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USHER SYNDROME
ABSTRACT -
Usher Syndrome is a common disorder that causes deafness or steady vision loss that deteriorate over a period of time. It can lead to eye disease called Retinitis Pigmentosa (RP) affecting the sensitive tissues at the backside of the eye i.e. retina
Types – Type 1 –- Deafness since birth
- Severe issues in balancing
- Vision problems gets developed during childhood
- Develops night vision
- Moderate to severe hearing loss in which hearing aids can help
- Steady hearing and vision loss
- Night blindness in and after puberty
CAUSES -
- Genetic factors
- DNA mutations
- Pre-existing conditions like Retinitis Pigmentosa(RP)
SIGNS AND SYMPTOMS –
- Hearing loss
- Loss of peripheral vision
DIAGNOSIS –
- Vision and Hearing tests includes physical examination
- Electroretinogram (ERG)
- Electronystagmogram (ENG)(RP)
TREATMENT –
Currently, there are no treatments available however early identification can help through:
- Hearing aids
- Assistive listening devices
- Cochlear Implants is an electronic medical device to help in providing sound signals to the brain
- Learning other sign languages
- Auditory training for better communication
EPIDEMIOLOGY –
- Estimation shows that both males and females get affected by Usher Syndrome irrespective of the gender.
- Children are majorly prone to get affected from usher’s syndrome due to genetic mutations.



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