U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Aplasia/Hypoplasia of the cochlea

MedGen UID:
868964
•Concept ID:
C4023378
•
Finding
Synonyms: Absent/small cochlea; Absent/underdeveloped cochlea
 
HPO: HP:0011395

Definition

Absence or underdevelopment of the cochlea, a spiral shaped cavity in the inner ear, owing to a developmental defect. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAplasia/Hypoplasia of the cochlea

Recent clinical studies

Etiology

Evaluation of Cochlear Implantation in Children With Cochlear Nerve Absence or Deficiency.
Shakhtour LB, Song S, Orobello NC, Garrett S, Ambrose T, Behzadpour HK, Vezina G, Preciado DA, Reilly BK
Otolaryngol Head Neck Surg 2024 Oct;171(4):1197-1204. Epub 2024 May 20 doi: 10.1002/ohn.827. PMID: 38769854
Cochlear Implant Outcomes in Cochlea Nerve Aplasia and Hypoplasia.
Birman CS, Powell HR, Gibson WP, Elliott EJ
Otol Neurotol 2016 Jun;37(5):438-45. doi: 10.1097/MAO.0000000000000997. PMID: 27050647
Auditory brainstem implant candidacy in the United States in children 0-17 years old.
Kaplan AB, Kozin ED, Puram SV, Owoc MS, Shah PV, Hight AE, Sethi RKV, Remenschneider AK, Lee DJ
Int J Pediatr Otorhinolaryngol 2015 Mar;79(3):310-315. Epub 2014 Dec 15 doi: 10.1016/j.ijporl.2014.11.023. PMID: 25577282Free PMC Article
The vestibulocochlear nerve: aplasia and hypoplasia in combination with inner ear malformations.
Giesemann AM, Kontorinis G, Jan Z, Lenarz T, Lanfermann H, Goetz F
Eur Radiol 2012 Mar;22(3):519-24. Epub 2011 Sep 30 doi: 10.1007/s00330-011-2287-z. PMID: 21960158
Cochlear implants in aplasia and hypoplasia of the cochleovestibular nerve.
Govaerts PJ, Casselman J, Daemers K, De Beukelaer C, Yperman M, De Ceulaer G
Otol Neurotol 2003 Nov;24(6):887-91. doi: 10.1097/00129492-200311000-00011. PMID: 14600469

Diagnosis

Congenital bilateral facial nerve hypoplasia with sensorineural hearing loss: a case report.
Hamizan AW, Yean KT, Abdullah A
Int J Pediatr Otorhinolaryngol 2012 Mar;76(3):455-9. Epub 2012 Jan 26 doi: 10.1016/j.ijporl.2012.01.002. PMID: 22281372

Prognosis

Evaluation of Cochlear Implantation in Children With Cochlear Nerve Absence or Deficiency.
Shakhtour LB, Song S, Orobello NC, Garrett S, Ambrose T, Behzadpour HK, Vezina G, Preciado DA, Reilly BK
Otolaryngol Head Neck Surg 2024 Oct;171(4):1197-1204. Epub 2024 May 20 doi: 10.1002/ohn.827. PMID: 38769854

Clinical prediction guides

Evaluation of Cochlear Implantation in Children With Cochlear Nerve Absence or Deficiency.
Shakhtour LB, Song S, Orobello NC, Garrett S, Ambrose T, Behzadpour HK, Vezina G, Preciado DA, Reilly BK
Otolaryngol Head Neck Surg 2024 Oct;171(4):1197-1204. Epub 2024 May 20 doi: 10.1002/ohn.827. PMID: 38769854
Cochlear Implant Outcomes in Cochlea Nerve Aplasia and Hypoplasia.
Birman CS, Powell HR, Gibson WP, Elliott EJ
Otol Neurotol 2016 Jun;37(5):438-45. doi: 10.1097/MAO.0000000000000997. PMID: 27050647

Recent systematic reviews

Auditory brainstem implant candidacy in the United States in children 0-17 years old.
Kaplan AB, Kozin ED, Puram SV, Owoc MS, Shah PV, Hight AE, Sethi RKV, Remenschneider AK, Lee DJ
Int J Pediatr Otorhinolaryngol 2015 Mar;79(3):310-315. Epub 2014 Dec 15 doi: 10.1016/j.ijporl.2014.11.023. PMID: 25577282Free PMC Article

Supplemental Content

Table of contents

    Clinical resources

    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...