Auditory brainstem responses in young children with Down's syndrome. 1986

K Kaga, and R R Marsh

Auditory brainstem evoked responses (ABRs) were studied in 37 Japanese infants and children with Down's syndrome to determine the level of the lesion causing their unresponsiveness to sound stimuli. ABR thresholds, peak latencies of waves I and the peak interval latency of wave I-V were measured. For comparison with ABR thresholds, behavioral audiometry was performed. Thirty-two percent of the patients (12 cases) with Down's syndrome showed no responses by ABR as well as behavioral audiometry. The other ABR abnormalities with respect to age-matched controls were classified into 6 types: shorter wave I latencies (2 cases, 5%), shorter wave V latencies (7 cases, 19%), shorter wave I-V intervals (8 cases, 22%), wave I prolongation (13 cases, 35%), wave V prolongation (5 cases, 14%) and prolongation of wave I-V peak interval (no case, 0%). Our results demonstrate a high incidence of hearing loss at middle and inner ear levels, and suggest some anomaly within the auditory brainstem in Japanese cases of Down's syndrome.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D008297 Male Males
D001933 Brain Stem The part of the brain that connects the CEREBRAL HEMISPHERES with the SPINAL CORD. It consists of the MESENCEPHALON; PONS; and MEDULLA OBLONGATA. Brainstem,Truncus Cerebri,Brain Stems,Brainstems,Cerebri, Truncus,Cerebrus, Truncus,Truncus Cerebrus
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D004314 Down Syndrome A chromosome disorder associated either with an extra CHROMOSOME 21 or an effective TRISOMY for chromosome 21. Clinical manifestations include HYPOTONIA, short stature, BRACHYCEPHALY, upslanting palpebral fissures, epicanthus, Brushfield spots on the iris, protruding tongue, small ears, short, broad hands, fifth finger clinodactyly, single transverse palmar crease, and moderate to severe INTELLECTUAL DISABILITY. Cardiac and gastrointestinal malformations, a marked increase in the incidence of LEUKEMIA, and the early onset of ALZHEIMER DISEASE are also associated with this condition. Pathologic features include the development of NEUROFIBRILLARY TANGLES in neurons and the deposition of AMYLOID BETA-PROTEIN, similar to the pathology of ALZHEIMER DISEASE. (Menkes, Textbook of Child Neurology, 5th ed, p213) Mongolism,Trisomy 21,47,XX,+21,47,XY,+21,Down Syndrome, Partial Trisomy 21,Down's Syndrome,Partial Trisomy 21 Down Syndrome,Trisomy 21, Meiotic Nondisjunction,Trisomy 21, Mitotic Nondisjunction,Trisomy G,Downs Syndrome,Syndrome, Down,Syndrome, Down's
D005072 Evoked Potentials, Auditory The electric response evoked in the CEREBRAL CORTEX by ACOUSTIC STIMULATION or stimulation of the AUDITORY PATHWAYS. Auditory Evoked Potentials,Auditory Evoked Response,Auditory Evoked Potential,Auditory Evoked Responses,Evoked Potential, Auditory,Evoked Response, Auditory,Evoked Responses, Auditory,Potentials, Auditory Evoked
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D001300 Audiometry, Evoked Response A form of electrophysiologic audiometry in which an analog computer is included in the circuit to average out ongoing or spontaneous brain wave activity. A characteristic pattern of response to a sound stimulus may then become evident. Evoked response audiometry is known also as electric response audiometry. Audiometry, Electroencephalic Response,Electrocochleography,Evoked Response Audiometry,Audiometries, Electroencephalic Response,Audiometries, Evoked Response,Electrocochleographies,Electroencephalic Response Audiometries,Electroencephalic Response Audiometry,Evoked Response Audiometries,Response Audiometries, Electroencephalic,Response Audiometries, Evoked,Response Audiometry, Electroencephalic,Response Audiometry, Evoked

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