or deafened, and 527 were hearing individuals who had either a deaf parent or child. The results showed that 21% of deaf, 39% of hard of hearing and deafened, and 49% of hearing participants said they would consider PND for deafness. Six percent of deaf, 11% of hard of hearing and deafened, and 16% of hearing participants said they would consider a TOP if the fetus was found to be deaf. Two percent of deaf participants said they would prefer to have deaf children and would consider a TOP if the fetus was found to be hearing.     

The Resilience of Structure Built Around the Predicate: Homesign Gesture Systems in Turkish and American Deaf Children

Deaf children whose hearing losses prevent them from accessing spoken language and whose hearing parents have not exposed them to sign language develop gesture systems, called homesigns, which have many of the properties of natural language—the so-called resilient properties of language. We explored the resilience of structure built around the predicate—in particular, how manner and path are mapped onto the verb—in homesign systems developed by deaf children in Turkey and the United States. We also asked whether the Turkish homesigners exhibit sentence-level structures previously identified as resilient in American and Chinese homesigners. We found that the Turkish and American deaf children used not only the same production probability and ordering patterns to indicate who does what to whom, but also used the same segmentation and conflation patterns to package manner and path. The gestures that the hearing parents produced did not, for the most part, display the patterns found in the children's gestures. Although cospeech gesture may provide the building blocks for homesign, it does not provide the blueprint for these resilient properties of language.     

Disordered Communication and Grieving in Deaf Member Families

When a deaf child is born to hearing parents, a grieving process is initiated in the parents. Unresolved grieving over their child's deafness often makes it difficult for hearing parents to accept the importance of signing, thus increasing the child's problems — further source of grief for these parents. Clinical illustrations are provided of (1) the reciprocal relationship between disruption of the mourning process and disturbance of communication between family members, and (2) the transmission of the dysfunctional relationship between hearing parents and deaf children to the subsequent relationship between the deaf children, when they reach adulthood, and their hearing children.     

Early interactions and the socioemotional development of deaf infants

Studies of the early face-to-face interactions and later attachment behaviours between infants and their caregivers are reviewed, with a particular focus on implications for deaf infants. Since the majority of deaf infants are born to hearing parents, it is to be expected that many parental responses will rely on vocalizations which are inaccessible to the deaf infant. Are other sensory modalities enhanced or used in a compensatory manner by hearing parents so as to make their communication with a deaf infant more effective? Is the deaf infant's heightened need for visual and tactile input adequately met during normal interactions with their caregivers? Does the deaf infant develop the same strategies for signalling distress and coping with separation as does the hearing infant? Although the research literature on this population is sparse, there are beginning to be more reports addressing these questions as early identification becomes increasingly possible. Therefore, this paper will cover issues of the role of eye contact and mutual coordination in early interactions, compensatory parenting behaviours from the perspective of the ‘intuitive parenting’ model, and precursors to later attachment behaviours in deaf infants.     

Preference for consonance over dissonance by hearing newborns of deaf parents and of hearing parents

Behavioral preferences for consonance over dissonance were tested in hearing infants of deaf parents and in hearing infants of hearing parents when they were 2 days old. Using a modified visual-fixation-based, auditory-preference procedure, I found that both 2-day-old infants of deaf parents and those of hearing parents looked longer at a visual stimulus when looking produced the original version of a Mozart minuet as opposed to a version altered to contain many dissonant intervals. The relative magnitude of such preference did not significantly differ whether their parents were deaf or hearing. Infants prefer consonance over dissonance, and the preference is present from birth and is not dependent on any specific prenatal or early postnatal experience.     

Judgments of speech intelligibility and speech annoyance by mothers of children who are deaf or hard of hearing

14 mothers of children who were deaf or hard of hearing provided magnitude estimation scaling responses for the speech intelligibility and speech annoyance of narrative speech samples produced by children who were deaf or hard of hearing. Analysis indicated that listeners scaled intelligibility and annoyance the same. As samples became more difficult to understand, they also became more annoying to these listeners. Implications for further research are discussed.     

