Imaging detection of endolymphatic sac tumor–associated hydrops

Clinical article

Restricted access

Object

To determine if physiologically based MRI sequences can be used to detect endolymphatic sac tumor (ELST)–associated hydrops, the authors performed contrast-enhanced delayed FLAIR imaging in consecutive ELST patients with clinical findings consistent with hydrops.

Methods

Consecutive patients with von Hippel-Lindau (VHL) disease and clinical findings of endolymphatic hydrops and ELSTs underwent contrast-enhanced delayed FLAIR MRI. Clinical, audiological, operative, and imaging findings were analyzed.

Results

Three patients (2 male, 1 female) with 4 ELSTs (1 patient had bilateral ELSTs) were identified who had clinical findings consistent with endolymphatic hydrops. Computed tomography and MRI evidence of an ELST was found in all patients. Their mean age at initial evaluation was 39.7 years (range 28–51 years). All patients demonstrated progressive sensorineural hearing loss that was associated with episodic vertigo and tinnitus. Contrast-enhanced delayed FLAIR MRI clearly demonstrated dilation of the membranous labyrinth consistent with hydrops in the affected ears but not the unaffected ears. Two patients underwent resection of the associated ELST that resulted in stabilization of progressive hearing loss, as well as amelioration of tinnitus and vertigo.

Conclusions

Contrast-enhanced delayed FLAIR MRI can be used to detect ELST-associated hydrops. Noninvasive MRI detection of hydrops can permit earlier detection of ELSTs in patients with VHL disease and provides direct insight into a mechanism that underlies ELST-associated audiovestibular morbidity.

Abbreviations used in this paper:ELST = endolymphatic sac tumor; VHL = von Hippel-Lindau.

Article Information

Address correspondence to: Russell R. Lonser, M.D., Department of Neurological Surgery, The Ohio State University, N1019 Doan Hall, 410 West 10th Avenue, Columbus, Ohio 43210. email: russell.lonser@osumc.edu.

Please include this information when citing this paper: published online March 8, 2013; DOI: 10.3171/2013.2.JNS12608.

© AANS, except where prohibited by US copyright law.

Headings

Figures

  • View in gallery

    Magnetic resonance imaging of endolymphatic hydrops in the cochlea (A, arrowheads) and vestibule (E, arrowheads) of a right inner ear (Case 1, Table 1) that was affected by an ELST (A, C, E, and G; asterisks). Endolymph is characterized by low signal (black) on delayed MR-FLAIR sequences (A, B, E, and F) and high signal on the corresponding T2-weighted sequences (C, D, G, and H). Perilymph is characterized by high signal on both the delayed MR-FLAIR sequences and the T2-weighted sequences. In the cochlea contralateral to the ELST (B and D), there is no expansion of the endolymph, and high signal intensity in the perilymph on delayed FLAIR sequences (B) matches the high signal intensity on T2-weighted sequences (D). In the right cochlea, which is on the side of the ELST (A), “filling defects” (A, arrowheads) represent expansion of the endolymph of the scala media. In the vestibule contralateral to the ELST, the normal utricle and saccule (F, arrows) are seen as low signal intensity endolymph “filling defects” surrounded by a halo of perilymph. In the vestibule ipsilateral to the ELST, the membranous labyrinth “filling defect” (E, arrowheads) is expanded such that the perilymph is completely effaced from the vestibule. Bar = 1 cm.

