[Diagnostic procedures for hearing disorders in infants].
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Biomedical subjects
Publications and source records attributed to M Kaga.
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The auditory brainstem response (ABR) was studied in an infant with Gaucher's disease. The infant was normal until the age of three months. His illness began with stridor, strabismus, inguinal hernia and failure to thrive. Thereafter, muscular rigidity with opisthotonus, ocular palsies, difficulty in swallowing and respiratory failure due to central origin developed. He died of respiratory failure due to central origin developed. He died of respiratory failure at the age of one year and four months. The ABR was abnormal at six and eight months of age. Initially, at the age of six months, there was a lengthening of the peak latencies of wave I, II and III and disappearance of the waves after IV. But at eight months, more marked lengthening of the peak latencies of the waves and the disappearance of waves after III were noted as his general condition deteriorated. The autopsy revealed relative preservation of the nuclei and tracts of the auditory pathways in the brainstem. The ABR was useful for monitoring the progress of the disease in this patient because it made detection of brainstem lesions possible.
Carcinogenicity study of cholestyramine, an anti-hypercholesterolemic agent, was carried out by feeding B6C3F1 mice of both sexes with the pellet diet in which cholestyramine was admixed at the rates of 1.25, 2.5 and 5%. The animals were fed on the drug-admixed diet for 18 months and on a normal diet for subsequent 3 months. After 32 weeks the mortality of male mice began to increase in the 5% cholestyramine group and the number of dead or moribund mice increased markedly after 60 weeks. Hemorrhage recognized in pleural cavity, heart and other organs, was suggested to be the main cause of death. Some kinds of tumors occurred in each group, but the tumor-incidences seen in mice of 1.25 and 2.5% cholestyramine groups were similar to those in mice of the control group and the occurrence of the specific tumor or an acceleration of tumor-development by feeding cholestyramine were not observed. Furthermore, the tumor-incidence in mice fed on the 5% cholestyramine diet was less than that in mice of the other three groups.
A child with auditory agnosia for verbal and nonverbal sounds was reported. He was born without difficulty and his early development was normal. At the age of 14 months, he suffered from herpes simplex encephalitis and fell in a trance. Soon after he recovered from the illness, he found not to pay any attention to sounds and not to speak any words. He was referred to Teikyo University hospital at 2.5 years of age for the hearing measurement. The average threshold of conditioned orientation reflex audiometry at 500, 1000 and 2000 Hz were 85 dB, while the threshold of auditory brain stem response was 15 dB which is normal. CT scan demonstrated bilateral lesions of superior temporal gyrus. Neurological examination revealed nothing particular except the troubles with hearing and speech. He was made a diagnosis of auditory agnosia. The speech therapy was started at two years old. During the first three years, he was taught how to communicate with gestures and lip reading. However, it was difficult for him to understand the meaning of gestures and lip reading. During these years he could acquire lip reading of simple words. As to environmental sounds, he could respond the telephone-bell and the organ at three years and seven months old, but could not discriminate other sounds. At five years old, finger spelling for his language education was introduced. He appeared to have learned finger spelling more easily and could read and write several letters through finger spelling within a month. One year after introduction of finger spelling, he acquired about 60 words (54 nouns and 6 verbs) and could read words and two-words sentences. However, the speed of his acquisition of language was very slow for his age. Now, he can understand mother's simple instructions and communicate with his mother and his speech therapist through finger spelling, but his responses toward environmental sounds are not stable yet. The course of this patient suggests that language acquisition of children with auditory agnosia might be different from that of deaf children. The prognosis of auditory agnosia in children is controversial. In this patient, the development of language is not good as well as in most previous reports, perhaps because of extensive lesions of language area. The patients with auditory agnosia is frequently mistaken for deaf or mentally retarded children. We emphasize that the early diagnosis and early speech therapy with visual communication are needed for these children.
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Age-hardening and metal-to-ceramic bonding were studied on Ni-Ta alloy for dental application. In the first experiment, it was shown the precipitation-hardening was recognized in Ni-33 wt% Ta, Ni-34 wt% Ta except for Ni-30 wt% Ta. The precipitates were of a Widmamstätten type structure and appeared to be a intermetallic compound, namely Ni3Ta, from the results of X-ray diffraction (debyesherrer) despite of the close proximity of the diffraction patterns of Ni and Ni2Ta. In the second experiment, it was shown that the bonding strength was 176 kg/cm2 for Ni-30 wt% Ta-ceramic system, compared with that of 222 kg/cm2 of Wiron S-ceramic system. The oxide layer was as wide as 3 approximately 5 micron at the Ni-30 wt% Ta-ceramic interface, in which high concentrations of Ni, Ta, and low concentrations of Al, Si were observed. The coefficient of thermal expansion, which was larger than that of porcelain, was 17.4 x 10(-6)/degrees C for Ni-30 wt% Ta.
