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Incomplete partition type III revisited—long-term results following cochlear implant

  • A. Alballaa
  • A. Aschendorff
  • S. Arndt
  • T. Hildenbrand
  • C. Becker
  • F. Hassepass
  • R. Laszig
  • R. Beck
  • I. Speck
  • T. Wesarg
  • M. C. KettererEmail author
Original articles



Incomplete partition type III (IP III) is defined by a missing lamina cribrosa between the cochlea and the internal auditory canal (IAC). Cochlear implantation (CI) may result in an insertion of the electrode array into the IAC. The aim of this study is to evaluate CI surgery protocols, long-term audiological outcome, mapping and electrophysiological data after CI in IP III patients.

Materials and methods

Nine IP III patients were implanted with perimodiolar electrode arrays between 1999 and 2014; eight of them were included in this study. We evaluated mapping data, stapedius reflexes, electrode impedances and ECAP thresholds. We matched them with 3 CI patients each with normal cochlear morphology regarding sex, age, side, implant type and surgical date. Speech discrimination was evaluated with the Oldenburger sentence test for adults, Göttingen audiometric speech test for children and the Freiburger monosyllabic word test.


3 years after CI IP III patients showed a significant increase in pulse width, calculated electric load and electrode impedances in basal electrodes. Intraoperative electrically-evoked stapedius reflexes could be measured in all patients. Speech recognition scores were lower than average scores for matched patients, but without statistical significance.


The significant increase of pulse width, electric load and electrode impedances of basal electrodes over time seem to be characteristic for IP III patients probably occurring due to fibrosis and neurodegeneration of the cochlear nerve. The long term audiological results are stable. Intraoperative imaging and stapedius reflexes are highly recommended to control the right position of the electrode array.


Mapping Cochlear implant Mapping data Incomplete partition type III Longterm results 

„Incomplete partition type III“ – Langzeitergebnisse nach Cochleaimplantation



The authors thank the support organisation “Taube Kinder lernen Hören e. V.”, which has supported the cochlear implant rehabilitation center in Freiburg.

Author Contribution

Ketterer, MC, M.D. analyzed data and wrote the paper. Alballaa, A. provided statistical analysis together with Becker, Ch., M.D. Wesarg, T, PhD., contributed to data analysis and provided critical revision; Aschendorff, A., M.D., PhD, provided critical revision and surgical analysis. The other coauthors provided critical revision.

Compliance with ethical guidelines

Conflict of interest

A. Aschendorff received travelling expenses and financial support for research from Advanced Bionics, Stäfa, Switzerland; financial support for research and travelling expenses from Cochlear Ltd, Australia; financial support for research and travelling expenses from Med-El, Innsbruck, Austria; travelling expenses and financial support for research from Oticon, Copenhagen, Denmark. S. Arndt received travelling expenses from Advanced Bionics, Stäfa, Switzerland; financial support for research and travelling expenses from Cochlear Ltd, Australia; financial support for research and travelling expenses from Med-El, Innsbruck, Austria and travelling expenses from Oticon, Copenhagen, Denmark. R. Laszig received financial support for research and travelling expenses from Advanced Bionics, Stäfa, Switzerland; financial support for research, travelling expenses and consultancy fees from Cochlear Ltd, Australia; travelling expenses from Oticon, Copenhagen, Denmark; financial support for research from Med-El, Innsbruck, Austria; financial support for research and travelling expenses from ARRIAG Munich, Germany; travelling expenses from Otologics Boulder, USA; travelling expenses from SonovaHolding, Stäfa, Switzerland; travelling expenses from the General Secretary of the German ENT Society; contract fees, consultancy fees and travelling costs from Medupdate and fees from Springer Medicine EiC. R. Beck received travelling expenses from Cochlear Ltd, Australia. This study was not sponsored by industry. M.C. Ketterer, C. Becker, T. Hildenbrand, F. Hassepass, T. Wesarg, A. Alballaa and I. Speck declare that they have no competing interests.

Retrospective study; compliance with ethical standards is given and the authors have no funding, financial relationships or conflicts of interest to declare.

The supplement containing this article is not sponsored by industry.


