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Vestibular hypofunction

MedGen UID:
336378
Concept ID:
C1848606
Finding
Synonym: Vestibular hyporeflexia
 
HPO: HP:0001756

Definition

A general descriptive term that describes impaired functioning of the vestibular apparatus that leads to manifestations such as dizziness or postural imbalance [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVVestibular hypofunction

Conditions with this feature

Usher syndrome type 1C
MedGen UID:
338506
Concept ID:
C1848604
Disease or Syndrome
Usher syndrome type I (USH1) is characterized by congenital, bilateral, profound sensorineural hearing loss, vestibular areflexia, and adolescent-onset retinitis pigmentosa (RP). Unless fitted with a cochlear implant, individuals do not typically develop speech. RP, a progressive, bilateral, symmetric degeneration of rod and cone functions of the retina, develops in adolescence, resulting in progressively constricted visual fields and impaired visual acuity.
Autosomal recessive nonsyndromic hearing loss 89
MedGen UID:
462701
Concept ID:
C3151351
Disease or Syndrome
Any autosomal recessive nonsyndromic deafness in which the cause of the disease is a mutation in the KARS gene.
Autosomal recessive nonsyndromic hearing loss 84B
MedGen UID:
767073
Concept ID:
C3554159
Disease or Syndrome
Any autosomal recessive nonsyndromic deafness in which the cause of the disease is a mutation in the OTOGL gene.
Autosomal recessive nonsyndromic hearing loss 18B
MedGen UID:
767077
Concept ID:
C3554163
Disease or Syndrome
Any autosomal recessive nonsyndromic deafness in which the cause of the disease is a mutation in the OTOG gene.
Lichtenstein-Knorr syndrome
MedGen UID:
898996
Concept ID:
C4225383
Disease or Syndrome
Lichtenstein-Knorr syndrome is an autosomal recessive neurologic disorder characterized by postnatal onset of severe progressive sensorineural hearing loss and progressive cerebellar ataxia. Features usually develop in childhood or young adulthood (summary by Guissart et al., 2015). Some patients with SLC9A1 mutations may not have deafness (Iwama et al., 2018)

Professional guidelines

PubMed

Hall CD, Herdman SJ, Whitney SL, Anson ER, Carender WJ, Hoppes CW, Cass SP, Christy JB, Cohen HS, Fife TD, Furman JM, Shepard NT, Clendaniel RA, Dishman JD, Goebel JA, Meldrum D, Ryan C, Wallace RL, Woodward NJ
J Neurol Phys Ther 2022 Apr 1;46(2):118-177. doi: 10.1097/NPT.0000000000000382. PMID: 34864777Free PMC Article
van de Berg R, Ramos A, van Rompaey V, Bisdorff A, Perez-Fornos A, Rubinstein JT, Phillips JO, Strupp M, Della Santina CC, Guinand N
J Vestib Res 2020;30(3):213-223. doi: 10.3233/VES-200701. PMID: 32651339Free PMC Article
Hall CD, Herdman SJ, Whitney SL, Cass SP, Clendaniel RA, Fife TD, Furman JM, Getchius TS, Goebel JA, Shepard NT, Woodhouse SN
J Neurol Phys Ther 2016 Apr;40(2):124-55. doi: 10.1097/NPT.0000000000000120. PMID: 26913496Free PMC Article

Recent clinical studies

Etiology

Lilios A, Chimona T, Papadakis C, Chatziioanou I, Nikitas C, Skoulakis C
Otol Neurotol 2023 Apr 1;44(4):e246-e255. Epub 2023 Feb 16 doi: 10.1097/MAO.0000000000003836. PMID: 36946364
Pröpper EJ, van Eijsden HMK, Schermer TR, Bruintjes T
J Int Adv Otol 2022 Jul;18(4):327-333. doi: 10.5152/iao.2022.21407. PMID: 35894529Free PMC Article
Jahn K
Adv Otorhinolaryngol 2019;82:143-149. Epub 2019 Jan 15 doi: 10.1159/000490283. PMID: 30947233
Dunlap PM, Holmberg JM, Whitney SL
Curr Opin Neurol 2019 Feb;32(1):137-144. doi: 10.1097/WCO.0000000000000632. PMID: 30461465
Schniepp R, Möhwald K, Wuehr M
J Neurol 2017 Oct;264(Suppl 1):87-92. Epub 2017 Apr 10 doi: 10.1007/s00415-017-8482-3. PMID: 28397001

Diagnosis

Hall CD, Herdman SJ, Whitney SL, Anson ER, Carender WJ, Hoppes CW, Cass SP, Christy JB, Cohen HS, Fife TD, Furman JM, Shepard NT, Clendaniel RA, Dishman JD, Goebel JA, Meldrum D, Ryan C, Wallace RL, Woodward NJ
J Neurol Phys Ther 2022 Apr 1;46(2):118-177. doi: 10.1097/NPT.0000000000000382. PMID: 34864777Free PMC Article
Starkov D, Strupp M, Pleshkov M, Kingma H, van de Berg R
J Neurol 2021 Jan;268(1):377-385. Epub 2020 Aug 7 doi: 10.1007/s00415-020-10139-4. PMID: 32767115Free PMC Article
Meldrum D, Jahn K
J Neurol 2019 Sep;266(Suppl 1):11-18. Epub 2019 Aug 5 doi: 10.1007/s00415-019-09459-x. PMID: 31385017
Dunlap PM, Holmberg JM, Whitney SL
Curr Opin Neurol 2019 Feb;32(1):137-144. doi: 10.1097/WCO.0000000000000632. PMID: 30461465
Lempert T
Semin Neurol 2013 Jul;33(3):212-8. Epub 2013 Sep 21 doi: 10.1055/s-0033-1354596. PMID: 24057824

