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Cochlear degeneration

MedGen UID:
338612
•Concept ID:
C1849095
•
Finding
Synonym: Progressive cochlear degeneration
 
HPO: HP:0005102

Definition

Deterioration or loss of the tissues of the cochlea. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCochlear degeneration

Conditions with this feature

Opticocochleodentate degeneration
MedGen UID:
101046
•Concept ID:
C0520711
•
Disease or Syndrome
See: Condition Record
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 recessive cerebellar ataxia-blindness-deafness syndrome
MedGen UID:
338611
•Concept ID:
C1849094
•
Disease or Syndrome
A rare autosomal recessive syndromic cerebellar ataxia with the association of early-onset cerebellar ataxia, hearing loss and blindness. Patients may also present demyelinating peripheral motor neuropathy. Cerebral MRI shows alterations of the cerebellar white matter without cerebellar atrophy.
See: Condition Record
Autosomal dominant nonsyndromic hearing loss 9
Autosomal recessive cerebellar ataxia-blindness-deafness syndrome
Opticocochleodentate degeneration

Professional guidelines

PubMed

Defective HSV-1 vector expressing BDNF in auditory ganglia elicits neurite outgrowth: model for treatment of neuron loss following cochlear degeneration.
Geschwind MD, Hartnick CJ, Liu W, Amat J, Van De Water TR, Federoff HJ
Hum Gene Ther 1996 Jan 20;7(2):173-82. doi: 10.1089/hum.1996.7.2-173. PMID: 8788168

Recent clinical studies

Etiology

Age-Related Hearing Loss: The Link between Inflammaging, Immunosenescence, and Gut Dysbiosis.
Kociszewska D, Vlajkovic S
Int J Mol Sci 2022 Jul 1;23(13) doi: 10.3390/ijms23137348. PMID: 35806352Free PMC Article
Extended High-frequency Hearing Impairment Despite a Normal Audiogram: Relation to Early Aging, Speech-in-noise Perception, Cochlear Function, and Routine Earphone Use.
Mishra SK, Saxena U, Rodrigo H
Ear Hear 2022 May/Jun;43(3):822-835. doi: 10.1097/AUD.0000000000001140. PMID: 34700326
Chronic Conductive Hearing Loss Leads to Cochlear Degeneration.
Liberman MC, Liberman LD, Maison SF
PLoS One 2015;10(11):e0142341. Epub 2015 Nov 18 doi: 10.1371/journal.pone.0142341. PMID: 26580411Free PMC Article
Age-dependent degeneration of the stria vascularis in human cochleae.
Suzuki T, Nomoto Y, Nakagawa T, Kuwahata N, Ogawa H, Suzuki Y, Ito J, Omori K
Laryngoscope 2006 Oct;116(10):1846-50. doi: 10.1097/01.mlg.0000234940.33569.39. PMID: 17003714
Cockayne syndrome and xeroderma pigmentosum.
Rapin I, Lindenbaum Y, Dickson DW, Kraemer KH, Robbins JH
Neurology 2000 Nov 28;55(10):1442-9. doi: 10.1212/wnl.55.10.1442. PMID: 11185579Free PMC Article

