Acuidade visual e eletrorretinografia de campo total em pacientes com síndrome de Usher

Detalhes bibliográficos
Autor(a) principal: Mendieta, Luana [UNIFESP]
Data de Publicação: 2005
Outros Autores: Berezovsky, Adriana [UNIFESP], Salomão, Solange Rios [UNIFESP], Sacai, Paula Yuri [UNIFESP], Pereira, Josenilson Martins [UNIFESP], Fantini, Sérgio Costa [UNIFESP]
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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spelling 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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