A Virtual Reality Orientation and Mobility Test for Inherited Retinal Degenerations: Testing a Proof-of-Concept After Gene Therapy

Tomas S Aleman,1– 3 Alexander J Miller,4 Katherine H Maguire,1,2 Elena M Aleman,2 Leona W Serrano,1,2 Keli B O’Connor,1,2 Emma C Bedoukian,5 Bart P Leroy,3,6– 8 Albert M Maguire,1– 3 Jean Bennett1,2 1Scheie Eye Institute at the Perelman Center for Advanced Med...

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Autores principales:	Aleman TS, Miller AJ, Maguire KH, Aleman EM, Serrano LW, O'Connor KB, Bedoukian EC, Leroy BP, Maguire AM, Bennett J
Formato:	article
Lenguaje:	EN
Publicado:	Dove Medical Press 2021
Materias:	virtual reality mobility orientation gene therapy lca rpe65 Ophthalmology RE1-994
Acceso en línea:	https://doaj.org/article/400aba714cfe4b78b045bc5bef8c3a88
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id	oai:doaj.org-article:400aba714cfe4b78b045bc5bef8c3a88
record_format	dspace
institution	DOAJ
collection	DOAJ
language	EN
topic	virtual reality mobility orientation gene therapy lca rpe65 Ophthalmology RE1-994
spellingShingle	virtual reality mobility orientation gene therapy lca rpe65 Ophthalmology RE1-994 Aleman TS Miller AJ Maguire KH Aleman EM Serrano LW O'Connor KB Bedoukian EC Leroy BP Maguire AM Bennett J A Virtual Reality Orientation and Mobility Test for Inherited Retinal Degenerations: Testing a Proof-of-Concept After Gene Therapy
description	Tomas S Aleman,1– 3 Alexander J Miller,4 Katherine H Maguire,1,2 Elena M Aleman,2 Leona W Serrano,1,2 Keli B O’Connor,1,2 Emma C Bedoukian,5 Bart P Leroy,3,6– 8 Albert M Maguire,1– 3 Jean Bennett1,2 1Scheie Eye Institute at the Perelman Center for Advanced Medicine, University of Pennsylvania, Philadelphia, PA, USA; 2Center for Advanced Retinal and Ocular Therapeutics, University of Pennsylvania, Philadelphia, PA, USA; 3Division of Ophthalmology at the Children’s Hospital of Philadelphia of the Department of Ophthalmology, University of Pennsylvania, Philadelphia, PA, USA; 4Neurology Virtual Reality Laboratory of the Department of Neurology, University of Pennsylvania, Philadelphia, PA, USA; 5Division of Human Genetics, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA; 6Department of Pediatrics, Ghent University, Ghent, Belgium; 7Department of Ophthalmology, Ghent University, Ghent, Belgium; 8Center for Medical Genetics, Ghent University Hospital, Ghent, BelgiumCorrespondence: Tomas S AlemanPerelman Center for Advanced Medicine, University of Pennsylvania, 3400 Civic Center Boulevard, Philadelphia, PA, 19104, USATel +1 215 662 8676Fax +1 215 615 0527Email aleman@pennmedicine.upenn.eduPurpose: To test the ability of a virtual reality (VR) orientation and mobility (O&M) protocol to serve a measure of functional vision for patients with inherited retinal degenerations (IRDs).Methods: A VR-O&M protocol designed using a commercially available VR hardware was tested in normally sighted control subjects (n=7; ages 10– 35yo; Average 22.5yo) and patients with RPE65-associated Leber Congenital Amaurosis (n=3; ages 7– 18yo; Average 12.7yo), in two of them before and after gene therapy. Patients underwent perimetry and full-field sensitivity testing. VR-O&M parameters correlated with the visual dysfunction.Results: Visual acuities in RPE65 patients were on average worse than 20/200, dark-adapted sensitivity losses > 5 log units, and fields constricted between 20° and 40°. Before treatment, patients required &sim; 1000-fold brighter environment to navigate, had at least x4 more collisions, and were slower both to orient and navigate compared to control subjects. Improvements in cone- (by 1– 2 L.u.) and rod-mediated (by > 4 L.u.) sensitivities post-treatment led to fewer collisions (at least by half) at &sim; 100-fold dimmer luminances, and to x4 times faster navigation times.Conclusion: This study provides proof-of-concept data in support for the use of VR-O&M systems to quantify the impact that the visual dysfunction and improvement of vision following treatments has on functional vision in IRDs. The VR-O&M was useful in potentially challenging scenarios such as in pediatric patients with severe IRDs.Translational Relevance: A VR-O&M test will provide much needed flexibility, both in its deployment as well as in the possibility to test various attributes of vision that may be impacted by gene therapy in the setting of translational studies.Precis: This study provides proof-of-concept data in support for the use of a virtual reality orientation and mobility test to quantify the impact of the disease and of treatments thereof on functional vision in inherited retinal degenerations.Keywords: virtual reality, mobility, orientation, gene therapy, LCA, RPE65
format	article
author	Aleman TS Miller AJ Maguire KH Aleman EM Serrano LW O'Connor KB Bedoukian EC Leroy BP Maguire AM Bennett J
author_facet	Aleman TS Miller AJ Maguire KH Aleman EM Serrano LW O'Connor KB Bedoukian EC Leroy BP Maguire AM Bennett J
author_sort	Aleman TS
title	A Virtual Reality Orientation and Mobility Test for Inherited Retinal Degenerations: Testing a Proof-of-Concept After Gene Therapy
