Usually hearing loss is the first symptom to become apparent. Balanceīecause this syndrome damages the ear, it naturally affects the balance of the body, which can lead to problem in movements, such as sitting or walking. The hearing damage depends on what type of Usher syndrome the person is suffering from and the phase of diagnosis. For some people, this loss can be managed through hearing aids, others might lose their hearing capability completely. This syndrome adversely damages the ears, causing hearing problems. Most people suffering from retinitis pigmentosa will lose their sight with time. This condition worsens with time, removing most of your side (peripheral) vision until you are left with a tiny bit of central vision. Usher syndrome causes a condition called retinitis pigmentosa, a severe eye condition this condition starts by affecting night vision and makes it hard for the person to see things when it’s dark or dim, causing loss of peripheral vision. What are the symptoms of Usher syndrome? Sight loss About half of the people experience a problem with balance. Vision loss begins in early to mid-adulthood, which grows with age. People with type 3 syndrome have normal hearing at birth which starts to decline in late childhood. Children with this type of Usher syndrome do not face balance problems. Night vision loss starts in teenagers and slowly affects their vision with time. People with type 2 Usher syndrome are born with moderate to severe hearing loss (however, less severe than type 1). Most babies with this type will not start walking until 18 months because of affected balance. Balance issues, such as problems in walking, sitting, or standing up are faced because of type 1 Usher syndrome. As a result, they might lose night vision by age 10, which worsens with time. People with Type 1 Usher syndrome are either born with deafness or have severe to profound hearing loss, making sight loss very evident in childhood. Type 1 and type 2 are most common, whereas type 3 is very rare. There are three types of Usher syndrome, each type causes different degrees of hearing and vision loss. The gene mutation affects the nerve cells in the spiral structure of the inner ear called the cochlea, stopping them from transmitting sound normally, causing hearing problems. As these cells are affected, with time, they go bad, starting with blurring outer edges and only leaving the centre part (central vision) which eventually might lead to blindness. The vision is affected as the gene mutation in this syndrome affects the rods and cone’s cells (light-sensitive cells). Usher’s syndrome affects both vision and hearing. The most common genes contributing towards type 1 and 2 are MYO7A and USH2A. Type 1: MYO7A, USH1C, CDH23, PCDH15, USH1G, CIB2 The research is still ongoing, however, so far, the below genes have been associated with this syndrome: This syndrome is caused by mutation in specific genes. As this disease affects hearing, it also causes problems with balance for many people. Since it is genetic, it is passed on through genes. The Usher syndrome is not a disease that someone gets with time, people are born with it. Damage to hearing causes deafness/hearing loss, whereas an eye disease called Retinitis Pigmentosa (RP) causes vision loss. According to Sense, Usher syndrome affects between 4-7 people in 100,000 people worldwide. Usher syndrome is a rare genetic, inherited condition affecting both hearing and vision. Medically reviewed by Tina Patel on 31 January 2023. Usher Syndrome – symptoms, diagnosis and treatment
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