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Der erweiterte Ductus und Saccus endolymphaticus

Teil 2: Klinische Manifestationen

Large endolymphatic duct and sac syndrome

Part 2: Clinical manifestations

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Zusammenfassung

Hintergrund

Ziel der Studie war die Beschreibung klinischer Verläufe des im deutschsprachigen Raum relativ wenig bekannten erweiterten Ductus und Saccus endolymphaticus („large endolymphatic duct and sac syndrome“, LEDS) als häufigste radiologisch nachweisbare Innenohrfehlbildung (IO-FB), assoziiert mit sensorineuraler Schwerhörigkeit (SNHL). Daraus sollten Hinweise für die klinische Betreuung abgeleitet werden.

Methoden

Wir analysierten klinisch-audiologische und Schnittbildbefunde von 169 Patienten (P) mit IO-FB-Verdacht zwischen 1994 und 2003. Nach Identifikation aller LEDS-Fälle wurden die funktionsdiagnostischen Daten seriell hinsichtlich Ausprägungs- und Verlaufsformen untersucht.

Ergebnisse

Von 169 P zeigten 17 P (medianes Alter: 12 Jahre, 12 weiblich) bzw. 28 Ohren ein LEDS. Klinisch dominierte eine prä- oder perilingual beginnende, progrediente oder fluktuierend progrediente höhergradige, hochtonbetonte SNHL oder Taubheit. Hörstürze stellten ein relativ häufiges Phänomen dar. Eine Korrelation zwischen morphologischem Defekt und SNHL-Grad war nicht nachweisbar.

Fazit

Das Zeitfenster der Progredienz der SNHL und der Hörsturzepisoden lag hauptsächlich in der späteren Kindheit und Adoleszenz. Dieser Verlauf sollte bei der Patientenaufklärung und Therapieplanung frühzeitig berücksichtigt werden.

Abstract

Objective

The aim of this study was to clarify the clinical course of large endolymphatic duct and sac syndrome (LEDS). Although LEDS is the most common form of radiologically detectable inner ear malformation associated with sensorineural hearing loss (SNHL), it is relatively unknown in the German-speaking countries. We hoped to derive useful pointers for clinical care.

Methods

We evaluated the clinical audiometric records and imaging findings of 169 patients with clinically suspected inner ear malformation seen between 1994 and 2003. Following identification of all LEDS cases, we serially investigated the anamnestic and functional data with regard to severity and course.

Results

Among 169 patients, 17 (median age 12 years; 12 females) showed LEDS. A total of 28 ears were affected. Clinically, most cases were of prelingual or perilingual onset and displayed steady or fluctuatingly progressive severe SNHL with emphasis on the high frequencies or deafness. Episodes of sudden hearing loss were relatively frequent. No correlation could be demonstrated between the severity of morphological changes and the degree of SNHL.

Conclusion

Progression of SNHL and episodes of sudden hearing loss were seen mainly in late childhood or adolescence. This should be taken into account when informing and advising patients and planning therapy.

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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. Abteilung für Stimm-, Sprach- und Hörstörungen (Phoniatrie/Audiologie), Kopfkliniken am Bayrischen Platz, Klinik und Poliklinik für HNO-Heilkunde, Universitätsklinikum Leipzig AöR, Leipzig, Deutschland

    M. Fuchs & S. Meuret

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

    B. Amaya

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

    S. Kösling

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

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Bartel-Friedrich, S., Fuchs, M., Amaya, B. et al. Der erweiterte Ductus und Saccus endolymphaticus. HNO 56, 225–230 (2008). https://doi.org/10.1007/s00106-007-1665-y

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