Endolymphatic Hydropsin a Patient with a Small Vestibular Schwannoma Suggests a Peripheral Origin of Vertigo

Case Report

Austin J Radiol. 2015; 2(6): 1033.

Endolymphatic Hydropsin a Patient with a Small Vestibular Schwannoma Suggests a Peripheral Origin of Vertigo

Claudia Jerin1,3, Eike Krause1,3, Birgit Ertl- Wagner2 and Robert Gürkov1,3*

¹Department of Otorhinolaryngology Head and Neck Surgery, University of Munich Marchioninistr, Germany

²German Centre for Vertigo and Balance Disorders, University of Munich Marchioninistr, Germany

³Grosshadern Medical Centre, University of Munich Marchioninistr, Germany

*Corresponding author: Robert Gürkov, Department of Otorhinolaryngology Head and Neck Surgery, Grosshadern Medical Centre, University of Munich Marchioninistr. 15, 81377 Munich, Germany

Received: June 03, 2015; Accepted: August 27, 2015; Published: August 31, 2015


Objective: To present audio vestibular and MR imaging findings in a case of vestibular schwannoma with endolymphtic hydrops.

Design: Case report with 9-years follow-up.

Study Sample: A 43 year-old male patient with a small stable intrameatal vestibular schwannoma who developed recurrent vertigo attacks.

Results: Inner ear MRI revealed vestibular endolymphatic hydrops. oVEMP responses showed an altered frequency tuning suggestive of endolymphatic hydrops. Audiometry revealed mild high frequency hearing loss and corresponding high-frequency loss of otoacoustic emissions, suggesting a cochlear lesion.

Conclusion: This case study supports a labyrinthine origin of audio vestibular function deficits and symptoms. Detection of endolymphatic hydrops in such patients may be clinically relevant for the management of vertigo symptoms possibly due to endolymphatic hydrops.

Keywords: Vestibular schwannoma; Vertigo; Hearing loss; Endolymphatic hydrops; MRI


LEIM: Locally Enhanced Inner ear MRI; MRI: Magnetic Resonance Imaging; ELH: Endolymphatic Hydrops; MD: Menière’s Disease; VS: Vestibular Schwannoma; VEMP: Vestibular Evoked Myogenic Potential; DPOAE: Distortion Product Otoacoustic Emissions


Recently, MR imaging after intratympanic contrast agent application (Locally Enhanced Inner ear MR Imaging, LEIM) has been used to visualize Endolymphatic Hydrops (ELH) in patients with Menière’s Disease (MD) in vivo. The extent of ELH as assessed by inner ear MRI has recently been quantified volumetrically [1] and it has been shown to correlate with the dysfunction of various inner ear function parameters like hearing loss, caloric response and saccular function examined by cervical vestibular evoked myogenic potentials [2,3]. Furthermore, ELH has been detected in various inner ear syndromes, e.g. in delayed endolymphatic hydrops, in sudden sensorineural hearing loss and in a typical MD, i.e. MD with purely cochlear or purely vestibular symptoms [4].

In Vestibular Schwannoma (VS), the origin of audio vestibular symptoms might not be solely of retrocochlear origin, i.e. due to the tumor mass effect within the internal acoustic meatus. Therefore, we examined a patient with a stable VS and vertigo with LEIM in order to evaluate the presence of ELH as a possible cause for his symptoms.

Case Report

The 43-year-old patient had presented to our institution nine years before, with one attack of rotatory vertigo lasting for one day. A subsequent MR Scan revealed an intrameatal vestibular schwannoma (size 9 x 5 mm). Audiometry showed a mild high-frequency hearing loss ipsilaterally. Brainstem evoked response audiometry latencies of Jewett peaks I and V were within normal limits bilaterally. In the following years, the patient did not suffer from any complaints except for a pre-existing longstanding bilateral tinnitus. Nine years after the first vertigo attack, recurrent vertigo attacks of variable duration (mostly several minutes) occurred. Simultaneous aural symptoms were not reported, and the patient still did not report any subjective hearing impairment.

A Gadolinium-based contrast medium, Gadopentetate dimuglumine (Magnograf, Marotrast, Jena, Germany), was diluted 8-fold in saline solution and injected intratympanically (0.4 ml) under microscopic control, as described previously [2]. The patient remained in a supine position for further 30 minutes with the head turned 45 degrees toward the contralateral side, instructed not to speak or chew during this period. MRI scans were performed 24 hours after application of the contrast agent on a 3T MR scanner (Magnetom Verio, Siemens Healthcare, Erlangen, Germany) using a commercially available 32-channel head coil. A 3D-Real-IR sequence was acquired with the following parameters: TR 6000 ms, TE 155 ms, TI 1500 ms, fat saturation, constant flip angle of 180 degrees, echo train length of 35, echo train followed by a 90° restore pulse, matrix size of 320 × 320, 36 acquired slices (with 11.1% slice oversampling), 0.5 x 0.5 mm² in-plane resolution at 0.5 mm slice thickness, receiver bandwidth 195 Hz/pixel, and number of excitations 1. The scan time was 15 minutes. For imaging of the vestibular schwannoma, conventional T1 weighted contrast enhanced MR scans were acquired.

Nine years after the onset of symptoms and after the detection of the vestibular schwannoma in MR imaging, the follow-up MRI showed a stable tumor without evidence of growth (Figure 1A). By use of LEIM (Figure 1B), endolymphatic hydrops in the vestibulum could be visualized as an enlarged hypointense area within the contrast-enhanced perilymphatic space (greater than 40% endolymph/perilymph area ratio). There was no evidence of cochlear endolymphatic hydrops. In this case, clinical symptoms were mainly vestibular, which corresponds well to the LEIM that showed a vestibular endolymphatic hydrops. Caloric videonystagmography showed regular horizontal semicircular canal function. Cervikal Vestibular Evoked Myogenic Potential (cVEMP) testing revealed normal results. Ocular Vestibular Evoked Myogenic Potentials (oVEMP) revealed normal absolute amplitudes. However, oVEMP testing also revealed an ipsilaterally decreased 500/1000 Hz ratio of 0.87 (Figure 2); this shift in oVEMP frequency tuning has been shown to be indicative of ELH [2]. Audiometry merely showed a mild ipsilateral high-frequency hearing loss (Figure 1C). Otoacoustic emissions correlated well with the pure tone audiogram, with a loss of DPOAE above 4 kHz, indicating a cochlear rather than a retrocochlear damage (Figure 1D).

Citation: Jerin C, Krause E, Ertl-Wagner B and Gürkov R. Endolymphatic Hydropsin a Patient with a Small Vestibular Schwannoma Suggests a Peripheral Origin of Vertigo. Austin J Radiol. 2015; 2(6): 1033. ISSN :2473-0637