M. Noterdaeme (1), H. Amorosa (1), K. Mildenberger (2), S. Sitter (2), F. Minow (3)
Children with autism and children with a specific language disorder show additional atention deficits. The literature on the neuropsychological investigation of attention in both groups of children suggests that the nature of their attention problems might be different. The purpose of this study is to examine the attention test profiles in these two groups of children with developmental disorders. Nineteen children and adolescents with autism, 17 subjects with a specific language disorder and 19 control subjects participated in the study. Non-verbal intelligence was normal for all subjects. The "Testbatterie zur Aufmerksamkeitsprüfung" was administered to all subjects. This instrument provides the possibility to examine a wide range of attention functions and executive functions. The results showed that the autistic individuals had deficits in executive functions, whereas the language impaired children had deficits in auditory sustained attention, in auditory selective attention, and in the domain of executive functions. It is concluded that although both groups of developmentally impaired subjects showed attention problems, the deficits are not the same in both groups. The different neuropsychological profiles probably reflect different mechanisms in the pathogenesis of the attention deficits in both types of developmental disorders. Article in PDF format (84 KB) Online publication: March 15, 2001 LINK Helpdesk © Steinkopff Verlag 2001
Selz PA; Girardi M; Konrad HR; Hughes LF
Division of Otolaryngology, Department of Surgery, Southern Illinois University School of Medicine, Springfield, 62794-9230, USA.
Considerable knowledge has been accumulated regarding acquired and congenital deafness in children. However, despite the intimate relationship between the auditory and vestibular systems, data are limited regarding the status of the balance system in these children. Using a test population of 15 children, aged 8 to 17 years, we performed electronystagmography testing. The test battery consisted of the eye-tracking (gaze nystagmus, spontaneous nystagmus, saccade, horizontal pursuit and optokinetic) tests, positional/positioning (Dix-Hallpike and supine) tests, and rotational chair tests. With age-matched controls, five children were tested in each of the following three categories: normal hearing, hereditary deafness, and acquired deafness. The children in the hereditary deafness category were congenitally deaf and had a family history of deafness. Those subjects in the acquired deafness category had hearing loss before the age of 2 years, after meningitis. Analysis of variance demonstrated significant differences between the two deaf groups and the control subjects in the gaze nystagmus test, saccade latencies, horizontal pursuit phase, and Dix-Hallpike and supine positionally provoked nystagmus. Also, significant differences were found in rotational chair gain and phase between the deaf and normal-hearing children. The children with acquired deafness exhibited the most profound results. In addition, there were significant differences in rotational chair gain between the acquired and congenitally deaf children. No differences were noted in horizontal pursuit gains, saccade accuracies, or saccade asymmetries. These preliminary data demonstrate that the etiologic factors responsible for congenital and acquired deafness in children may indeed affect the balance system as well. These findings of possible balance disorders in conjunction with the profound hearing loss in this patient population will have prognostic implications in the future evaluation, treatment, and rehabilitation of these patients.
Murdoch H
School of Education, University of Birmingham, England.
Stereotyped behaviors frequently cause anxiety to parents and educators, and behaviors that appear stereotyped may prompt negative reactions. There has been little research on whether deaf or hard of hearing children display such behaviors. This study surveyed 390 students aged 3-24 years attending 3 residential schools for the deaf and hard of hearing in the United Kingdom. At least 1 additional disability was reported in 79% of the students. The main findings were that: deaf and hard of hearing children without additional disabilities did not show apparently stereotyped behaviors; approximately 35% of children with additional disabilities did show such behaviors; some disabilities, in addition to hearing loss, were more likely than others to be associated with stereotyped behaviors. Implications of these findings for further research are considered with a view toward making recommendations for educational practice.
Sign language tics in a prelingually deaf man.
Morris HR, Thacker AJ, Newman PK, Lees AJ National Hospital for Neurology and Neurosurgery, London, UK.
