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By representing image content using probabilistic models of an object's appearance we can obtain semantics-preserving compression of the image data. Such compact representations of an image's salient features allow rapid computer searches of even large image databases. Examples are shown for databases of face images, a video of American sign language (ASL), and a video of facial expressions.
Children with special needs typically require family accommodation to those needs. We explore here the extent to which cultural forces shape the accommodations mothers make when communicating with young deaf children. Sixteen mother-child dyads (8 Chinese, 8 American) were videotaped at home. In each culture, 4 mothers interacted with their deaf children, and 4 interacted with their hearing children. None of the deaf children knew sign language, nor spoke at age level. We found that mothers adjusted their communicative behaviors to their deaf children, but in every case, those adjustments were calibrated to cultural norms. American mothers, for example, increased their use of gesture with deaf children but stopped far short of the Chinese range--despite the obvious potential benefits of gesturing to children who cannot hear. These findings provide the first cross-cultural demonstration that children are, first and foremost, inculcated into their cultures and, only within that framework, then treated as special cases.
The aim of this study was to establish rates of behavioural and emotional problems, and of social maladjustment, in a population of deaf children, particularly in relation to different methods of communication. The parents of 84 children who attended two schools for the deaf took part. They completed the parents' checklist (PCL), a behaviour rating scale for deaf children, and the Child Behaviour Checklist (CBCL), a measure widely used in the general population. The two instruments were significantly correlated on the severity of behavioural and emotional problems, but their previously established cut-off scores detected different rates of possible clinical cases, i.e. children with mental health disorders. According to the CBCL, 40% of children were within the clinical range, and 82% were socially dysfunctional compared with the general population. The PCL identified a much higher percentage (77%) of caseness. Behavioural and emotional problems were significantly higher in Asian children. Although all subjects used sign language, the additional use of speech, which may indicate increased hearing ability, had a protective effect for adolescents. The findings are discussed in relation to the validation of the instruments and the development of intervention programmes for deaf children.
We describe 2 patients with Angelman syndrome (AS) due to paternal uniparental disomy (UPD). One patient is a female aged 30 years and the other a male aged 4 1/2 years. Both have the characteristic wide mouth and big chin, moderate mental retardation, virtually no speech but some 30 words of sign language and a happy disposition with outbursts of laughter. Ataxia is minimal in both patients, manifesting mainly when they are excited or running. Both patients are tall (height around 90th percentile), have a head circumference around 75th percentile and are overweight (weight over the 97th percentile). These cases add to the knowledge of the possibility of normal or increased growth parameters, particularly weight, in AS when the genetic mechanism is paternal UPD.
The present study investigated self- and teacher-perceptions of deaf adolescents in relation to cultural identity. Fifty-one deaf adolescents completed the Porteous Checklist and Deaf Identity Scale presented in British Sign Language. Subjects were assigned to deaf, hearing or dual identity groups. Results suggest that deaf adolescents' self-perceived concerns are not dissimilar in content or severity to those of their hearing peers, although certain issues may assume a particular significance in the presence of deafness. The hypothesis that the hearing identity group would report most problems was not supported. Teachers rated the dual identity group as having the fewest difficulties.
Psychiatric screening questionnaires for deaf children and adolescents were piloted in a group of 62 children, aged 11-16 years, attending a residential school. The questionnaires, Parent's Checklist (PCL) and Teacher's Checklist (TCL) were then used to screen a group of 93 children attending one Deaf School and three Hearing Impaired Units (HIU). Psychiatric assessments were conducted with a highly structured diagnostic interview, the Child Assessment Schedule (CAS). The interview with signing deaf children was conducted with a sign language interpreter. The prevalence of psychiatric disorder in the whole group was 50.3%, 42.4% in the group attending the Deaf School and 60.9% for the group attending the HIUs. An aetiological model of psychiatric disorder in this group is proposed.
