Molekularbiologie des Gehörs

Zusammenfassung

Das Innenohr ist das empfindlichste Sinnesorgan des Menschen und kann in drei funktionelle Abschnitte unterteilt werden: Corti-Organ, Stria vascularis und Spiralganglion. Der adäquate Reiz für das Hörorgan ist Schall, der durch den äußeren Gehörgang an das Trommelfell gelangt und über das Mittelohr auf das Innenohr übertragen wird. Dort befinden sich die inneren Haarzellen, die für den entscheidenden Schritt des Hörvorgangs, die Mechanotransduktion, verantwortlich sind. Dieser Mechanismus ermöglicht die Umwandlung des mechanischen Schallsignals in ein bioelektrisches bzw. biochemisches Signal. Die Stria vascularis bildet das endokochleäre Potenzial und ist verantwortlich für die Aufrechterhaltung der kochleären Ionenhomöostase. Das Spiralganglion bildet synaptische Kontakte mit den Haarzellen und ist aufgebaut aus Neuronen, die elektrische Signale von der Cochlea ins zentrale Nervensystem übermitteln.

In den vergangenen Jahren konnten entscheidende Fortschritte bei der Erforschung der molekularen Grundlagen des Hörvorgangs erzielt werden. Immer mehr Gene und Proteine, die für den Vorgang des Hörens verantwortlich sind, können identifiziert und charakterisiert werden. Das ständig wachsende Wissen über diese Gene hilft nicht nur, den Mechanismus des Hörens besser zu verstehen, sondern trägt auch zu einem tieferen Verständnis der molekularen Grundlagen von genetisch bedingten Hörstörungen bei. Diese Grundlagenforschung stellt die Voraussetzung für die Entwicklung molekulargenetischer Diagnoseverfahren sowie moderner Therapieformen für die Schwerhörigkeit dar.

Abstract

Molecular Biology of Hearing

The inner ear is our most sensitive sensory organ and can be subdivided into 3 functional units: organ of Corti, stria vascularis and spiral ganglion. The appropriate stimulus for the organ of hearing is sound which travels through the external auditory canal to the middle ear where it is transmitted to the inner ear. The inner ear habors the hair cells, the sensory cells of hearing. The inner hair cells are capable of mechanotransduction, the transformation of mechanical force into an electrical signal, which is the basic principle of hearing. The stria vascularis generates the endocochlear potential and maintains the ionic homeostasis of the endolymph. The dendrites of the spiral ganglion form synaptic contacts with the hair cells. The spiral ganglion is composed of neurons that transmit the electrical signals from the cochlea to the central nervous system. In the past years there was significant progress in research on the molecular basis of hearing. More and more genes and proteins which are related to hearing can be identified and characterized. The increasing knowledge on these genes contributes not only to a better understanding of the mechanism of hearing but also to a deeper understanding of the molecular basis of hereditary hearing loss. This basic research is a prerequisite for the development of molecular diagnostics and novel therapies for hearing loss.

Schlüsselwörter

Innenohr - Cochlea - Haarzelle - Corti-Organ - Spiralganglion - Taubheit

Key words

inner ear - cochlea - hair cell - organ of Corti - spiral ganglion - deafness

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