Gilles de la Tourette syndrome is characterized by vocal and motor tics starting in childhood. Vocal tics may be either noises or words, and the vocal language tics may consist of obscenities (coprolalia) and repetitions of speech that has been heard (echolalia). We describe a prelingually deaf man who has the full array of tics seen in Gilles de la Tourette syndrome, but in whom vocal language tics are replaced by equivalent sign language tics. This is, to our knowledge, the first report of sign language tics in a person with prelingual deafness. The implications of this phenomenon for the separation between language and ideas in tics and the equivalence of sign language to spoken language is discussed.
Autism and hearing loss
Rosenhall U, Nordin V, Sandstrom M, Ahlsen G, Gillberg C
Department of Audiology, Karolinska Hospital, Karolinska Institute, Stockholm, Sweden. uro@ent.ks.se
A group of 199 children and adolescents (153 boys, 46 girls) with autistic disorder was audiologically reviewuated. Mild to moderate hearing loss was diagnosed in 7.9% and unilateral hearing loss in 1.6% of those who could be tested appropriately. Pronounced to profound bilateral hearing loss or deafness was diagnosed in 3.5% of all cases, representing a prreviewence considerably above that in the general population and comparable to the prreviewence found in populations with mental retardation. Hearing deficits in autism occurred at similar rates at all levels of intellectual functioning, so it does not appear that the covariation with intellectual impairment per se can account for all of the variance of hearing deficit in autism. Hyperacusis was common, affecting 18.0% of the autism group and 0% in an age-matched nonautism comparison group. In addition, the rate of serous otitis media (23.5%) and related conductive hearing loss (18.3%) appeared to be increased in autistic disorder. The study emphasizes the need for auditory reviewuation of individuals with autism in order to refer those with pronounced to profound hearing loss for aural habilitation and to follow those with mild to moderate hearing loss because of the risk of deterioration.
Minor malformations and physical measurements in autism: data from Nova Scotia.
Rodier PM, Bryson SE, Welch JP
Department of Obstetrics and Gynecology, University of Rochester, New York 14642, USA.In the context of an epidemiological study of autism in Nova Scotia, subjects were evaluated for minor physical anomalies and physical measurements. Normal control children, children with autism and their siblings, and children with developmental disabilities and their siblings were compared. Posterior rotation of the external ears was found to be a characteristic related to autism specifically, rather than to developmental disabilities in general. Small feet and normal-to-large hands also were observed in the autism group. Children with autism had a significant reduction in interpupillary distance, but not intercanthic distance or head circumference. In contrast, children with other developmental disabilities were notable for general small stature, which affected the hands, feet, eyes, and head size, as well as height. Abnormal ear configuration was the minor malformation most characteristic of the developmental disability group, and the subset of Down syndrome children had single transverse creases of the palm and epicanthic folds that resulted in significantly increased rates of these anomalies in the developmentally disabled controls. Siblings of the two disabled groups were not significantly different from normal controls on any of the measures that characterized children with autism or other developmental disabilities. The results agree with those of several previous studies, which have suggested that abnormalities of the ears are the general category of minor anomalies most associated with autism. Recent evidence regarding the embryological origin of autism suggests that the ear effects may be an important marker of the initiating events that lead to the disorder.
Fractionation of visual memory: evidence from a case with multiple neurodevelopmental impairments.
Cipolotti L, Robinson G, Blair J, Frith U Department of Clinical Neuropsychology, National Hospital for Neurology and Neurosurgery, Queen Square, London, UK. l.cipolotti@ion.ucl.ac.uk
It is known that the adult visual memory system is fractionable into functionally independent cognitive subsystems, selectively susceptible to brain damage. However, it is unclear whether these cognitive subsystems can fractionate developmentally. The present study describes an investigation of visual memory of a patient (PE) with multiple developmental disorders. PE was congenitally deaf, had Gilles de la Tourette syndrome and autism, with non-verbal ability in the normal range. The patient presented with a recognition memory impairment for unknown human faces. This contrasted with his superior recognition memory for unknown buildings, landscapes and outdoor scenes. PE's memory impairment for faces could not be explained by a general deficit in face processing. Interestingly, PE also showed a recognition memory impairment for animals. These findings indicate that different domains of the visual memory system can be fractionated developmentally. In particular, it demonstrates that topographical memory can develop independently from other aspects of visual memory.
Auditory brainstem response abnormalities in autistic children.
