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Large vestibular aqueduct syndrome and endolymphatic hydrops: two presentations of a common primary inner-ear dysfunction?

Published online by Cambridge University Press:  08 March 2017

J H Spiegel  and
A K Lalwani
J H Spiegel
Affiliation:
Department of Otolaryngology – Head and Neck Surgery, Boston University School of Medicine, Massachusetts, USA
A K Lalwani*
Affiliation:
Department of Otolaryngology, New York University, USA
*
Address for correspondence: Professor Anil K Lalwani, Mendik Foundation Professor and Chair, Department of Otolaryngology, New York University, 550 First Avenue, NBV 5 East 5, New York, NY 10016, USA. Fax: +1 212 263 8257 E-mail: [email protected]
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Abstract

Objective:

To present the theory that large vestibular aqueduct syndrome (i.e. the recognised existence of an enlarged vestibular aqueduct with progressive sensorineural hearing loss) and endolymphatic hydrops are due to a common primary dysfunction of inner-ear fluid homeostasis.

Method:

Case report and review of the world literature concerning large vestibular aqueduct syndrome and endolymphatic hydrops.

Results:

We report a family in which one sibling suffered from large vestibular aqueduct syndrome while the other had classic Ménière's disease. This suggests that large vestibular aqueduct syndrome and endolymphatic hydrops, in some cases, may be due to a common primary dysfunction of inner-ear fluid homeostasis.

Conclusion:

To our knowledge, this is the first report in the world literature to postulate that variation in the relative compliance of inner-ear membranes could be the factor that determines the manifestation of the disorder as either endolymphatic hydrops or large vestibular aqueduct syndrome.

Keywords

Meniere's DiseaseInner EarVertigoVestibular Aqueduct
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 123 , Issue 8 , August 2009 , pp. 919 - 921
Copyright
Copyright © JLO (1984) Limited 2008

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References

1 Kodama, A, Sando, I. Postnatal development of the vestibular aqueduct and endolymphatic sac. Ann Otol Rhinol Laryngol 1982;91(suppl 96):3–12Google Scholar
2 Wibrand, HF, Rask-Anderson, H, Gilstring, D. The vestibular aqueduct and paravestibular canal: an anatomic and radiologic investigation. Acta Radiol (Stockh) 1974;15:337–55Google Scholar
3 Valvassori, GE, Clemis, JD. The large vestibular aqueduct syndrome. Laryngoscope 1978;88:723–8CrossRefGoogle ScholarPubMed
4 Emmett, JR. The large vestibular aqueduct syndrome. Am J Otol 1985;6:387–403Google ScholarPubMed
5 Zalzal, GH, Tomaski, SM, Vezina, LG, Bjornsti, P, Grundfast, KM. Enlarged vestibular aqueduct and sensorineural hearing loss in childhood. Arch Otolaryngol Head Neck Surg 1995;121:23–8CrossRefGoogle ScholarPubMed
6 Jackler, RK, De La Cruz, A. The large vestibular aqueduct syndrome. Laryngoscope 1989;99:1238–43CrossRefGoogle ScholarPubMed
7 Nakagawa, O, Ito, S, Hanyu, O, Yamazaki, M, Urishiyama, M, Tani, N et al. Female siblings with Pendred's syndrome. Internal Medicine 1994;33:369–72CrossRefGoogle ScholarPubMed
8 Gussen, R. The endolymphatic sac in Mondini disorder. Arch Otorhinolaryngol 1985;242:71–6CrossRefGoogle ScholarPubMed
9 Yamamoto, E, Mizukami, C, Isono, M, Ohmura, M, Hirono, Y. Observation of the external aperture of the vestibular aqueduct using three-dimensional surface reconstruction imaging. Laryngoscope 1991;101:480–3CrossRefGoogle ScholarPubMed
10 Shea, JJ. The classification and treatment of Meniere's disease. Acta Otol Rhinol Laryngol (Belg) 1993;47:303–10Google ScholarPubMed
11 Talbot, JM, Wilson, DF. Computed tomographic diagnosis of X-linked congenital mixed deafness, fixation of the stapedia footplate, and perilymphatic gusher. Am J Otol 1994;15:177–82Google Scholar
12 Belenky, W, Madgy, DN, Leider, S, Becker, CJ, Hotaling, AJ. The enlarged vestibular aqueduct syndrome (EVA syndrome). ENT J 1993;72:746–51Google ScholarPubMed
13 Levenson, MJ, Parisier, SC, Jacobs, M, Edelstein, DR. The large vestibular aqueduct syndrome in children. Arch Otolaryngol Head Neck Surg 1989;115:54–8CrossRefGoogle ScholarPubMed
14 Carlborg, B, Farmer, J. Effects of hypobaric pressure on the labyrinth. Acta Otolaryngol (Stockh) 1990;110:386–93CrossRefGoogle ScholarPubMed
15 Kitahara, M, Suzuki, M, Kodama, A. Equilibrium of inner and middle ear pressures. Acta Otolaryngol Suppl (Stockh) 1994;510:113–15CrossRefGoogle Scholar
16 Okumura, T, Takahashi, H, Honjo, I, Takagi, A, Azato, R. Sensorineural hearing loss in patients with large vestibular aqueduct. Laryngoscope 1995;105:289–94CrossRefGoogle ScholarPubMed
17 Carlborg, B, Farmer, J. Transmission of cerebrospinal fluid pressure via the cochlear aqueduct and endolymphatic sac. Am J Otolaryngol 1983;4:273–82CrossRefGoogle ScholarPubMed
18 Bohmer, A. Hydrostatic pressure in the inner ear fluid compartments and its effects on inner ear function. Acta Otolaryngol Suppl (Stockh) 1993;507:3–24Google ScholarPubMed
19 Schuknecht, HF. Correlation of pathology with symptoms of Meniere's disease. Otolaryngol Clin North Am 1968;1:433–440CrossRefGoogle Scholar
20 Shirazi, A, Fenton, JE, Fagan, PA. Large vestibular aqueduct syndrome and stapes fixation. J Laryngol Otol 1994;108:177–82CrossRefGoogle ScholarPubMed
21 Nance, WE, Setleff, R, McLeod, A, Sweeney, A, Cooper, C, McConnell, F. X-linked mixed deafness with congenital fixation of the stapedial footplate and perilymphatic gusher. Birth Defects 1971;7:64–9Google Scholar
22 Griffith, AJ, Arts, A, Downs, C, Innis, JW, Shepard, NT, Sheldon, S et al. Familial large vestibular aqueduct syndrome. Laryngoscope 1996;106:960–5CrossRefGoogle ScholarPubMed