Behavioural Adaptation to Hereditary Macular Dystrophy: A Systematic Review on the Effect of Early Onset Central...

Hereditary macular dystrophies (HMD) result in early onset central field loss. Evidence for cortical plasticity has been found in HMD, which may enhance peripheral visual abilities to meet the increased demands and reliance on the peripheral field, as has been found in congenitally deaf adults and habitual action video-game players. This is a qualitative synthesis of the literature on the effect of early onset central field loss on peripheral visual abilities. The knowledge gained may help in developing rehabilitative strategies that enable optimisation of remaining peripheral vision.

A systematic search performed on the Web of Science and PubMED databases yielded 728 records published between 1809 to 2020, of which seven case-control studies were eligible for qualitative synthesis.

The search highlighted an overall paucity of literature, which lacked validity due to small heterogeneous samples and deficiencies in reporting of methods and population characteristics. A range of peripheral visual abilities at different eccentricities were studied. Superior performance of HMD observers in the peripheral field or similarities between the preferred retinal loci (PRL) and normal fovea were observed in four of seven studies. Findings were often based on studies including a single observer. Further larger rigorous studies are required in this area.

Spontaneous perceptual learning through reliance on and repeated use of the peripheral field and PRL may result in some specific superior peripheral visual abilities. However, worse performance in some tasks could reflect unexpected rod disease, lack of intensive training, or persistent limitations due to the need for cones for specific tasks. Perceptual learning through training regimes could enable patients to optimise use of the PRL and remaining peripheral vision. However, further studies are needed to design optimal training regimes.