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Application of the Vibrant Soundbridge middle-ear implant for aural atresia in patients with Treacher Collins syndrome

Published online by Cambridge University Press:  23 October 2012

E Lesinskas ,
V Stankeviciute  and
M Petrulionis
E Lesinskas*
Affiliation:
Clinic of Ear, Nose, Throat and Eye Diseases, Faculty of Medicine, Vilnius University, Center of Ear, Nose and Throat Diseases, Vilnius University Hospital Santariskiu Clinics, Lithuania
V Stankeviciute
Affiliation:
Clinic of Ear, Nose, Throat and Eye Diseases, Faculty of Medicine, Vilnius University, Center of Ear, Nose and Throat Diseases, Vilnius University Hospital Santariskiu Clinics, Lithuania
M Petrulionis
Affiliation:
Clinic of Ear, Nose, Throat and Eye Diseases, Faculty of Medicine, Vilnius University, Center of Ear, Nose and Throat Diseases, Vilnius University Hospital Santariskiu Clinics, Lithuania
*
Address for correspondence: Prof Eugenijus Lesinskas, Clinic of Ear, Nose, Throat and Eye Diseases, Faculty of Medicine, Vilnius University, Santariskiu 2, LT – 08661, Vilnius, Lithuania E-mail: [email protected]
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Abstract

Objective:

To present results for the auditory rehabilitation of patients with Treacher Collins syndrome with bilateral osseous atresia, using middle-ear implantation with a Vibrant Soundbridge.

Methods:

Three patients underwent vibroplasty for aural atresia with moderate to severe conductive hearing loss. The pre-operative Jahrsdoerfer radiological score was 4 for all patients. Patients underwent active middle-ear implantation of a Vibrant Soundbridge implant (coupling the floating mass transducer to the rudimentary stapes or footplate distally, and positioning it adjacent to the round window membrane proximally), with audiological analysis as follow up.

Results:

After implant activation, the mean air conduction threshold ± standard deviation decreased to 22.8 ± 5.5 dB HL, representing a mean functional gain of 44.5 dB. The mean word recognition score (for bisyllabic words at 65 dB SPL) increased from 0 to 97 per cent.

Conclusion:

Vibrant Soundbridge implantation is an effective hearing rehabilitation procedure in patients with Treacher Collins syndrome with bilateral osseous atresia. This is a versatile implant which can achieve coupling even in cases of severe middle-ear malformation.

Keywords

Ear, MiddleTreacher Collins SyndromeMandibulofacial DysostosisAtresiaEar Abnormalities
Type
Main Articles
Information
The Journal of Laryngology & Otology , Volume 126 , Issue 12 , December 2012 , pp. 1216 - 1223
Copyright
Copyright © JLO (1984) Limited 2012

