AI Article Synopsis

  • This study assessed the long-term effectiveness of cochlear implants in individuals with specific genetic variants linked to hearing loss conditions, namely Wolfram-like syndrome and DFNA6/14/38.
  • The research involved a retrospective analysis of seven cochlear implant recipients, revealing high phoneme recognition scores that remained stable for up to ten years post-implantation.
  • Despite positive outcomes for most participants, one individual struggled significantly due to prior lack of auditory experience, emphasizing the importance of timely intervention and rehabilitation.

Article Abstract

Objective: This study aimed to evaluate long-term cochlear implant (CI) outcomes in individuals with mono-allelic pathogenic variants in WFS1, which is associated with both Wolfram-like syndrome and DFNA6/14/38.

Design: Retrospective case series.

Study Sample: Seven CI recipients, ranging from eight months to 58 years of age, were included in the study, including four with Wolfram-like syndrome and three with DFNA6/14/38. A total of ten cochlear implantations were performed among these subjects.

Results: At one-year post-implantation, a mean phoneme score of 90 ± 9% at 65 dB SPL in quiet was found, which remained stable up to ten years post-implantation with a mean phoneme score of 94 ± 6%. Despite these excellent outcomes, one subject achieved no speech recognition with CI and eventually became a non-user. This individual had a prolonged absence of auditory stimulation prior to implantation and encountered multiple challenges during rehabilitation.

Conclusion: Individuals with Wolfram-like syndrome or DFNA6/14/38 demonstrate consistently good outcomes following implantation, which remain stable over time. These findings affirm cochlear implantation as an effective rehabilitation option for these individuals. Furthermore, the stable and good CI outcomes contradict the suggested link between -associated sensorineural hearing loss and auditory neuropathy.

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Source
http://dx.doi.org/10.1080/14992027.2024.2411579DOI Listing

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