Attitudes and experiences regarding preventive strategies for the deaf population in Western New York.

People for whom English is a second language, such as the deaf population, often have unequal access to health information and low health literacy. In the context of a wider study on risk of tick-borne illness in deaf communities, we explored barriers, opportunities, and nuances to accessible health information and communication among deaf people. Semi-structured qualitative individual and group interviews were conducted with 40 deaf people in upstate New York, to explore factors associated with health literacy and health information accessibility. Interviews were conducted in American Sign Language (ASL) by a deaf researcher fluent in ASL. Data analysis included the translation of ASL signs into English words, systematic coding, and generation of themes. A total of 21 interview events (mean time per interview = 41 minutes) were conducted. Two main themes and multiple sub-themes emerged from the data: 1) Layers of obstacles faced by deaf people confirms (or reinforces) exclusion; and 2) preventive information is unavailable or inaccessible to deaf people. Sub- themes identified in the results were perceptions of the deaf community and deaf culture, complex layers of obstacles faced by deaf individuals, the digital divide, the culture of communication, awareness of tick and tick-borne disease (TBD) diseases, importance of using certified deaf interpreters (CDI), health information dissemination strategies and collaborations with the education system, and physical/virtual community engagement. The data suggested several challenges to health literacy in the deaf population, including healthcare and education inequalities and negative perceptions of deaf people by both deaf and hearing people. Improving health literacy in the deaf population requires more interpreters who themselves are deaf ("certified deaf interpreters"), provision of health information in ASL, and a greater engagement with the deaf population by education and healthcare systems.

Deaf Children Need Rich Language Input from the Start: Support in Advising Parents.

Bilingual bimodalism is a great benefit to deaf children at home and in schooling. Deaf signing children perform better overall than non-signing deaf children, regardless of whether they use a cochlear implant. Raising a deaf child in a speech-only environment can carry cognitive and psycho-social risks that may have lifelong adverse effects. For children born deaf, or who become deaf in early childhood, we recommend comprehensible multimodal language exposure and engagement in joint activity with parents and friends to assure age-appropriate first-language acquisition. Accessible visual language input should begin as close to birth as possible. Hearing parents will need timely and extensive support; thus, we propose that, upon the birth of a deaf child and through the preschool years, among other things, the family needs an adult deaf presence in the home for several hours every day to be a linguistic model, to guide the family in taking sign language lessons, to show the family how to make spoken language accessible to their deaf child, and to be an encouraging liaison to deaf communities. While such a support program will be complicated and challenging to implement, it is far less costly than the harm of linguistic deprivation.

Who Goes First? Deaf People and CRISPR Germline Editing.

The development of CRISPR technology has catapulted the issue of germline editing to the forefront of a debate between the goals of medical advancement and promotion of human diversity. The US National Academy of Sciences and the National Academy of Medicine recommended in a joint report that germline editing should be tightly regulated and pursued only for "serious diseases." A follow-up statement from an international summit on human genome editing emphasized a more general point that "the risks [are] too great to permit clinical trials of germline editing at this time." Here we review their recommendations in the context of genetic deafness, a condition that historically has been viewed by the medical community as a pathology. Deafness does not meet the standard of "serious disease" for experiments with human germline editing, but there is a real concern that scientists may soon begin to do germline editing with deaf individuals because, as we will discuss, they are in many ways ideal subjects for a clinical study of CRISPR, though their condition is neither fatal nor debilitating. In light of this, we worry about the potential for medical overreach and expediency. Drawing from examples of living deaf communities around the world, we propose an expansive view of human diversity that recognizes the value of genetic, linguistic, and cultural diversity to the future health of humankind.

Discourses of prejudice in the professions: the case of sign languages.

