High-resolution MRI of the inner ear enables syndrome differentiation and specific treatment of cerebellar downbeat nystagmus and secondary endolymphatic hydrops in a postoperative ELST patient

  • V. Kirsch
  • B. Ertl-Wagner
  • A. Berman
  • J. Gerb
  • M. Dieterich
  • S. Becker-Bense
Letter to the Editors

Dear Sirs,

We report the case of a 48-year-old, right-handed female who had been operated on for an endolymphatic sac tumor (ELST) in the right petrosal bone (Fig.  1a–d) 15 months prior to the first presentation in our interdisciplinary dizziness unit. She was suffering considerably from three different clinical vestibular symptoms: (i) a persistent swaying sensation predominately in fore-after direction even at rest, (ii) reproducible position-dependent short-rotational vertigo attacks (30 s to several minutes) accompanied by an inconsistent nystagmus downward and to the right when lying on her back or her side ( R >  L), and (iii) spontaneous rotational vertigo attacks lasting for several hours associated with ear pressure on the operated right side, nausea, vomiting, and diarrhea. ELST is a very rare form of locally invasive, but very slowly growing papillary epithelial neoplasm originating from the endolymphatic sac and/or duct [ 1], which can occur sporadically or as part of von...



Partially funded by the Society for the Advancement of Science and Research at the Medical Faculty of the Ludwig-Maximilian University, Munich, the Graduate School of Systemic Neurosciences (GSN), the German Foundation for Neurology (DSN) and the German Federal Ministry of Education and Research (German Center for Vertigo and Balance Disorders, Grant Code 01EO140). We thank Katie Göttlinger for copyediting the manuscript.

Compliance with ethical standards

Conflicts of interest

The authors declare they have no competing financial interests.

Ethical standards

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all individual participants prior to measurement.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Neurology, University HospitalLudwig-Maximilians-UniversitätMunichGermany
  2. 2.German Center for Vertigo and Balance Disorders-IFBLudwig-Maximilians-UniversitätMunichGermany
  3. 3.Graduate School of Systemic NeuroscienceLudwig-Maximilians-UniversitätMunichGermany
  4. 4.Department of Radiology, University HospitalLudwig-Maximilians-UniversitätMunichGermany
  5. 5.Munich Cluster for Systems Neurology (SyNergy)MunichGermany

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