Pediatric Nephrology

, Volume 19, Issue 11, pp 1194–1201

Genetic disorders of transporters/channels in the inner ear and their relation to the kidney

  • Theo A. Peters
  • Leo A. H. Monnens
  • Cor W. R. J. Cremers
  • Jo H. A. J. Curfs
Review

DOI: 10.1007/s00467-004-1626-6

Cite this article as:
Peters, T.A., Monnens, L.A.H., Cremers, C.W.R.J. et al. Pediatr Nephrol (2004) 19: 1194. doi:10.1007/s00467-004-1626-6

Abstract

Inner ear physiology is reviewed with emphasis on features common to renal physiology. Genetic disorders in transporters/channels for chloride (ClC-K), bicarbonate (Cl-/HCO3- exchanger), protons (H+-ATPase), sodium (ENaC, NKKC1, NBC3, NHE3), potassium (KCNQ1/KCNE1, Kcc4), and water (AQP4) in the inner ear and their relation to the kidney are discussed. Based on data from human disorders (with or without mouse counterparts) and mouse models (without human counterparts) this article focuses on the involvement of these transporters/channels in hearing loss.

Keywords

Deafness Endolymph Hereditary Inner ear Transport disorders 

Copyright information

© IPNA 2004

Authors and Affiliations

  • Theo A. Peters
    • 1
  • Leo A. H. Monnens
    • 2
  • Cor W. R. J. Cremers
    • 1
  • Jo H. A. J. Curfs
    • 1
  1. 1.Department of OtorhinolaryngologyUniversity Medical Center NijmegenThe Netherlands
  2. 2.Department of Pediatric NephrologyUniversity Medical Center NijmegenThe Netherlands

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