Hearing loss and renal syndromes

Paul J. Phelan, Michelle N. Rheault

Research output: Contribution to journalReview articlepeer-review

13 Scopus citations


The association between ear and kidney abnormalities has long been recognized; however, the connection between these two disparate organs is not always straightforward. Although Alport syndrome is the most well-known, there are over 20 disorders that need to be considered in the differential diagnosis of patients with both ear and kidney abnormalities. Commonalities are present between the kidney and ear in a number of structural proteins, developmentally important transcription factors, ciliary proteins, and channel proteins, and mutations in these pathways can lead to disease in both organ systems. This manuscript reviews the congenital disorders with both hearing and kidney manifestations.

Original languageEnglish (US)
Pages (from-to)1671-1683
Number of pages13
JournalPediatric Nephrology
Issue number10
StatePublished - Oct 1 2018

Bibliographical note

Publisher Copyright:
© 2017, IPNA.

Copyright 2018 Elsevier B.V., All rights reserved.


  • Alport syndrome
  • Congenital anomalies of the kidney and urinary tract
  • Sensorineural hearing loss


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