Kinetics of visual field loss in Usher syndrome Type II.
OBJECTIVE
To characterize the kinetics of visual field decay in Usher syndrome type II.


METHODS
The area of 137 Goldmann visual fields (GVFs) delimited with the I4e and V4e targets was measured in each eye of 19 patients with an established diagnosis of Usher syndrome type II, and the average interocular GVF area for each patient at each time point was calculated. The average follow-up was 5.58 years. Symptomatic disease duration was defined as years elapsed after symptoms were first noted. The data set (n = 67 for the I4e target; n = 70 for the V4e target) was analyzed with a random coefficient mixed model to identify the best-fit model describing the decay of visual field size over time. The half-life of the residual visual field area (t(0.5)) was also calculated.


RESULTS
The variable that best explained the decay of the GVF area was the duration of symptomatic disease. In an exponential model, the slope estimate for the natural log of the GVF area was -0.172 for the I4e target and -0.136 for the V4e target for each year of symptomatic disease. Accordingly, t(0.5) was approximately 4 years for the I4e target and 5 years for the V4e target. These estimates are very similar to those in previous studies of nonsyndromic retinitis pigmentosa (RP).


CONCLUSIONS
This study suggests that the kinetics of GVF decline in Usher syndrome type II are, on average, very similar to other forms of RP and that, once the disease becomes symptomatic, GVF deterioration follows stereotyped kinetics, even in patients with late-onset retinal disease.
Department of Ophthalmology, University of Tennessee Health Science Center, Memphis, TN 38163, USA. iannacca@utmem.edu
Alessandro
A
Iannaccone
Stephen B
SB
Kritchevsky
Maria Laura
ML
Ciccarelli
Salvatore A
SA
Tedesco
Claudio
C
Macaluso
William J
WJ
Kimberling
Grant W
GW
Somes
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D005260:Female; D006311:Hearing Disorders / Q000151:congenital / Q000503:physiopathology*; D006801:Humans; D007700:Kinetics; D008297:Male; D008875:Middle Aged; D012174:Retinitis Pigmentosa / Q000235:genetics / Q000503:physiopathology*; D014786:Vision Disorders / Q000503:physiopathology*; D014794:Visual Fields