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Curr Eye Res. 2018 Oct;43(10):1295-1301. doi: 10.1080/02713683.2018.1501804. Epub 2018 Jul 30.

Usher Syndrome and Color Vision.

Author information

a Centre for Ophthalmology, Institute for Ophthalmic Research , University of Tuebingen , Tuebingen , Germany.
b Institut de la Vision , INSERM UMRS 1120 , Paris , France.
c Complexité du vivant , UPMC-Sorbonnes Universités , Paris , France.
d Centre d'Investigation Clinique, Direction de l'Hospitalisation et de l'Organisation des Soins , Centre Hospitalier National d'Ophtalmologie des Quinze-Vingts , Paris , France.
e Ophtalmologie , Institut Arthur Vernes , Paris , France.
f Eye Hospital , University Medical Centre Ljubljana , Ljubljana , Slovenia.
g Eye Clinic, Multidisciplinary Department of Medical, Surgical and Dental Sciences , University of Campania Luigi Vanvitelli , Naples , Italy.
h , Collège de France , Paris , France.
i Génétique et Physiologie de l`Audition , Institut Pasteur , Paris , France.
j Werner Reichardt Centre for Integrative Neuroscience (CIN) , University of Tübingen , Tübingen , Germany.



The aim of this study is to report on the results of color vision testing in a European cohort of patients with Usher syndrome (USH). We describe the results in relation to Usher type (USH1 and USH2), age and visual acuity.


The color vision of 220 genetically confirmed adult USH patients, aged 18-70 years, was analyzed with one of three methods: the Farnsworth D-15 Dichotomous test (D-15) along with the Lanthony desaturated 15 Hue tests (D-15d), the Roth 28-Hue test, or the Ishihara 14-plate test. Visual acuity was measured with either the ETDRS or the SNELLEN charts. The Confusion index, the Selectivity index and the Confusion angle were calculated for the panel tests and used for analysis. The numbers of plates that could not be read were analyzed for the Ishihara test.


For the panel tests, the degree of color loss (Confusion index) is similar in both subtypes of USH, but the polarization of error scores (Selectivity index) is significantly lower in USH1 than USH2, showing more diffuse errors than those found in USH2. There is no significant correlation between logMAR visual acuity and the Confusion or the Selectivity indices. Additionally, we find a significant correlation between patient age and the degree and the polarity of the loss only in USH2. There was no difference between USH1 and USH2 in the results of the Ishihara test.


The examination of color vision in patients with USH shows a significant difference in the pattern of color vision loss in USH1 and USH2 patients, but not in the severity of the loss. In USH2, we find a correlation between patient age and the degree and the polarity of the loss. These results may be due to differences in the pathogenesis of retinal dystrophy in USH1 and USH2.


Usher syndrome; color vision; retinal dystrophy; visual acuity

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