KEGG   DISEASE: Bilateral sudden sensorineural hearing loss
Entry
H01705                      Disease                                
Name
Bilateral sudden sensorineural hearing loss
  Supergrp
Deafness, autosomal dominant [DS:H00604]
Description
Bilateral sudden sensorineural hearing loss (SNHL) represents a rare disease entity, constituting less than 5 % of all sudden SNHL cases. Unlike unilateral disease, bilateral sudden SNHL appears to be mostly related to serious systemic pathology, and is associated with a more severe degree of hearing loss, poorer prognosis following treatment, and more significant impairment in morbidity and overall quality of life.
Category
Nervous system disease
Brite
Human diseases in ICD-11 classification [BR:br08403]
 10 Diseases of the ear or mastoid process
  Disorders with hearing impairment
   AB51  Acquired hearing impairment
    H01705  Bilateral sudden sensorineural hearing loss
Pathway
hsa04015  Rap1 signaling pathway
hsa04810  Regulation of actin cytoskeleton
hsa04210  Apoptosis
hsa04530  Tight junction
Gene
ACTG1 [HSA:71] [KO:K05692]
CDH23 [HSA:64072] [KO:K06813]
COCH [HSA:1690] [KO:K23574]
KCNQ4 [HSA:9132] [KO:K04929]
TECTA [HSA:7007] [KO:K18273]
TMPRSS3 [HSA:64699] [KO:K09634]
WFS1 [HSA:7466] [KO:K14020]
EYA4 [HSA:2070] [KO:K17622]
MYO6 [HSA:4646] [KO:K10358]
MYO15A [HSA:51168] [KO:K10361]
POU4F3 [HSA:5459] [KO:K09366]
Comment
See also H01704 Sudden sensorineural hearing loss, H00604 Deafness, autosomal dominant, and H00605 Deafness, autosomal recessive.
Other DBs
ICD-11: AB51.1
MeSH: D006312
Reference
  Authors
Sara SA, Teh BM, Friedland P
  Title
Bilateral sudden sensorineural hearing loss: review.
  Journal
J Laryngol Otol 128 Suppl 1:S8-15 (2014)
DOI:10.1017/S002221511300306X
Reference
  Authors
Chen YH, Young YH
  Title
Bilateral simultaneous sudden sensorineural hearing loss.
  Journal
J Neurol Sci 362:139-43 (2016)
DOI:10.1016/j.jns.2016.01.029
Reference
  Authors
Smith RJH, Shearer AE, Hildebrand MS, Van Camp G
  Title
Deafness and Hereditary Hearing Loss Overview
  Journal
GeneReviews (1993)
Reference
  Authors
Eshraghi AA, Polineni SP, Davies C, Shahal D, Mittal J, Al-Zaghal Z, Sinha R, Jindal U, Mittal R
  Title
Genotype-Phenotype Correlation for Predicting Cochlear Implant Outcome: Current Challenges and Opportunities.
  Journal
Front Genet 11:678 (2020)
DOI:10.3389/fgene.2020.00678
LinkDB

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