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Outcomes of Late Implantation in Usher Syndrome Patients

Introduction Usher syndrome (US) is an autosomal recessive disorder characterized by hearing loss and progressive visual impairment. Some deaf Usher syndrome patients learn to communicate using sign language. During adolescence, as they start losing vision, they are usually referred to cochlear impl...

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Autores principales: Hoshino, Ana Cristina H., Echegoyen, Agustina, Goffi-Gomez, Maria Valéria Schmidt, Tsuji, Robinson Koji, Bento, Ricardo Ferreira
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Thieme-Revinter Publicações Ltda 2016
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5375700/
https://www.ncbi.nlm.nih.gov/pubmed/28382120
http://dx.doi.org/10.1055/s-0036-1583306
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author Hoshino, Ana Cristina H.
Echegoyen, Agustina
Goffi-Gomez, Maria Valéria Schmidt
Tsuji, Robinson Koji
Bento, Ricardo Ferreira
author_facet Hoshino, Ana Cristina H.
Echegoyen, Agustina
Goffi-Gomez, Maria Valéria Schmidt
Tsuji, Robinson Koji
Bento, Ricardo Ferreira
author_sort Hoshino, Ana Cristina H.
collection PubMed
description Introduction Usher syndrome (US) is an autosomal recessive disorder characterized by hearing loss and progressive visual impairment. Some deaf Usher syndrome patients learn to communicate using sign language. During adolescence, as they start losing vision, they are usually referred to cochlear implantation as a salvage for their new condition. Is a late implantation beneficial to these children? Objective The objective of this study is to describe the outcomes of US patients who received cochlear implants at a later age. Methods This is a retrospective study of ten patients diagnosed with US1. We collected pure-tone thresholds and speech perception tests from pre and one-year post implant. Results Average age at implantation was 18.9 years (5–49). Aided average thresholds were 103 dB HL and 35 dB HL pre and one-year post implant, respectively. Speech perception was only possible to be measured in four patients preoperatively, who scored 13.3; 26.67; 46% vowels and 56% 4-choice. All patients except one had some kind of communication. Two were bilingual. After one year of using the device, seven patients were able to perform the speech tests (from four-choice to close set sentences) and three patients abandoned the use of the implant. Conclusion We observed that detection of sounds can be achieved with late implantation, but speech recognition is only possible in patients with previous hearing stimulation, since it depends on the development of hearing skills and the maturation of the auditory pathways.
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spelling pubmed-53757002017-04-05 Outcomes of Late Implantation in Usher Syndrome Patients Hoshino, Ana Cristina H. Echegoyen, Agustina Goffi-Gomez, Maria Valéria Schmidt Tsuji, Robinson Koji Bento, Ricardo Ferreira Int Arch Otorhinolaryngol Introduction Usher syndrome (US) is an autosomal recessive disorder characterized by hearing loss and progressive visual impairment. Some deaf Usher syndrome patients learn to communicate using sign language. During adolescence, as they start losing vision, they are usually referred to cochlear implantation as a salvage for their new condition. Is a late implantation beneficial to these children? Objective The objective of this study is to describe the outcomes of US patients who received cochlear implants at a later age. Methods This is a retrospective study of ten patients diagnosed with US1. We collected pure-tone thresholds and speech perception tests from pre and one-year post implant. Results Average age at implantation was 18.9 years (5–49). Aided average thresholds were 103 dB HL and 35 dB HL pre and one-year post implant, respectively. Speech perception was only possible to be measured in four patients preoperatively, who scored 13.3; 26.67; 46% vowels and 56% 4-choice. All patients except one had some kind of communication. Two were bilingual. After one year of using the device, seven patients were able to perform the speech tests (from four-choice to close set sentences) and three patients abandoned the use of the implant. Conclusion We observed that detection of sounds can be achieved with late implantation, but speech recognition is only possible in patients with previous hearing stimulation, since it depends on the development of hearing skills and the maturation of the auditory pathways. Thieme-Revinter Publicações Ltda 2016-05-04 2017-04 /pmc/articles/PMC5375700/ /pubmed/28382120 http://dx.doi.org/10.1055/s-0036-1583306 Text en © Thieme Medical Publishers
spellingShingle Hoshino, Ana Cristina H.
Echegoyen, Agustina
Goffi-Gomez, Maria Valéria Schmidt
Tsuji, Robinson Koji
Bento, Ricardo Ferreira
Outcomes of Late Implantation in Usher Syndrome Patients
title Outcomes of Late Implantation in Usher Syndrome Patients
title_full Outcomes of Late Implantation in Usher Syndrome Patients
title_fullStr Outcomes of Late Implantation in Usher Syndrome Patients
title_full_unstemmed Outcomes of Late Implantation in Usher Syndrome Patients
title_short Outcomes of Late Implantation in Usher Syndrome Patients
title_sort outcomes of late implantation in usher syndrome patients
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5375700/
https://www.ncbi.nlm.nih.gov/pubmed/28382120
http://dx.doi.org/10.1055/s-0036-1583306
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