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Der erweiterte Ductus und Saccus endolymphaticus

Teil 1: Analyse bildgebender Befunde

Large endolymphatic duct and sac syndrome (LEDS)

Part I: Analysis of imaging findings

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Zusammenfassung

Hintergrund

Das Syndrom des erweiterten Ductus und Saccus endolymphaticus („large endolymphatic duct and sac syndrome“, LEDS) stellt die häufigste radiologisch nachweisbare Innenohrfehlbildung (IO-FB) assoziiert mit sensorineuraler Schwerhörigkeit (SNHL) dar. Dennoch ist das LEDS im deutschsprachigen Raum relativ wenig bekannt.

Methoden

Unter allen Patienten (P), die bei IO-FB-Verdacht zwischen 1994 and 2003 eine Schnittbildgebung erhielten, identifizierten wir sämtliche LEDS-Fälle. Retrospektiv wurden die klinisch-audiologischen Daten sowie die durchgeführten HR-CT und MRT evaluiert.

Ergebnisse

Von 169 P zeigten 17 P (medianes Alter: 12 Jahre, 12 weiblich) bzw. 28 Ohren ein LEDS. Bei 10 P (6%; 15 Ohren) bestand ein isoliertes LEDS, 7 P (4%, 13 Ohren) zeigten weitere IO-FB. Am häufigsten fanden sich Vestibulumdysplasien (13/13 Ohren), eine Mondini-Deformität (10/13 Ohren) und Bogengangsdilatationen (7/13 Ohren). Schwerere kochleäre und Vestibulum-Bogengangs-IO-FB betrafen 3/13 Ohren. Eine Korrelation zwischen morphologischem Defekt und SNHL-Grad war nicht nachweisbar.

Fazit

Das LEDS kann eine evtl. bisher zu wenig beachtete Ursache einer SNHL sein. Zur Diagnosesicherung ist die Bildgebung erforderlich.

Abstract

Objective

Large endolymphatic duct and sac (LEDS) syndrome is the most common radiologically detectable form of inner ear malformation (IEM) associated with sensorineural hearing loss (SNHL). Nevertheless, it is relatively unknown in the German-speaking countries.

Methods

Among all patients referred from ear, nose, and throat specialists to the radiology department with clinically suspected IEM between 1994 and 2003, we identified the cases of LEDS. The retrospective study included clinical records, high-resolution computed tomography, and magnetic resonance imaging.

Results

Of 169 patients, 17 (median age 12 years; 12 females) showed LEDS. A total of 28 ears were affected. Ten patients (6%; 15 ears) had isolated LEDS, while seven patients showed additional IEM (4%; 13 ears). The most frequent of these was dysplastic vestibule (13/13 ears), followed by Mondini deformity (10/13 ears) and dilated semicircular canals (7/13 ears). Three of 13 ears revealed severe IEM of the cochlea, vestibule, and semicircular canals. No correlation could be demonstrated between the severity of morphological changes and the degree of SNHL.

Conclusion

LEDS may be an underestimated cause of SNHL. Imaging is necessary for a confident diagnosis.

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Authors and Affiliations

  1. Abteilung Phoniatrie und Pädaudiologie, Klinik und Poliklinik für Hals-Nasen-Ohren-Heilkunde, Kopf- und Halschirurgie, Universitätsklinikum der Martin-Luther-Universität Halle-Wittenberg, Magdeburger Straße 12, 06097, Halle/Saale, Deutschland

    S. Bartel-Friedrich & C. Rasinski

  2. Institut für Radiologie am Asklepios-Krankenhaus Weißenfels-Hohenmölsen, Weißenfels, Deutschland

    B. Amaya

  3. Abteilung für Stimm-, Sprach- und Hörstörungen (Phoniatrie/Audiologie), Klinik und Poliklinik für HNO-Heilkunde, Universitätsklinikum Leipzig, Leipzig, Deutschland

    M. Fuchs

  4. Klinik und Poliklinik für Diagnostische Radiologie, Universitätsklinikum der Martin-Luther-Universität Halle-Wittenberg, Halle/Saale, Deutschland

    S. Kösling

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  1. S. Bartel-Friedrich
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  2. B. Amaya
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  3. C. Rasinski
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  4. M. Fuchs
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  5. S. Kösling
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Correspondence to S. Bartel-Friedrich.

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Bartel-Friedrich, S., Amaya, B., Rasinski, C. et al. Der erweiterte Ductus und Saccus endolymphaticus. HNO 56, 219–224 (2008). https://doi.org/10.1007/s00106-007-1664-z

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  • DOI: https://doi.org/10.1007/s00106-007-1664-z

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