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Outcomes of cochlear implantation in children with Usher syndrome: a long-term observation

  • Otology
  • Published:
European Archives of Oto-Rhino-Laryngology Aims and scope Submit manuscript

Abstract

Purpose

To evaluate auditory performance and speech intelligibility of children with Usher syndrome up to 10 years after cochlear implantation.

Methods

Thirty-five children with USH were compared to 46 non-syndromic patients regarding age at implantation. Auditory performance and speech intelligibility was assessed with standard tools. Genetic counseling, vestibular tests, imaging studies, and ophthalmological findings were evaluated, depending on the availability.

Results

The mean age of implantation in USH children was 6.3 years (SD 4.6, range 0.3–17.6 years). Post-implantation values of the studied parameters were compared between USH and NS children and presented as follows: PTA = 25.0 dB HL vs. 28.4, CAP = 5.3 vs. 5.1, SIR = 4.1 vs. 3.9, MAIS = 82.3% vs. 80.5%, MUSS = 81.8% vs. 76.6%. There were no statistically significant differences between the USH and NS groups (p > 0.005). USH patients reached a higher score ceiling earlier compared to NS patients. Children implanted before 3 years of age achieved significantly higher results than older children in USH and NS groups (p < 0.005). In all patients with USH, the electroretinogram was abnormal. Vestibular examination was abnormal in 29 of 31 patients with USH1. Imaging studies revealed no inner ear or auditory nerve anomalies in patients with USH.

Conclusion

Cochlear implantation successfully improves auditory performance and speech intelligibility in patients with USH, especially those implanted under 3 years of age. The electroretinogram is the only reliable test to establish a diagnosis of USH. Logopedic outcomes are associated with early implantation, and early diagnosis of USH contributes to optimizing speech therapy.

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Authors and Affiliations

  1. Clarós Clinic, Cochlear Implant Centre, c./Vergós 31, 08017, Barcelona, Spain

    Agnieszka Remjasz-Jurek, Pedro Clarós, Astrid Clarós-Pujol, Carmen Pujol & Andrés Clarós

  2. Department of Otorhinolaryngology, Stefan Zeromski Specialist Hospital, Cracow, Poland

    Agnieszka Remjasz-Jurek

  3. Scholarship in Clarós Clinic, Barcelona, Spain

    Agnieszka Remjasz-Jurek

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  1. Agnieszka Remjasz-Jurek
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Correspondence to Pedro Clarós.

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Remjasz-Jurek, A., Clarós, P., Clarós-Pujol, A. et al. Outcomes of cochlear implantation in children with Usher syndrome: a long-term observation. Eur Arch Otorhinolaryngol 280, 2119–2132 (2023). https://doi.org/10.1007/s00405-022-07670-7

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  • DOI: https://doi.org/10.1007/s00405-022-07670-7

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