Therapy

Lilios A, Chimona T, Papadakis C, Chatziioanou I, Nikitas C, Skoulakis C
Otol Neurotol 2023 Apr 1;44(4):e246-e255. Epub 2023 Feb 16 doi: 10.1097/MAO.0000000000003836. PMID: 36946364
Hall CD, Herdman SJ, Whitney SL, Anson ER, Carender WJ, Hoppes CW, Cass SP, Christy JB, Cohen HS, Fife TD, Furman JM, Shepard NT, Clendaniel RA, Dishman JD, Goebel JA, Meldrum D, Ryan C, Wallace RL, Woodward NJ
J Neurol Phys Ther 2022 Apr 1;46(2):118-177. doi: 10.1097/NPT.0000000000000382. PMID: 34864777Free PMC Article
Schniepp R, Möhwald K, Wuehr M
J Neurol 2017 Oct;264(Suppl 1):87-92. Epub 2017 Apr 10 doi: 10.1007/s00415-017-8482-3. PMID: 28397001
Hall CD, Herdman SJ, Whitney SL, Cass SP, Clendaniel RA, Fife TD, Furman JM, Getchius TS, Goebel JA, Shepard NT, Woodhouse SN
J Neurol Phys Ther 2016 Apr;40(2):124-55. doi: 10.1097/NPT.0000000000000120. PMID: 26913496Free PMC Article
Herdman SJ
Curr Opin Neurol 2013 Feb;26(1):96-101. doi: 10.1097/WCO.0b013e32835c5ec4. PMID: 23241567

Prognosis

Nayak N, Kellermeyer B, Dornton L, Heyd C, Kim CS, Wazen JJ
Am J Otolaryngol 2022 Jan-Feb;43(1):103171. Epub 2021 Aug 6 doi: 10.1016/j.amjoto.2021.103171. PMID: 34509078
Batuecas-Caletrio A, Trinidad-Ruiz G, Rey-Martinez J, Matiño-Soler E, Martin Sanz E, Perez Fernandez N
Ear Hear 2020 Mar/Apr;41(2):323-329. doi: 10.1097/AUD.0000000000000760. PMID: 31517671
Rah YC, Park JH, Park JH, Choi BY, Koo JW
Eur Arch Otorhinolaryngol 2016 Nov;273(11):3615-3621. Epub 2016 Mar 21 doi: 10.1007/s00405-016-3988-3. PMID: 27001256
Herdman SJ
Curr Opin Neurol 2013 Feb;26(1):96-101. doi: 10.1097/WCO.0b013e32835c5ec4. PMID: 23241567
Schubert MC, Minor LB
Phys Ther 2004 Apr;84(4):373-85. PMID: 15049730

Clinical prediction guides

Lilios A, Chimona T, Papadakis C, Chatziioanou I, Nikitas C, Skoulakis C
Otol Neurotol 2023 Apr 1;44(4):e246-e255. Epub 2023 Feb 16 doi: 10.1097/MAO.0000000000003836. PMID: 36946364
Müller KJ, Becker-Bense S, Strobl R, Grill E, Dieterich M
Eur J Neurol 2022 Jun;29(6):1825-1835. Epub 2022 Mar 13 doi: 10.1111/ene.15308. PMID: 35239246
Micarelli A, Viziano A, Pistillo R, Granito I, Micarelli B, Alessandrini M
Laryngoscope 2021 Oct;131(10):2341-2347. Epub 2021 Jun 30 doi: 10.1002/lary.29719. PMID: 34191310
Frejo L, Giegling I, Teggi R, Lopez-Escamez JA, Rujescu D
J Neurol 2016 Apr;263 Suppl 1:S45-53. Epub 2016 Apr 15 doi: 10.1007/s00415-015-7988-9. PMID: 27083884Free PMC Article
Herdman SJ
Curr Opin Neurol 2013 Feb;26(1):96-101. doi: 10.1097/WCO.0b013e32835c5ec4. PMID: 23241567

Recent systematic reviews

Chari DA, Madhani A, Sharon JD, Lewis RF
J Neurol 2022 Nov;269(11):5831-5842. Epub 2022 Aug 5 doi: 10.1007/s00415-022-11289-3. PMID: 35930032
Lilios A, Chimona T, Nikitas C, Papadakis C, Chatziioannou I, Skoulakis C
Otol Neurotol 2021 Dec 1;42(10):e1422-e1431. doi: 10.1097/MAO.0000000000003354. PMID: 34510122
Waissbluth S, Sepúlveda V
Otol Neurotol 2021 Jun 1;42(5):646-658. doi: 10.1097/MAO.0000000000003022. PMID: 33492062
Hillier S, McDonnell M
Eur J Phys Rehabil Med 2016 Aug;52(4):541-56. Epub 2016 Jul 12 PMID: 27406654
Porciuncula F, Johnson CC, Glickman LB
J Vestib Res 2012;22(5-6):283-98. doi: 10.3233/VES-120464. PMID: 23302709

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