Acuidade visual e eletrorretinografia de campo total em pacientes com síndrome de Usher
Autor(a) principal: | |
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Data de Publicação: | 2005 |
Outros Autores: | , , , , |
Tipo de documento: | Artigo |
Idioma: | por |
Título da fonte: | Repositório Institucional da UNIFESP |
Texto Completo: | http://dx.doi.org/10.1590/S0004-27492005000200004 http://repositorio.unifesp.br/handle/11600/2490 |
Resumo: | PURPOSE: Usher's syndrome (US) is a group of genetically distinct autossomal conditions, characterized by sensorineural hearing loss accompanied by a retinal dystrophy indistinguishable from retinitis pigmentosa (RP). The purpose of this study was to analyze full-field electroretinography (ERG) and visual acuity (VA) among patients with type I and II Usher's syndrome. METHODS: Electroretinography responses and visual acuity were studied in 22 patients (mean age at test = 26.8±16.8 years). Seventeen patients had SU type I and 5 patients were diagnosed as Usher's syndrome type II. RESULTS: Mean visual acuity was 0.9 logMAR (20/160, Snellen equivalent) for patients with Usher's syndrome type I and 0.4 logMAR (20/50, Snellen equivalent) for patients with Usher's syndrome type II. Scotopic rod and maximal responses were non-detectable in both groups. Mean amplitude for oscillatory potentials was 14.5 µV ±6.1 in Usher's syndrome type I and 12.6 µV±5.2 in Usher's syndrome type II. Cone responses were non-detectable in 95% of the patients with Usher's syndrome I and in 100% of patients with Usher's syndrome II. Mean amplitude for 30 Hz flicker photopic cone response was 3.1 µV±4.1 for Usher's syndrome type I and 1.0 µV±0.6 for type II with mean implicit time of 34.0 ms±6.2 (US I) and 35.8 ms±3.1 (type II). CONCLUSIONS: Visual acuity was relatively preserved in both groups, however Usher's syndrome II group showed better visual acuity results. Electroretinography findings were severely reduced in both groups, with most patients showing non-detectable rod and cone responses. |
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Acuidade visual e eletrorretinografia de campo total em pacientes com síndrome de UsherVisual acuity and full-field electroretinography in patients with Usher's syndromeRetinitis pigmentosaUsher's syndromeElectroretinographyVisual acuityElectrophysiologyRetinal diseasesVisually impaired personsRetinite pigmentosaSíndrome de UsherEletrorretinografiaAcuidade visualEletrofisiologiaDoenças retinianasPortadores de deficiência visualPURPOSE: Usher's syndrome (US) is a group of genetically distinct autossomal conditions, characterized by sensorineural hearing loss accompanied by a retinal dystrophy indistinguishable from retinitis pigmentosa (RP). The purpose of this study was to analyze full-field electroretinography (ERG) and visual acuity (VA) among patients with type I and II Usher's syndrome. METHODS: Electroretinography responses and visual acuity were studied in 22 patients (mean age at test = 26.8±16.8 years). Seventeen patients had SU type I and 5 patients were diagnosed as Usher's syndrome type II. RESULTS: Mean visual acuity was 0.9 logMAR (20/160, Snellen equivalent) for patients with Usher's syndrome type I and 0.4 logMAR (20/50, Snellen equivalent) for patients with Usher's syndrome type II. Scotopic rod and maximal responses were non-detectable in both groups. Mean amplitude for oscillatory potentials was 14.5 µV ±6.1 in Usher's syndrome type I and 12.6 µV±5.2 in Usher's syndrome type II. Cone responses were non-detectable in 95% of the patients with Usher's syndrome I and in 100% of patients with Usher's syndrome II. Mean amplitude for 30 Hz flicker photopic cone response was 3.1 µV±4.1 for Usher's syndrome type I and 1.0 µV±0.6 for type II with mean implicit time of 34.0 ms±6.2 (US I) and 35.8 ms±3.1 (type II). CONCLUSIONS: Visual acuity was relatively preserved in both groups, however Usher's syndrome II group showed better visual acuity results. Electroretinography findings were severely reduced in both groups, with most patients showing non-detectable rod and cone responses.A síndrome de Usher (SU) é doença autossômica recessiva caracterizada por perda auditiva neuro-sensorial acompanhada de retinose pigmentária (RP). OBJETIVO: Analisar a eletrorretinografia de campo total (ERG) e a acuidade visual (AV) em pacientes com síndrome de Usher tipos I e