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Update zur Physiologie und Pathophysiologie des Innenohrs

Pathomechanismen der sensorineuralen Schwerhörigkeit

Update on physiology and pathophysiology of the inner ear

Pathomechanisms of sensorineural hearing loss

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Zusammenfassung

Die Schwerhörigkeit ist die häufigste humane Sinnesbehinderung. Der sensorineuralen Schwerhörigkeit (SNSH), auf die etwa 70% der Schwerhörigkeiten entfallen, liegen verschiedene pathologische Veränderungen im Innenohr und im Hörnerv zugrunde. Die individuelle Beeinträchtigung durch die SNSH und der Erfolg einer apparativen Rehabilitation hängen kritisch von den zugrunde liegenden Pathomechanismen ab. In dieser Übersicht stellen wir aktuelle Erkenntnisse über die zelluläre Pathophysiologie der SNSH vor, die überwiegend aus Studien an Mauslinien mit gezielten genetischen Modifikationen gewonnen wurden. Zuerst werden interessante Einsichten aus Experimenten von Mausmutanten mit spezifischen Defekten der kochleären Ionenhomöostase oder Verstärkung dargestellt. Wir wenden uns dann ausführlich krankhaften Veränderungen der afferenten Synapse der inneren Haarzellen (auditorische Synaptopathie) und des Hörnerven (auditorische Neuropathie) zu. Beide nosologischen Entitäten haben in den letzten Jahren auch große klinische Beachtung gefunden. Diesen SNSH-Varianten ist die gestörte zeitliche Verarbeitung von Schallsignalen gemeinsam. Daraus resultiert ein besonders schlechtes Sprachverständnis, dessen Störung oft über das von der Tonschwelle erwartete Maß hinausgeht. Das Ausmaß der Schwerhörigkeit reicht von milden Formen mit Defizit ausschließlich in der zeitlichen Verarbeitung von Schallsignalen bis hin zur Taubheit. Dabei sind otoakustische Emissionen als Zeichen einer normalen kochleären Verstärkung zumindest initial häufig zu beobachten.

Zusammenfassend kann gesagt werden, dass gut charakterisierte Tiermodelle unser pathophysiologisches Verständnis der SNSH vertiefen. Sie leisten wertvolle Hilfe bei der Etablierung neuer audiologischer Protokolle zur Differenzierung der einer individuellen SNSH zugrunde liegenden Pathomechanismen. Auf diese Weise tragen sie zur gezielten Diagnostik und Rehabilitation von SNSH-Patienten bei.

Abstract

Hearing impairment is the most common form of human sensory deficit. The most frequent form, sensorineural hearing loss (SNHL), which accounts for approximately 70% of cases, encompasses various pathologies in both the inner ear and the auditory nerve. The individual hearing impairment and its outcome following aiding with hearing devices critically depend on the underlying disorder. Here recent progress in our understanding of the cellular mechanisms of SNHL in genetically engineered mouse models is reviewed. First, insights gained from models for specific defects in cochlear sound amplification and ion homeostasis are discussed followed by a focus on disorders of the inner hair cell synapses (auditory synaptopathy) and the auditory nerve (auditory neuropathy). Both nosological entities have also attracted substantial clinical interest in recent years and share an impaired temporal processing of auditory stimuli. This results in poor speech recognition, often out of proportion to the pure tone threshold. Hearing loss can range from mild variants with exclusive deficits of temporal processing to complete deafness. At least initially, signs of normal outer hair cell function such as evoked otoacoustic emissions can be found.

In summary, well-characterized animal models allow us to refine our pathophysiological understanding of SNHL and offer invaluable help in defining toolboxes for investigating the mechanism(s) underlying the SNHL of affected individuals. Together, this will contribute to custom-tailored diagnostics and rehabilitation of SNHL patients.

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Danksagung

Wir bedanken uns bei Frau B. Higazi für das Korrekturlesen und bei Dr. Regis Nouvian für seine Unterstützug bei den Abbildungen 1, 2 und 4. Die Arbeit der Gruppe wurde durch die DFG (FZT Center for Molecular Physiology of the Brain), das BMBF (Bernstein Center for Computational Neuroscience), die EU (Eurohear), das Human Frontier Science Program (T.M.) und Anschubfinanzierungen der medizinischen Fakultät der Universität Göttingen (N.S., A.M.) gefördert.

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Authors and Affiliations

  1. Eaton Peabody Laboratory, MEEI, Harvard University, Boston, USA

    N. Strenzke

  2. InnerEarLab, Department of Otolaryngology and Center for Molecular Physiology of the Brain, Universität Göttingen, Göttingen, Deutschland

    N. Strenzke, D. Pauli-Magnus, A. Meyer, A. Brandt &  T. Moser

  3. InnerEarLab, Department of Otolaryngology and Center for Molecular Physiology of the Brain, Universität Göttingen, Robert-Koch-Straße 40, 37075, Göttingen, Deutschland

    T. Moser

  4. HNO-Universitätsklinik, Hamburg-Eppendorf, Deutschland

    H. Maier

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  1. N. Strenzke
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  2. D. Pauli-Magnus
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  3. A. Meyer
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  4. A. Brandt
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  5. H. Maier
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Correspondence to T. Moser.

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Strenzke, N., Pauli-Magnus, D., Meyer, A. et al. Update zur Physiologie und Pathophysiologie des Innenohrs. HNO 56, 27–36 (2008). https://doi.org/10.1007/s00106-007-1640-7

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