There is no evidence that learning a natural human language is cognitively harmful to children. To the contrary, multilingualism has been argued to be beneficial to all. Nevertheless, many professionals advise the parents of deaf children that their children should not learn a sign language during their early years, despite strong evidence across many research disciplines that sign languages are natural human languages. Their recommendations are based on a combination of misperceptions about (1) the difficulty of learning a sign language, (2) the effects of bilingualism, and particularly bimodalism, (3) the bona fide status of languages that lack a written form, (4) the effects of a sign language on acquiring literacy, (5) the ability of technologies to address the needs of deaf children and (6) the effects that use of a sign language will have on family cohesion. We expose these misperceptions as based in prejudice and urge institutions involved in educating professionals concerned with the healthcare, raising and educating of deaf children to include appropriate information about first language acquisition and the importance of a sign language for deaf children. We further urge such professionals to advise the parents of deaf children properly, which means to strongly advise the introduction of a sign language as soon as hearing loss is detected.

The effect of being human and the basis of grammatical word order: Insights from novel communication systems and young sign languages.

This study identifies a central factor that gives rise to the different word orders found in the world's languages. In the last decade, a new window on this long-standing question has been provided by data from young sign languages and invented gesture systems. Previous work has assumed that word order in both invented gesture systems and young sign languages is driven by the need to encode the semantic/syntactic roles of the verb's arguments. Based on the responses of six groups of participants, three groups of hearing participants who invented a gestural system on the spot, and three groups of signers of relatively young sign languages, we identify a major factor in determining word order in the production of utterances in novel and young communication systems, not suggested by previous accounts, namely the salience of the arguments in terms of their human/animacy properties: human arguments are introduced before inanimate arguments ('human first'). This conclusion is based on the difference in word order patterns found between responses to depicted simple events that vary as to whether both subject and object are human or whether the subject is human and the object inanimate. We argue that these differential patterns can be accounted for uniformly by the 'human first' principle. Our analysis accounts for the prevalence of SOV order in clauses with an inanimate object in all groups (replicating results of previous separate studies of deaf signers and hearing gesturers) and the prevalence of both SOV and OSV in clauses with a human object elicited from the three groups of participants who have the least interference from another linguistic system (nonliterate deaf signers who have had little or no exposure to another language). It also provides an explanation for the basic status of SOV order suggested by other studies, as well as the scarcity of the OSV order in languages of the world, despite its appearance in novel communication systems. The broadest implication of this study is that the basic cognitive distinction between humans and inanimate entities is a crucial factor in setting the wheels of word ordering in motion.

Tools for language: patterned iconicity in sign language nouns and verbs.

When naming certain hand-held, man-made tools, American Sign Language (ASL) signers exhibit either of two iconic strategies: a handling strategy, where the hands show holding or grasping an imagined object in action, or an instrument strategy, where the hands represent the shape or a dimension of the object in a typical action. The same strategies are also observed in the gestures of hearing nonsigners identifying pictures of the same set of tools. In this paper, we compare spontaneously created gestures from hearing nonsigning participants to commonly used lexical signs in ASL. Signers and gesturers were asked to respond to pictures of tools and to video vignettes of actions involving the same tools. Nonsigning gesturers overwhelmingly prefer the handling strategy for both the Picture and Video conditions. Nevertheless, they use more instrument forms when identifying tools in pictures, and more handling forms when identifying actions with tools. We found that ASL signers generally favor the instrument strategy when naming tools, but when describing tools being used by an actor, they are significantly more likely to use more handling forms. The finding that both gesturers and signers are more likely to alternate strategies when the stimuli are pictures or video suggests a common cognitive basis for differentiating objects from actions. Furthermore, the presence of a systematic handling/instrument iconic pattern in a sign language demonstrates that a conventionalized sign language exploits the distinction for grammatical purpose, to distinguish nouns and verbs related to tool use.

Bilingualism: A Pearl to Overcome Certain Perils of Cochlear Implants.

