July 2019
Volume 60, Issue 9
Free
ARVO Annual Meeting Abstract  |   July 2019
First data of a prospective study comparing the impact of Retinitis Pigmentosa linked to Usher Syndrome 1B caused by MYO7A mutations and non-syndromic on daily living one year apart
Author Affiliations & Notes
  • Karine Becker
    Streetlab, Paris, France
  • Emmanuel Gutman
    Streetlab, Paris, France
  • Caroline Segaut-Prevost
    Sanofi, Chilly-Mazarin, France
  • Patrick Benoit
    Sanofi, Chilly-Mazarin, France
  • Caroline Cohen
    Sanofi, Chilly-Mazarin, France
  • Isabelle S Audo
    Department of Genetics, Inst de la Vision/INSERM/UPMC/CNRS/CHNO, Paris, France
  • Saddek Mohand-Said
    CHNO Quinze-Vingts / CIC Inserm, Paris, France
  • Jose Alain Sahel
    Institut de la Vision, Paris, France
  • Footnotes
    Commercial Relationships   Karine Becker, None; Emmanuel Gutman, None; Caroline Segaut-Prevost, Sanofi (E); Patrick Benoit, Sanofi (E); Caroline Cohen, Sanofi (E); Isabelle Audo, None; Saddek Mohand-Said, None; Jose Sahel, None
  • Footnotes
    Support  Sanofi
Investigative Ophthalmology & Visual Science July 2019, Vol.60, 4959. doi:
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      Karine Becker, Emmanuel Gutman, Caroline Segaut-Prevost, Patrick Benoit, Caroline Cohen, Isabelle S Audo, Saddek Mohand-Said, Jose Alain Sahel; First data of a prospective study comparing the impact of Retinitis Pigmentosa linked to Usher Syndrome 1B caused by MYO7A mutations and non-syndromic on daily living one year apart. Invest. Ophthalmol. Vis. Sci. 2019;60(9):4959.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose : To investigate the impact over time of reduced visual function on daily living in Usher Syndrome 1B (USH1B) patients compared to non-syndromic retinitis pigmentosa (RP) patients.

Methods : 9 fully-sighted controls (CO), 10 USH1B and 15 RP patients performed two tasks at t0 and t+1year in the same experimental conditions. The mobility task consisted of moving around avoiding obstacles under 500 and 7.5 lux in an artificial street (Streetlab platform). The visual search task aimed to look for and grasp target objects among 48 placed in a kitchen in an experimental apartment (Homelab platform) under 500 lux. Errors and time parameters were measured. We compared the data collected at t0 and t+1year, analyzing separately those from patients whose vision remained stable (USH1Bst; RPst) and those from patients whose visual acuity (VA) has decreased by at least 0.1 logMAR and / or visual field area (VF) has been reduced by at least 30% (USH1Bdet; RPdet).

Results : At t+1year, 3 USH1B and 6 RP patients’ vision has deteriorated (VA: p=0.042; VF: p=0.031). As at t0, RP and USH1B showed reduced Pourcentage of Preferred Walking Speed (PPWS) (p<0.001) and greater number of collisions than CO (p<0.05) at t+1year during the mobility task. There was no significant difference in PPWS and collisions between t0 and t+1year among USH1Bst and RPst patients, nor among CO. At 7.5 lux, PPWS of RPst increased by 3.9% from t0 to t+1year, whereas PPWS of RPdet decreased by 4.1% (ΔPPWSt0-PPWSt+1year: p=0.043). Trajectory errors increased by 64.3% from t0 to t+1year in RPdet and by 57.1% in USH1Bdet. At t+1year as at t0, visual search task showed that searching time (ST) in RP and USH1B was slower than in CO (p=0.0001) and USH1B made more identification errors than CO (p=0.031). No significant difference between t0 and t+1year was observed in CO, RPst and USH1Bst. At t+1year, identification time of USH1Bst decreased by 26% – so they were faster – and made less errors than at t0, whereas the one of USH1Bdet increased by 9.7% (ΔSTt0-STt+1year: p=0.017; ΔErrorst0-Errorst+1year: p<0.0001).

Conclusions : The subjects' performance have an opposite evolution depending on whether or not their vision deteriorates. Further data are expected before the summer 2019 to confirm the results observed.

This abstract was presented at the 2019 ARVO Annual Meeting, held in Vancouver, Canada, April 28 - May 2, 2019.

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