What you need to know about cone-rod dystrophy

Cone-rod dystrophy is considered a rare inherited eye disorder that affects the retina and causes vision loss. The retina captures, or “sees”, images and converts them into electronic signals that are sent to the brain for translation and identification. The cells in the retina that are sensitive to light are called cones and rods. Cone-rod dystrophy causes the cones and rods in the retina to deteriorate, inhibiting the retinas ability to work properly. Unfortunately, most people with the disorder are legally blind by the time they are middle aged adults.

Scientists say there are more than 30 types of genetic mutations in several different genes that may cause the disorder. It is a progressive disease and there is no cure, however there may be ways to slow its progression.

Symptoms appear at young ages

The symptoms of cone-rod dystrophy usually begin to appear in childhood. The first signs are usually a decrease in visual acuity, or sharpness of vision, and an abnormal sensitivity to light. As cone-rod dystrophy progresses, it can cause loss of color perception, loss of peripheral vision and blind spots in the central field of vision. Here is a list of the symptoms and the frequency with which they occur, according to the National Center for Advancing Translational Sciences

80 to 99 percent of people have:

  • Abnormal retinal pigmentation; causing changes in the color of the retina
  • Night blindness; the inability to see well at night, or in poor light
  • Extreme sensitivity to light; exposure to bright light causes discomfort or pain in the eyes

30 to 70 percent of people have:

  • Abnormal color vision; the inability to see the difference between colors

5 to 29 percent of people have:

  • Visual impairment; defined as significant limitation of vision

Treatments for cone-rod dystrophy

Currently there are no treatments for cone-rod dystrophy. Scientists are conducting research on the disorder and they believe that in the future, gene therapy, stem cell therapy, and retinal implants may potentially be treatments. The Foundation Fighting Blindness, of which we are a board member, raises funds that drive the research to find cures, treatments, and preventions for the spectrum of inherited retinal diseases that lead to blindness, including cone-rod dystrophy. We hope that working together we can find a cure.

Until that time, it is believed that restricting exposure to bright light may help to slow the progression of the disease. In addition, low vision devices, like the ones we provide our patients, can also help to enhance remaining vision. There are many advanced, wearable technologies designed specifically to enhance low vision caused by retinal disorders and diseases. They include:

IrisVision: The software magnifies the central field of vision, the area that often deteriorates from retinal eye disease, using a 16 MP camera. The screen displays 10X more pixels per inch than an HD TV and provides a 70° field of view, the widest on the market today. Adjustable settings include contrast, ambient levels, interpupilar distance and more. Each device can be customized by the wearer to adjust magnification and field of vision.

Telescopic eyeglasses: These are miniature binoculars that are inserted into an individual’s eyeglasses. The person’s eyeglasses prescription as well as the highest quality optics are used together in the system for maximum clarity.  Telescopic spectacles can be for one eye (monocular) or two eyes (binocular).

Microscopic eyeglasses: This is a double lens system that combine the patient’s eyeglass prescription with the amount of magnification needed. The amount of magnification depends upon the level of remaining vision and the size of the object to be seen.

We believe that low vision can be enhanced. We believe that people should not be told that nothing more can be done to enhance their vision. Every day we provide people with advanced technology that enhances their low vision and expands their lived environment. If you or a loved one lives with low vision, call us. Let’s discuss how we can help you.