Open Access
CC BY-NC-ND 4.0 · Laryngorhinootologie 2020; 99(S 02): S260
DOI: 10.1055/s-0040-1711099
Abstracts
Otology

Diagnostics prior cochlear implant surgery in children with consanguineous parents

Authors

  • D Guderian

    1   Hals-Nasen-Ohrenklinik des Universitätsklinikums Frankfurt Frankfurt/M.

  • S Helbig

    1   Hals-Nasen-Ohrenklinik des Universitätsklinikums Frankfurt Frankfurt/M.

  • S Kramer

    2   Hals-Nasen-Ohrenklinik des Universitätsklinikums Frankfurt, Schwerpunkt Phoniatrie und Pädaudiologie Frankfurt/M.

  • I Burck

    3   Institut für diagnostische und interventionelle Radiologie des Universitätsklinikums Frankfurt Frankfurt/M.

  • T Stöver

    1   Hals-Nasen-Ohrenklinik des Universitätsklinikums Frankfurt Frankfurt/M.

  • M Diensthuber

    1   Hals-Nasen-Ohrenklinik des Universitätsklinikums Frankfurt Frankfurt/M.
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Introduction Consanguinity is associated with an increased risk for genetic diseases and malformations which can manifest in various ways. A small but significant part of all paediatric cochlear implant candidates are children of consanguineous parents.

Methods For this study we retrospectively analysed patient files from children ≤ 6 years (n=370) who underwent diagnostics prior cochlear implant surgery. Using parents’ self-disclosure we identified children with consanguine background. Results of radiological (CT/MRI) and audiological findings as well as the recommended interventions for hearing rehabilitation of these patients were analyzed.

Results 48 patients of the patient group where children of consanguine parents (13,0%). Of these, 13 patients (27,1%) had an inner ear malformation. Hypoplasia of the cochlear nerve was most prevalent (n=3; 23,1%). In 42 patients (87,5%) profound hearing impairment or deafness was diagnosed. 33 (68,8%) patients were recommended to undertake cochlear implant surgery.

Conclusion Our data show, that a significant fraction of the patients with consanguineous background showed an inner ear malformation. Cochlear implant treatment is therefore often a particular challenge in these patients.

Poster-PDF A-1785.PDF


Dr. med. Daniela Guderian
Hals-Nasen-Ohrenklinik des Universitätsklinikums Frankfurt
Theodor-Stern-Kai 7
60590 Frankfurt/M.

Publication History

Article published online:
10 June 2020

© 2020. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).

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