A Genetic Murine Model of Endolymphatic Hydrops: The Phex Mouse

Cameron C. Wick, Maroun T. Semaan, Qing Yin Zheng, Cliff A. Megerian

Research output: Contribution to journalReview articlepeer-review

7 Scopus citations

Abstract

Animal models of endolymphatic hydrops (ELH) provide critical insight into the pathophysiology of Meniere’s disease (MD). A new genetic murine model, called the Phex mouse, circumvents prior need for a time and cost-intensive surgical procedure to create ELH. The Phex mouse model of ELH, which also has X-linked hypophosphatemic rickets, creates a postnatal, spontaneous, and progressive ELH whose phenotype has a predictable decline of vestibular and hearing function reminiscent of human MD. The Phex mouse enables real-time histopathologic analysis to assess diagnostic and therapeutic interventions as well as further our understanding of ELH’s adverse effects. Already the model has validated electrocochleography and cervical vestibular evoked myogenic potential as useful diagnostic tools. New data on caspase activity in apoptosis of the spiral ganglion neurons may help target future therapeutic interventions. This paper highlights the development of the Phex mouse model and highlights its role in characterizing ELH.

Original languageEnglish
Pages (from-to)144-151
Number of pages8
JournalCurrent Otorhinolaryngology Reports
Volume2
Issue number3
DOIs
StatePublished - Sep 2014

Keywords

  • Apoptosis
  • Electrocochleography
  • Endolymphatic hydrops
  • Meniere’s disease
  • Phex
  • Vestibular evoked myogenic potential

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