[Deafness and ataxia due to meningitis in children].
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Biomedical subjects
Publications and source records attributed to K Kaga.
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The patient was a right-handed boy. Pregnancy and delivery were normal. There were no neonatal complications. His development was normal until the age of 6 years and 10 months when he suddenly fell into coma. He was admitted to a hospital, where he was diagnosed as having encephalopathy of unknown cause. It was found that he did not respond to verbal stimuli after recovery from coma. He was referred to our hospital for detailed examination of hearing at the age of 7 years, and received neuroradiological and audiological examinations including X-ray CT, positron-CT, behavioral audiometry, auditory evoked response audiometry as well as other hearing and speech tests. Pure tone audiometry showed that auditory thresholds for pure tones ranging from 125 to 8,000 Hz were normal, while speech audiometry demonstrated that he was unable to discriminate test words. However, he accurately recognized environmental sounds and noises as well as sounds produced by musical instruments. The decreased uptake of 11C-glucose at the left temporal lobe was demonstrated by positron CT, although no abnormality was showed by X-ray CT. WISC-R showed he had normal intelligence. ITPA demonstrated that the psycholinguistic ability via visual channel remained intact, whereas auditory memory and auditory closure were markedly impaired. The boy had no difficulties in reading, naming and writing. Speech therapy was started as soon as diagnosis of word deafness was made. Until 6 months after onset, he never understood and repeated what was said to him.(ABSTRACT TRUNCATED AT 250 WORDS)
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Auditory brain stem evoked responses (ABRs) and behavioral thresholds were studied in 30 infants with infantile spasms to determine the level of the lesion causing their inattention to sound stimuli. ABR thresholds, peak latencies of waves I and V and the peak interval latency of wave V-I were measured; behavioral audiometric thresholds were determined through conditioned-orientation-reflex (COR) audiometry. Thirty per cent of the infants (9 cases) with infantile spasms showed ABR abnormalities with respect to age-matched controls. The ABR abnormalities were classified into partial disappearance of latter waves (5 cases), prolongation of the wave V-I peak interval (3 cases) and no response (1 case). These findings are clear evidence that brain stem involvement may occur in infantile spasms. Eighty-six per cent of the patients showed threshold elevation by behavioral audiometry which may reflect by cortical dysfunction inherent in psychomotor retardation, as there was no peripheral hearing loss. Our results reveal that brain stem involvement can occur in patients with infantile spasms and their poor responses to sound are due to psychomotor retardation.
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.
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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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A forty years old woman with hysterical deafness is reported. Chief complaints were bilateral hearing loss. Nothing particular was found in her past and family history. In 1977, on the 11th day of May, she was admitted to A city hospital because of headache and paresis of right limb. As angiography revealed an aneurysms of her anterior communicating artery, she was undertaken the surgery of clipping and coating of the aneurysms. Post-operatively, left hemiparalysis appeared and paresis of right limb developed because of spasm of right middle cerebral artery. On the 14th day of August, ventricular-peritoneal shunt's operation was performed. As soon as she recovered from postoperative coma, she complained of bilateral hearing loss. Because pure tone audiometry demonstrated complete loss of her hearing, she was referred to ENT department of Teikyo University Hospital. Findings were as follows: 1) She had a queer way of hearing because she could understand to hear limited persons' speech (her doctor and husband). 2) Pure tone audiometry showed complete loss of her hearing but the thresholds of auditory brain stem responses were 15 dB and those of slow vertex responses were 45 dB. These results suggested no lesion in cochlea and brain stem. 3) Rorschach test and sentence complete test were performed. The results of these tests suggested hysterical state or neurotic state. 4) Total intelligent quotients by WAIS were 69 which indicated borderline level. However, this value appeared to be incorrect because she was uncooperative. 5) CT scan revealed low density areas at right temporo-parietal lobes and left temporal lobe which were localized and small. Our findings suggested hysterical deafness but not auditory agnosia. During three years, she was referred to several hospitals for rehabilitation but didn't become well at all. On the third year of the onset, her husband became sick and admitted to her room of the same hospital. During that period, suddenly, she talked her hearing to improve and the pure tone audiometry demonstrated decrease in threshold. In conclusion, this event could give a final diagnosis of hysterical deafness but not auditory agnosia.
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Development correlates of auditory brainstem evoked responses (ABRs) and behavioral audiometry in a total of 112 normal subjects consisting of 78 infants (age, 1 to 18 months), 24 children (age, 2 to 5 years), and ten adults (age, 18 to 22 years) were studied to provide normative data for audiological and neurological applications. Thresholds of ABR, as determined by minimum stimulus intensities evoking wave V, decreased with age. Neonates had the highest ABR thresholds, and adults had the lowest. A similar pattern was observed for behavioral sensitivity as that for the ABR thresholds. Response functions determined by both methods converged with age. They crossed between the ages of 2 and 3 years. There was a trend for all peak latencies to decrease with age. This effect was particularly pronounced for the later ABR components.
Twenty cases which showed partial loss of ABR waves were reported. Only wave I was recorded in 18 ears tested. Wave I and II; I-III; and I-IV responses were observed in four, eight, and five ears tested, respectively. Cases included pontine glioma (8), acoustic tumor (4), West syndrome (2), leukodystrophy (2), facial neurinoma (1), epidermoid tumor (1), pontine bleeding (1), and unknown cause (1). Fourteen of 20 were tumor cases. This fact indicates that if the ABR shows partial loss of waves, the examiner should first suspect the space-occupying lesions of the brain stem.
Serial studies of auditory brainstem evoked responses (ABR) and slow vertex responses (SVR) were obtained during the progress of adrenoleukodystrophy in a 6-year-old boy. This child was normal until 5 years of age. His illness began with a gait disturbance, dysarthria, and hearing difficulty. Later, spastic paralysis, serious deafness, and blindness appeared. He died of respiratory failure 2 years after the onset. The ABR was normal at onset but changed to an abnormal pattern. Initially, there was lengthening of the wave V-I interpeak interval. This was followed by the disappearance of the later components as his general condition deteriorated. At the terminal stage, only a prolonged wave I was recordable. The postmortem pathology revealed demyelination of auditory nerves and remarkable neuronal loss in the auditory pathways of the brainstem; in addition, there was a variety of extensive degeneration throughout the cerebrum, in particular the complete degeneration of the white matter with secondarily occurring ganglionic cell changes. These date suggest that degeneration of the brainstem from rostral to caudal levels occurred.
The origin of the scalp-recorded auditory evoked potential, Pa, was examined in cats anesthetized with chloralose-urethane and immobilized with gallamine triethiodide. This potential is a prominent positive wave which peaks approximately 12--15 msec following click stimuli. Mapping revealed that Pa is distributed on the scalp in the region overlying cortical area AI, contralateral to the stimulated ear. The cortical potential recorded from AI was a surface-positive wave, restricted to the anterior portion of AI. Laminar analysis of the cortical evoked potentials demonstrated the existence of a dipole generator at that area. The onset of this potential coincided with the onset of the scalp-recorded Pa. Comparison of the scalp and the cortex-recorded potentials showed that both the amplitude-intensity function and the amplitude-rate function for the scalp-recorded potential closely paralleled those recorded from AI. Acute and chronic lesion studies showed that extirpation of AI (particularly the anterior part) almost completely abolished the Pa response. This evidence indicates that the scalp-recorded Pa of cats is generated almost entirely from the anterior part of the contralateral AI.