Int Arch Otorhinolaryngol 2015; 19(04): 359-363
DOI: 10.1055/s-0034-1395791
Case Report
Thieme Publicações Ltda Rio de Janeiro, Brazil

Cochlear Implantation in Isolated Large Vestibular Aqueduct Syndrome: Report of Three Cases and Literature Review

Rabindra Pradhananga
1   Department of ENT-Head and Neck Surgery, Tribhuvan University Teaching Hospital, Kathmandu, Nepal
2   Department of Implantation Otology, Madras ENT Research Foundation, Chennai, Tamil Nadu, India
,
Kiran Natarajan
3   Department of Otorhinolaryngology, Madras ENT Research Foundation, Chennai, Tamil Nadu, India
,
AmarNath Devarasetty
3   Department of Otorhinolaryngology, Madras ENT Research Foundation, Chennai, Tamil Nadu, India
,
Mohan Kameswaran
2   Department of Implantation Otology, Madras ENT Research Foundation, Chennai, Tamil Nadu, India
› Author Affiliations
Further Information

Publication History

24 August 2014

11 October 2014

Publication Date:
14 November 2014 (online)

Abstract

Introduction Large vestibular aqueduct syndrome (LVAS) is characterized by the enlargement of the vestibular aqueduct associated with sensorineural hearing loss. It is the most common radiographically detectable inner ear anomaly in congenital hearing loss. LVAS may occur as an isolated anomaly or in association with other inner ear malformations.

Objective To report three cases of isolated LVAS with a focus on preoperative assessment, surgical issues, and short-term postoperative follow-up with preliminary auditory habilitation outcomes.

Resumed Report One girl and two boys with LVAS were assessed and cochlear implantation was performed for each. Various ways of intraoperative management of cerebrospinal fluid gusher and postoperative care and outcomes are reported.

Conclusion Cochlear implantation in the deaf children with LVAS is feasible and effective.

 
  • References

  • 1 Valvassori GE, Clemis JD. The large vestibular aqueduct syndrome. Laryngoscope 1978; 88 (5) 723-728
  • 2 Becker TS, Vignaud J, Sultan A, Lachman M. The vestibular aqueduct in congenital deafness: evaluation by the axial projection. Radiology 1983; 149 (3) 741-744
  • 3 Bagger-Sjoback D, Jansson B, Friberg U, Rask-Andersen H. Three-dimensional anatomy of the human endolymphatic sac. Arch Otolaryngol Head Neck Surg 1990; 116 (3) 345-349
  • 4 Dahlen RT, Harnsberger HR, Gray SD , et al. Overlapping thin-section fast spin-echo MR of the large vestibular aqueduct syndrome. AJNR Am J Neuroradiol 1997; 18 (1) 67-75
  • 5 Abe S, Usami S, Shinkawa H. Three familial cases of hearing loss associated with enlargement of the vestibular aqueduct. Ann Otol Rhinol Laryngol 1997; 106 (12) 1063-1069
  • 6 Aschendorff A, Marangos N, Laszig R. Large vestibular aqueduct syndrome and its implication for cochlear implant surgery. Am J Otol 1997; 18 (6, Suppl): S57
  • 7 Miyamoto RT, Bichey BG, Wynne MK, Kirk KI. Cochlear implantation with large vestibular aqueduct syndrome. Laryngoscope 2002; 112 (7 Pt 1) 1178-1182
  • 8 Chen X, Liu B, Liu S , et al. The development of auditory skills in infants with isolated Large Vestibular Aqueduct Syndrome after cochlear implantation. Int J Pediatr Otorhinolaryngol 2011; 75 (7) 943-947
  • 9 Emmett JR. The large vestibular aqueduct syndrome. Am J Otol 1985; 6 (5) 387-415
  • 10 Fahy CP, Carney AS, Nikolopoulos TP, Ludman CN, Gibbin KP. Cochlear implantation in children with large vestibular aqueduct syndrome and a review of the syndrome. Int J Pediatr Otorhinolaryngol 2001; 59 (3) 207-215
  • 11 Jackler RK, De La Cruz A. The large vestibular aqueduct syndrome. Laryngoscope 1989; 99 (12) 1238-1242, discussion 1242–1243
  • 12 Govaerts PJ, Casselman J, Daemers K, De Ceulaer G, Somers T, Offeciers FE. Audiological findings in large vestibular aqueduct syndrome. Int J Pediatr Otorhinolaryngol 1999; 51 (3) 157-164
  • 13 Natarajan K, Raghunandhan S, Senthilvadivu SM, Kameswaran M. The pulsatile stapes: an unusual sign in large vestibular aqueduct syndrome. Indian Journal of Otolaryngology & Head & Neck Surgery 2010; 62 (Suppl. 01) 16-18
  • 14 Harker LA, Vanderheiden S, Veazey D, Gentile N, McCleary E. Multichannel cochlear implantation in children with large vestibular aqueduct syndrome. Ann Otol Rhinol Laryngol Suppl 1999; 177: 39-43
  • 15 Loundon N, Leboulanger N, Maillet J , et al. Cochlear implant and inner ear malformation. Proposal for an hyperosmolar therapy at surgery. Int J Pediatr Otorhinolaryngol 2008; 72 (4) 541-547