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What is Usher Syndrome and how does it affect vision?

What is Usher Syndrome and how does it affect vision?

02 November 2023

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Usher Syndrome is named after British physician Charles Usher, who first described the condition in 1914. It is an inherited disease caused by mutations in several genes that play a crucial role in the development and maintenance of sensory cells. of the inner ear and the photoreceptor cells of the retina.

There are three main types of Usher Syndrome, called Usher type 1, type 2 and type 3, depending on the severity of the hearing loss and the age of onset of visual symptoms.

Hearing loss is one of the most common symptoms of Usher Syndrome. Those affected may be born with moderate to profound hearing loss or develop it during childhood. In the case of Usher type 1, hearing loss is severe from birth. On the other hand, in type 2, the hearing loss is less severe and usually occurs in adolescence or early adulthood. Finally, in type 3, hearing loss begins later, often in adolescence.

The most distinctive feature of Usher Syndrome is its impact on vision. Progressive retinal degeneration, known as retinitis pigmentosa, is an integral part of this disease. The retina's photoreceptors, the cells that detect light and transmit visual information to the brain, gradually become damaged.

This process of retinal degeneration leads to visual symptoms such as decreased night vision (night blindness), reduced peripheral visual field, and decreased visual acuity. As the disease progresses, central vision may also be affected, making activities such as reading and recognizing faces increasingly difficult.

It is important to note that the severity and rate of progression of symptoms can vary considerably among people with Usher Syndrome. Some may experience vision loss more quickly than others, and hearing loss can also vary in severity and time of onset. This makes each case unique and requires a personalized approach in terms of medical care and support.

Currently, there is no definitive cure for Usher Syndrome. However, there are treatments and management strategies that can help affected people adapt and improve their quality of life.

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