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Aplasia of the vestibular nerve.

MedGen UID:
892296
•Concept ID:
C4021153
•
Anatomical Abnormality
Synonyms: Absent cranial nerve viii; Absent the eighth cranial nerve; Absent vestribular nerve; Aplasia of cranial nerve VIII; Aplasia of the eighth cranial nerve
 
HPO: HP:0011393

Definition

Absence of the vestibular nerve [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAplasia of the vestibular nerve.

Professional guidelines

PubMed

Diagnosis, Management, and New Therapeutic Options in Childhood Neurofibromatosis Type 2 and Related Forms.
Ruggieri M, Praticò AD, Evans DG
Semin Pediatr Neurol 2015 Dec;22(4):240-58. Epub 2015 Oct 28 doi: 10.1016/j.spen.2015.10.008. PMID: 26706012
The clinical utility of search coil horizontal vestibulo-ocular reflex testing.
Kessler P, Zarandy MM, Hajioff D, Tomlinson D, Ranalli P, Rutka J
Acta Otolaryngol 2008 Jan;128(1):29-37. doi: 10.1080/00016480701299642. PMID: 17851913
Diagnosis and management of drop attacks of vestibular origin: Tumarkin's otolithic crisis.
Black FO, Effron MZ, Burns DS
Otolaryngol Head Neck Surg 1982 Mar-Apr;90(2):256-62. doi: 10.1177/019459988209000221. PMID: 6810273

Recent clinical studies

Etiology

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
The auditory and vestibular toxicities induced by antiepileptic drugs.
Hamed SA
Expert Opin Drug Saf 2017 Nov;16(11):1281-1294. Epub 2017 Aug 29 doi: 10.1080/14740338.2017.1372420. PMID: 28838247
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
Vestibular neuritis affects both superior and inferior vestibular nerves.
Taylor RL, McGarvie LA, Reid N, Young AS, Halmagyi GM, Welgampola MS
Neurology 2016 Oct 18;87(16):1704-1712. Epub 2016 Sep 30 doi: 10.1212/WNL.0000000000003223. PMID: 27694256
Tinnitus: presence and future.
Møller AR
Prog Brain Res 2007;166:3-16. doi: 10.1016/S0079-6123(07)66001-4. PMID: 17956767

Diagnosis

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
Neuroimaging in cerebellar ataxia in childhood: A review.
Serrallach BL, Orman G, Boltshauser E, Hackenberg A, Desai NK, Kralik SF, Huisman TAGM
J Neuroimaging 2022 Sep;32(5):825-851. Epub 2022 Jun 24 doi: 10.1111/jon.13017. PMID: 35749078
Neurofibromatosis type 2.
Slattery WH
Otolaryngol Clin North Am 2015 Jun;48(3):443-60. doi: 10.1016/j.otc.2015.02.005. PMID: 26043141
Neuro-otological emergencies.
Seemungal BM
Curr Opin Neurol 2007 Feb;20(1):32-9. doi: 10.1097/WCO.0b013e3280122514. PMID: 17215686
Vestibular neuritis.
Nadol JB Jr
Otolaryngol Head Neck Surg 1995 Jan;112(1):162-72. doi: 10.1016/S0194-59989570316-0. PMID: 7816452

Therapy

Histopathology of Inner Ear Malformations: Potential Pitfalls for Cochlear Implantation.
Monsanto RDC, Sennaroglu L, Uchiyama M, Sancak IG, Paparella MM, Cureoglu S
Otol Neurotol 2019 Sep;40(8):e839-e846. doi: 10.1097/MAO.0000000000002356. PMID: 31361687Free PMC Article
The auditory and vestibular toxicities induced by antiepileptic drugs.
Hamed SA
Expert Opin Drug Saf 2017 Nov;16(11):1281-1294. Epub 2017 Aug 29 doi: 10.1080/14740338.2017.1372420. PMID: 28838247
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
Initial Results of a Safety and Feasibility Study of Auditory Brainstem Implantation in Congenitally Deaf Children.
Wilkinson EP, Eisenberg LS, Krieger MD, Schwartz MS, Winter M, Glater JL, Martinez AS, Fisher LM, Shannon RV; Los Angeles Pediatric ABI Team
Otol Neurotol 2017 Feb;38(2):212-220. doi: 10.1097/MAO.0000000000001287. PMID: 27898605Free PMC Article
Vestibular paroxysmia: a treatable neurovascular cross-compression syndrome.
Brandt T, Strupp M, Dieterich M
J Neurol 2016 Apr;263 Suppl 1:S90-6. Epub 2016 Apr 15 doi: 10.1007/s00415-015-7973-3. PMID: 27083889Free PMC Article

