ORIGINAL ARTICLE
COCHLEAR IMPLANTATION IN CONGENITALLY DEAF CHILDREN WITH ISOLATED LARGE VESTIBULAR AQUEDUCT: A SURGEON’S PERSPECTIVE
 
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Department of Implantation Otology, Madras ENT Research Foundation, Chennai, India
CORRESPONDING AUTHOR
Rabindra B. Pradhananga   

Rabindra B. Pradhananga, e-mail: rabindrabp@yahoo.com
Publication date: 2020-04-16
 
J Hear Sci 2014;4(3):20–25
 
KEYWORDS
ABSTRACT
Background:
The vestibular aqueduct is a bony canal containing the endolymphatic duct. Its average diameter is 0.6 to 1.5 mm at its midpoint between the crus communes and its opening at the posterior cranial fossa. If the radiographic diameter at this point is more than 1.5 mm, it is considered a case of large vestibular aqueduct (LVA). It is the most common radiographically detectable inner ear anomaly in congenital hearing loss. LVA with hearing loss is described as LVA syndrome (LVAS). In children with LVA, cochlear implantation has been proven to be effective in increasing both auditory perception and speech and language development.

Material and Methods:
The study was carried out from March 2009 to March 2014 on 9 isolated LVAS cases among 406 congenitally deaf children who had been implanted at the Madras ENT Research Foundation (MERF), Chennai, India. This observational study is focused on pre-operative assessment, surgical issues, and postoperative follow-up.

Results:
Five males and four females less than six years old were detected with LVAS. There was a pulsatile stapes in five cases and a CSF gusher in eight cases. CSF gushers were well controlled during surgery by tightly sealing the insertion site with soft tissue. An intraoperative mannitol drip was used in six patients and fibrin glue in three. Postoperative oral acetazolamide was used routinely in cases of CSF gusher. No early postoperative complications were seen. Intraoperative impedance and neural telemetry were all satisfactory.

Conclusions:
Cochlear implantation in LVAS is feasible and effective. However, CSF gushers are common and require appropriate management and follow-up.

 
REFERENCES (20)
1.
Valvassori GE, Clemis JD. The large vestibular aqueduct syndrome. Laryngoscope, 1978; 88: 723–8.
 
2.
Becker TS, Vignaud J, Sultan A, Lachman M. The vestibular aqueduct in congenital deafness: evaluation by the axial projection. Radiology, 1983; 149: 741–4.
 
3.
Dahlen RT, Harnsberger HR, Gray SD et al. Overlapping thin section fast spin echo MR of the large vestibular aqueduct syndrome: comparison with CT. Am J Neuroradiol, 1997; 18: 67–75.
 
4.
Fugazzola L, Mannavola D, Cerutti N et al. Molecular analysis of the Pendred’s syndrome gene and magnetic resonance imaging studies of the inner ear are essential for the diagnosis of true Pendred’s syndrome. J Clin Endocrinol Metab, 2000; 85: 2469–75.
 
5.
Stinckens C, Standaert L, Casselman JW et al. The presence of a widened vestibular aqueduct and progressive sensorineural hearing loss in the branchio-oto-renal syndrome. A family study. Int J Pediatr Otorhinolaryngol, 2001; 59: 163–72.
 
6.
Murofushi T, Ouvrier RA, Parker GD, Graham RI, da Silva M, Halmagyi GM. Vestibular abnormalities in CHARGE association. Ann Otol Rhinol Laryngol, 1997; 106(2): 129–34.
 
7.
Abe S, Usami S, Shinkawa H et al. Three familial cases of hearing loss associated with enlargement of the vestibular aqueduct. Ann Otol Rhinol Laryngol 1997;106: 1063–9.
 
8.
Aschendorff A, Marangos N, Laszig R. Large vestibular aqueduct syndrome and its implication for cochlear implant surgery. Am J Otol, 1997; 18: S57.
 
9.
Emmett JR. The large vestibular aqueduct syndrome. Am J Otol, 1985; 6: 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): 207–15.
 
11.
Jackler RK, De La Cruz A. The large vestibular aqueduct syndrome. Laryngoscope, 1989; 99(12): 1238–42.
 
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): 157–64.
 
13.
Ko HC, Liu TC, Lee LA, Chao WC et al. Timing of surgical intervention with cochlear implant in patients with large vestibular aqueduct syndrome. PLoS One, 2013; 8(11): e81568.
 
14.
Natarajan K, Raghunandhan S, Senthilvadivu, Sathiya M, Kameswaran M. The pulsatile stapes: an unusual sign in large vestibular aqueduct syndrome. Indian J Otolaryngol Head Neck Surg, 2010; 62(Suppl.1): 16–8.
 
15.
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.
 
16.
Wootten CT, Backous DD, Haynes DS. Management of cerebrospinal fluid leakage from cochleostomy during cochlear implant surgery. Layngoscope, 2006; 116(11): 2055–9.
 
17.
Loundon N, Leboulanger N, Maillet J, Riggouzzo A et al. Cochlear implant and inner ear malformation. Proposal for an hyperosmolar therapy at surgery. Int J Pediatr Otorhinolaryngol, 2008; 72(4): 541–7.
 
18.
Miyamoto RT, Bichey BG, Wynne MK, Kirk KI. Cochlear implantation with large vestibular aqueduct syndrome. Laryngoscope, 2002; 112: 1178–82.
 
19.
Vassoler TMF, Bergonse GFR, Meira S, Bevilacqua MC, Costa Filho OA. Cochlear implant and large vestibular aqueduct syndrome in children. Braz J Otorhinolaryngol, 2008; 74(2): 260–4.
 
20.
Chen X, Liu B Liu S, Mo L et al. The development of auditory skills in infants with isolated large vestibular aqueduct syndrome after cochlear implantation. Int J Pediatr Otorhinolaryngol, 2011; 75: 943–7.