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A Mouse Model Validates the Utility of Electrocochleography in Verifying Endolymphatic Hydrops

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Abstract

Endolymphatic hydrops (ELH) is a disorder of the inner ear that causes tinnitus, vertigo, and hearing loss. An elevated ratio of the summating potential (SP) to the action potential (AP) measured by electrocochleography has long been considered to be the electrophysiological correlate of ELH-related clinical conditions, such as Meniere’s disease, but in vivo confirmation and correlation between an elevated SP/AP ratio and ELH has not yet been possible. Confirming this relationship will be important to show that elevated SP/AP ratio is indeed diagnostic of ELH. Here, we sought to confirm that an elevated SP/AP ratio is associated with ELH and test the hypothesis that severity of ELH and hearing loss would also correlate with the SP/AP ratio in vivo using the Phex Hyp-Duk /Y mouse model of postnatal ELH. In addition, we describe a minimally invasive approach for electrocochleography in mice. Auditory brainstem responses and electrocochleography data were collected from controls and Phex Hyp-Duk/Y mutants at postnatal day 21 and the mice (all male) were euthanized immediately for cochlear histology. Our results show that (1) the SP/AP ratio was significantly elevated in mice with histological ELH compared to controls, (2) the SP/AP ratio was not correlated with the severity of histological ELH or hearing loss, and (3) the severity of hearing loss correlated with the severity of histological ELH. Our study demonstrates that an elevated SP/AP ratio is diagnostic of ELH and that the severity of hearing loss is a better predictor of the severity of ELH than is the SP/AP ratio.

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Acknowledgments

This study was supported in part by grants from the National Institutes of Health (R01DC009246 to Q.Y.Z; R01DC01816 to K.N.A.) and funds from the Maniglia Endowed Chair, University Hospitals Case Medical Center (to K.N.A) and Pogue Endowed Chair (To C.A.M). We thank Heping Yu for her assistance in histological processing and animal breeding.

Conflict of Interest

The authors declare that they have no conflict of interest.

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Authors and Affiliations

  1. Ear Nose and Throat Institute, University Hospitals Case Medical Center, Case Western Reserve University, Cleveland, OH, 44106, USA

    Sami J Melki, Yiping Li, Maroun T Semaan, Qing Yin Zheng, Cliff A. Megerian & Kumar N Alagramam

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  1. Sami J Melki
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  2. Yiping Li
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  3. Maroun T Semaan
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  4. Qing Yin Zheng
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  5. Cliff A. Megerian
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  6. Kumar N Alagramam
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Corresponding author

Correspondence to Kumar N Alagramam.

Additional information

S.J.M. and Y.L. are co-first authors.

C.A.M and K.N.A are co-senior authors.

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Melki, S.J., Li, Y., Semaan, M.T. et al. A Mouse Model Validates the Utility of Electrocochleography in Verifying Endolymphatic Hydrops. JARO 15, 413–421 (2014). https://doi.org/10.1007/s10162-014-0445-0

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  • DOI: https://doi.org/10.1007/s10162-014-0445-0

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