title: Outcomes of Late Implantation in Usher Syndrome Patients.
abstract: IntroductionUsher 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?ObjectiveThe objective of this study is to describe the outcomes of US patients who received cochlear implants at a later age.MethodsThis 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.ResultsAverage 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.ConclusionWe 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.
authors.affiliation: Department of Otolaryngology, Hospital das Clínicas, São Paulo, SP, Brazil.
authors.forename: Ana Cristina H
authors.initials: AC
authors.lastname: Hoshino
authors.affiliation: Department of Otolaryngology, Hospital das Clínicas, São Paulo, SP, Brazil.
authors.forename: Agustina
authors.initials: A
authors.lastname: Echegoyen
authors.affiliation: Cochlear Implant Group, School of Medicine, Hospital das Clínicas, Universidade de São Paulo, São Paulo, SP, Brazil.
authors.forename: Maria Valéria Schmidt
authors.initials: MV
authors.lastname: Goffi-Gomez
authors.affiliation: Department of Otolaryngology, Universidade de São Paulo, São Paulo, SP, Brazil.
authors.forename: Robinson Koji
authors.initials: RK
authors.lastname: Tsuji
authors.affiliation: Department of Otolaryngology, Universidade de São Paulo, São Paulo, SP, Brazil.
authors.forename: Ricardo Ferreira
authors.initials: RF
authors.lastname: Bento
keywords: cochlear implant; delayed diagnosis; quality of life; usher syndromes