OBJECTIVE: This study sought to determine the oral health care knowledge and practices of a group of deaf adolescents in Lagos. METHODS: The study involved 50 students of Wesley School 1 for the Deaf, Lagos (26 males and 24 females, aged 10-19 years, mean 13.3 +/- 2.8). Information about previous dental care, oral hygiene, and snacking habits were obtained through a questionnaire and sign language by the teachers. RESULTS: Only 12 percent of pupils had received dental care. Eight percent and 72 percent, respectively, gave correct answers to causes of tooth decay and bleeding gums. Ninety-four percent brushed their teeth once daily, with no significant sex difference (P > .05). Reported dental problems include bleeding gums (36%), tooth discoloration, and tooth decay. The majority of pupils (60%) preferred biscuits and soft drinks as snacks. More than 90 percent were willing to have a dental check-up. CONCLUSIONS: The oral health knowledge and practices of this group of children will improve through a controlled school-based oral health education program.
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Bengkala is an Indonesian village located on the north shore of Bali that has existed for over 700 years. Currently, 2.2% of the 2185 people in this village have profound congenital deafness. In response to the high incidence of deafness, the people of Bengkala have developed a village specific sign language which is used by many of the hearing and deaf people. Deafness in Bengkala is congenital, sensorineural, non-syndromal, and caused by a fully penetrant autosomal recessive mutation at the DFNB3 locus. The frequency of the DFNB3 mutation is estimated to be 9.4% among hearing people who have a 17.2% chance of being heterozygous for DFNB3.
Angelman syndrome (AS) is a complex neurological disorder with different genetic aetiologies. It is not known whether the clinical features vary depending on the genetic mechanism. We report four patients with AS owing to uniparental disomy (UPD). There were two males and two females, with a mean age of 8 years (range 7 to 11 years). All patients had a happy disposition, hyperactive behaviour, and the characteristic facial phenotype of AS, but in three there was a normal head circumference, two had epilepsy, ataxic movements were mild in three, the mean age of onset of walking was 2.4 years, and there was some sign language in all four patients. Our cases add further weight to the previously reported impressions of a milder phenotype in cases of AS resulting from UPD than in deleted AS patients. Patients suspected of having AS, but who are considered atypical, warrant DNA testing.
For autosomal recessive nonsyndromic hearing impairment over 30 loci have been mapped and 19 genes have been identified. DFNB38, a novel locus for autosomal recessive nonsyndromic hearing impairment, was localized in a consanguineous Pakistani kindred to 6q26-q27. The affected family members present with profound prelingual sensorineural hearing impairment and use sign language for communications. Linkage was established to microsatellite markers located on chromosome 6q26-q27 (Multipoint lod score 3.6). The genetic region for DFNB38 spans 10.1 cM according to the Marshfield genetic map and is bounded by markers D6S980 and D6S1719. This genetic region corresponds to 3.4 MB on the sequence-based physical map.
OBJECTIVE: The authors investigated knowledge, attitudes, and beliefs about mental illness and providers held by a group of deaf adults. METHOD: The American Sign Language interviews of 54 deaf adults were analyzed. RESULTS: Recurrent themes included mistrust of providers, communication difficulty as a primary cause of mental health problems, profound concern with communication in therapy, and widespread ignorance about how to obtain services. CONCLUSIONS: Deaf consumers' views need due consideration in service delivery planning. Outreach regarding existing programs is essential.
The increasing digital skill made manifest in the development of instrumental music is reviewed and supports the hypothesis that the fifth digit was belatedly used even when a suitable instrument had already become established. With notable exceptions, such as the harp, it appears that progressive improvements in instrument design was influenced by the need to utilize the fifth digits. Its greater use was accomplished in overlapping stages of increasing dexterity, of which the highest is exemplified by the left hand of the violinist. The apparent evolutional background of these observations is discussed. It is proposed that man has an inherent atavistic tendency to ignore the fifth digit and this view agrees with its neglect in gesture and sign language.
