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Abnormality of the cochlear nerve

MedGen UID:
868963
•Concept ID:
C4023377
•
Anatomical Abnormality
HPO: HP:0011396

Definition

Any anomaly of the cohclear nerve, which is the part of the vestibulocochlear nerve that is responsible for transmitting auditory signals from the inner ear to the cochlear nuclei within the brainstem and ultimately to the primary auditory cortex within the temporal lobe. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAbnormality of the cochlear nerve

Professional guidelines

PubMed

Management of Cochlear Nerve Hypoplasia and Aplasia.
Freeman SR, Sennaroglu L
Adv Otorhinolaryngol 2018;81:81-92. Epub 2018 Apr 6 doi: 10.1159/000485542. PMID: 29794457
The management of pediatric hearing loss caused by auditory neuropathy spectrum disorder.
Pham NS
Curr Opin Otolaryngol Head Neck Surg 2017 Oct;25(5):396-399. doi: 10.1097/MOO.0000000000000390. PMID: 28708635
Early complications following cochlear implantation in children and their management.
Li S, Qin Z, Zhang F, Li L, Qi S, Liu L
Int J Pediatr Otorhinolaryngol 2014 Jul;78(7):1040-4. Epub 2014 Apr 12 doi: 10.1016/j.ijporl.2014.03.036. PMID: 24809767

Recent clinical studies

Etiology

Midline Facial Nerve Monitoring: Single-Center Experience and Review of Literature.
Jomah MA, Hajr EA
J Int Adv Otol 2022 Jan;18(1):38-42. doi: 10.5152/iao.2022.20098. PMID: 35193844Free PMC Article
Auditory phenotype of Smith-Lemli-Opitz syndrome.
Zalewski CK, Sydlowski SA, King KA, Bianconi S, Dang Do A, Porter FD, Brewer CC
Am J Med Genet A 2021 Apr;185(4):1131-1141. Epub 2021 Feb 2 doi: 10.1002/ajmg.a.62087. PMID: 33529473Free PMC Article
Management of Cochlear Nerve Hypoplasia and Aplasia.
Freeman SR, Sennaroglu L
Adv Otorhinolaryngol 2018;81:81-92. Epub 2018 Apr 6 doi: 10.1159/000485542. PMID: 29794457
Classification and Current Management of Inner Ear Malformations.
Sennaroğlu L, Bajin MD
Balkan Med J 2017 Sep 29;34(5):397-411. Epub 2017 Aug 25 doi: 10.4274/balkanmedj.2017.0367. PMID: 28840850Free PMC Article
Cochlear synaptopathy in acquired sensorineural hearing loss: Manifestations and mechanisms.
Liberman MC, Kujawa SG
Hear Res 2017 Jun;349:138-147. Epub 2017 Jan 10 doi: 10.1016/j.heares.2017.01.003. PMID: 28087419Free PMC Article

Diagnosis

Management of Cochlear Nerve Hypoplasia and Aplasia.
Freeman SR, Sennaroglu L
Adv Otorhinolaryngol 2018;81:81-92. Epub 2018 Apr 6 doi: 10.1159/000485542. PMID: 29794457
Electrophysiology of Cranial Nerve Testing: Auditory Nerve.
Legatt AD
J Clin Neurophysiol 2018 Jan;35(1):25-38. doi: 10.1097/WNP.0000000000000421. PMID: 29298210
Auditory neuropathy--neural and synaptic mechanisms.
Moser T, Starr A
Nat Rev Neurol 2016 Mar;12(3):135-49. Epub 2016 Feb 19 doi: 10.1038/nrneurol.2016.10. PMID: 26891769
Pre-, Intra- and Post-Operative Imaging of Cochlear Implants.
Vogl TJ, Tawfik A, Emam A, Naguib NN, Nour-Eldin A, Burck I, Stöver T
Rofo 2015 Nov;187(11):980-9. Epub 2015 Sep 1 doi: 10.1055/s-0035-1553413. PMID: 26327670
Auditory neuropathy.
Starr A, Picton TW, Sininger Y, Hood LJ, Berlin CI
Brain 1996 Jun;119 ( Pt 3):741-53. doi: 10.1093/brain/119.3.741. PMID: 8673487

Therapy

Bony Cochlear Nerve Canal as a Predictor for Cochlear Nerve Status in Prelingually Deaf Children.
Dokoska M, Nikolova S, Kiteva-Trencevska G, Tilman K, Netkovski J
Pril (Makedon Akad Nauk Umet Odd Med Nauki) 2023 Mar 1;44(1):155-162. Epub 2023 Mar 29 doi: 10.2478/prilozi-2023-0017. PMID: 36987763
Investigating Facial Nerve Stimulation After Cochlear Implantation in Adult and Pediatric Recipients.
Alzhrani F, Halawani R, Basodan S, Hudeib R
Laryngoscope 2021 Feb;131(2):374-379. Epub 2020 Mar 28 doi: 10.1002/lary.28632. PMID: 32222081
Cochlear Implantation in Inner Ear Malformations: Systematic Review of Speech Perception Outcomes and Intraoperative Findings.
Farhood Z, Nguyen SA, Miller SC, Holcomb MA, Meyer TA, Rizk HG
Otolaryngol Head Neck Surg 2017 May;156(5):783-793. Epub 2017 Apr 4 doi: 10.1177/0194599817696502. PMID: 28374626
Cochlear synaptopathy in acquired sensorineural hearing loss: Manifestations and mechanisms.
Liberman MC, Kujawa SG
Hear Res 2017 Jun;349:138-147. Epub 2017 Jan 10 doi: 10.1016/j.heares.2017.01.003. PMID: 28087419Free PMC Article
Management of surgical difficulties during cochlear implant with inner ear anomalies.
Aldhafeeri AM, Alsanosi AA
Int J Pediatr Otorhinolaryngol 2017 Jan;92:45-49. Epub 2016 Nov 3 doi: 10.1016/j.ijporl.2016.11.001. PMID: 28012532

