[Large endolymphatic duct and sac syndrome (LEDS) : part I: analysis of imaging findings].
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.


CONCLUSIONS
LEDS may be an underestimated cause of SNHL. Imaging is necessary for a confident diagnosis.
Bartel-Friedrich
S
S
Abteilung Phoniatrie und Pädaudiologie, Klinik und Poliklinik für Hals-Nasen-Ohren-Heilkunde, Kopf- und Halschirurgie, Universitätsklinikum der Martin-Luther-Universität Halle-Wittenberg, Deutschland. sylva.bartel-friedrich@medizin.uni-halle.de
Amaya
B
B
Rasinski
C
C
Fuchs
M
M
Kösling
S
S
D002648:Child; D007758:Ear, Inner / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000473:pathology; D004711:Endolymphatic Duct / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000473:pathology; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D013577:Syndrome; D014057:Tomography, X-Ray Computed