Early diagnosis of Usher syndrome in children

Marilyn B Mets*, Nancy M Young, A. Pass, Janice Lasky, S. H. Day, A. J. Flach, M. T. Miller

*Corresponding author for this work

Research output: Contribution to journalArticle

33 Scopus citations

Abstract

Purpose: To screen severe to profound, preverbal hearing-impaired children for Usher syndrome by ophthalmologic examinations, including electroretinographic testing. These patients are especially good candidates for early cochlear implants, which will improve listening and spoken language skills. Methods: Consecutive patients over 2 years of age, given a diagnosis of severe to profound, preverbal hearing loss, were screened for Usher syndrome by a complete ophthalmologic examination including an electroretinogram. Results: Five of 48 patients screened (10.4%) were diagnosed with Usher syndrome and received cochlear implants. Conclusion: All children with severe to profound, preverbal sensorineural hearing loss should be screened for Usher syndrome by ophthalmologic examination including electroretinogram.

Original languageEnglish (US)
Pages (from-to)237-245
Number of pages9
JournalTransactions of the American Ophthalmological Society
Volume98
StatePublished - Dec 1 2000

ASJC Scopus subject areas

  • Ophthalmology

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