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Diagnostik und Therapie der auditorischen Synaptopathie/Neuropathie

Diagnosis and therapy of auditory synaptopathy/neuropathy

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Zusammenfassung

Die audiologische Konstellation von pathologischen frühen akustisch evozierten Potenzialen (fehlend, erhöhte Schwelle und gestörte Kurvenform) trotz nachweisbarer otoakustischer Emissionen geht häufig mit einer von schlechtem Sprachverständnis geprägten Schwerhörigkeit bzw. mit Taubheit einher. Diese als auditorische Neuropathie erstbeschriebene, heterogene Erkrankungsgruppe beinhaltet peripher-auditorische Störungen der synaptischen Schallkodierung durch innere Haarzellen (Synaptopathie) und/oder der Erregungsbildung und -weiterleitung im Hörnerv (Neuropathie). Dieses Konsensuspapier gibt aktuelle Hintergrundinformationen sowie Empfehlungen zur Diagnostik und Therapie im deutschsprachigen Raum. Es nimmt dabei Bezug auf aktuelle internationale Statements.

Abstract

Pathological auditory brainstem responses (lack of responses, elevated thresholds and perturbed waveforms) in combination with present otoacoustic emissions are typical audiometric findings in patients with a hearing impairment that particularly affects speech comprehension or complete deafness. This heterogenous group of disorders first described as “auditory neuropathy” includes dysfunction of peripheral synaptic coding of sound by inner hair cells (synaptopathy) and/or of the generation and propagation of action potentials in the auditory nerve (neuropathy). This joint statement provides prevailing background information as well as recommendations on diagnosis and treatment. The statement focuses on the handling in the german language area but also refers to current international statements.

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Abb. 1

Notes

  1. Diagnosekriterien: nachweisbare otoakustische Emissionen (OAE) und/oder kochleäre Mikrophonpotenziale bei Pegeln <80 dB HL (CM); Schwelle der frühen akustisch evozierten Potenziale (FAEP) schlechter als 80 dB HL mit schlechter Reproduzierbarkeit der Kurven.

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Authors and Affiliations

  1. HNO-Universitätsklinik Göttingen, Robert-Koch-Straße 40, 37075, Göttingen, Germany

    T. Moser, N. Strenzke & A. Meyer

  2. HNO-Klinik, Medizinische Hochschule Hannover, Germany

    A. Lesinski-Schiedat & T. Lenarz

  3. HNO-Universitätsklinik Köln, Germany

    D. Beutner, A. Foerst, R. Lang-Roth, H. von Wedel & M. Walger

  4. Klinik für Audiologie und Phoniatrie, Charité – Universitätsmedizin Berlin, Germany

    M. Gross

  5. Univ.-Klinik für Kommunikationsstörungen Mainz, Germany

    A. Keilmann & A. Limberger

  6. HNO-Universitätsklinik Regensburg, Germany

    T. Steffens & J. Strutz

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  1. T. Moser
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Correspondence to T. Moser.

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Moser, T., Strenzke, N., Meyer, A. et al. Diagnostik und Therapie der auditorischen Synaptopathie/Neuropathie. HNO 54, 833–841 (2006). https://doi.org/10.1007/s00106-006-1450-3

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