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Magnetic resonance imaging features of large endolymphatic sac compartments: audiological and clinical correlates

Published online by Cambridge University Press:  30 May 2012

S E J Connor*
Affiliation:
Department of Radiology, Guy's and St Thomas' Hospital, London, UK Department of Neuroradiology, King's College Hospital, London, UK
A Siddiqui
Affiliation:
Department of Radiology, Guy's and St Thomas' Hospital, London, UK Department of Neuroradiology, King's College Hospital, London, UK
R O'Gorman
Affiliation:
Department of Neuroradiology, King's College Hospital, London, UK MR-Center, University Children's Hospital, Zurich, Switzerland
J R Tysome
Affiliation:
Ear Nose and Throat Department and Auditory Implantation Centre, Guy's and St Thomas' Hospital, London, UK
A Lee
Affiliation:
Ear Nose and Throat Department and Auditory Implantation Centre, Guy's and St Thomas' Hospital, London, UK
D Jiang
Affiliation:
Ear Nose and Throat Department and Auditory Implantation Centre, Guy's and St Thomas' Hospital, London, UK
A Fitzgerald-O'Connor
Affiliation:
Ear Nose and Throat Department and Auditory Implantation Centre, Guy's and St Thomas' Hospital, London, UK
*
Address for correspondence: Dr S E J Connor, Neuroradiology Department, Ruskin Wing, King's College Hospital, London SE5 9RS, UK E-mail: steve.connor@nhs.net

Abstract

Objectives:

(1) To study the prevalence and characteristics of large endolymphatic sac internal compartments on thin-section T2- and T2*-weighted magnetic resonance imaging, and to relate these to other large endolymphatic sac magnetic resonance imaging features, and (2) to correlate the compartment imaging features, endolymphatic sac size and labyrinthine anomalies with the patients' clinical and audiological data.

Method:

Magnetic resonance imaging studies for 38 patients with large endolymphatic sac anomalies were retrospectively reviewed in a tertiary referral centre. Endolymphatic sac compartment presence, morphology and imaging signal were assessed. Endolymphatic sac size and labyrinthine anomalies were also recorded. Endolymphatic sac compartments and other imaging features were correlated with clinical and audiological data.

Results:

Compartments were present in 57 per cent of the imaged endolymphatic sacs, but their presence alone did not correlate with other imaging features or clinical data. The endolymphatic sac : internal auditory meatus signal ratio was associated with a history of sudden or fluctuating hearing loss. Hearing loss correlated with opercular and extraosseous endolymphatic sac size measurements. A larger midpoint intraosseous endolymphatic sac size was associated with clear fluid loss at cochlear implantation.

Conclusion:

The magnetic resonance imaging characteristics of large endolymphatic sac compartments have been defined. The endolymphatic sac size and distal compartment signal should be recorded, as these provide prognostic information and assist the planning of appropriate interventions.