The case of a 5-month-old male infant with 18p- mosaic, who has intractable seizures and severe ophthalmological abnormalities in addition to many clinical manifestations usually described in the 18p- syndrome, is reported. The proportions of abnormal cells are 7-8% in blood and 55% in skin. About 35% of the short arm of chromosome 18 is deleted. to our knowledge the present report is the fifth one of 18p-mosaic. The main interest of this case resides in the fact that it shows a serious clinical picture despite the low proportion of abnormal cells in blood and the small degree of deletion of the short arm of chromosome 18.
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Findings for auditory brainstem response (ABR) before and after surgical removal of a cerebellar tumor in a 10-year-old female are presented. ABR improved markedly, although the tumor showed no direct invasion to the brainstem. The cause of the ABR change and the origin of wave III are discussed.
Visual and somatosensory evoked potentials (VEPs and SEPs) were studied in 9 patients with Rett syndrome and compared with those in 6 patients with photosensitive progressive myoclonus epilepsy (PPME). In Rett syndrome, a giant III-IV amplitude of VEPs was present in 8 patients, although none exhibited giant II-III amplitudes. Four of 6 patients with Rett syndrome who demonstrated giant SEPs did not have a C reflex. Conversely, the patients with PPME demonstrated giant II-III and III-IV amplitudes in VEPs, and giant SEPs with concurrent positive C reflexes. It is concluded that the mechanism of altered cortical excitability in Rett syndrome is different from that in PPME.
Serial auditory evoked responses were investigated in 3 children with Krabbe disease. Auditory brainstem responses revealed prolongation of each wave component and interpeak latency with decreased amplitudes in later components which finally disappeared except for wave I. Long-latency auditory responses (LLRs) persisted in the advanced stage when all wave components of middle-latency auditory responses (MLRs) had disappeared. The results of auditory brainstem responses and MLRs are compatible with magnetic resonance imaging findings and a review of pathologic findings in Krabbe disease, including extensive involvement of brainstem and subcortical structures. It is suggested that the source of LLR waves is different from that of MLR because of the persistent existence of LLR waves. It is speculated that the cerebral cortex and/or subcortical U fibers, which are spared in Krabbe disease, have an important role in generating LLR wave components.
OBJECTIVES: The purpose of this study was to determine (1) the weakest zone of resin-dentin bonds and (2) the relation between bond strength and failure mode to clarify the effect of demineralized dentin. METHODS: Human premolars were sectioned to expose the dentin surfaces, and the dentin surfaces were conditioned with phosphoric acid for 15, 60, 120, or 180s. Resin-dentin bonded specimens were produced using two adhesives: One-Step (Bisco) and OptiBond Solo (Kerr). Each sample was sectioned to produce a beam (adhesive area: 0.9mm(2)). Microtensile bond tests were then conducted, and the mean bond strengths (n=12 for each group) were statistically compared using two-way ANOVA and Duncan's multiple-range test (p<0.05). The fractured surfaces of all specimens were examined using SEM, and the areas of failure were measured using an image analyzer. RESULTS: For One-Step, the bond strength decreased with increase in acid-conditioning time (15s: 50.7+/-9.7, 60s: 40.8+/-11.0, 120s: 23.6+/-4.9 and 180s: 12.1+/-4.6MPa) (p<0.05). For OptiBond Solo, the bond strength in the case of 15s acid-conditioning time (42.6+/-7.9MPa) was significantly greater than that for the other times (60s: 31.9+/-10.3, 120s: 31.8+/-14.4 and 180s: 31.8+/-7.4MPa) (p<0.05). Fractography showed that the area percentage of the hybrid layer increased with increase in etching time for both systems. CONCLUSIONS: The integrity of the hybrid layer, especially the top part, has an effect on bond strength.
An 8-year-old girl with chronic idiopathic intestinal pseudo-obstruction (CIIP), who is the first case of CIIP in Japan, has been receiving total parenteral nutrition (TPN) for more than 6 years. During this time, she experienced deficiencies of copper, zinc, vitamin A, vitamin B12, folic acid, and biotin, and an excess of vitamin A; she exhibited a series of signs and symptoms due to these deficiencies and vitamin A overdosage. Nevertheless, careful monitoring of serum levels of trace elements and vitamins and appropriate therapy have almost solved these problems. She has achieved normal physical and mental development and goes to school, while receiving home parenteral nutrition with an ambulatory infusion system.
A two year and ten-month-old girl with cerebello-brain stem leukodystrophy is presented. She was hypotonic with spasticity and showed delayed motor development until she was twelve months old, when regression of motor activities began. Progressive hypotonia, dysphagia and fatal respiratory abnormalities developed. Auditory brain stem response was absent. Pathological examination revealed primary demyelination with a predilection for the cerebellum, brain stem and spinal cord, in association with changes of the cerebellar cortex, pontine nuclei, inferior olivary nuclei and part of the basal ganglia. Clinical, pathological and biochemical studies revealed that this disease is different from metachromatic leukodystrophy, Krabbe's globoid cell leukodystrophy and adrenoleukodystrophy. The clinical entity of cerebello-brain stem orthochromatic leukodystrophy is discussed.