Article abstract-Objective: To assess early visual impairment related to vigabatrin prospectively in patients with and without visual symptoms. Background: Vigabatrin acts as an inhibitor of gamma-aminobutyric acid (GABA) transaminase. GABA-induced ion transport changes in the retinal pigment epithelium have been described. The electro-oculogram (EOG) is a clinical test that reflects photoreceptor and pigment epithelium function. Patients and methods: Of the 22 consecutive patients presenting with a history of partial seizures currently treated with vigabatrin, 20 were included in the study. A complete clinical ophthalmologic and neurologic examination was performed, including static 100-point perimetry, EOG, and electroretinogram (ERG). Results: In 14 of 20 patients, the light/dark ratio (Arden ratio) of the standard EOG was reduced in at least one eye. The a-and b-wave amplitudes and implicit time of the ERG were within the normal range in all patients; however, ERG oscillatory potentials could not be recorded in 10 patients. Twelve patients had visual field constriction; five complained of visual symptoms. The most severe visual impairment was observed in patients treated with both vigabatrin and valproate. Conclusions: There is some evidence of outer retinal dysfunction in the patients treated with vigabatrin. EOG, a more sensitive diagnostic tool than ERG for screening vigabatrin-treated patients, also appears to be more specific.

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Objective: To describe the genetic and phenotypic spectrum of Usher syndrome after 6 years of studies by next-generation sequencing, and propose an up-to-date classification of Usher genes in patients with both visual and hearing impairments suggesting Usher syndrome, and in patients with seemingly isolated deafness. Study Design: The systematic review and meta-analysis protocol was based on Cochrane and Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. We performed 1) a meta-analysis of data from 11 next-generation sequencing studies in 684 patients with Usher syndrome; 2) a meta-analysis of data from 21 next-generation studies in 2,476 patients with seemingly isolated deafness, to assess the involvement of Usher genes in seemingly nonsyndromic hearing loss, and thus the proportion of patients at high risk of subsequent retinitis pigmentosa (RP); 3) a statistical analysis of differences between parts 1) and 2). Results: In patients with both visual and hearing impairments, the biallelic disease-causing mutation rate was assessed for each Usher gene to propose a classification by frequency: USH2A: 50% (341/684) of patients, MYO7A: 21% (144/684), CDH23: 6% (39/684), ADGRV1: 5% (35/684), PCDH15: 3% (21/684), USH1C: 2% (17/684), CLRN1: 2% (14/684), USH1G: 1% (9/684), WHRN: 0.4% (3/684), PDZD7 0.1% (1/684), CIB2 (0/684). In patients with seemingly isolated sensorineural deafness, 7.5% had disease-causing mutations in Usher genes, and are therefore at high risk of developing RP. These new findings provide evidence that usherome dysfunction is the second cause of genetic sensorineural hearing loss after connexin dysfunction. Conclusion: These results promote generalization of early molecular screening for Usher syndrome in deaf children.

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Background: Three-dimensional (3D) visualization systems, also known as heads-up systems, are now available for eye surgery and as with every new device there is need for a specific evaluation. Objectives: The aim of this study was to compare the efficiency, surgical comfort, and safety of a 3D visualization system to a standard binocular microscope (BM) in routine ophthalmologic procedures. Method: After a 4-week training period, a 3D visualization system (Ngenuity, Alcon ®) available in one of the Robert Debré Hospital Ophthalmology Departments' operating rooms was compared to a standard BM (OPMI LUMIRA 700, Zeiss ®), in the process of a call for new device evaluation. From December 2017 to March 2018, 5 surgeons and their respective residents were asked to fill in a questionnaire for all procedures. Before the surgery, the surgeon recorded: (i) the type of surgery (cataract [PK], retinal detachment [RD], epiretinal membrane peeling [ERM], macular hole, vitreous haemorrhage [VH]), (ii) the type of visualization system chosen (3D or BM), and (iii) the estimated surgical risk (low, intermediate, or high grade). At the end of the procedure, the primary surgeon recorded