Visual field asymmetries for motion processing in deaf and hearing signers

Recently, we reported a strong right visual field/left hemisphere advantage for motion processing in deaf signers and a slight reverse asymmetry in hearing nonsigners (Bosworth & Dobkins, 1999). This visual field asymmetry in deaf signers may be due to auditory deprivation or to experience with a visual-manual language, American Sign Language (ASL). In order to separate these two possible sources, in this study we added a third group, hearing native signers, who have normal hearing and have learned ASL from their deaf parents. As in our previous study, subjects performed a direction-of-motion discrimination task at different locations across the visual field. In addition to investigating differences in left vs right visual field asymmetries across subject groups, we also asked whether performance differences exist for superior vs inferior visual fields and peripheral vs central visual fields. Replicating our previous study, a robust right visual field advantage was observed in deaf signers, but not in hearing nonsigners. Like deaf signers, hearing signers also exhibited a strong right visual field advantage, suggesting that this effect is related to experience with sign language. These results suggest that perceptual processes required for the acquisition and comprehension of language (motion processing in the case of ASL) are recruited by the left, language-dominant, hemisphere. Deaf subjects also exhibited an inferior visual field advantage that was significantly larger than that observed in either hearing group. In addition, there was a trend for deaf subjects to perform relatively better on peripheral than on central stimuli, while both hearing groups showed the reverse pattern. Because deaf signers differed from hearing signers and nonsigners along these domains, the inferior and peripheral visual field advantages observed in deaf subjects is presumably related to auditory deprivation. Finally, these visual field asymmetries were not modulated by attention for any subject group, suggesting they are a result of sensory, and not attentional, factors.     

Provision of Genetic Services for Hearing Loss: Results from a National Survey and Comparison to Insights Obtained from Previous Focus Group Discussions

Hearing loss is a common sensory deficit and more than 50% of affected individuals have a genetic etiology. The discovery of 40 genes and more than 100 loci involved in hearing loss has made genetic testing for some of these genes widely available. Genetic services for deafness are also being sought more often due to the early identification of hearing loss through newborn screening services. The motivations for pursuing genetic testing, and how genetic services are provided to the client may differ among individuals. Additionally, information obtained through genetic testing can be perceived and used in different ways by parents of deaf children and deaf adults. This study aimed to follow up on focus group studies published earlier with a quantitative survey instrument and assess the preference of consumers for provision of genetic services. We conducted a national survey of hearing and deaf parents of children with hearing loss and of deaf adults. Data was compared and analyzed by hearing status of the participant, their community affiliation and the genetic testing status using nominal logistic regression. Consistent with our focus group results, the survey participants thought that a genetic counselor/geneticist would be the most appropriate professional to provide genetics services. Statistically significant differences were noted in the preferred choice of provider based on the genetic testing status. Parents preferred that genetic evaluation, including testing, occur either immediately at or a few months after the audiologic diagnosis of hearing loss. This data should help providers in clinical genetics keep patient preferences at the helm and provide culturally competent services.     

Effects of signability and imagery on word recall of deaf and hearing students

This study examined 40 deaf and 20 hearing students' free recall of visually presented words varied systematically with respect to signability (i.e., words that could be expressed by a single sign) and visual imagery. Half of the deaf subjects had deaf parents, while the other half had hearing parents. For deaf students, recall was better for words that had sign-language equivalents and high-imagery values. For the hearing students, recall was better for words with high-imagery values, but there was no effect of signability. Over-all, the hearing students recalled significantly more words than the deaf students in both immediate and delayed free-recall conditions. In immediate recall, deaf students with deaf parents reported using a sign-language coding strategy more frequently and recalled more words correctly than deaf students with hearing parents. Serial-position curves indicated several differences in patterns of recall among the groups. These results underline the importance of sign language in the memory and recall of deaf persons.     

