Fluctuating high-frequency hearing loss with vertigo: is it Menière’s disease? A case report
Article information
Abstract
This study describes an unusual case of fluctuating unilateral high-frequency hearing loss with vertigo resembling Menière’s disease. The current diagnostic criteria for definite Menière’s disease include audiometrically documented low- to medium-frequency sensorineural hearing loss on at least one occasion before, during, or after an episode of vertigo. This case presented a diagnostic dilemma. Brain MRI was nonspecific, and a bithermal caloric test showed unilateral weakness of 44% on the affected side. The results of electrocochleography and cervical vestibular evoked myogenic potential tests were within the normal ranges. Persistent geotropic or ageotropic positional nystagmus was observed during each vertigo attack; the mechanism underlying this characteristic nystagmus needs further investigation.
INTRODUCTION
Menière’s disease (MD) is characterized by recurrent attacks of vertigo with fluctuating sensorineural hearing loss. Prosper Menière first described a patient with vertigo, balance problems, and hearing loss due to an inner ear disorder in 1861. The diagnostic criteria for definite MD include (1) two or more spontaneous episodes of vertigo each lasting 20 minutes to 12 hours; (2) audiometrically documented low- to medium-frequency sensorineural hearing loss in one ear, defining the affected ear, on at least one occasion before, during, or after one of the episodes of vertigo; (3) fluctuating aural symptoms (hearing loss, tinnitus, or ear fullness); and (4) not better accounted for by another vestibular diagnosis [1,2]. Low-frequency sensorineural hearing loss is defined as a bone-conduction threshold in pure tone audiometry that is greater in the affected ear than in the contralateral ear by at least 30 dB hearing loss or more at two contiguous frequencies below 2,000 Hz. In MD, as the disease progresses, hearing loss can involve middle and high frequencies after several episodes of vertigo, leading to pan-tonal hearing loss. The current diagnostic criteria rule out definite MD unless documented hearing loss involves the low-frequency range, regardless of high-frequency involvement. However, in patients with recurrent isolated high-frequency hearing loss with episodic vertigo, a diagnosis of MD may be suspected. This report presents a case of fluctuating isolated high-frequency hearing loss with vertigo and describes detailed characteristics of audiometry and positional nystagmus.
CASE REPORT
This study was approved by the Institutional Review Board (IRB) of Konkuk University Medical Center (No. 2021-03-073). Informed consent from the patient was waived by the IRB.
A 33-year-old woman with severe vertigo lasting for hours visited our outpatient clinic. Nausea and vomiting accompanied the vertigo, with no neurological deficits observed. The patient did not complain of hearing loss, aural fullness, or tinnitus in either ear. Although headache did not occur during this attack, the patient had been diagnosed with migraine several years prior and had been on intermittent medication since then. Her mother and sister also had migraines. The patient denied any past medical history of other neurological disorders, otologic diseases, or previous ear surgery. She reported being admitted to another hospital with acute vertigo, left-sided hearing loss, and high-pitched tinnitus 14 months prior. At that time, pure tone audiometry revealed left-sided sensorineural hearing loss in the high-frequency range (Fig. 1A, upper panel). The patient was diagnosed with sudden hearing loss with vertigo, and treatment including systemic corticosteroids was administered, resulting in improvements in hearing loss (Fig. 1A, lower panel) and vertigo symptoms.
Serial demonstration of pure tone audiometry. (A) At the first episode of vertigo attack, middle- to high-frequency hearing loss was shown in the left ear (upper panel), which improved after the treatment (lower panel). (B and C) High-frequency hearing loss was shown in the left ear at the second (B) and third (C) episode of vertigo attack (upper panel), which recovered after the treatment (lower panel).
Neurological examinations, including a cerebellar function test, revealed no abnormalities. A video Frenzel examination demonstrated right-beating spontaneous nystagmus, and a supine head-roll test showed persistent ageotropic positional nystagmus with greater intensity on the left head roll (Supplementary Video 1). The patient was diagnosed with right lateral semicircular canal cupulolithiasis, and a cupulolith repositioning maneuver was performed. Two days later, the patient returned to our clinic complaining of sudden-onset high-pitched tinnitus in the left ear with aggravated vertigo symptoms lasting for 4 hours. No focal neurological deficits were noted. The patient exhibited vigorous left-beating spontaneous nystagmus, and persistent geotropic positional nystagmus with a torsional component was observed during the supine head-roll test (Supplementary Video 2). Pure tone audiometry revealed a high-frequency hearing loss in the left ear (Fig. 1B, upper panel), with a speech discrimination score of 100%. A supine head-roll test conducted 2 hours after the last test elicited persistent ageotropic positional nystagmus (Supplementary Video 3). Temporal bone MRI was nonspecific (Fig. 2A, B), and a bithermal caloric test showed unilateral weakness of 44% on the left side (Fig. 2C). Auditory brainstem response revealed that thresholds were 5 dB higher on the left than on the right side without difference in latency (Fig. 2D). The results of electrocochleography and cervical vestibular evoked myogenic potential (cVEMP) tests were within the normal ranges. Treatment including systemic corticosteroids and conservative management for vertigo symptoms was started, and vertigo symptoms and hearing loss improved within a few days (Fig. 1B, lower panel).