Prognosis

The Results of Cochlear Implantation in the Inner Ear Malformations.
Onan E, Tuncer U, Surmelioglu O, Dagkiran M, Ozdemir S, Tarkan O, Kiroglu MM
J Int Adv Otol 2022 May;18(3):203-209. doi: 10.5152/iao.2022.20134. PMID: 35608487Free PMC Article
Otopathologic Abnormalities in CHARGE Syndrome.
da Costa Monsanto R, Knoll RM, de Oliveira Penido N, Song G, Santos F, Paparella MM, Cureoglu S
Otolaryngol Head Neck Surg 2022 Feb;166(2):363-372. Epub 2021 Apr 20 doi: 10.1177/01945998211008911. PMID: 33874787
Cochlear Nerve Aplasia and Hypoplasia: Predictors of Cochlear Implant Success.
Peng KA, Kuan EC, Hagan S, Wilkinson EP, Miller ME
Otolaryngol Head Neck Surg 2017 Sep;157(3):392-400. Epub 2017 Jul 4 doi: 10.1177/0194599817718798. PMID: 28675079
Cochlear Implantation in Inner Ear Malformations: Systematic Review of Speech Perception Outcomes and Intraoperative Findings.
Farhood Z, Nguyen SA, Miller SC, Holcomb MA, Meyer TA, Rizk HG
Otolaryngol Head Neck Surg 2017 May;156(5):783-793. Epub 2017 Apr 4 doi: 10.1177/0194599817696502. PMID: 28374626
Sudden deafness: is it viral?
Merchant SN, Durand ML, Adams JC
ORL J Otorhinolaryngol Relat Spec 2008;70(1):52-60; discussion 60-2. Epub 2008 Feb 1 doi: 10.1159/000111048. PMID: 18235206Free PMC Article

Clinical prediction guides

Auditory phenotype of Smith-Lemli-Opitz syndrome.
Zalewski CK, Sydlowski SA, King KA, Bianconi S, Dang Do A, Porter FD, Brewer CC
Am J Med Genet A 2021 Apr;185(4):1131-1141. Epub 2021 Feb 2 doi: 10.1002/ajmg.a.62087. PMID: 33529473Free PMC Article
Noise-induced Cochlear Synaptopathy and Signal Processing Disorders.
Chen H, Shi L, Liu L, Yin S, Aiken S, Wang J
Neuroscience 2019 May 21;407:41-52. Epub 2018 Sep 27 doi: 10.1016/j.neuroscience.2018.09.026. PMID: 30267832
Management of Cochlear Nerve Hypoplasia and Aplasia.
Freeman SR, Sennaroglu L
Adv Otorhinolaryngol 2018;81:81-92. Epub 2018 Apr 6 doi: 10.1159/000485542. PMID: 29794457
Classification and Current Management of Inner Ear Malformations.
Sennaroğlu L, Bajin MD
Balkan Med J 2017 Sep 29;34(5):397-411. Epub 2017 Aug 25 doi: 10.4274/balkanmedj.2017.0367. PMID: 28840850Free PMC Article
Cochlear Nerve Aplasia and Hypoplasia: Predictors of Cochlear Implant Success.
Peng KA, Kuan EC, Hagan S, Wilkinson EP, Miller ME
Otolaryngol Head Neck Surg 2017 Sep;157(3):392-400. Epub 2017 Jul 4 doi: 10.1177/0194599817718798. PMID: 28675079

Recent systematic reviews

Surgical Management of Endolymphatic Sac Tumor: A Systematic Review and Meta-Analysis.
Gioacchini FM, Chiarella G, Kaleci S, Iannella G, Viola P, Pisani D, Scarpa A, Tulli M, Re M
J Int Adv Otol 2023 Jun;19(3):248-254. doi: 10.5152/iao.2023.22957. PMID: 37272644Free PMC Article
Systematic review of cochlear implantation in patients with inner ear malformations.
Shah S, Walters R, Langlie J, Davies C, Finberg A, Tuset MP, Ebode D, Mittal R, Eshraghi AA
PLoS One 2022;17(10):e0275543. Epub 2022 Oct 21 doi: 10.1371/journal.pone.0275543. PMID: 36269710Free PMC Article
Dysfunction of the Auditory System in Children With Hypothyroidism: A Systematic Review and Meta-Analysis.
Zhang K, Fried J, Nguyen SA, Meyer TA, White DR
Ear Hear 2022 Jan/Feb;43(1):23-31. doi: 10.1097/AUD.0000000000001082. PMID: 34282088
Systematic review of cochlear implantation in CHARGE syndrome.
Amin N, Sethukumar P, Pai I, Rajput K, Nash R
Cochlear Implants Int 2019 Sep;20(5):266-280. Epub 2019 Jul 7 doi: 10.1080/14670100.2019.1634857. PMID: 31282293
Cochlear Implantation in Inner Ear Malformations: Systematic Review of Speech Perception Outcomes and Intraoperative Findings.
Farhood Z, Nguyen SA, Miller SC, Holcomb MA, Meyer TA, Rizk HG
Otolaryngol Head Neck Surg 2017 May;156(5):783-793. Epub 2017 Apr 4 doi: 10.1177/0194599817696502. PMID: 28374626

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