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 (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.
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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
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.
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.
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.
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.
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
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