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Posted by -Dr-kamlesh

Usher Syndrome / 31 Jul 2019

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
Type 2 –
  • Develops night vision
  • Moderate to severe hearing loss in which hearing aids can help
Type 3 –
  • Steady hearing and vision loss
  • Night blindness in and after puberty

CAUSES -

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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