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
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
Vestibular neuritis affects both superior and inferior vestibular nerves.
Taylor RL, McGarvie LA, Reid N, Young AS, Halmagyi GM, Welgampola MS
Neurology 2016 Oct 18;87(16):1704-1712. Epub 2016 Sep 30 doi: 10.1212/WNL.0000000000003223. PMID: 27694256
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

Clinical prediction guides

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
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
Identification of Novel Candidate Genes and Variants for Hearing Loss and Temporal Bone Anomalies.
Santos-Cortez RLP, Yarza TKL, Bootpetch TC, Tantoco MLC, Mohlke KL, Cruz TLG, Chiong Perez ME, Chan AL, Lee NR, Tobias-Grasso CAM, Reyes-Quintos MRT, Cutiongco-de la Paz EM, Chiong CM
Genes (Basel) 2021 Apr 13;12(4) doi: 10.3390/genes12040566. PMID: 33924653Free PMC Article
Epidemiologic, Imaging, Audiologic, Clinical, Surgical, and Prognostic Issues in Common Cavity Deformity: A Narrative Review.
Brotto D, Avato I, Lovo E, Muraro E, Bovo R, Trevisi P, Martini A, Manara R
JAMA Otolaryngol Head Neck Surg 2019 Jan 1;145(1):72-78. doi: 10.1001/jamaoto.2018.2839. PMID: 30419122
Vestibular neuronitis. An otoneurological evaluation.
Silvoniemi P
Acta Otolaryngol Suppl 1988;453:1-72. doi: 10.3109/00016488809098974. PMID: 3068952

Recent systematic reviews

Assessment of the Clinical Benefit of Imaging in Children With Unilateral Sensorineural Hearing Loss: A Systematic Review and Meta-analysis.
Ropers FG, Pham ENB, Kant SG, Rotteveel LJC, Rings EHHM, Verbist BM, Dekkers OM
JAMA Otolaryngol Head Neck Surg 2019 May 1;145(5):431-443. doi: 10.1001/jamaoto.2019.0121. PMID: 30946449Free PMC Article
Diagnostic yield of MRI for pediatric hearing loss: a systematic review.
Kachniarz B, Chen JX, Gilani S, Shin JJ
Otolaryngol Head Neck Surg 2015 Jan;152(1):5-22. Epub 2014 Nov 11 doi: 10.1177/0194599814555837. PMID: 25389321Free PMC Article
Indications and contraindications of auditory brainstem implants: systematic review and illustrative cases.
Merkus P, Di Lella F, Di Trapani G, Pasanisi E, Beltrame MA, Zanetti D, Negri M, Sanna M
Eur Arch Otorhinolaryngol 2014 Jan;271(1):3-13. Epub 2013 Feb 13 doi: 10.1007/s00405-013-2378-3. PMID: 23404468
Cochlear implantation in children with anomalous cochleovestibular anatomy: a systematic review.
Pakdaman MN, Herrmann BS, Curtin HD, Van Beek-King J, Lee DJ
Otolaryngol Head Neck Surg 2012 Feb;146(2):180-90. Epub 2011 Dec 1 doi: 10.1177/0194599811429244. PMID: 22140206
Antioxidant micronutrient impact on hearing disorders: concept, rationale, and evidence.
Haase GM, Prasad KN, Cole WC, Baggett-Strehlau JM, Wyatt SE
Am J Otolaryngol 2011 Jan-Feb;32(1):55-61. Epub 2009 Nov 7 doi: 10.1016/j.amjoto.2009.09.002. PMID: 20015808

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