A hidden pathophysiology of endolymphatic hydrops: case report of a patient with spontaneous intracranial hypotension presenting with sudden sensorineural hearing loss with vertigo

Jong Kwan Kim; Ho Yun Lee; Ha Youn Kim; Min Young Kwak

doi:10.3389/fneur.2024.1394534

A hidden pathophysiology of endolymphatic hydrops: case report of a patient with spontaneous intracranial hypotension presenting with sudden sensorineural hearing loss with vertigo

Front Neurol. 2024 Apr 5:15:1394534. doi: 10.3389/fneur.2024.1394534. eCollection 2024.

Authors

Jong Kwan Kim^#¹, Ho Yun Lee^#², Ha Youn Kim³, Min Young Kwak⁴

Affiliations

¹ Department of Otorhinolaryngology-Head and Neck Surgery, Daejeon Eulji Medical Center, Eulji University, Daejeon, Republic of Korea.
² Department of Otolaryngology-Head and Neck Surgery, College of Medicine, Ewha Womans University, Seoul, Republic of Korea.
³ Department of Radiology, Daejeon Eulji Medical Center, Eulji University, Daejeon, Republic of Korea.
⁴ Department of Otorhinolaryngology-Head and Neck Surgery, Hallym University Dongtan Sacred Heart Hospital, Hallym University College of Medicine, Hwaseong, Republic of Korea.

^# Contributed equally.

Abstract

Spontaneous intracranial hypotension (SIH) is characterized by decreased cerebrospinal fluid (CSF) volume due to leakage through the dural membrane. We present the case of a patient with SIH manifested by fluctuating low-frequency hearing loss, tinnitus, and vertigo. In this patient, endolymphatic hydrops in the cochlea and saccule were visualized by means of a special sequence of inner ear magnetic resonance imaging scans, with a gadolinium-based contrast agent administered intravenously. Endolymphatic hydrops is a potential underlying pathophysiology of SIH-associated hearing impairment. We hypothesize that SIH may be a rare cause of endolymphatic hydrops.

Keywords: MRI; endolymphatic hydrops; hearing loss; intracranial hypotension; vertigo.

Grants and funding

The author(s) declare that financial support was received for the research, authorship, and/or publication of this article. This research was supported by a National Research Foundation of Korea (NRF) grant funded by the Korean government (MSIT) (No. RS-2022-00165906) and a grant of the Korea Health Technology R&D Project through the Korea Health Industry Development Institute (KHIDI), funded by the Ministry of Health & Welfare, Republic of Korea (grant number: HI21C1574040021).