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CC BY-NC-ND 4.0 · Int Arch Otorhinolaryngol 2017; 21(02): 140-143
DOI: 10.1055/s-0036-1583306
Original Research
Thieme-Revinter Publicações Ltda Rio de Janeiro, Brazil

Outcomes of Late Implantation in Usher Syndrome Patients

Ana Cristina H. Hoshino
1   Department of Otolaryngology, Hospital das Clínicas, São Paulo, SP, Brazil
,
Agustina Echegoyen
1   Department of Otolaryngology, Hospital das Clínicas, São Paulo, SP, Brazil
,
Maria Valéria Schmidt Goffi-Gomez
2   Cochlear Implant Group, School of Medicine, Hospital das Clínicas, Universidade de São Paulo, São Paulo, SP, Brazil
,
Robinson Koji Tsuji
3   Department of Otolaryngology, Universidade de São Paulo, São Paulo, SP, Brazil
,
Ricardo Ferreira Bento
3   Department of Otolaryngology, Universidade de São Paulo, São Paulo, SP, Brazil
› Author Affiliations
Further Information

Publication History

09 November 2015

20 March 2016

Publication Date:
04 May 2016 (online)

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Abstract

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.

Keywords

usher syndromes - cochlear implant - delayed diagnosis - quality of life
 

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