Abstract
The etiology and pathogenesis of cochlear nerve deficiency (CND) are unclear and are generally thought to be related to congenital development and acquired degeneration. Congenital developments include early developmental insult that affects both the hindbrain and cochlear formation and later localized insult that is limited to the cochlear inner hair cells, spiral ganglion, or cochlear nerve itself. Acquired degeneration includes cochlear hair cell loss, and the amount of degeneration may differ considerably between the two ears. Furthermore, congenital cytomegalovirus infection may be associated with CND, but the exact mechanism remains to be further explored.
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References
Adunka OF, Roush PA, Teagle HF, Brown CJ, Zdanski CJ, Jewells V, Buchman CA. Internal auditory canal morphology in children with cochlear nerve deficiency. Otol Neurotol. 2006;27(6):793–801.
Bradford RD, Yoo YG, Golemac M, Pugel EP, Jonjic S, Britt WJ. Murine CMV-induced hearing loss is associated with inner ear inflammation and loss of spiral ganglia neurons. PLoS Pathog. 2015;11(4):e1004774.
Carner M, Colletti L, Shannon R, Cerini R, Barillari M, Mucelli RP, Colletti V. Imaging in 28 children with cochlear nerve aplasia. Acta Otolaryngol. 2009;129(4):458–61.
Chen J, Feng Y, Chen L, Liu H, Wang L, Wang X, Xiao J, Liu T, Yin Z, Chen S. Murine model for congenital CMV infection and hearing impairment. Virol J. 2011;8:70.
Chinnadurai V, Sreedhar CM, Khushu S. Assessment of cochlear nerve deficiency and its effect on normal maturation of auditory tract by diffusion kurtosis imaging and diffusion tensor imaging: a correlational approach. Magn Reson Imaging. 2016;34:1305.
Ciorba A, Bovo R, Trevisi P, Bianchini C, Arboretti R, Martini A. Rehabilitation and outcome of severe profound deafness in a group of 16 infants affected by congenital cytomegalovirus infection. Eur Arch Otorhinolaryngol. 2009;266(10):1539–46.
Elangovan S, Spankovich C. Diabetes and auditory-vestibular pathology. Semin Hear. 2019;40(4):292–9.
Glastonbury CM, Davidson HC, Harnsberger HR, Butler J, Kertesz TR, Shelton C. Imaging findings of cochlear nerve deficiency. Am J Neuroradiol. 2002;23(4):635–43.
Huang BY, Roche JP, Buchman CA, Castillo M. Brain stem and inner ear abnormalities in children with auditory neuropathy spectrum disorder and cochlear nerve deficiency. Am J Neuroradiol. 2010;31(10):1972–9.
Levi J, Ames J, Bacik K, Drake C, Morlet T, O’Reilly RC. Clinical characteristics of children with cochlear nerve dysplasias. Laryngoscope. 2013;123(3):752–6.
Li L, Wu W, Huang W, Hu G, Yuan W, Li W. NF-κB RNAi decreases the Bax/Bcl-2 ratio and inhibits TNF-α-induced apoptosis in human alveolar epithelial cells. Inflamm Res. 2013;62(4):387–97.
Li X, Shi X, Wang C, Niu H, Zeng L, Qiao Y. Cochlear spiral ganglion neuron apoptosis in neonatal mice with murine cytomegalovirus-induced sensorineural hearing loss. J Am Acad Audiol. 2016;27(4):345–53.
Mohan S, Abdelwahab SI, Kamalidehghan B, Syam S, May KS, Harmal NSM, Shafifiyaz N, Hadi AHA, Hashim NM, Rahmani M, Taha MME, Cheah SC, Zajmi A. Involvement of NF-κB and Bcl2/Bax signaling pathways in the apoptosis of MCF7 cells induced by a xanthone compound Pyranocycloartobiloxanthone A. Phytomedicine. 2012;19(11):1007–15.
Pagarkar W, Gunny R, Saunders DE, Yung W, Rajput K. The bony cochlear nerve canal in children with absent or hypoplastic cochlear nerves. Int J Pediatr Otorhinolaryngol. 2011;75(6):764–73.
Paul A, Marlin S, Parodi M, Rouillon I, Guerlain J, Pingault V, Couloigner V, Garabedian EN, Denoyelle F, Loundon N. Unilateral sensorineural hearing loss: medical context and etiology. Audiol Neurootol. 2017;22(2):83–8.
Rubel EW, Fritzsch B. Auditory system development: primary auditory neurons and their targets. Annu Rev Neurosci. 2002;25:51–101.
Sha Y, Luo DH, Li HG, Head and neck imaging. In: Otorhinolaryngology head and neck surgery, vol. 42. Beijing: People’s Medical Publishing House; 2014.
Shepherd RK, Roberts LA, Paolini AG. Long-term sensorineural hearing loss induces functional changes in the rat auditory nerve. Eur J Neurosci. 2004;20(11):3131–40.
Stevens G, Flaxman S, Brunskill E, Mascarenhas M, Mathers CD, Finucane M. Global Burden of Disease Hearing Loss Expert Group Global and regional hearing impairment prevalence: an analysis of 42 studies in 29 countries. Eur J Public Health. 2013;23(1):146–52.
Sung CYW, Seleme MC, Payne S, Jonjic S, Hirose K, BrittW. Virus-induced cochlear inflammation in newborn mice alters auditory function. JCI Insight. 2019;4(17):e128878.
Usamia S, Kitoha R, Moteki H, Nishio SY, Kitano T, Kobayashi M, Shinagawa J, Yokota Y, Sugiyama K, Watanabe K. Etiology of single-sided deafness and asymmetrical hearing loss. Acta Otolaryngol. 2017;137(Suppl 565):S2–7.
Vos SB, Haakma W, Versnel H, Froeling M, Speleman L, Dik P, Viergever MA, Leemans A, Grolman W. Diffusion tensor imaging of the auditory nerve in patients with long-term single-sided deafness. Hear Res. 2015;323:1–8.
Wang QJ, Starr A. Hereditary auditory neuropathies: stepping into precision management from the discovery . Chin J Otorhinolaryngol Head Neck Surg 2018, 53:161.
Wilkins A, Prabhu SP, Huang L, Ogando PB, Kenna MA. Frequent association of cochlear nerve canal stenosis with pediatric sensorineural hearing loss. Arch Otolaryngol Head Neck Surg. 2012;138(4):383–8.
Zhang XY, Fang F. Congenital human cytomegalovirus infection and neurologic diseases in newborns. Chin Med J (Engl). 2019;132(17):2109–18.
Zhao JF, Zhao X, Zhang XA, Tao JH, Guo J. MRI manifestations of cochlear nerve dysplasia and concomitant abnormalities. J Clin Radiol. 2021;40(3):582–5.
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Huang, L., Wen, C., Xie, J., Yu, Y., Li, Y. (2022). Pathogenesis of Cochlear Nerve Deficiency. In: Li, Y. (eds) Cochlear Implantation for Cochlear Nerve Deficiency. Springer, Singapore. https://doi.org/10.1007/978-981-19-5892-2_2
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