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Auditorische Synaptopathie/Neuropathie

Klinik und Diagnostik

Auditory synaptopathy/neuropathy

Clinical findings and diagnosis

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Zusammenfassung

Die auditorische Synaptopathie/Neuropathie (AS/AN) stellt eine in ihrer Häufigkeit unterschätzte Form der sensorineuralen Schwerhörigkeit mit sehr heterogener klinischer Ausprägung dar. Bei vielen Betroffenen besteht die Hörstörung seit Geburt, bei einigen erst im Erwachsenenalter. Symptome sind der schwankende, meist beidseitig auftretende Hörverlust und die oft starke Einschränkung des Sprachverstehens, insbesondere im Störgeräusch. Otoakustische Emissionen (OAE) und cochleäre Mikrofonpotenziale (CM) lassen sich nachweisen, Stapediusreflexe fehlen und frühe akustisch evozierte Potenziale (FAEP) sind nicht nachweisbar oder stark verändert. Durch ein rein OAE-basiertes Neugeborenen-Hörscreening werden Kinder mit AS/AN nicht erkannt. Klinische Befunde, transtympanale Elektrocochleographie (ECochG) und weiterführende Diagnostik lassen die individuelle Ausprägung der AS/AN erkennen. Im Einzelfall können der Einsatz von Hörgeräten und/oder FM-Anlagen die Hör- und Kommunikationsfähigkeit verbessern. Sind diese Maßnahmen in Verbindung mit einer intensiven Hör-, Sprech- und Sprachfrühförderung unzureichend, stellen die CI-Versorgung oder der Einsatz alternativer Kommunikationsformen sinnvolle Optionen dar.

Abstract

Auditory synaptopathy/neuropathy (AS/AN) is a special subtype of sensorineural hearing disorders with heterogenous phenotypes and underestimated incidence. AS/AN generally develops in infancy, occasionally in adulthood. Symptoms include fluctuating, mostly bilateral hearing loss and abnormally reduced speech comprehension, especially in noisy environments. Within audiological assessments, patients with AS/AN present otoacoustic emissions (TEOAE; DPOAE) and cochlear microphonics (CM), absence of stapedius reflexes (SR) as well as absent or pathologically altered auditory evoked brainstem potentials (ABR). Children with AS/AN cannot be identified within OAE-based newborn hearing screening programs. Clinical findings, transtympanic electrocochleography (ECoG) and further diagnostic tools permit further identification of individual characteristics. In individual cases conventional amplification and the use of FM systems may improve hearing and communication skills. If these interventions, accompanied by intensive hearing, speech and language therapy are unsuccessful, cochlear implants (CI) or alternative forms of communication may be useful options for rehabilitation.

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Authors and Affiliations

  1. Audiologie und Pädaudiologie, Cochlear Implant Centrum Köln, HNO-Universitätsklinik Köln, Kerpener Straße 62, 50937, Köln, Deutschland

    M. Walger, A. Foerst, D. Beutner, B. Streicher, K. Stürmer & R. Lang-Roth

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  1. M. Walger
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  2. A. Foerst
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  3. D. Beutner
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  4. B. Streicher
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  5. K. Stürmer
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  6. R. Lang-Roth
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Correspondence to M. Walger.

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Walger, M., Foerst, A., Beutner, D. et al. Auditorische Synaptopathie/Neuropathie. HNO 59, 414–424 (2011). https://doi.org/10.1007/s00106-011-2301-4

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  • DOI: https://doi.org/10.1007/s00106-011-2301-4

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