Research in Vestibular Science

Review Article

Persistent geotropic direction-changing positional nystagmus: a systematic review: Dong-Han Lee, Chang-Hee Kim; Res Vestib Sci. 2025;24(3):153-176. Published online September 15, 2025; DOI: https://doi.org/10.21790/rvs.2025.002

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Abstract PDF: Persistent geotropic direction-changing positional nystagmus (DCPN) is a unique clinical phenomenon characterized by persistent nystagmus observed during the supine head roll test. Unlike other DCPNs caused by canalolithiasis or cupulolithiasis, this persistent geotropic form is not fully explained by conventional mechanisms. The “light cupula” hypothesis, introduced in the early 2000s, proposes that reduced cupula density relative to the surrounding endolymph is a key explanation for cases of peripheral origin. However, other mechanisms, such as heavier endolymph, light debris, utricular dysfunction, and perilymph-endolymph density difference have also been suggested. Persistent geotropic DCPN may also arise from central lesions, further complicating its diagnosis. This review examines persistent geotropic DCPN with a focus on the peripheral hypothesis, particularly the concept of the light cupula, exploring its origin, clinical characteristics, diagnostic approaches, proposed alternative mechanisms, and treatment. This review also explores cases of persistent geotropic DCPN linked to central lesions and coexisting conditions like vestibular migraine and Ménière disease, focusing on their distinctive characteristics as reported in the literature. The limited effectiveness of available treatments highlights the importance of advancing research to better understand and manage this condition.

Case Reports

Positional nystagmus in pneumolabyrinth: a case report: Dong-Han Lee, Do-Won Kwon, Minho Jang, Jung Eun Shin, Chang-Hee Kim; Res Vestib Sci. 2025;24(2):107-112. Published online June 15, 2025; DOI: https://doi.org/10.21790/rvs.2025.011

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Abstract PDF Supplementary Material: This report describes a patient with left-sided pneumolabyrinth following a temporal bone fracture and demonstrates characteristic positional nystagmus observed in this condition while discussing its mechanism. A previously healthy 38-year-old man presented with left-sided hearing loss, tinnitus, and positional vertigo after blunt head trauma. Temporal bone computed tomography revealed pneumolabyrinth in the ampulla of the left lateral semicircular canal (LSCC). Pure tone audiometry showed mixed hearing loss on the left (bone, 13.8 dB; air, 42.5 dB). Video Frenzel examination revealed direction-changing positional nystagmus: persistent left-beating nystagmus in the bow position, right-beating nystagmus upon moving from bowing to leaning, and persistent left-beating nystagmus in the left head-roll position only. We propose that in pneumolabyrinth involving the LSCC, changes in head position shift the air bubble, generating a buoyant force that indents the endolymphatic membrane and deflects the cupula, producing positional nystagmus. The direction of nystagmus may vary depending on the bubble’s location.

Fluctuating high-frequency hearing loss with vertigo: is it Menière’s disease? A case report: Minho Jang, Dong-Han Lee, Jiyeon Lee, Chang-Hee Kim; Res Vestib Sci. 2024;23(3):106-110. Published online September 15, 2024; DOI: https://doi.org/10.21790/rvs.2024.011

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Abstract PDF Supplementary Material: 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.

Rotatory Vertebral Artery Syndrome in Foramen Magnum Stenosis: Ileok Jung, Jin-Man Jung, Moon Ho Park; Res Vestib Sci. 2018;17(4):167-169. Published online December 21, 2018; DOI: https://doi.org/10.21790/rvs.2018.17.4.167

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1 Crossref

Abstract PDF

Rotatory vertebral artery syndrome (RVAS) is characterized by recurrent attacks of vertigo, nystagmus, and syncope induced by compression of the vertebral artery during head rotation. A 60-year-old man with atlas vertebrae fracture presented recurrent attacks of positional vertigo. Left-beat, upbeat and count clock-wise torsional nystagmus occurred after lying down and bilateral head roll (HR) showing no latency or fatigue. Magnetic resonance imaging revealed foramen magnum stenosis (FMS) and dominancy of right vertebral artery (VA). The flow of the right VA on transcranial Doppler decreased significantly during left HR. The slower the velocity was, the more the nystagmus was aggravated. RVAS can be evoked by FMS causing compression of the VA. And the nystagmus might be aggravated according to the blood flow insufficiency.

