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Etiology and therapy indication for cochlear implantation in children with single-sided deafness

Retrospective analysis

Ätiologie und Therapieindikation zur Cochleaimplantation bei Kindern mit unilateraler Taubheit

Retrospektive Analyse

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Abstract

Objective

The characteristics of children with single-sided deafness (SSD) who become candidates for unilateral cochlear implantation (uCI) were identified.

Study design

In all, 118 children with SSD presenting from 2013–2019 to a tertiary pediatric children’s hospital were retrospectively assessed regarding candidacy for uCI.

Results

Of the 118 children, 103 had completed uCI candidacy assessment, while 15 were undergoing this assessment at the time of review. More than half of children did not go on to implantation (63/103, 61%), with the 2 main reasons being (1) half (31/63) did not meet candidacy criteria for implantation, most commonly due to cochlear nerve aplasia/hypoplasia (31/82 who were assessed with MRI, 38%) and (2) families (30/103; 29%) declined participation in the surgical arm of the trial. The most common etiologies of SSD in the 37/103 (36%) children who both met candidacy and consented to implantation were congenital cytomegalovirus (cCMV; 16/37, 43%), unknown (6/37, 16%), cochleovestibular anomaly and trauma (each 5/37, 14%).

Conclusions

Many children with SSD who present for implant candidacy assessment do not ultimately receive uCI. Major factors contributing to noncandidacy are cochlear nerve aplasia and parental acceptance of the intervention. While approximately half of children with SSD in our cohort were candidates for implantation, only 1/3 of the total cohort proceeded with implantation with the main predictors of acceptability of this intervention being an etiology (i.e., cCMV) that carries risk of progressive deterioration in the better hearing ear or SSD that was sudden in onset. These findings provide important insight into this new population of cochlear implant users and the emerging acceptance of intervention in children with SSD.

Zusammenfassung

Ziel

Es wurden Merkmale von Kindern mit einseitiger Taubheit („single-sided deafness“, SSD) herausgearbeitet, bei denen eine unilaterale Cochleaimplantation (uCI) infrage kam.

Studiendesign

Insgesamt wurden die Fälle von 118 Kindern mit SSD, die sich in der Zeit von 2013–2019 in einem Kinderkrankenhaus der Tertiärversorgung vorstellten, retrospektiv in Bezug auf die Eignung als Kandidat für eine uCI ausgewertet.

Ergebnisse

Von den 118 Kindern hatten 103 die vollständigen Voruntersuchungen für eine uCI durchlaufen, während 15 diese Voruntersuchungen zum Zeitpunkt der vorliegenden Arbeit noch absolvierten. Bei mehr als der Hälfte der Kindern kam es nicht zur Implantation (63/103, 61 %), Folgendes waren die beiden Hauptgründe: Zum einen erfüllte die Hälfte (31/63) nicht die Kriterien als Kandidat für eine Implantation, zumeist aufgrund einer Aplasie/Hypoplasie des N. cochlearis (31/82 mit erfolgter MRT-Untersuchung, 38 %), und zum anderen lehnten manche Familien (30/103, 29 %) die Teilnahme am chirurgischen Arm der Studie ab. Die häufigsten Ätiologien der SSD bei den 37/103 (36 %) Kindern, die sowohl die Kriterien als Kandidat für eine Implantation erfüllten als auch der Implantation zustimmten, bestanden aus kongenitaler Zytomegalovirusinfektion (cCMV; 16/37, 43 %), unbekannten Ursachen (6/37, 16 %), cochleovestibulären Anomalien und Traumata (je 5/37, 14 %).

Schlussfolgerung

Bei vielen Kindern mit SSD, die sich zur Voruntersuchung für eine uCI vorstellen, wurde letztlich keine uCI durchgeführt. Hauptfaktoren dafür sind die Aplasie des N. cochlearis und die nicht erteilte Zustimmung der Eltern zu der Intervention. Während annähernd die Hälfte der Kinder mit SSD in der untersuchten Kohorte Kandidaten für eine uCI waren, kam es nur bei einem Drittel der Gesamtkohorte zur Implantation; dabei waren die Hauptprädiktoren der Akzeptanz dieser Intervention eine Ätiologie (z. B. cCMV), bei der das Risiko einer progredienten Verschlechterung auf dem besser hörenden Ohr besteht, oder eine plötzlich aufgetretene SSD. Aus den vorliegenden Ergebnissen lassen sich wichtige Erkenntnisse über diese neue Population der Nutzer von Cochleaimplantaten und die sich entwickelnde Akzeptanz einer Intervention bei Kindern mit SSD ziehen.

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Author information

Authors and Affiliations

  1. Department of Otolaryngology, Head and Neck Surgery, Hospital for Sick Children, Room 6103C Burton Wing, 555 University Avenue, M5G 1X8, Toronto, ON, Canada

    S. L. Cushing MD MSc. FRCSC, M. Sokolov & B. C. Papsin

  2. Department of Otolaryngology, Head and Neck Surgery, University of Toronto, Toronto, ON, Canada

    S. L. Cushing MD MSc. FRCSC, K. A. Gordon, M. Sokolov & B. C. Papsin

  3. Archie’s Cochlear Implant Laboratory, Hospital for Sick Children, Toronto, ON, Canada

    S. L. Cushing MD MSc. FRCSC, K. A. Gordon, M. Sokolov, M. Polonenko & B. C. Papsin

  4. Department of Communication Disorders, Hospital for Sick Children, Toronto, ON, Canada

    K. A. Gordon & V. Papaioannou

  5. Department of Neurosciences & Mental Health, Hospital for Sick Children, Toronto, ON, Canada

    M. Polonenko

  6. Institute of Medical Science, University of Toronto, Toronto, ON, Canada

    M. Polonenko

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  1. S. L. Cushing MD MSc. FRCSC
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Correspondence to S. L. Cushing MD MSc. FRCSC.

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Conflict of interest

S.L. Cushing: Speaker’s Bureau Cochlear Corporation. Oticon, Interacoustics. K.A. Gordon and B.C. Papsin: Speaker’s Bureau Cochlear Corporation. M. Sokolov, V. Papaioannou and M. Polonenko declare that they have no competing interests.

For this article no studies with human participants or animals were performed by any of the authors. All studies performed were in accordance with the ethical standards indicated in each case.

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Cushing, S.L., Gordon, K.A., Sokolov, M. et al. Etiology and therapy indication for cochlear implantation in children with single-sided deafness. HNO 67, 750–759 (2019). https://doi.org/10.1007/s00106-019-00729-8

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