  • View in gallery

    Magnetic resonance imaging of endolymphatic hydrops in the cochlea (B, arrowheads) and vestibule (F, arrowhead) of a left inner ear (Case 2, Table 1) that was affected by an ELST (B, D, F, and H; asterisks). Endolymph is characterized by low signal (black) on delayed MR-FLAIR sequences (A, B, E, and F) and high signal on the matching T2-weighted sequences (C, D, G, and H). Perilymph is characterized by high signal intensity on both the delayed MR-FLAIR sequences and the T2-weighted sequences. In the cochlea contralateral to the ELST (A and C), there is no expansion of the endolymph, and high signal intensity in the perilymph on delayed FLAIR sequences (A) matches the high signal intensity on T2-weighted sequences (C). In the cochlea ipsilateral to the ELST (B), “filling defects” (B, arrowheads) represent expansion of the endoymph of the scala media. In the vestibule contralateral to the ELST, the normal utricle and saccule (E, arrows) are seen as low signal intensity endolymph “filling defects” surrounded by a halo of perilymph. In the vestibule ipsilateral to the ELST, the membranous labyrinth “filling defect” (F, arrowhead) is expanded such that the perilymph is completely effaced from the vestibule. Bar = 1 cm.

References

1

Butman JAKim HJBaggenstos MAmmerman JMDambrosia JPatsalides A: Mechanisms of morbid hearing loss associated with tumors of the endolymphatic sac in von Hippel-Lindau disease. JAMA 298:41482007

2

Choo DIShotland LMastroianni MGlenn GMvan Waes CLinehan WM: Endolymphatic sac tumors in von Hippel-Lindau disease. J Neurosurg 100:4804872004

3

Heffner DK: Low-grade adenocarcinoma of probable endolymphatic sac origin. A clinicopathologic study of 20 cases. Cancer 64:229223021989

4

Jagannathan JButman JALonser RRVortmeyer AOZalewski CKBrewer C: Endolymphatic sac tumor demonstrated by intralabyrinthine hemorrhage. Case report. J Neurosurg 107:4214252007

5

Kim HJButman JABrewer CZalewski CVortmeyer AOGlenn G: Tumors of the endolymphatic sac in patients with von Hippel-Lindau disease: implications for their natural history, diagnosis, and treatment. J Neurosurg 102:5035122005

6

Lonser RRBaggenstos MKim HJButman JAVortmeyer AO: The vestibular aqueduct: site of origin of endolymphatic sac tumors. J Neurosurg 108:7517562008

7

Lonser RRGlenn GMWalther MChew EYLibutti SKLinehan WM: von Hippel-Lindau disease. Lancet 361:205920672003

8

Lonser RRKim HJButman JAVortmeyer AOChoo DIOldfield EH: Tumors of the endolymphatic sac in von Hippel-Lindau disease. N Engl J Med 350:248124862004

9

Manski TJHeffner DKGlenn GMPatronas NJPikus ATKatz D: Endolymphatic sac tumors. A source of morbid hearing loss in von Hippel-Lindau disease. JAMA 277:146114661997

10

Megerian CAHaynes DSPoe DSChoo DIKeriakas TJGlasscock ME III: Hearing preservation surgery for small endolymphatic sac tumors in patients with von Hippel-Lindau syndrome. Otol Neurotol 23:3783872002

11

Nakashima TNaganawa SPyykko IGibson WPSone MNakata S: Grading of endolymphatic hydrops using magnetic resonance imaging. Acta Otolaryngol Suppl 560582009

12

Nakashima TNaganawa STeranishi MTagaya MNakata SSone M: Endolymphatic hydrops revealed by intravenous gadolinium injection in patients with Ménière's disease. Acta Otolaryngol 130:3383432010

13

Tagaya MYamazaki MTeranishi MNaganawa SYoshida TOtake H: Endolymphatic hydrops and blood-labyrinth barrier in Ménière's disease. Acta Otolaryngol 131:4744792011

14

Vortmeyer AOChoo DPack SDOldfield EZhuang Z: von Hippel-Lindau disease gene alterations associated with endolymphatic sac tumor. J Natl Cancer Inst 89:9709721997

TrendMD

Metrics

Metrics

All Time Past Year Past 30 Days
Abstract Views 79 79 21
Full Text Views 131 131 14
PDF Downloads 67 67 5
EPUB Downloads 0 0 0

PubMed

Google Scholar