Electroretinography and inherited macular dystrophies

With judicious application, conventional electroretinographic recordings can provide meaningful diagnostic information on patients with hereditary macular dystrophies. In some patients, characteristic fundus lesions in addition to already known information on predictably normal electrophysiologic findings should obviate the need for implementation of electroretinographic investigations. In other macular dystrophies, such recordings are necessary for a definitive diagnosis or for monitoring photoreceptor cell deterioration. This review discusses six of the most frequently encountered inherited macular dystrophies with consideration as to when and why electroretinographic recordings should be implemented in the study of these disorders.