In many ways this version of Usher Syndrome Type 3 is the least common version of this disease only affecting approximately 2 percent of all Usher Syndrome cases. One of the major symptoms of this version asĬompared to those of Type 1 is that there are no abnormalities that develop within the inner ear. Unlike the severe hearing lossĮxperienced in Type 1, Type 2 can range from mild to severe versions, limiting the ability to hear high-frequency sounds. Usher Syndrome Type 2 is categorized by hearing loss at birth and vision loss being expressed during adolescence or young adulthood. Impact on the performance of the most remedial of tasks, such as walking. This condition is relatively more severe than the other two because of the age at which it is expressed and its exhibits severe hearing loss from birthĪ person diagnosed with Usher Syndrome Type 1 experiences abnormalities within their inner ear, whichĭirectly impacts their spatial orientation and balance.Usher Syndrome Type 1Ī person is classified as having Usher Syndrome Type 1 if they meet three conditions: These classifications consists of three different categories of the disease ranging from type 1, type 2 and type 3.Īn individual's respective category will be classified based upon the severity of the condition and its symptoms, which include balance, vision and hearing. That result in different classifications of this disorder. There are in fact multiple genetic mutations These hair cells normally help transmit sound from the inner ear to the brain. These proteins directly impact the proper function of the photoreceptors found within the retina,Īnd the development of the hair cells located in the ear. Proteins required in promoting normal healthy vision, hearing and balance. These mutated genes are originally responsible for producing the necessary Gene, resulting in the disorder being expressed in the offspring carrying both copies. Usher syndrome has been linked to an autosomal recessive gene where each parent carries one copy of a mutation of the To the formation of blind spots and even tunnel vision. Most people impacted by this type of vision loss usually experience loss of night vision first, which then gradually and eventually leads That damages and deteriorates the light-sensing region of the retina, which is located at the back of the eye. Leading to hearing reduction while also causing the onset of retinitis pigmentosa in the eye. These abnormalities affect the inner ear, ultimately
Gradual loss of vision and hearing over time due to abnormalities found within the ears and eyes. According to the National Institute of Health, Usher Syndrome is defined as a condition that causes a
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