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Cochlear implantation in Bjornstad syndrome: a case series with literature review

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Abstract

Background

To report the presentation, diagnostic process, management and results of cochlear implantation of patients diagnosed with Bjornstad syndrome with profound sensorineural hearing loss (SNHL).

Case presentation and management

A retrospective report of two siblings with Bjornstad syndrome suffering profound SNHL unresponsive to conventional hearing aids treated with bilateral simultaneous cochlear implantation.

Setting

Tertiary-referral center.

Results

Cochlear implant surgeries of two siblings (four ears) with profound SNHL and bilateral inner ear anomaly (incomplete partition type 1) were performed without complications. Postoperative audiometric measurements showed a significiant improvement in pure-tone threshold and a word recognition score. In the literature review, no previous case of Bjornstad syndrome treated with cochlear implantation has been reported.

Conclusions

Cochlear implantation is an effective, safe, and ultimate treatment option for Bjornstad syndrome with profound SNHL not responding to hearing aids.

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Data availability

The data that support the findings of this study are available from the authors, upon reasonable request.

References

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Funding

No funding was received for this research.

Author information

Authors and Affiliations

  1. Department of Otolaryngology, Head and Neck Surgery, Dr Ersin Arslan Training and Research Hospital, Eyüpoğlu Mah. Hürriyet Cad. No: 40, Şahinbey, Gaziantep, Turkey

    Secaattin Gülşen

  2. Private Clinic, Şahinbey, Gaziantep, Turkey

    Sercan Çıkrıkcı

Authors
  1. Secaattin Gülşen
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  2. Sercan Çıkrıkcı
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Contributions

SG: analyzing the data, coordinating the study, conceptualization of study design and writing the manuscript; SÇ: literature search, data collection, interpretation, designing the study, writing the manuscript. All co-authors of the study take full responsibility for the integrity of the final version of the manuscript.

Corresponding author

Correspondence to Sercan Çıkrıkcı.

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Conflict of interest

The author declares that he has no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. This article does not contain any studies with animals performed by any of the authors.

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Informed consent was obtained from all individual participants included in the study.

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Gülşen, S., Çıkrıkcı, S. Cochlear implantation in Bjornstad syndrome: a case series with literature review. Eur Arch Otorhinolaryngol 281, 1047–1052 (2024). https://doi.org/10.1007/s00405-023-08265-6

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  • DOI: https://doi.org/10.1007/s00405-023-08265-6

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