Turkish Journal of Medical Sciences




Background/aim: 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). Materials and 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. Conclusion: 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.


Consanguinity, ophthalmologic screening, retinitis pigmentosa, sensorineural deafness, Usher?s syndrome

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