Type
Main Articles
Copyright
Copyright © JLO (1984) Limited 2012

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References

1Jackler, RK, De La Cruz, A. The large vestibular aqueduct syndrome. Laryngoscope 1989;99:1238–43CrossRefGoogle ScholarPubMed
2Mafee, MF, Charlette, D, Kumar, A, Belmount, H. Large vestibular aqueduct and congenital sensorineural hearing loss. AJNR Am J Neuroradiol 1992;13:805–19Google ScholarPubMed
3Valvassori, GE, Clemis, JD. The large vestibular aqueduct syndrome. Am J Otol 1985;6:387415Google Scholar
4Naganawa, S, Koshikawa, T, Iwayama, E, Fukatsu, H, Ishiguchi, T, Ishigaki, T et al. MR imaging of the enlarged endolymphatic duct and sac syndrome by use of a 3D fast asymmetric spin-echo sequence: volume and signal-intensity measurement of the endolymphatic duct and sac and area measurement of the cochlear modiolus. AJNR Am J Neuroradiol 2000;21:1664–9Google ScholarPubMed
5Colvin, I, Beale, T, Harrop-Griffiths, K. Long term follow up of hearing loss in children and young adults with enlarged vestibular aqueducts: relationship to radiologic findings and Pendred syndrome diagnosis. Laryngoscope 2006;116:2027–36CrossRefGoogle Scholar
6Lemmerling, MM, Mancuso, AA, Antonelli, PJ, Kubilis, PS. Normal modiolus: CT appearance in patients with a large vestibular aqueduct. Radiology 1997;204:213–9CrossRefGoogle ScholarPubMed
7Hirsch, BE, Weissman, JL, Curtin, H, Kamerer, DB. Magnetic resonance imaging of the large vestibular aqueduct. Arch Otolaryngol Head Neck Surg 1992;118:1124–7CrossRefGoogle ScholarPubMed
8Boston, M, Halsted, M, Meinzen-Derr, J, Bean, J, Vijayasakaren, S, Arjmand, E et al. The large vestibular aqueduct: a new definition based on audiologic and computed tomography correlation. Otolaryngol Head Neck Surg 2007;136:972–7CrossRefGoogle ScholarPubMed
9Koesling, S, Rsainski, C, Amaya, B. Imaging and clinical findings in large endolymphatic duct and sac syndrome. Eur J Radiol 2006;57:5462CrossRefGoogle ScholarPubMed
10Naganawa, S, Ito, T, Iwayama, E. MR imaging of the cochlear modiolus: area measurement in healthy subjects and in patients with a large endolymphatic duct and sac. Radiology 1999;213:819–23CrossRefGoogle ScholarPubMed
11Romo, LV, Casselman, JW, Robson, CD. Temporal bone: congenital anomalies. In: Som, PS, Curtin, HD, eds. Head and Neck Imaging, 4th edn.St Louis: CV Mosby, 2003;2:1109–71Google Scholar
12Vijayasekaren, S, Halsted, MJ, Boston, M, Meinzen-Derr, J, Bardo, DME, Greinwald, J, Benton, C. When is the vestibular aqueduct enlarged? A statistical analysis of the normative distribution of vestibular aqueduct size. AJNR Am J Neuroradiol 2007;28:1133–8CrossRefGoogle Scholar
13Davidson, HC, Harnsberger, HR, Lemmerling, MM, Mancuso, AA, White, DK, Tong, KA et al. MR evaluation of vestibulocochlear anomalies associated with large endolymphatic duct and sac. AJNR Am J Neuroradiol 1999;20:1435–41Google ScholarPubMed
14Bagger-Sjoback, D, Jansson, B, Friberg, U, Rask-Anderson, H. Three dimensional anatomy of the human endolymphatic sac. Arch Otolaryngol Head Neck Surg 1990;116:345–9CrossRefGoogle ScholarPubMed
15Danckwardt-Lilliestrom, N, Laurent, C, Hellstrom, S, Friberg, U, Kinnefors, A, Rask-Andersen, H. Localisation of hyaluronan in the human endolymphatic sac. Acta Otolaryngol 1994;114:382–6CrossRefGoogle ScholarPubMed
16Gill, H, Michaels, L, Phelps, PD, Reardon, W. Histopathological findings suggest the diagnosis in an atypical case of Pendred syndrome. Clin Otolaryngol 1999;24:523–6CrossRefGoogle Scholar
17Gussen, R. The endolymphatic sac in Mondini disorder. Eur Arch Otorhinolaryngol 1985;242:71–6CrossRefGoogle ScholarPubMed
18Okamoto, K, Ito, J, Furusawa, T, Sakai, K, Tokiguchi, S. Large vestibular aqueduct syndrome with high CT density and high MR signal intensity. AJNR Am J Neuroradiol 1997;18:482–4Google ScholarPubMed
19Couloigner, V, Grayeli, AB, Sterkers, O, Ferrary, E. Composition of endolymphatic sac luminal fluid in a patient with Mondini dysplasia. Ann Otol Rhinol Laryngol 2008;117:123–6CrossRefGoogle Scholar
20Naganawa, S, Koshikawa, T, Fukatsu, H, Ishigaki, T, Nukashima, T. Serial MR imaging studies in enlarged endolymphatic duct and sac syndrome. Eur Radiol 2002;12:114–7CrossRefGoogle Scholar
21Naganawa, S, Sone, M, Otake, H, Kakashima, T. Endolymphatic hydrops of the labyrinth visualized on noncontrast MR imaging: a case report. Magn Reson Med Sci 2009;8:43–6CrossRefGoogle ScholarPubMed
22Oh, SH, Choi, BY, Son, KR, Lee, KJ, Chang, SO, Kim, CS. Can magnetic resonance imaging provide clues to the inner ear functional status of enlarged vestibular aqueduct subjects with PDS mutation? Otol Neurotol 2008;29:593600CrossRefGoogle Scholar
23Antonelli, PJ, Nall, AV, Lemmerling, MM, Mancuso, AA, Kubilis, PC. Hearing loss with cochlear modiolar defects and large vestibular aqueducts. Am J Otol 1998;19:306–12Google ScholarPubMed
24Au, G, Gibson, W. Cochlear implantation in children with large vestibular aqueduct syndrome. Otol Neurotol 1999;20:183–6Google ScholarPubMed