U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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

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
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
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
Neural correlates of sensorineural hearing loss.
Salvi RJ, Henderson D, Hamernik R, Ahroon WA
Ear Hear 1983 May-Jun;4(3):115-29. doi: 10.1097/00003446-198305000-00001. PMID: 6345246

Prognosis

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
Adaptation of the acoustic reflex.
Fowler CG, Wilson RH
Ear Hear 1984 Sep-Oct;5(5):281-8. doi: 10.1097/00003446-198409000-00004. PMID: 6389238

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
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
Synchronous auditory nerve activity in the carboplatin-chinchilla model of auditory neuropathy.
Cowper-Smith CD, Dingle RN, Guo Y, Burkard R, Phillips DP
J Acoust Soc Am 2010 Jul;128(1):EL56-62. doi: 10.1121/1.3453764. PMID: 20649190Free PMC Article

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

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Reviews

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...