Citations

Citations to this article as recorded by

A Case of Rotational Vertebral Artery Syndrome after Vertebral Artery Dissection
Song Jae Lee, Ha Young Byun, Seung Hwan Lee, Jae Ho Chung
Korean Journal of Otorhinolaryngology-Head and Nec.2020; 63(5): 228. CrossRef

Symposium III

Central Positional Vertigo: Dong Uk Kim; Res Vestib Sci. 2013;12:S85-S88. Published online June 1, 2013

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Abstract PDF: Positional vertigo and positional nystagmus are usually due to peripheral vestibular disorders; there is no doubt that the most common etiology is benign paroxysmal positional vertigo. However, it should be cautious that the differential diagnosis for patients presenting with a findings of positional vertigo or positional nystagmus. In this review, we discuss uncommon causes of positional vertigo or positional nystagmus that result from lesion or dysfunction of central vestibular system, not peripheral vestibular structures.

Original Article

Clinical Characteristics of Horizontal Canal Benign Paroxysmal Positional Vertigo with Persistent Geotropic Direction Changing Positional Nystagmus: Kyung Min Ko, Mee Hyun Song, Jin Woo Park, Joon Hee Lee, Yong Gook Shin, Dae Bo Shim; Res Vestib Sci. 2015;14(4):117-122.

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Abstract PDF: Objective: The purpose of this study was to identify the clinical characteristics of horizontal canal benign paroxysmal positional vertigo (h-BPPV) with persistent geotropic direction changing positional nystagmus (DCPN).
Methods
One hundred thirty two patients diagnosed as the geotropic subtype of h-BPPV were analyzed retrospectively. Patients were classified into two groups: persistent h-BPPV (ph-BPPV) group which means h-BPPV showing persistent (＞1 minute) geotropic DCPN and short duration h-BPPV (sh-BPPV) group that means h-BPPV with short duration (≤1 minute) geotropic DCPN. We compared the clinical characteristics and treatment outcomes between the two groups.
Results
The study included 34 patients with ph-BPPV and 98 patients with sh-BPPV. There were no differences between the two groups in age, distribution of sex and the affected side. The ph-BPPV group had higher secondary BPPV preponderance and dizziness handicap index (DHI) score compared to the sh-BPPV group. The ph-BPPV group required higher number of canalith repositioning procedures (CRPs) until resolution and higher multiple/single CRP ratio than the sh-BPPV group. In addition, the ph-BPPV group showed longer duration until the remission of subjective symptoms (vertigo, dizziness) compared to the sh-BPPV group.
Conclusion
ph-BPPV was more frequently associated with secondary causes of BPPV and demonstrated higher DHI score, total number of CRP, and longer remission duration of subjective symptoms compared to sh-BPPV. This information may be helpful for clinicians in counseling and managing the patients with persistent geotropic DCPN h-BPPV.

Case Reports

Apogeotropic Positional Nystagmus in Pontine Infarction: Hye Ran Son, Jae Yun Jung, Myung Whan Suh; Res Vestib Sci. 2012;11(3):105-109.

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Abstract PDF: It is thought that horizontal canal benign paroxysmal positional vertigo (BPPV) is the most common cause of apogeotropic direction-changing positional nystagmus (DCPN). But there are many reports about cerebellar or brainstem lesions as the cause of apogeotropic DCPN. We also report a 72-year-old male patient who showed apogeotropic DCPN, but was proven to have a pontine infarction. The patients complained of disequilibrium which has lasted for 3-4 years and aggravated recently. The symptom was present only when he stood up, and was absent as soon as he sat down. He was not able to successfully perform the Romberg test and tandem gait on physical examination. Vestibular function test revealed apogeotropic DCPN without spontaneous nystagmus. Rotation chair test and caloric test results were all within normal limit. On the brain magnetic resonance imaging, newly detected infarction in the left basal ganglia, pons and right parietal lobe was found. Although horizontal canal BPPV is the most common cause of apogeotropic DCPN, we should be aware that there can be patients with central origin DCPN. In this report, we present the detailed history of this patient and tried to point out the clues to suspect central lesion in patients with apogeotropic DCPN.