Ethical issues in cochlear implant surgery: an exploration into disease, disability, and the best interests of the child

This paper examines ethical issues related to medical practices with children and adults who are members of a linguistic and cultural minority known as the DEAF-WORLD. Members of that culture characteristically have hearing parents and are treated by hearing professionals whose values, particularly concerning language, speech, and hearing, are typically quite different from their own. That disparity has long fueled a debate on several ethical issues, most recently the merits of cochlear implant surgery for DEAF children. We explore whether that surgery would be ethical if implants could deliver close to normal hearing for most implanted children, thereby diminishing the ranks of the DEAF-WORLD. The ethical implications of eugenic practices with the DEAF are explored, as are ethical quandaries in parental surrogacy for DEAF children, and their parallels in transracial adoption.     

Development of social-cognitive and communication skills in children born deaf

Central to the interface of social-cognitive and communicative development is the growth of a theory of mind (ToM). ToM is mastered by most hearing children and deaf children of signing deaf parents by the age of 5 or 6 but is often seriously delayed in deaf children of hearing parents. This paper reviews recently published research on deaf children's ToM development and presents an original study consisting of eight longitudinal case histories that collectively map late-signing deaf children's ToM performance from 44 to 158 months of age. While five tentative conclusions can be posited from the collective research so far, further investigation of each of these possibilities is clearly needed.     

The Short-Term Memory of Profoundly Deaf People for Words,Signs, and Abstract Spatial Stimuli

The short-term memory (STM) of 25 deaf and 20 hearing adults fluent in Australian Sign Language (Auslan) was tested using both free- and serial-recall versions of three tasks. On two tasks, where stimuli were presented as either written words or Auslan signs, hearing subjects performed significantly better than deaf subjects. This difference was attributed to the facility of the hearing subjects in translating these two classes of language-based stimuli into phonological codes, which have a preferred status in STM. On the third, language-free task, which was an adaptation of the Corsi Blocks test, the deaf and hearing subjects performed at comparable levels, indicating that differences in their STM became evident only with the introduction of language-based factors. Analyses restricted to the deaf subjects showed that performances on the language-based STM tasks correlated positively with scores on a reading comprehension test. Also, deaf subjects who reported an oral education outperformed their counterparts, who reported a total communication (oral plus signed English) education on the language-based STM tasks. Thus, for this diverse adult deaf sample, proficiency in STM for language-based material, skill in reading, and report of an oral rather than total communication education appear to covary.     

Consumer Motivations for Pursuing Genetic Testing and their Preferences for the Provision of Genetic Services for Hearing Loss

Genetic services for deafness are being increasingly sought due to the introduction of early hearing detection and intervention programs, as well as the rapid progress in the identification of deafness genes. This study aimed to assess the motivations of consumers for pursuing genetic testing as well as their preferences for provision of these services. We conducted 5 focus groups consisting of hearing parents of deaf children, deaf parents, and unmarried deaf adults. Motivations for pursuing genetic testing included determining the etiology, helping to alleviate the guilt associated with the diagnosis of hearing loss in a child, and acquiring information to help them and other family members prepare for the future. Most participants thought that a genetic counselor/geneticist would be the most appropriate professional to provide genetics services. For culturally Deaf individuals, the communication method was seen as more important than the type of professional. Parents preferred that genetic evaluation, including testing, occur either immediately at or a few months after the audiologic diagnosis of hearing loss.     