Congenital disorders of glycosylation, formerly called carbohydrate-deficient glycoprotein syndrome, may present in infancy with slowly progressive neurologic deficits including cognitive impairment, ataxia, pigmentary retinal degeneration, and neuropathy. The metabolic defect is in N-linked oligosaccharide synthesis, and diagnosis is made by a serum transferrin isoelectric focusing. We reviewed the neurologic course of 10 children with congenital disorders of glycosylation (ages 13 months to 7 years). All had severe developmental delay and ataxia; none walked unassisted, and the highest level of communication was simple sign language in one patient. Five of 10 children had seizures (absence, complex partial, tonic clonic). Only one patient has had strokelike episodes, despite reports that they are common in this population. The underlying basis of these episodes has been hypothesized to be coagulopathy due to dysfunctional, incorrectly glycosylated coagulation factors. This 5-year-old patient with congenital disorders of glycosylation type Ia had two strokelike episodes, with evolving hemiparesis over 5 to 6 days' duration, followed by focal tonic-clonic seizures. Coagulation studies were normal. Electroencephalography showed transient hemispheric polymorphous delta-range slowing and suppression. Magnetic resonance imaging revealed corresponding cortical swelling. Magnetic resonance angiography was normal. Magnetic resonance spectroscopy revealed a decrease in the N-acetylaspartate peak, suggesting neuronal loss, with normal lactate peak. The neuroradiologic data do not support a thrombotic, embolic, or hemorrhagic basis for strokelike episodes in carbohydrate-deficient glycoprotein syndrome; other mechanisms must be considered.
We report a case of dystonia with a partial deletion of the short arm (p) of chromosome 18 and androgen insensitivity. Neurologic findings in the 18p syndrome are reported to include mental retardation, seizures, incoordination, tremor, and chorea. A 15-year-old girl with a denovo 18p deletion [karyotype 46, XY, del (18)(p11.1)] developed progressive asymmetric dystonia. She had oromotor apraxia and partial expressive aphasia since childhood, and she was able to partially communicate through elementary sign language. At the age of 15 years, she developed subacute and progressive choreic movements of the right arm, severe dystonic posturing of the left arm, and spastic dystonia in both legs. Her response to parenteral or oral benzodiazepines, oral trihexyphenidyl, benztropine mesylate, baclofen, and L-dopa were brief and inadequate. The response to intrathecal baclofen has been sustained over 18 months. In all likelihood, the 18p deletion syndrome affecting this patient is significant in the pathogenesis of her acquired dystonia. Chronic intrathecal baclofen therapy via pump has been effective in this case and should be considered as a treatment modality in carefully selected patients with dystonia.
Transient mutism resolving to cerebellar speech after posterior fossa surgery is a well-recognized phenomenon, particularly in pediatric patients. The anatomic basis for this postoperative functional change is unclear but may reside in the dominant superior cerebellar hemisphere or the medial deep cerebellar nuclei. We report a case of an 8-year-old girl who presented for surgical resection of a cavernous malformation of the right pons (at the level of the middle cerebellar peduncle) after hemorrhage. Preoperatively, her complaints consisted of contralateral motor deficits. She had normal speech. Her lesion was resected through a subtemporal approach to the pons. She awoke unable to speak. She was able to communicate through a variety of verbal cues, including sign language. Her mutism lasted 12 days after which she underwent a prolonged period of slowly resolving cerebellar dysarthria. Her preoperative motor deficits also slowly resolved. This is the first reported case of mutism resolving to cerebellar dysarthria after a supratentorial approach to the brain stem. We discuss the anatomic basis for postoperative mutism in light of previous observations combined with the unusual finding of mutism after pontine surgery. In particular, reports of mutism after bilateral cerebellar hemispheric injury, bilateral or unilateral medial deep nuclear injury, and, now, pontine tegmental injury implicate the superior cerebellar hemispheres, the deep cerebellar nuclei, and the nuclear outflow through the superior cerebellar peduncle as the anatomic bases for cerebellar participation in the production of human speech.
This qualitative case study is a description of a young man with autism who communicated using speech, sign language, facilitated communication, body language, and his mother's conversational supports. Participant observation, interviews, and review of records were used to explore his current and past communication practices. These practices illustrate his preference for speaking and the complexity of choosing among communication means on an ongoing basis. Although the young man and his mother differed in their thinking about communication, they revealed a common goal: for Michael to participate in ordinary life activities as a member with a voice.
The aim of the present study was to advance the knowledge of the linguistic development of students with prelingual profound deafness, especially the acquisition and use of prepositions in Spanish, a lexical category with an important role in the verbal comprehension. The researchers sought to learn the level of mastery students with prelingual profound deafness can achieve in the command of prepositions, depending on the system of communication they have been exposed to: classic oralism, Cued Speech, or signed language. The results show that the different systems of communication contribute, to different degrees, to the acquisition of Spanish prepositions, with the best results being obtained with Cued Speech.