Linked Life Data

2294949

Source:http://linkedlifedata.com/resource/pubmed/id/2294949

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
1
pubmed:dateCreated
1990-2-20
pubmed:abstractText
It has been reported that cochlea is the lesion of hearing loss in FSH. However, the details of this lesion are not yet sufficiently known. We performed detailed audiologic studies to examine hearing loss in FSH. We experienced 2 cases of FSH associated with hearing loss. Case 1 was a girl aged 5 years, and case 2 a boy aged 15 years. Clinical findings, EMG and muscle biopsy gave a diagnosis of FSH in both cases. Hearing loss was evaluated by pure tone audiography, speech audiography, tympanometry, stapedial reflex, auditory brain stem response and electrocochleography. In case 1, pure tone audiograms revealed high tone hearing loss without an A-B gap. On speech audiography, the maximum articulation score was 100% and proved normal. The tympanogram was type A. Stapedial reflex was normal bilaterally. The threshold of the 5th wave increased markedly on auditory brain stem response. On electrocochleography, the H-curve of the input-output function curves of action potential was recorded, but the L-curve was absent. There were no complaints of hearing loss in case 2, but pure tone audiograms revealed high-tone hearing loss without an A-B gap. The tympanogram was type A. Stapedial reflex was normal bilaterally. On auditory brain stem response, threshold was increased and latency was prolonged when intensity was lowered. The electrocochleograms were almost normal. It has been reported that, in electrocochleography, the L-curve represents the function of the outer hair cells and the H-curve that of the inner hair cells. The electrocochleograms in case 1 showed damage to the outer hair cells.(ABSTRACT TRUNCATED AT 250 WORDS)
pubmed:language
jpn
pubmed:journal
pubmed:citationSubset
IM
pubmed:status
MEDLINE
pubmed:month
Jan
pubmed:issn
0029-0831
pubmed:author
pubmed:issnType
Print
pubmed:volume
22
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
24-9
pubmed:dateRevised
2008-12-25
pubmed:meshHeading
pubmed:year
1990
pubmed:articleTitle
[Facioscapulohumeral muscular dystrophy (FSH) and hearing loss].
pubmed:affiliation
Department of Pediatrics, University of Tsukuba.
pubmed:publicationType
Journal Article, English Abstract, Case Reports