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Kochleaimplantat bei Innenohrfehlbildungen

Cochlear implant for malformations of the inner ear

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Zusammenfassung

Die radiologische Evaluation des Felsenbeins bei Kochleaimplantat-Voruntersuchungen deckt in bis zu 20% der Fälle Fehlbildungen des Innenohrs auf. Ziel der vorgelegten Untersuchung war die retrospektive Analyse und Klassifikation der mit einem Kochleaimplantat versorgten Patienten mit einer Innenohrfehlbildung an der Universitäts-HNO-Klinik Freiburg im Zeitraum 2001–2009. Dabei wurden Fehlbildungen des Innenohrs einschließlich der Dysplasien des inneren Gehörgangs bei Kindern in 12,7% und bei Erwachsenen in 3,4% der Fälle gefunden. Mondini-Dysplasien wurden mit 45% am häufigsten beobachtet. Das chirurgische Vorgehen wurde adaptiert, entsprechend der jeweiligen Fehlbildung. Von Bedeutung sind dabei die Modifikation des operativen Zugangs, das Management des intraoperativen Gusher, die Auswahl der zu implantierenden Elektrode, die intraoperative Bildgebung sowie der Einsatz der Navigation. Die postoperativen Ergebnisse sind als sehr positiv zu bewerten und entsprechen der Erwartung in Abhängigkeit von der Dauer der Taubheit, wenn keine zusätzlichen Entwicklungsbehinderungen bestehen.

Abstract

The radiologic evaluation of the temporal bone in cochlear implant candidates can detect malformations of the inner ear in up to 20% of cases. The aim of our study was to analyze and classify malformations of the inner ear in patients with cochlear implants carried out from 2001 to 2009. Malformations of the inner ear, including malformations of the internal auditory canal were detected in 12.7% of children and 3.4% of adults. Mondini dysplasia was most common and occurred in 45% of cases. The surgical procedure had to be adapted according to the individual malformation. Modification of surgical access, management of intraoperative CSF gusher, choice of electrode array, intraoperative imaging and the use of navigation were the most important factors. Rehabilitation results were generally very positive and corresponded to the expectation depending on the duration of deafness, if no additional handicaps were present.

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Literatur

  1. Aschendorff A, Klenzner T, Laszig R (2002) Strategien zur Cochlear-Implant-Versorgung bei Fehlbildungen des Labyrinths – Vorschlag einer neuen Klassifikation. HNO-Informationen 27:85

    Google Scholar 

  2. Aschendorff A, Kromeier J, Klenzner T, Laszig R (2007) Quality control after insertion of the nucleus contour and contour advance electrode in adults. Ear Hear 28:75S–79S

    Article  PubMed  Google Scholar 

  3. Aschendorff A, Maier W, Jaekel K et al (2009) Radiologically assisted navigation in cochlear implantation for X-linked deafness malformation. Cochlear Implants Int 10S:14–18

    Google Scholar 

  4. Birkenhäger R, Zimmer AJ, Maier W et al (2007) Hinweise für ein zusätzliches Gen für das LAV-Syndrom. Laryngol Rhino Otol 86:102–106

    Article  Google Scholar 

  5. Bloom JD, Rizzi MD, Germiller JA (2009) Real-time intraoperative computed tomography to assist cochlear implant placement in the malformed inner ear. Otol Neurotol 30:23–26

    Article  PubMed  Google Scholar 

  6. Buchman CA, Copeland BJ, Yu KK et al (2004) Cochlear implantation in children with congenital inner ear malformations. Laryngoscope 114:309–316

    Article  PubMed  Google Scholar 

  7. Casselman JW, Offeciers FE, Govaerts PJ et al (1997) Aplasia and hypoplasia of the vestibulocochlear nerve: Diagnosis with MR imaging. Radiology 202:773–781

    PubMed  CAS  Google Scholar 

  8. Glastonbury CM, Davidson HC, Harnsberger HR et al (2002) Imaging findings of cochlear nerve deficiency. AJNR Am J Neuroradiol 23:635–643

    PubMed  Google Scholar 

  9. Herlitschke C (2006) Erstellung einer klinischen Datenbank in ACCESS für die Dokumentation von Cochlear Implant Patienten. mdi Forum der Medizin_Dokumentation und medizin_Informatik 8:87–90

    Google Scholar 

  10. Jackler RK, Luxford WM, House WF (1987) Congenital malformations of the inner ear: A classification based on embryogenesis. Laryngoscope 97:2–14

    Article  PubMed  CAS  Google Scholar 

  11. Kempf HG, Tempel S, Johann K, Lenarz T (1999) Komplikationen der Cochlear Implant-Chirurgie bei Kindern und Erwachsenen. Laryngorhinootologie 78:529–537

