Diagnostik und Therapie der auditorischen Neuropathie

Zusammenfassung

Die auditorische Neuropathie (AN) zeichnet sich durch nachweisbare evozierbare otoakustische Emissionen (EOAE) und nicht ableitbare bzw. nur mit erhöhten Schwellen ableitbare auditorisch evozierte Hirnstammpotenziale aus. Als Ursache mus eine Funktionsstörung der inneren Haarzellen und/oder eine Störung der neuronalen Reizweiterleitung bei intakten äußeren Haarzellen angenommen werden.

Betroffene Patienten verhalten sich in schweren Fällen wie Schwerhörige mit einer hochgradigen kochleären Schwerhörigkeit, in milderen Fällen kann das Tonschwellenaudiogramm weitgehend normal sein, das Sprachverständnis ist meistens jedoch deutlich eingeschränkt.

Die auditorische Neuropathie scheint häufiger zu sein als noch vor einiger Zeit angenommen. Bei anamnestisch zu vermutender Schwerhörigkeit, insbesondere bei Kindern mit einem Sprachentwicklungsrückstand – gerade, wenn ein Sprachaudiogramm noch nicht durchführbar erscheint – gutem Tonschwellenaudiogramm und nachweisbaren EOAE ist die auditorische Neuropathie immer diffenzialdiagnostisch zu berücksichtigen.

Summary

The combined findings of „normal evoked otoacoustic emissions (EOAE) and absent or markedly disturbed auditory evoked potentials from the brainstem has been named auditory neuropathy. The pure tone thresholds in these patients range from mild to severe loss. The loss of speech comprehension is out of proportion in comparison to the pure tone threshold elevation. Presumably, cochlear function and in particular outer hair cell function, is normal in these patients but also suggests that the inner hair cell/VIII nerve functional unit is abnormal. Disruption in neural synchrony has previously been postulated as a reasonable explanation for the absent auditory brainstem response.

The entire set of findings, consistently seen in these patients, supports the importance of the crosscheck principle in diagnostic evaluation especially in children with delayed and/or impaired language acquisition. It is of ponderous importance that this disorder is not being confused with sensory hearing loss.

Schlüsselwörter

Otoakustische Emissionen - auditorisch evozierte Potenziale - auditorische Neuropathie

Keywords

Otoacoustic emissions - auditory evoked potentials - auditory neuropathy

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