title: Ophthalmic pathologies in female subjects with bilateralcongenital sensorineural hearing loss.
abstract: OBJECTIVE
  The high prevalence of ophthalmologic pathologies in hearing-disabled subjects necessitates early screening of other sensory deficits, especially visual function. The aim of this study is to determine the frequency and clinical characteristics of ophthalmic pathologies in patients with congenital bilateral sensorineural hearing loss (SNHL).
  
  
  METHODS
  This descriptive study is a prospective analysis of 78 young female SNHL subjects who were examined at a tertiary care university hospital with a detailed ophthalmic examination, including electroretinography (ERG) and visual field tests as needed.
  
  
  RESULTS
  The mean age was 19.00 ± 1.69 years (range: 15 to 24 years). A total of 39 cases (50%) had at least one ocular pathology. Refractive errors were the leading problem, found in 35 patients (44.9%). Anterior segment examination revealed heterochromia iridis or Waardenburg syndrome in 2 cases (2.56%). Dilated fundus examination revealed retinal pathologies in 15 cases (19.23%), including retinitis pigmentosa or Usher's syndrome in 8 cases (10.25%). Most of the Usher's syndrome cases (87.5%) had consanguinity.
  
  
  CONCLUSIONS
  Screening for congenital SNHL in the early years of life and routine yearly follow-ups are essential for maximizing the rehabilitation of this disabled group. The high rate of visually debilitating syndromic ocular pathologies associated with high frequency of consanguinity doubles the importance.
authors: Department of Ophthalmology, Tatvan Military Hospital, Bitlis, Turkey.
authors: Mehmet Talay
authors: MT
authors: Köylü
authors: Department of Ophthalmology, Kayseri Military Hospital, Kayseri, Turkey.
authors: Gökçen
authors: G
authors: Gökçe
authors: Department of Ophthalmology, Faculty of Medicine, Hacettepe University, Ankara, Turkey.
authors: Güngor
authors: G
authors: Sobaci
authors: Department of Public Health, Gülhane Military Medical School, Ankara, Turkey.
authors: Fahrettin Güven
authors: FG
authors: Oysul
authors: Department of Ophthalmology, Gülhane Military Medical School, Ankara, Turkey.
authors: Dorukcan
authors: D
authors: Akincioğlu
mesh_terms: D000293:Adolescent; D003241:Consanguinity; D005260:Female; D006319:Hearing Loss, Sensorineural; D006801:Humans; D011446:Prospective Studies; D012174:Retinitis Pigmentosa; D055815:Young Adult
keywords: Consanguinity; Usher’s syndrome; ophthalmologic screening; retinitis pigmentosa; sensorineural deafness