title_short	A Virtual Reality Orientation and Mobility Test for Inherited Retinal Degenerations: Testing a Proof-of-Concept After Gene Therapy
title_full	A Virtual Reality Orientation and Mobility Test for Inherited Retinal Degenerations: Testing a Proof-of-Concept After Gene Therapy
title_fullStr	A Virtual Reality Orientation and Mobility Test for Inherited Retinal Degenerations: Testing a Proof-of-Concept After Gene Therapy
title_full_unstemmed	A Virtual Reality Orientation and Mobility Test for Inherited Retinal Degenerations: Testing a Proof-of-Concept After Gene Therapy
title_sort	virtual reality orientation and mobility test for inherited retinal degenerations: testing a proof-of-concept after gene therapy
publisher	Dove Medical Press
publishDate	2021
url	https://doaj.org/article/400aba714cfe4b78b045bc5bef8c3a88
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spelling	oai:doaj.org-article:400aba714cfe4b78b045bc5bef8c3a882021-12-02T13:33:13ZA Virtual Reality Orientation and Mobility Test for Inherited Retinal Degenerations: Testing a Proof-of-Concept After Gene Therapy1177-5483https://doaj.org/article/400aba714cfe4b78b045bc5bef8c3a882021-03-01T00:00:00Zhttps://www.dovepress.com/a-virtual-reality-orientation-and-mobility-test-for-inherited-retinal--peer-reviewed-article-OPTHhttps://doaj.org/toc/1177-5483Tomas S Aleman,1– 3 Alexander J Miller,4 Katherine H Maguire,1,2 Elena M Aleman,2 Leona W Serrano,1,2 Keli B O’Connor,1,2 Emma C Bedoukian,5 Bart P Leroy,3,6– 8 Albert M Maguire,1– 3 Jean Bennett1,2 1Scheie Eye Institute at the Perelman Center for Advanced Medicine, University of Pennsylvania, Philadelphia, PA, USA; 2Center for Advanced Retinal and Ocular Therapeutics, University of Pennsylvania, Philadelphia, PA, USA; 3Division of Ophthalmology at the Children’s Hospital of Philadelphia of the Department of Ophthalmology, University of Pennsylvania, Philadelphia, PA, USA; 4Neurology Virtual Reality Laboratory of the Department of Neurology, University of Pennsylvania, Philadelphia, PA, USA; 5Division of Human Genetics, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA; 6Department of Pediatrics, Ghent University, Ghent, Belgium; 7Department of Ophthalmology, Ghent University, Ghent, Belgium; 8Center for Medical Genetics, Ghent University Hospital, Ghent, BelgiumCorrespondence: Tomas S AlemanPerelman Center for Advanced Medicine, University of Pennsylvania, 3400 Civic Center Boulevard, Philadelphia, PA, 19104, USATel +1 215 662 8676Fax +1 215 615 0527Email aleman@pennmedicine.upenn.eduPurpose: To test the ability of a virtual reality (VR) orientation and mobility (O&M) protocol to serve a measure of functional vision for patients with inherited retinal degenerations (IRDs).Methods: A VR-O&M protocol designed using a commercially available VR hardware was tested in normally sighted control subjects (n=7; ages 10– 35yo; Average 22.5yo) and patients with RPE65-associated Leber Congenital Amaurosis (n=3; ages 7– 18yo; Average 12.7yo), in two of them before and after gene therapy. Patients underwent perimetry and full-field sensitivity testing. VR-O&M parameters correlated with the visual dysfunction.Results: Visual acuities in RPE65 patients were on average worse than 20/200, dark-adapted sensitivity losses > 5 log units, and fields constricted between 20° and 40°. Before treatment, patients required &sim; 1000-fold brighter environment to navigate, had at least x4 more collisions, and were slower both to orient and navigate compared to control subjects. Improvements in cone- (by 1– 2 L.u.) and rod-mediated (by > 4 L.u.) sensitivities post-treatment led to fewer collisions (at least by half) at &sim; 100-fold dimmer luminances, and to x4 times faster navigation times.Conclusion: This study provides proof-of-concept data in support for the use of VR-O&M systems to quantify the impact that the visual dysfunction and improvement of vision following treatments has on functional vision in IRDs. The VR-O&M was useful in potentially challenging scenarios such as in pediatric patients with severe IRDs.Translational Relevance: A VR-O&M test will provide much needed flexibility, both in its deployment as well as in the possibility to test various attributes of vision that may be impacted by gene therapy in the setting of translational studies.Precis: This study provides proof-of-concept data in support for the use of a virtual reality orientation and mobility test to quantify the impact of the disease and of treatments thereof on functional vision in inherited retinal degenerations.Keywords: virtual reality, mobility, orientation, gene therapy, LCA, RPE65Aleman TSMiller AJMaguire KHAleman EMSerrano LWO'Connor KBBedoukian ECLeroy BPMaguire AMBennett JDove Medical Pressarticlevirtual realitymobilityorientationgene therapylcarpe65OphthalmologyRE1-994ENClinical Ophthalmology, Vol Volume 15, Pp 939-952 (2021)

A Virtual Reality Orientation and Mobility Test for Inherited Retinal Degenerations: Testing a Proof-of-Concept After Gene Therapy

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