The genetics of deafness

Deafness is an etiologically heterogeneous trait with many known genetic and environmental causes. Genetic factors account for at least half of all cases of profound congenital deafness, and can be classified by the mode of inheritance and the presence or absence of characteristic clinical features that may permit the diagnosis of a specific form of syndromic deafness. The identification of more than 120 independent genes for deafness has provided profound new insights into the pathophysiology of hearing, as well as many unexpected surprises. Although a large number of genes can clearly cause deafness, recessive mutations at a single locus, GJB2 or Connexin 26, account for more than half of all genetic cases in some, but not all populations. The high frequency may well be related to the greatly improved social, educational, and economic circumstances of the deaf that began with the introduction of sign language 300-400 years ago, along with a high frequency of marriages among the deaf in many countries. Similar mechanisms may account for the rapid fixation of genes for speech after the first mutations appeared 50,000-100,000 years ago. Molecular studies have shown that mutations involving several different loci may be the cause for the same form of syndromic deafness. Even within a single locus, different mutations can have profoundly different effects, leading to a different pattern of inheritance in some cases, or isolated hearing loss without the characteristic syndromic features in others. Most cases of genetic deafness result from mutations at a single locus, but an increasing number of examples are being recognized in which recessive mutations at two loci are involved. For example, digenic interactions are now known to be an important cause of deafness in individuals who carry a single mutation at the Connexin 26 locus along with a deletion involving the functionally related Connexin 30 locus. This mechanism complicates genetic evaluation and counseling, but provides a satisfying explanation for Connexin 26 heterozygotes who, for previously unknown reasons, are deaf. A specific genetic diagnosis can sometimes be of great clinical importance, as in the case of the mitochondrial A1555G mutation which causes gene carriers to be exquisitely sensitive to the ototoxic effects of aminoglycosides. This potentially preventable genetic-environmental interaction was the most common cause of genetic deafness in countries where these antibiotics were used indiscriminately in the past. Advances in genetic knowledge along with the use of cochlear implants have posed unique ethical dilemmas for society as well as the deaf community. Since most deaf children are born to hearing parents, it seems likely that deaf culture, and intermarriages among those born with deafness will recede during this century. Will future critics view this as one of the medical triumphs of the 21(st) Century, or as an egregious example of cultural genocide? On the other hand, genetics can provide empowering knowledge to the deaf community that for the first time can allow many deaf couples to know whether their children will be hearing or deaf even before they are conceived.     

How children make language out of gesture: morphological structure in gesture systems developed by American and Chinese deaf children

When children learn language, they apply their language-learning skills to the linguistic input they receive. But what happens if children are not exposed to input from a conventional language? Do they engage their language-learning skills nonetheless, applying them to whatever unconventional input they have? We address this question by examining gesture systems created by four American and four Chinese deaf children. The children's profound hearing losses prevented them from learning spoken language, and their hearing parents had not exposed them to sign language. Nevertheless, the children in both cultures invented gesture systems that were structured at the morphological/word level. Interestingly, the differences between the children's systems were no bigger across cultures than within cultures. The children's morphemes could not be traced to their hearing mothers' gestures; however, they were built out of forms and meanings shared with their mothers. The findings suggest that children construct morphological structure out of the input that is handed to them, even if that input is not linguistic in form.     

Does language about similarity play a role in fostering similarity comparison in children?

Commenting on perceptual similarities between objects stands out as an important linguistic achievement, one that may pave the way towards noticing and commenting on more abstract relational commonalities between objects. To explore whether having a conventional linguistic system is necessary for children to comment on different types of similarity comparisons, we observed four children who had not been exposed to usable linguistic input - deaf children whose hearing losses prevented them from learning spoken language and whose hearing parents had not exposed them to sign language. These children developed gesture systems that have language-like structure at many different levels. Here we ask whether the deaf children used their gestures to comment on similarity relations and, if so, which types of relations they expressed. We found that all four deaf children were able to use their gestures to express similarity comparisons (point to cat + point to tiger) resembling those conveyed by 40 hearing children in early gesture + speech combinations (cat + point to tiger). However, the two groups diverged at later ages. Hearing children, after acquiring the word like, shifted from primarily expressing global similarity (as in cat/tiger) to primarily expressing single-property similarity (as in crayon is brown like my hair). In contrast, the deaf children, lacking an explicit term for similarity, continued to primarily express global similarity. The findings underscore the robustness of similarity comparisons in human communication, but also highlight the importance of conventional terms for comparison as likely contributors to routinely expressing more focused similarity relations.     

Cochlear implants and the claims of culture? A response to Lane and Grodin

Because I reject the notion that physical characteristics constitute cultural membership, I argue that, even if the claim were persuasive that deafness is a culture rather than a disability, there is no reason to fault hearing parents who choose cochlear implants for their deaf children.