Cochlear implants (CI) have demonstrated success in improving young deaf children's speech and low-level speech awareness across a range of auditory functions, but this success is highly variable, and how this success correlates to high-level language development is even more variable. Prevalence on the success rate of CI as an outcome for language development is difficult to obtain because studies vary widely in methodology and variables of interest, and because not all cochlear implant technology (which continues to evolve) is the same. Still, even if the notion of treatment failure is limited narrowly to those who gain no auditory benefit from CI in that they cannot discriminate among ambient noises, the reported treatment failure rate is high enough to call into question the current lack of consideration of alternative approaches to ensure young deaf children's language development. Recent research has highlighted the risks of delaying language input during critical periods of brain development with concomitant consequences for cognitive and social skills. As a result, we propose that before, during, and after implantation deaf children learn a sign language along with a spoken language to ensure their maximal language development and optimal long-term developmental outcomes.

Competing iconicities in the structure of languages.

The paper examines the role that iconicity plays in the structuring of grammars. Two main points are argued for: (a) Grammar does not necessarily suppress iconicity; rather, iconicity and grammar can enjoy a congenial relation in that iconicity can play an active role in the structuring of grammars. (b) Iconicity is not monolithic. There are different types of iconicity and languages take advantage of the possibilities afforded by them. We examine the interaction between iconicity and grammar by focusing on the ways in which sign languages employ the physical body of the signer as a rich iconic resource for encoding a variety of grammatical notions. We show that the body can play three different roles in iconic forms in sign languages: it can be used as a naming device where body parts represent body parts; it can represent the subject argument of verbal signs, and it can stand for first person. These strategies interact and sometimes compete in the languages under study. Each language resolves these competitions differently, which results in different grammars and grammatical structures. The investigation of the ways in which grammar and iconicity interact in these languages provides insight into the nature of both systems.

The right to language.

We argue for the existence of a state constitutional legal right to language. Our purpose here is to develop a legal framework for protecting the civil rights of the deaf child, with the ultimate goal of calling for legislation that requires all levels of government to fund programs for deaf children and their families to learn a fully accessible language: a sign language.

Language acquisition for deaf children: Reducing the harms of zero tolerance to the use of alternative approaches.

Children acquire language without instruction as long as they are regularly and meaningfully engaged with an accessible human language. Today, 80% of children born deaf in the developed world are implanted with cochlear devices that allow some of them access to sound in their early years, which helps them to develop speech. However, because of brain plasticity changes during early childhood, children who have not acquired a first language in the early years might never be completely fluent in any language. If they miss this critical period for exposure to a natural language, their subsequent development of the cognitive activities that rely on a solid first language might be underdeveloped, such as literacy, memory organization, and number manipulation. An alternative to speech-exclusive approaches to language acquisition exists in the use of sign languages such as American Sign Language (ASL), where acquiring a sign language is subject to the same time constraints of spoken language development. Unfortunately, so far, these alternatives are caught up in an "either - or" dilemma, leading to a highly polarized conflict about which system families should choose for their children, with little tolerance for alternatives by either side of the debate and widespread misinformation about the evidence and implications for or against either approach. The success rate with cochlear implants is highly variable. This issue is still debated, and as far as we know, there are no reliable predictors for success with implants. Yet families are often advised not to expose their child to sign language. Here absolute positions based on ideology create pressures for parents that might jeopardize the real developmental needs of deaf children. What we do know is that cochlear implants do not offer accessible language to many deaf children. By the time it is clear that the deaf child is not acquiring spoken language with cochlear devices, it might already be past the critical period, and the child runs the risk of becoming linguistically deprived. Linguistic deprivation constitutes multiple personal harms as well as harms to society (in terms of costs to our medical systems and in loss of potential productive societal participation).

The gestures ASL signers use tell us when they are ready to learn math.

The manual gestures that hearing children produce when explaining their answers to math problems predict whether they will profit from instruction in those problems. We ask here whether gesture plays a similar role in deaf children, whose primary communication system is in the manual modality. Forty ASL-signing deaf children explained their solutions to math problems and were then given instruction in those problems. Children who produced many gestures conveying different information from their signs (gesture-sign mismatches) were more likely to succeed after instruction than children who produced few, suggesting that mismatch can occur within-modality, and paving the way for using gesture-based teaching strategies with deaf learners.

