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.
PURPOSE: To screen severe to profound, preverbal hearing-impairedchildren 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.
Authors: S Wayne; V M Der Kaloustian; M Schloss; R Polomeno; D A Scott; J F Hejtmancik; V C Sheffield; R J Smith Journal: Hum Mol Genet Date: 1996-10 Impact factor: 6.150
Authors: M Hmani; A Ghorbel; A Boulila-Elgaied; Z Ben Zina; W Kammoun; M Drira; M Chaabouni; C Petit; H Ayadi Journal: Eur J Hum Genet Date: 1999-04 Impact factor: 4.246
Authors: E M Sankila; L Pakarinen; H Kääriäinen; K Aittomäki; S Karjalainen; P Sistonen; A de la Chapelle Journal: Hum Mol Genet Date: 1995-01 Impact factor: 6.150
Authors: R J Smith; E C Lee; W J Kimberling; S P Daiger; M Z Pelias; B J Keats; M Jay; A Bird; W Reardon; M Guest Journal: Genomics Date: 1992-12 Impact factor: 5.736
Authors: J Kaplan; S Gerber; D Bonneau; J M Rozet; O Delrieu; M L Briard; H Dollfus; I Ghazi; J L Dufier; J Frézal Journal: Genomics Date: 1992-12 Impact factor: 5.736
Authors: Richard J Vivero; Kenneth Fan; Simon Angeli; Thomas J Balkany; Xue Z Liu Journal: Int J Pediatr Otorhinolaryngol Date: 2010-07-22 Impact factor: 1.675
Authors: Magnus Teschner; Juergen Neuburger; Roland Gockeln; Thomas Lenarz; Anke Lesinski-Schiedat Journal: Eur Arch Otorhinolaryngol Date: 2007-12-06 Impact factor: 2.503
Authors: William J Kimberling; Michael S Hildebrand; A Eliot Shearer; Maren L Jensen; Jennifer A Halder; Karmen Trzupek; Edward S Cohn; Richard G Weleber; Edwin M Stone; Richard J H Smith Journal: Genet Med Date: 2010-08 Impact factor: 8.822
Authors: Greta Glover; Kaspar P Mueller; Christian Söllner; Stephan C F Neuhauss; Teresa Nicolson Journal: Mol Vis Date: 2012-09-05 Impact factor: 2.367