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Aplasia/Hypoplasia of the inner ear

MedGen UID:
870191
•Concept ID:
C4024625
•
Finding
Synonyms: Absent/small inner ear; Absent/underdeveloped inner ear
 
HPO: HP:0008774

Definition

Aplasia or developmental hypoplasia of the inner ear. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Aplasia/Hypoplasia of the inner ear

Recent clinical studies

Etiology

Bimodal stimulation in children with inner ear malformation: One side cochlear implant and contralateral auditory brainstem implant.
Batuk MO, Cinar BC, Yarali M, Aslan F, Ozkan HB, Sennaroglu G, Yucel E, Bajin MD, Bilginer B, Sennaroglu L
Clin Otolaryngol 2020 Mar;45(2):231-238. Epub 2020 Jan 22 doi: 10.1111/coa.13499. PMID: 31854074
Ratio of Vestibular Endolymph in Patients with Isolated Lateral Semicircular Canal Dysplasia.
Naganawa S, Kawai H, Sone M, Ikeda M
Magn Reson Med Sci 2015;14(3):203-10. Epub 2015 Mar 31 doi: 10.2463/mrms.2014-0112. PMID: 25833266
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

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
Bimodal stimulation in children with inner ear malformation: One side cochlear implant and contralateral auditory brainstem implant.
Batuk MO, Cinar BC, Yarali M, Aslan F, Ozkan HB, Sennaroglu G, Yucel E, Bajin MD, Bilginer B, Sennaroglu L
Clin Otolaryngol 2020 Mar;45(2):231-238. Epub 2020 Jan 22 doi: 10.1111/coa.13499. PMID: 31854074
A Challenge for Cochlear Implantation: Duplicated Internal Auditory Canal.
Binnetoğlu A, Bağlam T, Sarı M, Gündoğdu Y, Batman Ç
J Int Adv Otol 2016 Aug;12(2):199-201. doi: 10.5152/iao.2016.1440. PMID: 27716607
Radiological Imaging Findings of Patients with Congenital Totally Hearing Loss.
Dağkıran M, Dağkıran N, Sürmelioğlu Ö, Ballı T, Tuncer Ü, Akgül E, Çetik F
J Int Adv Otol 2016 Apr;12(1):43-8. doi: 10.5152/iao.2015.1450. PMID: 27340982
Ratio of Vestibular Endolymph in Patients with Isolated Lateral Semicircular Canal Dysplasia.
Naganawa S, Kawai H, Sone M, Ikeda M
Magn Reson Med Sci 2015;14(3):203-10. Epub 2015 Mar 31 doi: 10.2463/mrms.2014-0112. PMID: 25833266

Therapy

Ratio of Vestibular Endolymph in Patients with Isolated Lateral Semicircular Canal Dysplasia.
Naganawa S, Kawai H, Sone M, Ikeda M
Magn Reson Med Sci 2015;14(3):203-10. Epub 2015 Mar 31 doi: 10.2463/mrms.2014-0112. PMID: 25833266

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
Bimodal stimulation in children with inner ear malformation: One side cochlear implant and contralateral auditory brainstem implant.
Batuk MO, Cinar BC, Yarali M, Aslan F, Ozkan HB, Sennaroglu G, Yucel E, Bajin MD, Bilginer B, Sennaroglu L
Clin Otolaryngol 2020 Mar;45(2):231-238. Epub 2020 Jan 22 doi: 10.1111/coa.13499. PMID: 31854074
Radiological Imaging Findings of Patients with Congenital Totally Hearing Loss.
Dağkıran M, Dağkıran N, Sürmelioğlu Ö, Ballı T, Tuncer Ü, Akgül E, Çetik F
J Int Adv Otol 2016 Apr;12(1):43-8. doi: 10.5152/iao.2015.1450. PMID: 27340982

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
Bimodal stimulation in children with inner ear malformation: One side cochlear implant and contralateral auditory brainstem implant.
Batuk MO, Cinar BC, Yarali M, Aslan F, Ozkan HB, Sennaroglu G, Yucel E, Bajin MD, Bilginer B, Sennaroglu L
Clin Otolaryngol 2020 Mar;45(2):231-238. Epub 2020 Jan 22 doi: 10.1111/coa.13499. PMID: 31854074
Radiological Imaging Findings of Patients with Congenital Totally Hearing Loss.
Dağkıran M, Dağkıran N, Sürmelioğlu Ö, Ballı T, Tuncer Ü, Akgül E, Çetik F
J Int Adv Otol 2016 Apr;12(1):43-8. doi: 10.5152/iao.2015.1450. PMID: 27340982

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

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    • ClinVar
      Related medical variations
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      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

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