  1. 1.
    Aschendorff A, Maier W, Jaekel K, Wesarg T, Arndt S, Laszig R, Voss P, Metzger M, Schulze D (2009) Radiologically assisted navigation in cochlear implantation for X‑linked deafness malformation. Cochlear Implants International 10(Suppl 1):14–18CrossRefPubMedGoogle Scholar
  2. 2.
    Bademci G, Lasisi A, Yariz KO, Montenegro P, Menendez I, Vinueza R, Paredes R, Moreta G, Subasioglu A, Blanton S, Fitoz S, Incesulu A, Sennaroglu L, Tekin M (2015) Novel domain-specific POU3F4 mutations are associated with X‑linked deafness: Examples from different populations. BMC Med Genet 16:9CrossRefPubMedPubMedCentralGoogle Scholar
  3. 3.
    Cohen LT (2009) Practical model description of peripheral neural excitation in cochlear implant recipients: 4. Model development at low pulse rates: General model and application to individuals. Hear Res 248(1–2):15–30CrossRefPubMedGoogle Scholar
  4. 4.
    Cosetti MK, Friedmann DR, Heman-Ackah SE, Perez R, Waltzman SB, Roland JT Jr. (2015) Surgical techniques and outcomes of cochlear implantation in patients with radiographic findings consistent with X‑linked deafness. Int J Pediatr Otorhinolaryngol 79(10):1689–1693CrossRefPubMedGoogle Scholar
  5. 5.
    Incesulu A, Adapinar B, Kecik C (2008) Cochlear implantation in cases with incomplete partition type III (X-linked anomaly). Eur Arch Otorhinolaryngol 265(11):1425–1430CrossRefPubMedGoogle Scholar
  6. 6.
    Kang WS, Shim BS, Lee KS (2013) Audiologic performance after cochlear implantation in children with X‑linked deafness: Comparison with deaf children with a normal inner ear structure. Otol Neurotol 34(3):544–548CrossRefPubMedGoogle Scholar
  7. 7.
    Kozikowski GP, Cohen SP (2004) Lumbar puncture associated with pneumocephalus: Report of a case. Anesth Analg 98(2):524–526CrossRefPubMedGoogle Scholar
  8. 8.
    Lee HK, Lee SH, Lee KY, Lim EJ, Choi SY, Park RK, Kim UK (2009) Novel POU3F4 mutations and clinical features of DFN3 patients with cochlear implants. Clin Genet 75(6):572–575CrossRefPubMedGoogle Scholar
  9. 9.
    Nance WE, Setleff R, McLeod A, Sweeney A, Cooper C, McConnell F (1971) X‑linked mixed deafness with congenital fixation of the stapedial footplate and perilymphatic gusher. Birth Defects Orig Artic Ser 07(4):64–69PubMedGoogle Scholar
  10. 10.
    Papadaki E, Prassopoulos P, Bizakis J, Karampekios S, Papadakis H, Gourtsoyiannis N (1998) X‑linked deafness with stapes gusher in females. Eur J Radiol 29(1):71–75CrossRefPubMedGoogle Scholar
  11. 11.
    Phelps PD (1992) The basal turn of the cochlea. Br J Radiol 65(773):370–374CrossRefPubMedGoogle Scholar
  12. 12.
    Phelps PD, Reardon W, Pembrey M, Bellman S, Luxom L (1991) X‑linked deafness, stapes gushers and a distinctive defect of the inner ear. Neuroradiology 33(4):326–330CrossRefPubMedGoogle Scholar
  13. 13.
    Saeed H, Powell HR, Saeed SR (2016) Cochlear implantation in X‑linked deafness—How to manage the surgical challenges. Cochlear Implants International 17(4):178–183CrossRefPubMedGoogle Scholar
  14. 14.
    Saylisoy S, Incesulu A, Gurbuz MK, Adapinar B (2014) Computed tomographic findings of X‑linked deafness: A spectrum from child to mother, from young to old, from boy to girl, from mixed to sudden hearing loss. J Comput Assist Tomogr 38(1):20–24CrossRefPubMedGoogle Scholar
  15. 15.
    Sennaroglu L, Bajin MD (2017) Incomplete partition type III: A rare and difficult cochlear implant surgical indication. Auris Nasus Larynx 45(1):26–32CrossRefPubMedGoogle Scholar
  16. 16.
    Sennaroglu L, Sarac S, Ergin T (2006) Surgical results of cochlear implantation in malformed cochlea. Otol Neurotol 27(5):615–623CrossRefPubMedGoogle Scholar
  17. 17.
    Smeds H, Wales J, Asp F, Löfkvist U, Falahat B, Anderlid BM, Anmyr L, Karltorp E (2017) X‑linked malformation and cochlear implantation. Otol Neurotol 38(1):38–46CrossRefPubMedGoogle Scholar
  18. 18.
    Stankovic KM, Hennessey AM, Herrmann B, Mankarious LA (2010) Cochlear implantation in children with congenital X‑linked deafness due to novel mutations in POU3F4 gene. Ann Otol Rhinol Laryngol 119(12):815–822CrossRefPubMedGoogle Scholar
  19. 19.
    Syal R, Tyagi I, Goyal A (2005) Cerebrospinal fluid otorhinorrhea due to cochlear dysplasias. Int J Pediatr Otorhinolaryngol 69(7):983–988CrossRefPubMedGoogle Scholar
  20. 20.
    Van Camp G, Smith RJH (2010) Hereditary hearing loss homepageGoogle Scholar
  21. 21.
    Wootten CT, Backous DD, Haynes DS (2006) Management of cerebrospinal fluid leakage from cochleostomy during cochlear implant surgery. Laryngoscope 116(11):2055–2059CrossRefPubMedGoogle Scholar

Copyright information

© Springer Medizin Verlag GmbH, ein Teil von Springer Nature 2019

Authors and Affiliations

  • A. Alballaa
    • 1
    • 2
  • A. Aschendorff
    • 1
  • S. Arndt
    • 1
  • T. Hildenbrand
    • 1
  • C. Becker
    • 1
  • F. Hassepass
    • 1
  • R. Laszig
    • 1
  • R. Beck
    • 1
  • I. Speck
    • 1
  • T. Wesarg
    • 1
  • M. C. Ketterer
    • 1
    Email author
  1. 1.Department of Otorhinolaryngology—Head and Neck Surgery, Medical Center—University of Freiburg, Faculty of MedicineUniversity of FreiburgFreiburgGermany
  2. 2.Department of Otolaryngology, King Abdulaziz University Hospital, King Abdullah Ear Specialist Center (KAESC), College of MedicineKing Saud UniversityRiyadhSaudi Arabia

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