Case Report

Two Sisters With Usher Syndrome - Case Report

  • Onur Karadağ
  • Sülay Eraslan Özdil
  • Süleyman Kuğu
  • Arzu Taşkıran Çömez

Received Date: 05.07.2007 Accepted Date: 26.05.2008 Turk J Ophthalmol 2008;38(4):346-349

Two sisters of 39 and 43 ages, were admitted to Dr.Lutfi Kirdar Kartal Training and Research Hospital Eye Clinic with progressive decrease in visual acuity in both eyes. It was learned from their history that, decrease in visual acuity started in the ages of ten in both sisters, while they had deafness and ataxia since birth. The visual acuity in the patient 43 years old (patient 1) was 0.2 in the right eye and 2 meter finger count in the left eye while it was found to be 2 meter finger counts in both eyes of 39 years old patient (patient 2). Biomicroscopic evaluation revealed that both patients had posterior subcapsular cataracts in both eyes and hiperpigmentated spicule style lesions mostly in the peripheral fundus which was compatible with retinitis pigmentosa (RP). Because of retinitis pigmentosa, deafness and balance problems, these patients are thought to be Usher Syndrome. As a result; in patients with progressive decrease in visual acuity and deafness, US should also be kept in mind.

Keywords: Retinitis pigmentosa, deafness, Usher syndrome

Full Text (Turkish)