Download PDF
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)

Also available at
   Permissions and Reprints

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
 

  • References

  • 1 Bronya JB. . Keats, FACMG and Jennifer Lentz. Usher Syndrome Type I. GeneReviews® [Internet]. Initial Posting: December 10, 1999; Last Update: June 20, 2013. http://www.ncbi.nlm.nih.gov/books/NBK1265/ accessed in October,2015
  • 2 Loundon N, Marlin S, Busquet D. , et al. Usher syndrome and cochlear implantation. Otol Neurotol 2003; 24 (02) 216-221
    CrossrefGoogle Scholar
  • 3 Damen GW, Pennings RJ, Snik AF, Mylanus EA. Quality of life and cochlear implantation in Usher syndrome type I. Laryngoscope 2006; 116 (05) 723-728
    CrossrefGoogle Scholar
  • 4 Jatana KR, Thomas D, Weber L, Mets MB, Silverman JB, Young NM. Usher syndrome: characteristics and outcomes of pediatric cochlear implant recipients. Otol Neurotol 2013; 34 (03) 484-489
    CrossrefGoogle Scholar
  • 5 Henricson C, Wass M, Lidestam B, Möller C, Lyxell B. Cognitive skills in children with Usher syndrome type 1 and cochlear implants. Int J Pediatr Otorhinolaryngol 2012; 76 (10) 1449-1457
    CrossrefGoogle Scholar
  • 6 Liu XZ, Angeli SI, Rajput K. , et al. Cochlear implantation in individuals with Usher type 1 syndrome. Int J Pediatr Otorhinolaryngol 2008; 72 (06) 841-847
    CrossrefGoogle Scholar
  • 7 Pietola L, Aarnisalo AA, Abdel-Rahman A. , et al. Speech recognition and communication outcomes with cochlear implantation in Usher syndrome type 3. Otol Neurotol 2011; 33 (01) 38-41
    Google Scholar
  • 8 Goffi-Gomez MVS, Guedes MC, Sant'anna SBG. , et al. Critérios de seleção e avaliação médica e audiológico dos candidatos ao implante coclear. Protocolo HCFMUSP. Arquivos Int Otorrinolaringol. 2004; 8 (04) 303-323
    Google Scholar
  • 9 Lazard DS, Vincent C, Venail F. , et al. Pre-, per- and postoperative factors affecting performance of postlinguistically deaf adults using cochlear implants: a new conceptual model over time. PLoS ONE 2012; b 7 (11) e48739
    CrossrefGoogle Scholar
  • 10 Lee HJ, Giraud AL, Kang E. , et al. Cortical activity at rest predicts cochlear implantation outcome. Cereb Cortex 2007; 17 (04) 909-917
    CrossrefGoogle Scholar
  • 11 Lazard DS, Giraud AL, Gnansia D, Meyer B, Sterkers O. Understanding the deafened brain: implications for cochlear implant rehabilitation. Eur Ann Otorhinolaryngol Head Neck Dis 2012; a 129 (02) 98-103
    CrossrefGoogle Scholar
  • 12 Butler BE, Lomber SG. Functional and structural changes throughout the auditory system following congenital and early-onset deafness: implications for hearing restoration. Front Syst Neurosci 2013; 7: 92
    Google Scholar
  • 13 Sharma A, Nash AA, Dorman M. Cortical development, plasticity and re-organization in children with cochlear implants. J Commun Disord 2009; 42 (04) 272-279
    CrossrefGoogle Scholar
  • 14 Campbell J, Sharma A. Cross-modal re-organization in adults with early stage hearing loss. PLoS ONE 2014; 9 (02) e90594
    CrossrefGoogle Scholar
  • 15 Broomfield SJ, Bruce IA, Henderson L, Ramsden RT, Green KM. Cochlear implantation in children with syndromic deafness. Int J Pediatr Otorhinolaryngol 2013; 77 (08) 1312-1316
    CrossrefGoogle Scholar
  • 16 Wiley S, Jahnke M, Meinzen-Derr J, Choo D. Perceived qualitative benefits of cochlear implants in children with multi-handicaps. Int J Pediatr Otorhinolaryngol 2005; 69 (06) 791-798
    CrossrefGoogle Scholar
  • 17 Edwards LC. Children with cochlear implants and complex needs: a review of outcome research and psychological practice. J Deaf Stud Deaf Educ 2007; 12 (03) 258-268
    CrossrefGoogle Scholar
  • 18 Daneshi A, Hassanzadeh S. Cochlear implantation in prelingually deaf persons with additional disability. J Laryngol Otol 2007; 121 (07) 635-638
    CrossrefPubMedGoogle Scholar
  • 19 Vlahović S, Sindija B. The influence of potentially limiting factors on paediatric outcomes following cochlear implantation. Int J Pediatr Otorhinolaryngol 2004; 68 (09) 1167-1174
    CrossrefGoogle Scholar