A Case of Atypical Benign Paroxismal Positional Vertigo: Beom Gyu Kim, Jai Hyuk Chang, Il Seok Park, Yong Bok Kim; J Korean Bal Soc. 2004;3(2):428-430.

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Abstract PDF: Paroxysmal positional nystagmus is a common finding in patients with vertigo and can occur in typical and atypical forms.1) Atypical forms of paroxismal positional nystagmus are thought to represent conditions which are in fact not “benign”. This patient was diagnosed as right posterior semicircular canal BPPV at first. After modified Epley maneuver, the type of nystagmus was changed to atypical forms. After left cupulolith reposition maneuver (CRmM), the nystagmus and dizziness were disappeared finally.

Traumatic Perilymphatic Fistula Presenting with Direction-Changing Positional Nystagmus: Ja Won Koo, Si Whan Kim, Ji Soo Kim, Sung Wha Hong; J Korean Bal Soc. 2004;3(1):173-176.

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Abstract PDF: Diagnosis of perilymphatic fistula (PLF) is considered in the patient presenting hearing loss associated with ataxia after penetrating injury of the tympanic membrane. PLF accompanies mixed type hearing loss and paralytic nystagmus. If audiovestibular symptoms and signs are not definite for those patients, in whom PLF is highly suspicious, they can be induced by affected ear down position. The direction of nystagmus induced by position change was reported either toward or away from the affected ear. But the direction changing nature has not been noted in the previous literature. We report on a case of traumatic PLF presented with direction changing positional nystagmus and discuss the possible mechanism involved in this case. Key Words : Positional nystagmus, Perilymphatic fistula

Review

Central Positional Nystagmus from Focal Brain Lesion: Ja Won Koo, Kwang Dong Choi, So Young Moon, Seong Ho Park, Ji Soo Kim; J Korean Bal Soc. 2004;3(1):129-135.

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Abstract PDF: Department of Otolaryngology, Head and Neck Surgery1, and Neurology2, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea Background and Objectives : Central positional nystagmus is induced by positional changes from brainstem or cerebellar lesions. Differentiation central from peripheral positional nystagmus is important in clinical practice. To delineate characteristics of the positional nystagmus observed in central lesions, we analyzed the parameters of positional nystagmus from focal brain lesions. Materials and Methods : Ten patients with central positional nystagmus were recruited from the dizziness clinic of Seoul National University Bundang Hospital. All the patients had focal brainstem or cerebellar lesions documented by magnetic resonance imaging. The nystagmus was observed with and without fixation by using Frenzel glasses or Video Goggles. The nystagmus was videotaped or recorded with video-oculography. Provoking positional maneuvers, direction, latency, duration, phase reversal, and fatigue phenomenon of the nystagmus were analyzed.
Results
: Of the 10 patients, seven had infarctions in the lateral medulla or inferior cerebellum while two experienced cerebellar hemorrhage and remaining one showed a compression of the ventrolateral medulla by cavernous malformation of the vertebral artery. The directions of the positional nystagmus were variable depending on the lesions and provoking maneuvers. Most patients exhibited direction-changing nystagmus without latency, direction-reversal and fatigue phenomenon. However, some of the patients also showed patterns of nystagmus characteristic of peripheral positional nystagmus. In two of the four patients with infarction in the territory of medial posterior inferior cerebellar artery, the positional nystagmus was the only abnormal findings.
Conclusions
: Central positional nystagmus may share many characteristics with peripheral type of positional nystagmus. In individual cases, the patterns of nystagmus should be interpreted with caution in differentiating central from peripheral positional nystagmus. Considering the isolated positional nystagmus in some patients with caudal cerebellar lesions, systematic positional maneuvers should be applied to all the patients with vertigo Key Words : Positional nystagmus, Medulla, Cerebellum

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