The emergence of complexity in prosody and syntax.

The relation between prosody and syntax is investigated here by tracing the emergence of each in a new language, Al-Sayyid Bedouin Sign Language. We analyze the structure of narratives of four signers of this language: two older second generation signers, and two about 15 years younger. We find that younger signers produce prosodic cues to dependency between semantically related constituents, e.g., the two clauses of conditionals, revealing a type and degree of complexity in their language that is not frequent in that of the older pair. In these younger signers, several rhythmic and (facial) intonational cues are aligned at constituent boundaries, indicating the emergence of a grammatical system. There are no overt syntactic markers (such as complementizers) to relate clauses; prosody is the only clue. But this prosodic complexity is matched by syntactic complexity inside propositions in the younger signers, who are more likely to use pronouns as abstract grammatical markers of arguments, and to combine predicates with their arguments within in a constituent. As the prosodic means emerge for identifying constituent types and signaling dependency relations between them, the constituents themselves become increasingly complex. Finally, our study shows that the emergence of grammatical complexity is gradual.

The gradual emergence of phonological form in a new language.

The division of linguistic structure into a meaningless (phonological) level and a meaningful level of morphemes and words is considered a basic design feature of human language. Although established sign languages, like spoken languages, have been shown to be characterized by this bifurcation, no information has been available about the way in which such structure arises. We report here on a newly emerging sign language, Al-Sayyid Bedouin Sign Language, which functions as a full language but in which a phonological level of structure has not yet emerged. Early indications of formal regularities provide clues to the way in which phonological structure may develop over time.

Infants and children with hearing loss need early language access.

Around 96 percent of children with hearing loss are born to parents with intact hearing, who may initially know little about deafness or sign language. Therefore, such parents will need information and support in making decisions about the medical, linguistic, and educational management of their child. Some of these decisions are time-sensitive and irreversible and come at a moment of emotional turmoil and vulnerability (when some parents grieve the loss of a normally hearing child). Clinical research indicates that a deaf child's poor communication skills can be made worse by increased level of parental depression. Given this, the importance of reliable and up-to-date support for parents' decisions is critical to the overall well-being of their child. In raising and educating a child, parents are often offered an exclusive choice between an oral environment (including assistive technology, speech reading, and voicing) and a signing environment. A heated controversy surrounds this choice, and has since at least the late 19th century, beginning with the International Congress on the Education of the Deaf in Milan, held in 1880. While families seek advice from many sources, including, increasingly, the internet, the primary care physician (PCP) is the professional medical figure the family interacts with repeatedly. The present article aims to help family advisors, particularly the PCP and other medical advisors in this regard. We argue that deaf children need to be exposed regularly and frequently to good language models in both visual and auditory modalities from the time hearing loss is detected and continued throughout their education to ensure proper cognitive, psychological, and educational development. Since there is, unfortunately, a dearth of empirical studies on many of the issues families must confront, professional opinions, backed by what studies do exist, are the only option. We here give our strongly held professional opinions and stress the need for improved research studies in these areas.

Delayed recognition of profound hearing loss in a 7-year-old girl with a neurological condition.