II. MÉTODOS: Foram estudadas as respostas da eletrorretinografia de campo total e a acuidade visual de 22 pacientes (idade média = 26,8±16,8 anos). Destes, 17 tinham síndrome de Usher tipo I e 5 tinham síndrome de Usher tipo II. RESULTADOS: A acuidade visual média do grupo síndrome de Usher I foi de 0,9 logMAR (20/160, equivalente de Snellen) e do grupo síndrome de Usher II de 0,4 logMAR (20/50, equivalente de Snellen). As respostas dos bastonetes e as máximas respostas mostraram-se não detectáveis nos dois grupos. A amplitude média dos potenciais oscilatórios foi de 14,5 µV±6,1 na síndrome de Usher I e na síndrome de Usher II de 12,6 µV±5,2. As respostas de cones foram não detectáveis em 95% dos pacientes com síndrome de Usher I e em 100% dos pacientes com síndrome de Usher II. A amplitude média do flicker a 30 Hz nos pacientes com síndrome de Usher I foi de 3,1 µV±4,1 e do tempo de culminação de 34,0ms±6,2; nos pacientes com síndrome de Usher II a média de amplitude foi de 1,0 mV±0,6 e do tempo de culminação de 35,8 ms±3,1. CONCLUSÃO: A acuidade visual mostrou-se relativamente preservada nos dois grupos, porém com melhores resultados no grupo de síndrome de Usher II. Os achados eletrorretinográficos mostraram-se grandemente reduzidos em ambos os grupos, com a maioria dos pacientes apresentando respostas não detectáveis de bastonetes e cones.Universidade Federal de São Paulo (UNIFESP) Departamento de OftalmologiaUniversidade Federal de São Paulo (UNIFESP) Departamento de Oftalmologia Laboratório de Eletrofisiologia Visual ClínicaUniversidade Federal de São Paulo (UNIFESP)UNIFESP, Depto. de OftalmologiaUNIFESP, Depto. de Oftalmologia Laboratório de Eletrofisiologia Visual ClínicaUNIFESPSciELOConselho Brasileiro de OftalmologiaUniversidade Federal de São Paulo (UNIFESP)Mendieta, Luana [UNIFESP]Berezovsky, Adriana [UNIFESP]Salomão, Solange Rios [UNIFESP]Sacai, Paula Yuri [UNIFESP]Pereira, Josenilson Martins [UNIFESP]Fantini, Sérgio Costa [UNIFESP]2015-06-14T13:31:32Z2015-06-14T13:31:32Z2005-04-01info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersion171-176application/pdfhttp://dx.doi.org/10.1590/S0004-27492005000200004Arquivos Brasileiros de Oftalmologia. Conselho Brasileiro de Oftalmologia, v. 68, n. 2, p. 171-176, 2005.10.1590/S0004-27492005000200004S0004-27492005000200004.pdf0004-2749S0004-27492005000200004http://repositorio.unifesp.br/handle/11600/2490porArquivos Brasileiros de Oftalmologiainfo:eu-repo/semantics/openAccessreponame:Repositório Institucional da UNIFESPinstname:Universidade Federal de São Paulo (UNIFESP)instacron:UNIFESP2024-08-04T19:11:48Zoai:repositorio.unifesp.br/:11600/2490Repositório InstitucionalPUBhttp://www.repositorio.unifesp.br/oai/requestbiblioteca.csp@unifesp.bropendoar:34652024-08-04T19:11:48Repositório Institucional da UNIFESP - Universidade Federal de São Paulo (UNIFESP)false |
dc.title.none.fl_str_mv |
Acuidade visual e eletrorretinografia de campo total em pacientes com síndrome de Usher Visual acuity and full-field electroretinography in patients with Usher's syndrome |
title |
Acuidade visual e eletrorretinografia de campo total em pacientes com síndrome de Usher |
spellingShingle |
Acuidade visual e eletrorretinografia de campo total em pacientes com síndrome de Usher Mendieta, Luana [UNIFESP] Retinitis pigmentosa Usher's syndrome Electroretinography Visual acuity Electrophysiology Retinal diseases Visually impaired persons Retinite pigmentosa Síndrome de Usher Eletrorretinografia Acuidade visual Eletrofisiologia Doenças retinianas Portadores de deficiência visual |
title_short |
Acuidade visual e eletrorretinografia de campo total em pacientes com síndrome de Usher |
title_full |
Acuidade visual e eletrorretinografia de campo total em pacientes com síndrome de Usher |
title_fullStr |
Acuidade visual e eletrorretinografia de campo total em pacientes com síndrome de Usher |
title_full_unstemmed |
Acuidade visual e eletrorretinografia de campo total em pacientes com síndrome de Usher |
title_sort |
Acuidade visual e eletrorretinografia de campo total em pacientes com síndrome de Usher |
author |
Mendieta, Luana [UNIFESP] |
author_facet |
Mendieta, Luana [UNIFESP] Berezovsky, Adriana [UNIFESP] Salomão, Solange Rios [UNIFESP] Sacai, Paula Yuri [UNIFESP] Pereira, Josenilson Martins [UNIFESP] Fantini, Sérgio Costa [UNIFESP] |
author_role |
author |
author2 |