Axial views of three-dimensional FIESTA (A) and post-contrast T1-weighted (B) images of temporal bone magnetic resonance imaging showed no abnormal findings. (C) Bithermal caloric test showed a canal paresis of 44% on the left side. (D) Auditory brainstem response thresholds were 5 dB higher on the left than on the right side without a difference in latency.
Nineteen months after her initial visit to our hospital, the patient visited our clinic again with the same symptoms of acute vertigo with high-frequency hearing loss and tinnitus in the left ear (Fig. 1C, upper panel). A video Frenzel glasses examination revealed right-beating spontaneous nystagmus and persistent geotropic positional nystagmus during the supine head-roll test (Supplementary Video 4). After treatment with systemic corticosteroids, her hearing improved (Fig. 1C, lower panel).
DISCUSSION
The diagnostic guidelines for MD were first published in 1972 [3] and have been revised over time. The most recent diagnostic criteria suggest that although hearing loss may involve the high-frequency range in the late stage of MD, low- to medium-frequency sensorineural hearing loss is required for diagnosis [1,2]. Thus, according to the current diagnostic criteria, fluctuating high-frequency hearing loss with episodic vertigo cannot be diagnosed as definite MD even though there is a high congruence between changes in low- and high-frequency thresholds in MD, suggesting that a pathologic change occurs in the whole cochlea, not only in the apical turn [4]. Martin-Sanz et al. [5] reported 20 patients with ‘Menière-like’ syndrome characterized by episodic vertigo and isolated high-frequency sensorineural hearing loss, tentatively termed ‘high-frequency sensorineural hearing loss with vestibular episodic syndrome (HIVES)’. They observed no significant differences in the affected side, symptom duration, age, or sex between patients with HIVES and patients with definite MD. There were also no significant differences in the cVEMP or electrocochleography results [5]. Our patient had comorbid migraine and a family history of migraine. Strong associations between migraine and tinnitus and between migraine and hearing loss have been reported [6]. Additionally, a high incidence of coexisting MD and vestibular migraine has been reported [7]. However, the associations between migraine and fluctuating high-frequency hearing loss with episodic vertigo have not yet been reported.
Our patient presented with characteristic positional nystagmus during the attacks of vertigo, which has not been reported in patients with HIVES. Persistent ageotropic positional nystagmus was observed at the first visit to our hospital (Supplementary Video 1), and at the second visit, persistent geotropic nystagmus (Supplementary Video 2) was changed to vigorous ageotropic nystagmus (Supplementary Video 3). At the third visit, persistent geotropic positional nystagmus was observed (Supplementary Video 4). This direction-changing positional nystagmus has been observed in varying neurotologic disorders such as vestibular migraine [8], MD [9], sudden hearing loss with vertigo [10], Ramsay Hunt syndrome [11], and acute otitis media [12], and in patients without evidence of other specific neurotologic disorders [13,14]. Based on the patient’s past medical history of migraine, direction-changing positional nystagmus may be caused by vestibular migraine. The mechanism underlying direction-changing positional nystagmus requires further study.
Genetic conditions such as mutation of the COCH gene may cause a Menière-like syndrome with high-frequency hearing loss [15]. The vestibular phenotypes of patients with COCH gene mutations may include a wide spectrum ranging from bilateral total vestibular loss without episodic vertigo to MD-like features with devastating episodic vertigo [15].
In conclusion, we report a rare case of fluctuating high-frequency hearing loss with vertigo, mimicking the clinical manifestations of MD. Persistent geotropic or ageotropic positional nystagmus was observed during each episode of vertigo attack; the mechanism underlying this characteristic nystagmus needs further investigation.
Notes
Funding/Support
None.
Conflicts of Interest
No potential conflict of interest relevant to this article was reported.
Availability of Data and Materials
All data generated or analyzed during this study are included in this published article. For other data, these may be requested through the corresponding author.
Authors' Contributions
Conceptualization, Visualization: MJ, CHK; Data curation, Formal analysis, Methodology: MJ, DHL, JL; Investigation: All authors; Project administration, Supervision, Validation: CHK; Writing–Original Draft: MJ, DHL, JL; Writing–Review & Editing: CHK.
All authors read and approved the final manuscript.
Acknowledgments
The authors thank Professor Jose Antonio Lopez-Escamez for his helpful comments on our study.
SUPPLEMENTARY MATERIALS
Supplementary materials can be found via https://doi.org/10.21790/rvs.2024.011.
Supplementary Video 1.
On the first visit to our hospital, the patient complained of vertigo without hearing loss or tinnitus. A supine head-roll test elicited persistent ageotropic positional nystagmus.
Supplementary Video 2.
Two days after the first visit to our hospital, the patient experienced aggravation of vertigo and hearing loss with high-pitched tinnitus on the left side. A supine head-roll test elicited persistent geotropic positional nystagmus with a torsional component.
Supplementary Video 3.
Two hours after a video Frenzel test shown in Supplementary Video 2, a supine head-roll test elicited persistent ageotropic positional nystagmus.
Supplementary Video 4.
At the third episode of acute vertigo 19 months later, a supine head-roll test elicited persistent geotropic positional nystagmus.