Diagnosis

Predicting early auditory evoked potentials using a computational model of auditory-nerve processing.
Temboury-Gutierrez M, Encina-Llamas G, Dau T
J Acoust Soc Am 2024 Mar 1;155(3):1799-1812. doi: 10.1121/10.0025136. PMID: 38445986
The Stria Vascularis in Mice and Humans Is an Early Site of Age-Related Cochlear Degeneration, Macrophage Dysfunction, and Inflammation.
Lang H, Noble KV, Barth JL, Rumschlag JA, Jenkins TR, Storm SL, Eckert MA, Dubno JR, Schulte BA
J Neurosci 2023 Jul 5;43(27):5057-5075. Epub 2023 Jun 2 doi: 10.1523/JNEUROSCI.2234-22.2023. PMID: 37268417Free PMC Article
Progressive familial hearing loss in Muckle-Wells syndrome.
Koitschev A, Gramlich K, Hansmann S, Benseler S, Plontke SK, Koitschev C, Koetter I, Kuemmerle-Deschner JB
Acta Otolaryngol 2012 Jul;132(7):756-62. Epub 2012 Apr 12 doi: 10.3109/00016489.2012.656321. PMID: 22497426
Gap junctions and connexins in the inner ear: their roles in homeostasis and deafness.
Nickel R, Forge A
Curr Opin Otolaryngol Head Neck Surg 2008 Oct;16(5):452-7. doi: 10.1097/MOO.0b013e32830e20b0. PMID: 18797288
Cockayne syndrome and xeroderma pigmentosum.
Rapin I, Lindenbaum Y, Dickson DW, Kraemer KH, Robbins JH
Neurology 2000 Nov 28;55(10):1442-9. doi: 10.1212/wnl.55.10.1442. PMID: 11185579Free PMC Article

Therapy

Cochlear Pathology after stapedectomy.
Schuknecht HF, Mendoza AM
Am J Otolaryngol 1981 Aug;2(3):173-87. doi: 10.1016/s0196-0709(81)80014-2. PMID: 7283061

Prognosis

Predicting early auditory evoked potentials using a computational model of auditory-nerve processing.
Temboury-Gutierrez M, Encina-Llamas G, Dau T
J Acoust Soc Am 2024 Mar 1;155(3):1799-1812. doi: 10.1121/10.0025136. PMID: 38445986
Cochlear degeneration in leigh disease: histopathologic features.
Ulualp SO, Wright CG, Pawlowski K, Roland PS
Laryngoscope 2004 Dec;114(12):2239-42. doi: 10.1097/01.mlg.0000149465.80703.8e. PMID: 15564853
GFP-tagged expression and immunohistochemical studies to determine the subcellular localization of the tubby gene family members.
He W, Ikeda S, Bronson RT, Yan G, Nishina PM, North MA, Naggert JK
Brain Res Mol Brain Res 2000 Sep 30;81(1-2):109-17. doi: 10.1016/s0169-328x(00)00164-9. PMID: 11000483

Clinical prediction guides

Predicting early auditory evoked potentials using a computational model of auditory-nerve processing.
Temboury-Gutierrez M, Encina-Llamas G, Dau T
J Acoust Soc Am 2024 Mar 1;155(3):1799-1812. doi: 10.1121/10.0025136. PMID: 38445986
Extended High-frequency Hearing Impairment Despite a Normal Audiogram: Relation to Early Aging, Speech-in-noise Perception, Cochlear Function, and Routine Earphone Use.
Mishra SK, Saxena U, Rodrigo H
Ear Hear 2022 May/Jun;43(3):822-835. doi: 10.1097/AUD.0000000000001140. PMID: 34700326
Otopathology in Osteogenesis Imperfecta.
Santos F, McCall AA, Chien W, Merchant S
Otol Neurotol 2012 Dec;33(9):1562-6. doi: 10.1097/MAO.0b013e31826bf19b. PMID: 22996160Free PMC Article
Age-dependent degeneration of the stria vascularis in human cochleae.
Suzuki T, Nomoto Y, Nakagawa T, Kuwahata N, Ogawa H, Suzuki Y, Ito J, Omori K
Laryngoscope 2006 Oct;116(10):1846-50. doi: 10.1097/01.mlg.0000234940.33569.39. PMID: 17003714
GFP-tagged expression and immunohistochemical studies to determine the subcellular localization of the tubby gene family members.
He W, Ikeda S, Bronson RT, Yan G, Nishina PM, North MA, Naggert JK
Brain Res Mol Brain Res 2000 Sep 30;81(1-2):109-17. doi: 10.1016/s0169-328x(00)00164-9. PMID: 11000483

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