Taylor MJ, Rosenblatt B, Linschoten L
In an attempt to clarify issues of brainstem dysfunction and hearing thresholds in autistic children, we studied the Auditory Brainstem Responses (ABRs) in 32 children who clearly fit within the criteria of autism established by the National Society for Autistic Children (1977). ABRs were recorded between Cz and ipsilateral ear in response to click stimuli. Interwave latencies and auditory threshold in each ear were determined. Of the 32 children, 11 had moderate hearing loss (8 bilaterally) and 3 had severe to profound hearing loss, all bilaterally. 8 of the 14 with hearing loss also had associated features (e.g., perinatal encephalopathy). The I-III and I-V interwave latencies were significantly longer in the autistic children compared to normal control children; the increased conduction times were found mainly in the early portion of the auditory brainstem pathway. These data confirm some earlier reports of ABR abnormalities in autistic children and are concordant with some theories of the etiological basis of autism. The high incidence of hearing loss in these children is significant and routine ABR testing is recommended.
Hearing-impaired autistic children.
Jure R, Rapin I, Tuchman RF
Saul R. Korey Department of Neurology, Rose F. Kennedy Center
for Research in Mental Retardation and Human Development,
Albert Einstein College of Medicine, Bronx, NY.
The charts of 46 children diagnosed as deaf and autistic were reviewed. Nearly one-fifth had normal or near-normal non-verbal intelligence and only one-fifth had severe mental deficiency. The severity of the autistic behavior was related to the severity of the mental deficiency, but not to that of the hearing loss. In 11 of the 46 children, autism went unrecognized for over four years after the diagnosis of hearing loss, and in 10 the hearing loss went unrecognized for several years after the diagnosis of autism. The educational experience of some children was generally disastrous because of the frequently late and incorrect diagnoses and the lack of specialized facilities for hearing-impaired autistic children.
A comparative study by the behavioral observation for sterotypy in the exceptional children.
Sakuma M
1. Stereotyped behavior as a type of repetitive behavior was studied by observing four major groups of exceptional children by a natural observation method. The total 400 subjects are made up as follows: 46 with visual disturbance, 85 with auditory disturbance, 29 with infantile autism and 240 with mental retardation. 2. From analyses of our observational data, it was inductively proved that each group has its own characteristic sterotypy. There were marked differences in stereotypy between the auditory disturbance group and the visual disturbance group. Autistic children showed a perfect form of stereotypy and outstandingly bizarre characteristics. It was also found that blindism and autism have much in common. Stereotyped behavior shown by mentally retarded children differed with different intelligence levels. 3. There was no significant difference in the stereotypy appearance rate among the blind as well as the deaf-mute. Comparison between the blind with mental retardation and the blind with normal intelligence showed that there was significant difference of 0.5% or less. When taking the intelligence factor into account, significant differences aroused. As against the autism group, each group showed a significant difference of over 0.5%. In conclusion, it can be said that mental retardation factor has close bearing on the stereotypy appearance rate. 4. With respect to the cause of stereotyped behavior, the author based its argument on its own theory that blindism accrues from congenital visual disturbance. Weighing the fact that stereotypy differs between the blind and the deaf-mute, the author discussed with reference to the theory developed by Kaufman and Berkson that the inability of the child to monitor normal stimulation due to sensory disturbance produces frustration in him and this frustrated bodily need manifests itself in stereotyped movements. As for stereotypy of the mentally retarded children, the author pointed out that the arousal level theory is applicable only to the cases of severe-grade mental regardation, and that it fails to explain the parallelism between the differences of intelligence level and stereotypy. As regards stereotyped movements of the autistic children, due consideration was given to subtleties in action and bizarre nature of their behavior. Based on Orniz's theory that autistic children lack the constancy of central perceptual reception, the author maintained that the occurrence of stereotypy in autistic children suggests the underlying mechansims of perception are functioning pathologically as well as neurophysiologically. 5. As our observation as been conducted for a short period of time, the results might have been rather cross sectional. But the author thinks this shortcoming would have been made up for by Rutter's five-year follow-up study and the records available at the institutions which proved autism-stereotypy has continued to appear for a long period.
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