    Article  PubMed  CAS  Google Scholar 

  12. Kim AH, Kileny PR, Arts HA et al (2008) Role of electrically evoked auditory brainstem response in cochlear implantation of children with inner ear malformations. Otol Neurotol 29:626–634

    Article  PubMed  Google Scholar 

  13. Lanson BG, Green JE, Roland JT Jr et al (2007) Cochlear implantation in children with CHARGE syndrome: Therapeutic decisions and outcomes. Laryngoscope 117:1260–1266

    Article  PubMed  Google Scholar 

  14. Lehnhardt E, Aschendorff A (1993) Prognostic factors in 187 adults provided with the Nucleus cochlear mini-system 22. Adv Otorhinolaryngol 48:146–152

    PubMed  CAS  Google Scholar 

  15. Loundon N, Rouillon I, Munier N et al (2005) Cochlear implantation in children with internal ear malformations. Otol Neurotol 26:668–673

    Article  PubMed  CAS  Google Scholar 

  16. Miyamoto RT, Bichey BG, Wynne MK, Kirk KI (2002) Cochlear implantation with large vestibular aqueduct syndrome. Laryngoscope 112:1178–1182

    Article  PubMed  Google Scholar 

  17. Morimoto AK, Wiggins RH III, Hudgins PA et al (2006) Absent semicircular canals in CHARGE syndrome: Radioloc spectrum of findings. AJNR Am J Neuroradiol 27:1663–1671

    PubMed  CAS  Google Scholar 

  18. Mylanus EA, Rotteveel LJ, Leeuw RL (2004) Congenital malformation of the inner ear and pediatric cochlear implantation. Otol Neurotol 25:308–317

    Article  PubMed  Google Scholar 

  19. Papsin BC (2005) Cochlear implantation in children with anomalous cochleovestibular anatomy. Laryngoscope115:1S–26S

  20. Phelps PD, Reardon W, Pembrey ME et al (1991) X-linked deafness, stapes gushers and a distinctive defect of the inner ear. Neuroradiology 33:326–330

    Article  PubMed  CAS  Google Scholar 

  21. Phelps PD (1992) Cochlear implants for congenital deformities. J Laryngol Otol 106:967–970

    Article  PubMed  CAS  Google Scholar 

  22. Roland JT Jr (2000) Complications of cochlear implant surgery. In: Waltzman SB, Cohen NL (eds) Cochlear Implants. Thieme, New York, pp 171–175

  23. Schipper J, Aschendorff A, Arapakis I et al (2004) Navigation as a quality management tool in cochlear implant surgery. J Laryngol Otol 118:764–770

    PubMed  Google Scholar 

  24. Sennaroglu L, Saatci I (2002) A new classification for cochleovestiblar malformations. Laryngoscope 112:2230–2241

    Article  PubMed  Google Scholar 

  25. Sennaroglu L, Sarac S, Ergi T (2006) Surgical results of cochlear implantation in malformed cochlea. Otol Neurotol 27:615–623

    Article  PubMed  Google Scholar 

  26. Stieve M, Schwab B, Haupt C et al (2006) Intraoperative computed tomography in otorhinolaryngology. Acta Otolaryngol 126:82–87

    Article  PubMed  CAS  Google Scholar 

  27. Swarzt JD (2004) An overview of congenital/developmental sensorineural hearing loss with emphasis on the vestibular aqueduct syndrome. Sem Ultrasound CT MRI 25:353–368

    Article  Google Scholar 

  28. Zanetti D, Guida M, Barezzani MG (2006) Favorable outcome of cochlear implant in VIIIth nerve deficiency. Otol Neurotol 27:815–823

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. HNO-Klinik und Sektion Cochlear Implant, Universität Freiburg, Killianstraße 5, 79106, Freiburg, Deutschland

    A. Aschendorff, R. Laszig, W. Maier, R. Beck, C. Schild, R. Birkenhäger, T. Wesarg, S. Kröger & S. Arndt

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  1. A. Aschendorff
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  2. R. Laszig
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  3. W. Maier
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  4. R. Beck
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  5. C. Schild
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  6. R. Birkenhäger
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  7. T. Wesarg
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  8. S. Kröger
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  9. S. Arndt
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Correspondence to A. Aschendorff.

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Aschendorff, A., Laszig, R., Maier, W. et al. Kochleaimplantat bei Innenohrfehlbildungen. HNO 57, 533–541 (2009). https://doi.org/10.1007/s00106-009-1936-x

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  • DOI: https://doi.org/10.1007/s00106-009-1936-x

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