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色素性视网膜炎与耳聋。

Retinitis pigmentosa and deafness.

作者信息

Mills R P, Calver D M

出版信息

J R Soc Med. 1987 Jan;80(1):17-20. doi: 10.1177/014107688708000108.

DOI:10.1177/014107688708000108
PMID:3560119
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1290625/
Abstract

Seventeen patients with retinitis pigmentosa (RP) have been investigated audiologically. Of 9 found to have a significant hearing loss, 6 were examples of Usher's syndrome; these patients had a cochlear pattern of hearing loss. The other 3 were examples of Senior's syndrome, Kearne-Sayre syndrome and Lawrence-Moon-Biedle syndrome respectively. Two of these patients had absent stapedius reflexes. It is suggested that patients with different RP-deafness syndromes may have lesions in different parts of the auditory system.

摘要

对17例视网膜色素变性(RP)患者进行了听力检查。在9例发现有明显听力损失的患者中,6例为Usher综合征;这些患者有耳蜗型听力损失。另外3例分别为Senior综合征、Kearne-Sayre综合征和Lawrence-Moon-Biedle综合征。其中2例镫骨肌反射消失。提示不同的RP-耳聋综合征患者可能在听觉系统的不同部位有病变。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a2cd/1290625/7bb512e42c02/jrsocmed00179-0021-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a2cd/1290625/7bb512e42c02/jrsocmed00179-0021-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a2cd/1290625/7bb512e42c02/jrsocmed00179-0021-a.jpg

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引用本文的文献

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本文引用的文献

1
Juvenile familial nephropathy with tapetoretinal degeneration. A new oculorenal dystrophy.伴有视网膜色素变性的青少年家族性肾病。一种新的眼肾营养不良症。
Am J Ophthalmol. 1961 Nov;52:625-33. doi: 10.1016/0002-9394(61)90147-7.
2
Hereditary renal dysplasia and blindness.遗传性肾发育不全与失明。
Acta Paediatr (Stockh). 1961 Mar;50:177-84. doi: 10.1111/j.1651-2227.1961.tb08037.x.
3
Retinal degeneration combined with obesity, diabetes mellitus and neurogenous deafness: a specific syndrome (not hitherto described) distinct from the Laurence-Moon-Bardet-Biedl syndrome: a clinical, endocrinological and genetic examination based on a large pedigree.
视网膜变性合并肥胖、糖尿病和神经性耳聋:一种不同于劳伦斯-穆恩-巴德-比德尔综合征(迄今未描述过)的特定综合征:基于一个大家系的临床、内分泌学和遗传学检查
Acta Psychiatr Neurol Scand Suppl. 1959;129:1-35.
4
Retinitis pigmentosa, external ophthalmophegia, and complete heart block: unusual syndrome with histologic study in one of two cases.色素性视网膜炎、眼外肌麻痹和完全性心脏传导阻滞:一种不寻常的综合征,对两例中的一例进行了组织学研究。
AMA Arch Ophthalmol. 1958 Aug;60(2):280-9.
5
Morphogenesis of the retinal rods; an electron microscope study.视网膜视杆细胞的形态发生;电子显微镜研究
J Biophys Biochem Cytol. 1956 Jul 25;2(4 Suppl):209-18. doi: 10.1083/jcb.2.4.209.
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Heterogeneity of retinal degeneration and hearing impairment syndromes.
Am J Ophthalmol. 1980 Dec;90(6):755-67. doi: 10.1016/s0002-9394(14)75190-6.
7
Mitochondrial cytopathy. A multisystem disorder with ragged red fibres on muscle biopsy.线粒体细胞病。一种多系统疾病,肌肉活检可见破碎红纤维。
Arch Dis Child. 1981 Oct;56(10):741-52. doi: 10.1136/adc.56.10.741.
8
Cockayne syndrome--an audiologic and temporal bone analysis.科凯恩综合征——听觉及颞骨分析
Am J Otol. 1984 Apr;5(4):300-7.
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Vestibular and auditory function in Usher's syndrome.
Ann Otol Rhinol Laryngol. 1984 Nov-Dec;93(6 Pt 1):600-8. doi: 10.1177/000348948409300613.
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Hearing loss in retinitis pigmentosa.视网膜色素变性中的听力损失。
Pediatrics. 1967 Nov;40(5):875-80.