Berezovsky, Adriana [UNIFESP] Salomão, Solange Rios [UNIFESP] Sacai, Paula Yuri [UNIFESP] Pereira, Josenilson Martins [UNIFESP] Fantini, Sérgio Costa [UNIFESP] |
author2_role |
author author author author author |
dc.contributor.none.fl_str_mv |
Universidade Federal de São Paulo (UNIFESP) |
dc.contributor.author.fl_str_mv |
Mendieta, Luana [UNIFESP] Berezovsky, Adriana [UNIFESP] Salomão, Solange Rios [UNIFESP] Sacai, Paula Yuri [UNIFESP] Pereira, Josenilson Martins [UNIFESP] Fantini, Sérgio Costa [UNIFESP] |
dc.subject.por.fl_str_mv |
Retinitis pigmentosa Usher's syndrome Electroretinography Visual acuity Electrophysiology Retinal diseases Visually impaired persons Retinite pigmentosa Síndrome de Usher Eletrorretinografia Acuidade visual Eletrofisiologia Doenças retinianas Portadores de deficiência visual |
topic |
Retinitis pigmentosa Usher's syndrome Electroretinography Visual acuity Electrophysiology Retinal diseases Visually impaired persons Retinite pigmentosa Síndrome de Usher Eletrorretinografia Acuidade visual Eletrofisiologia Doenças retinianas Portadores de deficiência visual |
description |
PURPOSE: Usher's syndrome (US) is a group of genetically distinct autossomal conditions, characterized by sensorineural hearing loss accompanied by a retinal dystrophy indistinguishable from retinitis pigmentosa (RP). The purpose of this study was to analyze full-field electroretinography (ERG) and visual acuity (VA) among patients with type I and II Usher's syndrome. METHODS: Electroretinography responses and visual acuity were studied in 22 patients (mean age at test = 26.8±16.8 years). Seventeen patients had SU type I and 5 patients were diagnosed as Usher's syndrome type II. RESULTS: Mean visual acuity was 0.9 logMAR (20/160, Snellen equivalent) for patients with Usher's syndrome type I and 0.4 logMAR (20/50, Snellen equivalent) for patients with Usher's syndrome type II. Scotopic rod and maximal responses were non-detectable in both groups. Mean amplitude for oscillatory potentials was 14.5 µV ±6.1 in Usher's syndrome type I and 12.6 µV±5.2 in Usher's syndrome type II. Cone responses were non-detectable in 95% of the patients with Usher's syndrome I and in 100% of patients with Usher's syndrome II. Mean amplitude for 30 Hz flicker photopic cone response was 3.1 µV±4.1 for Usher's syndrome type I and 1.0 µV±0.6 for type II with mean implicit time of 34.0 ms±6.2 (US I) and 35.8 ms±3.1 (type II). CONCLUSIONS: Visual acuity was relatively preserved in both groups, however Usher's syndrome II group showed better visual acuity results. Electroretinography findings were severely reduced in both groups, with most patients showing non-detectable rod and cone responses. |
publishDate |
2005 |
dc.date.none.fl_str_mv |
2005-04-01 2015-06-14T13:31:32Z 2015-06-14T13:31:32Z |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://dx.doi.org/10.1590/S0004-27492005000200004 Arquivos Brasileiros de Oftalmologia. Conselho Brasileiro de Oftalmologia, v. 68, n. 2, p. 171-176, 2005. 10.1590/S0004-27492005000200004 S0004-27492005000200004.pdf 0004-2749 S0004-27492005000200004 http://repositorio.unifesp.br/handle/11600/2490 |
url |
http://dx.doi.org/10.1590/S0004-27492005000200004 http://repositorio.unifesp.br/handle/11600/2490 |
identifier_str_mv |
Arquivos Brasileiros de Oftalmologia. Conselho Brasileiro de Oftalmologia, v. 68, n. 2, p. 171-176, 2005. 10.1590/S0004-27492005000200004 S0004-27492005000200004.pdf 0004-2749 S0004-27492005000200004 |
dc.language.iso.fl_str_mv |
por |
language |
por |
dc.relation.none.fl_str_mv |
Arquivos Brasileiros de Oftalmologia |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
171-176 application/pdf |
dc.publisher.none.fl_str_mv |
Conselho Brasileiro de Oftalmologia |
publisher.none.fl_str_mv |
Conselho Brasileiro de Oftalmologia |
dc.source.none.fl_str_mv |
reponame:Repositório Institucional da UNIFESP instname:Universidade Federal de São Paulo (UNIFESP) instacron:UNIFESP |
instname_str |
Universidade Federal de São Paulo (UNIFESP) |
instacron_str |
UNIFESP |
institution |
UNIFESP |
reponame_str |
Repositório Institucional da UNIFESP |
collection |
Repositório Institucional da UNIFESP |
repository.name.fl_str_mv |
Repositório Institucional da UNIFESP - Universidade Federal de São Paulo (UNIFESP) |
repository.mail.fl_str_mv |
biblioteca.csp@unifesp.br |
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1814268336108732416 |