![]() ![]() ![]() With the right hearing aids, patients suffering from Usher syndrome will be enabled to lead a more fulfilling life, rich in sounds. Hence, it is very important that an experienced hearing specialist select the appropriate hearing system and perform an optimal fitting. As an artificial inner ear replacement, it enables the direct transduction of the acoustic signal to the auditory nerve.īy contrast, the reduced hearing ability with type 2 Usher syndrome can be effectively treated with binaural hearing aids, which amplify residual hearing. In the case of complete deafness, as with type 1 Usher syndrome, a cochlear implant can be considered, especially with children. They all aim at delaying the complete destruction of the photoreceptors. ![]() The situation is aggravated by the problem of what is typically referred to as “discrimination loss.” This means that sounds at a certain volume are still audible, but language at an equal volume cannot be discerned anymore.Ĭurrently, there are only insufficient and experimental treatment options available for the degeneration of the retina. The other types of Usher syndrome are usually characterized by a severe hearing disability in the high-frequency range and a moderate hearing impairment in the low-frequency range. No form of sound can be perceived, and those who are affected resort to lip reading or American Tactile Sign Language (TSL). Congenital deafness with type 1 is characterized by a complete loss of hearing. The hearing impairment depends on the respective type. Therefore, the task of reading does not pose any problems yet, but the sense of orientation in a room becomes difficult. As the illness progresses, a type of tunnel vision develops due to a restricted field of vision. (The necessary photoreceptors are located at the periphery of the retina and thus affected first.) Moreover, patients notice an increased sensitivity to glare, and contrasts cannot be adequately perceived. At first, it is noticeable when a person’s vision seems impaired at dawn and at night. However, the fact is that retinitis pigmentosa is often discovered very late. This is, of course, only possible when there is no, or just minor, visual impairment. ![]()
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