Researched by Silvia Le

Condition: light-sensing cells of the Early diagnosis of Usher Usher Syndrome a rare retina gradually Syndrome is very genetic disorder that is deteriorate. important. Diagnosis associated with a mutation includes the evaluation of in any one of 10 genes. hearing, balance, and Both parents must vision (three senses). contribute an appropriate gene for the syndrome to Treatment: appear. In the United Types: Usher syndrome is States about four babies in Usher I: usually born incurable at present time. every 100,000 birth have deaf and have It is helpful to diagnose this syndrome difficulties maintaining children before they their balance. Babies develop the characteristic are usually slow to night blindness. The develop motor skill such simplest approach to as walking. diagnose Usher syndrome Usher II: usally hard of is to test for the hearing rather than deaf, characteristic and their hearing does chromosomal mutations. not degrade over time. Maybe underdiagnosed Sources: or more difficult to 1. http://en.wikipedia.org/wiki / Usher _ syndrome detect. 2. www.boystownhospital.or Usher III: experience a g/ Usher /information Other Names: 3. www.nidcd.nih.gov/health/ Hallgren syndrome, progressive loss of hearing/ usher hearing and roughly half 4. Professional Guide to Usher-Hallgren syndrome, Diseases, Lippintcott vestibular loss. Williams & Wilkins pp. rp-dysacusis syndrome, 1180 and dystrophia retinae 5. A Gene for Usher dysacusis Causes of Usher Syndrome Type 1 Syndrome: (USH1A) Maps to Chromosome 14q: J. Usher syndrome is Kaplanm et al.: Genomics, What is it? (Symtoms) 1992, vol. 14, pp.979-987 Usher syndrome is passed from parents to responsible for the their children through majority of deaf-blindness. mutated genes. To have It affects hearing, balance, this syndrome a person and vision. The major must receive a mutated symptoms are hearing loss gene from each parent. and an eye disorder called If both parents are retinitis pigmentosa (RP). carriers of this mutated RP causes night-blindness gene, they will have a and a loss of peripheral one-in-four chance of vision (side vision) having a child with this through progressive syndrome. degeneration of the retina. Vision loss occurs as the Diagnosis: