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Abnormality of the vestibulocochlear nerve

MedGen UID:
867073
•Concept ID:
C4021431
•
Anatomical Abnormality
Synonyms: Abnormality of the eighth cranial nerve; Abnormality of the VIIIth cranial nerve
 
HPO: HP:0009591

Definition

Any structural anomaly of the vestibulocochlear nerve. The vestibulocochlear nerve consists of the vestibular and cochlear nerves, also known as cranial nerve eight (CN VIII). Each nerve has distinct nuclei within the brainstem. The vestibular nerve is primarily responsible for maintaining body balance and eye movements, while the cochlear nerve is responsible for hearing. [from HPO]

Term Hierarchy

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

Conditions with this feature

Autosomal dominant nonsyndromic hearing loss 9
MedGen UID:
371327
•Concept ID:
C1832425
•
Disease or Syndrome
DFNA9 is an autosomal dominant adult-onset form of progressive sensorineural hearing loss associated with variable vestibular dysfunction (summary by Robertson et al., 2006).
See: Condition Record
Autosomal dominant nonsyndromic hearing loss 9

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
Auditory neuropathy/dys-synchrony: diagnosis and management.
Berlin CI, Hood L, Morlet T, Rose K, Brashears S
Ment Retard Dev Disabil Res Rev 2003;9(4):225-31. doi: 10.1002/mrdd.10084. PMID: 14648814

Recent clinical studies

Etiology

Cochlear implantation programming characteristics and outcomes of cochlear nerve deficiency.
Wei X, Lu S, Chen B, Chen J, Zhang L, Li Y, Kong Y
Eur Arch Otorhinolaryngol 2023 Oct;280(10):4409-4418. Epub 2023 Apr 10 doi: 10.1007/s00405-023-07949-3. PMID: 37036510
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
Inferior vestibular neuritis.
Kim JS, Kim HJ
J Neurol 2012 Aug;259(8):1553-60. Epub 2012 Jan 4 doi: 10.1007/s00415-011-6375-4. PMID: 22215238

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
Neuro-otological emergencies.
Seemungal BM
Curr Opin Neurol 2007 Feb;20(1):32-9. doi: 10.1097/WCO.0b013e3280122514. PMID: 17215686
The vestibulocochlear nerve.
Spickler EM, Govila L
Semin Ultrasound CT MR 2002 Jun;23(3):218-37. doi: 10.1016/s0887-2171(02)90048-x. PMID: 12168998

Therapy

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
Hemorrhagic vestibular schwannoma: review of the literature.
Niknafs YS, Wang AC, Than KD, Etame AB, Thompson BG, Sullivan SE
World Neurosurg 2014 Nov;82(5):751-6. Epub 2013 Feb 27 doi: 10.1016/j.wneu.2013.02.069. PMID: 23454397
Histopathology of the inner ear relevant to cochlear implantation.
Nadol JB, Eddington DK
Adv Otorhinolaryngol 2006;64:31-49. doi: 10.1159/000094643. PMID: 16891835
Auditory neuropathy/dys-synchrony and its perceptual consequences.
Rance G
Trends Amplif 2005;9(1):1-43. doi: 10.1177/108471380500900102. PMID: 15920648Free PMC Article
Neuropharmacologic aspects of the ocular motor system and the treatment of abnormal eye movements.
Leigh RJ, Ramat S
Curr Opin Neurol 1999 Feb;12(1):21-7. doi: 10.1097/00019052-199902000-00004. PMID: 10097880

Prognosis

Vestibular function in children with vestibulocochlear nerve aplasia/hypoplasia.
Karpeta N, Asp F, Edholm K, Bonnard Å, Wales J, Karltorp E, Duan M, Verrecchia L
Acta Otolaryngol 2023 Oct;143(10):861-866. Epub 2024 Jan 5 doi: 10.1080/00016489.2023.2285453. PMID: 38063358
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
Some Remarks on Imaging of the Inner Ear: Options and Limitations.
Giesemann A, Hofmann E
Clin Neuroradiol 2015 Oct;25 Suppl 2:197-203. Epub 2015 Jul 8 doi: 10.1007/s00062-015-0422-y. PMID: 26153464
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
Audiovestibular manifestations of sarcoidosis: a review of the literature.
Colvin IB
Laryngoscope 2006 Jan;116(1):75-82. doi: 10.1097/01.mlg.0000184580.52723.9f. PMID: 16481814

Clinical prediction guides

Cochlear implantation programming characteristics and outcomes of cochlear nerve deficiency.
Wei X, Lu S, Chen B, Chen J, Zhang L, Li Y, Kong Y
Eur Arch Otorhinolaryngol 2023 Oct;280(10):4409-4418. Epub 2023 Apr 10 doi: 10.1007/s00405-023-07949-3. PMID: 37036510
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 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

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

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