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References

1Gorlin, RJ, Cohen, MM Jr, Hennekam, RCM. Syndromes of the Head and Neck. New York: Oxford University Press, 2001;799–802CrossRefGoogle Scholar
2Marsella, P, Scorpecci, A, Pacifico, C, Tieri, L. Bone-anchored hearing aid (BAHA) in patients with Treacher Collins syndrome: tips and pitfalls. Int J Pediatr Otorhinolaryngol 2011;75:1308–12CrossRefGoogle ScholarPubMed
3Marres, HA. Hearing loss in the Treacher-Collins syndrome. Adv Otorhinolaryngol 2002;61:209–15Google ScholarPubMed
4Pron, G, Galloway, C, Armstrong, D, Posnick, J. Ear malformation and hearing loss in patients with Treacher Collins syndrome. Cleft Palate Craniofac J 1993;30:97–103CrossRefGoogle ScholarPubMed
5Jahrsdoerfer, RA, Jacobson, JT. Treacher Collins syndrome: otologic and auditory management. J Am Acad Audio 1995;6:93–102Google ScholarPubMed
6Jahrsdoerfer, RA, Aguilar, EA, Yeakley, JW, Cole, RR. Treacher Collins syndrome: an otologic challenge. Ann Otol Rhinol Laryngol 1989;98:807–12CrossRefGoogle ScholarPubMed
7Frenzel, H, Hanke, F, Beltrame, M, Steffen, A, Schonweiler, R, Wollenberg, B. Application of the Vibrant Soundbridge to unilateral osseous atresia cases. Laryngoscope 2009;119:67–74CrossRefGoogle ScholarPubMed
8Colletti, L, Carner, M, Mandala, M, Veronese, S, Colletti, V. The floating mass transducer for external auditory canal and middle ear malformations. Otol Neurotol 2010;32:108–15CrossRefGoogle Scholar
9Colletti, V, Soli, S, Carner, M, Colletti, L. Treatment of mixed hearing losses via implantation of a vibratory transducer on the round window. Int J Audiol 2006;45:600–8CrossRefGoogle ScholarPubMed
10Jahrsdoerfer, RA, Yeakley, JW, Aquilar, EA, Cole, RR, Gray, LC. Grading system for the selection of patients with congenital aural atresia. Am J Otol 1992;13:6–12Google ScholarPubMed
11Evans, AK, Kazahaya, K. Canal atresia: “surgery or implantable hearing devices? The expert's question is revisited”. Int J Pediatr Otorhinolaryngol 2007;71:367–74CrossRefGoogle Scholar
12De La Cruz, A, Teufert, KB. Congenital aural atresia surgery: long-term results. Otolaryngol Head Neck Surg 2003;29:121–7Google Scholar
13Kiefer, J, Staudenmaier, R. Combined aesthetic and functional reconstruction of ear malformations. Adv Otorhinolaryngol 2010;68:81–94Google ScholarPubMed
14Tringali, S, Pergola, N, Ferber-Viart, C, Truy, E, Berger, P, Dubreuil, C. Fully implantable hearing device as a new treatment of conductive hearing loss in Franceschetti syndrome. Int J Pediatr Otorhinolaryngol 2008;72:513–17CrossRefGoogle ScholarPubMed
15Baumgartner, WD, Böheim, K, Hagen, R, Müller, J, Lenarz, T, Reiss, S et al. The Vibrant Soundbridge for conductive and mixed hearing losses: European multicenter study results. Adv Otorhinolaryngol 2010;69:38–50Google ScholarPubMed
16Hüttenbrink, KB, Beutner, D, Zahnert, T. Clinical results with an active middle ear implant in the oval window. Adv Otorhinolaryngol 2010;69:27–32Google ScholarPubMed
17Zahnert, T, Bornitz, M, Hüttenbrink, KB. Experiments on the coupling of an active middle ear implant to the stapes footplate. Adv Otorhinolaryngol 2010;69:32–7Google Scholar
18Mandalà, M, Colletti, L, Colletti, V. Treatment of the atretic ear with round window Vibrant Soundbridge implantation in infants and children: electrocochleography and audiologic outcomes. Otol Neurotol 2011;32:1250–5CrossRefGoogle ScholarPubMed
19Lupo, JE, Koka, K, Holland, NJ, Jenkins, HA, Tollin, DJ. Prospective electrophysiologic findings of round window stimulation in a model of experimentally induced stapes fixation. Otol Neurotol 2009;30:1215–24CrossRefGoogle Scholar
20Roman, S, Nicollas, R, Triglia, JM. Middle ear implant for mixed hearing loss with malformation in a 9-year-old child. Eur Ann Otorhinolaryngol Head Neck Dis 2010;127:11–14CrossRefGoogle Scholar
21Verhaert, N, Fuchsmann, C, Tringali, S, Lina-Granade, G, Truy, E. Strategies of active middle ear implants for hearing rehabilitation in congenital aural atresia. Otol Neurotol 2011;32:639–45CrossRefGoogle ScholarPubMed
22Hüttenbrink, KB, Zahnert, T, Bornitz, M, Beutner, D. TORP-vibroplasty: a new alternative for the chronically disabled middle ear. Otol Neurotol 2008;29:965–71CrossRefGoogle ScholarPubMed