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Voor andere vormen van AN dan congenitale hypoplasie is het resultaat van een CI minder goed voorspelbaar. Bij een vermoeden van presynaptische aandoening zijn de resultaten vergelijkbaar met die van cochleaire verliezen. Resultaten van postsynaptische AN zijn variabel, maar gemiddeld slechter dan voor cochleaire verliezen (presynaptisch 75% en postsynaptisch 33%). Zie Tabel 2.

Tabel 2. CI resultaten bij auditieve neuropathie.

Plaats van laesie Locatie Etiologie aantal patiënten (n = 101) Elektrisch opgewekte potentialen CI stroomsterkten Open-Set spraakverstaan % Referenties
eCAP eABR
Presynaptisch Cochleair IHC Hypoxie 13 Normaal Normaal Normaal 46–100

DIAPH3/AUNA1 mutatie 2 * Normaal * 58–65

IHC ribbon synaps OTOF mutatie 10 Normaal Normaal * 50–100

(Breneman et al., 2012)

Postsynaptisch Dendriet OPA1 mutatie 10 Abnormaal Normaal * 50–90

Dendrieten en axonen Erfelijke perifere zenuwafwijkingen FRDA 1 * * * 20
DDON 1 * * Verhoogd 0
CMT1 1 * * * 54
Ganglion cellen Kernicterus 21 Normaal Variabel Normaal of verhoogd 0–100 (Rance and Barker, 2009)

(Breneman et al., 2012)

Gehoorzenuw Congenitale hypoplasie 32 Typisch

afwezig

Typisch afwezig Geen responsie max. sterkte (>90%) cases 0 in most (>90%) cases (Buchman et al., 2006)

(Young et al., 2012)

Hersenstam Brughoektumor 10 * Abnormaal * 0–99

CI = Cochleair Implantaat; CMT1 = Charcot–Marie–Tooth; DDON = Deafness-Dystonia-Optic Neuropathy (Mohr-Tranebjaerg) syndroom; eABR = electrically evoked Auditory Brainstem Response; eCAP = electrically evoked Compound Action Potential; FRDA = FRieDreich Ataxie; IHC = Inner Hair Cell; OPA1 = Optic Otrophy 1 (autosomaal dominant optische atrofie). Vetgedrukte cellen verwijzen naar afwezige of afwijkende bevindingen.

References:

Breneman, A. I., Gifford, R. H., & DeJong, M. D. (2012). Cochlear Implantation in Children with Auditory Neuropathy Spectrum Disorder: Long-Term Outcomes. Journal of the American Academy of Audiology, 23(1), 5–17. https://doi.org/10.3766/jaaa.23.1.2
Brookes, J. T., Kanis, A. B., Tan, L. Y., Tranebjærg, L., Vore, A., & Smith, R. J. H. (2008). Cochlear implantation in deafness-dystonia-optic neuronopathy (DDON) syndrome. International Journal of Pediatric Otorhinolaryngology, 72(1), 121–126. https://doi.org/10.1016/j.ijporl.2007.08.019
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Mukherjee, P., Ramsden, J. D., Donnelly, N., Axon, P., Saeed, S., & Fagan, P. (2013). Cochlear Implants to Treat Deafness Caused by Vestibular Schwannomas. 34(7), 8.
Rance, G., & Barker, E. J. (2009). Speech and language outcomes in children with auditory neuropathy/dys-synchrony managed with either cochlear implants or hearing aids. International Journal of Audiology, 48(6), 313–320. https://doi.org/10.1080/14992020802665959
Santarelli, R., Starr, A., Castillo, I. del, Huang, T., Scimemi, P., Cama, E., Rossi, R., & Arslan, E. (2011). Presynaptic and postsynaptic mechanisms underlying auditory neuropathy in patients with mutations in the OTOF or OPA1 gene. Audiological Medicine, 9(2), 59–66. https://doi.org/10.3109/1651386X.2011.558764
Santarelli, R., Rossi, R., Scimemi, P., Cama, E., Valentino, M. L., La Morgia, C., Caporali, L., Liguori, R., Magnavita, V., Monteleone, A., Biscaro, A., Arslan, E., & Carelli, V. (2015). OPA1-related auditory neuropathy: site of lesion and outcome of cochlear implantation. Brain, 138(3), 563–576. https://doi.org/10.1093/brain/awu378
Schoen, C. J., Burmeister, M., & Lesperance, M. M. (2013). Diaphanous homolog 3 (Diap3) Overexpression Causes Progressive Hearing Loss and Inner Hair Cell Defects in a Transgenic Mouse Model of Human Deafness. PLOS ONE, 8(2), e56520. https://doi.org/10.1371/journal.pone.0056520
Starr, A., Isaacson, B., Michalewski, H. J., Zeng, F.-G., Kong, Y.-Y., Beale, P., Paulson, G. W., Keats, B. J. B., & Lesperance, M. M. (2004). A Dominantly Inherited Progressive Deafness Affecting Distal Auditory Nerve and Hair Cells. Journal of the Association for Research in Otolaryngology, 5(4), 411–426. https://doi.org/10.1007/s10162-004-5014-5
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