CASE: Kelly is a 7-year-old girl with a complex medical history including asthma, mild spastic diplegia, and seizure disorder that is controlled with carbamazepine. She had a significant receptive and expressive language impairment and milder delays in gross and fine motor skills. Kelly is currently repeating first grade in a self-contained classroom; she receives speech, occupational, and physical therapy. At the 7-year-old well child visit, her mother is worried about Kelly's poor progress in school, and she expresses concern about her daughter's hearing. Her pediatrician observes that Kelly is withdrawn, uses minimal language, and is fearful of the examination. Kelly was born full-term by Cesarean section because of placental abruption. She was in the neonatal intensive care nursery for 2 weeks with metabolic acidosis because of acute tubular necrosis. One day after arriving home, she had a cardiopulmonary arrest followed by emergency open-heart surgery for critical pulmonary hypertension. Her postoperative course was significant for renal failure, extracorporal membrane oxygenation, ventilator dependency, tracheostomy, and gastrostomy. By 3 years of age her medical condition stabilized, and the tracheostomy and gastrostomy tubes were removed. A review of Kelly's previous audiological tests revealed a failed otoacoustic emission test at 5 months. An auditory brain stem response test at 8 months recorded normal hearing in the right ear. At 4 years, behavioral audiometry was attempted but not completed because Kelly cried throughout the session. At 5 years, testing with ear inserts showed normal hearing bilaterally. Because of the concerns raised by Kelly's mother at the pediatric visit, she was referred to audiology for a reevaluation. Testing at this time revealed moderate to profound sensorineural hearing loss in both the ears, which was confirmed on subsequent examinations. Kelly was promptly fitted for hearing aids. Her individual education plan was changed to reflect the diagnosis of hearing impairment, and hearing services were implemented in the classroom. On a recent follow-up visit, Kelly was talkative, engaging, and cheerful.

Delayed recognition of profound hearing loss in a 7-year-old girl with a neurological condition.

Kelly is a 7-year-old girl with a complex medical history including asthma, mild spastic diplegia, and seizure disorder that is controlled with carbamazepine. She had a significant receptive and expressive language impairment and milder delays in gross and fine motor skills. Kelly is currently repeating first grade in a self-contained classroom; she receives speech, occupational, and physical therapy. At the 7-year-old well child visit, her mother is worried about Kelly's poor progress in school, and she expresses concern about her daughter's hearing. Her pediatrician observes that Kelly is withdrawn, uses minimal language, and is fearful of the examination.Kelly was born full-term by Cesarean section because of placental abruption. She was in the neonatal intensive care nursery for 2 weeks with metabolic acidosis because of acute tubular necrosis. One day after arriving home, she had a cardiopulmonary arrest followed by emergency open-heart surgery for critical pulmonary hypertension. Her postoperative course was significant for renal failure, extracorporal membrane oxygenation, ventilator dependency, tracheostomy, and gastrostomy. By 3 years of age her medical condition stabilized, and the tracheostomy and gastrostomy tubes were removed.A review of Kelly's previous audiological tests revealed a failed otoacoustic emission test at 5 months. An auditory brain stem response test at 8 months recorded normal hearing in the right ear. At 4 years, behavioral audiometry was attempted but not completed because Kelly cried throughout the session. At 5 years, testing with ear inserts showed normal hearing bilaterally.Because of the concerns raised by Kelly's mother at the pediatric visit, she was referred to audiology for a reevaluation. Testing at this time revealed moderate to profound sensorineural hearing loss in both the ears, which was confirmed on subsequent examinations. Kelly was promptly fitted for hearing aids. Her individual education plan was changed to reflect the diagnosis of hearing impairment, and hearing services were implemented in the classroom. On a recent follow-up visit, Kelly was talkative, engaging, and cheerful.

The Roots of Linguistic Organization in a New Language.

It is possible for a language to emerge with no direct linguistic history or outside linguistic influence. Al-Sayyid Bedouin Sign Language (ABSL) arose about 70 years ago in a small, insular community with a high incidence of profound prelingual neurosensory deafness. In ABSL, we have been able to identify the beginnings of phonology, morphology, syntax, and prosody. The linguistic elements we find in ABSL are not exclusively holistic, nor are they all compositional, but a combination of both. We do not, however, find in ABSL certain features that have been posited as essential even for a proto-language. ABSL has a highly regular syntax as well as word-internal compounding, also highly regular but quite distinct from syntax in its patterns. ABSL, however, has no discernable word-internal structure of the kind observed in more mature sign languages: no spatially organized morphology and no evident duality of phonological patterning.