Objectives Vestibular paroxysmia (VP) is characterized by brief episodes of vertigo due to neurovascular cross-compression (NVCC) of the eighth cranial nerve. This study aimed to analyze the clinical features of VP patients in a tertiary neurotologic clinic using the 2008 and 2016 diagnostic criteria and to compare these features.
Methods A retrospective review was conducted on patients diagnosed with definite or probable VP at the Asan Medical Center from May 2012 to May 2013. Patients underwent comprehensive evaluations including history taking, physical examination, audiometry, vestibular function tests, and magnetic resonance imaging (MRI). The 2008 and 2016 diagnostic criteria for VP were applied, and clinical characteristics were compared.
Results Nineteen patients were included (14 females and five males; mean age, 57.9±14.5 years). According to the 2008 criteria, 17 patients were diagnosed with definite VP and two with probable VP; however, using the 2016 criteria, nine were definite and two were probable VP. NVCC was observed in 88.9% of patients under the 2016 criteria. MRI revealed NVCC predominantly involving the anterior inferior cerebellar artery. Patients with cerebellopontine angle (CPA) tumors presenting with VP-like symptoms responded to medication.
Conclusions The 2016 diagnostic criteria for VP allow for a syndromic diagnosis based solely on clinical features. Neurovascular contact is commonly observed in VP patients, and CPA tumor can present VP-like symptoms and respond to carbamazepine. Although MRI is not included in the current criteria, it can be beneficial in diagnosis by identifying neurovascular contact and distinguishing CPA tumors.
Vertebrobasilar dolichoectasia (VBD) is a rare vascular condition characterized by the elongation, widening, and tortuosity of the vertebrobasilar artery. It can lead to various symptoms due to compression of cranial nerves and brainstem structures. We report a rare case of VBD presenting as sudden sensorineural hearing loss (SSNHL) with vertigo and spontaneous downbeat nystagmus in a 65-year-old woman with a history of hypertension. Magnetic resonance imaging revealed a tortuous and dilated right vertebrobasilar artery compressing the brainstem and left 8th cranial nerve root entry zone, without signs of acute cerebellar stroke. The compression of the anterior inferior cerebellar artery and cervicomedullary junction was also noted, suggesting a vascular cause for her symptoms. Following high-dose steroid treatment, the patient showed significant hearing improvement. This case underscores the importance of considering VBD in patients with unexplained SSNHL and vertigo, highlighting the role of detailed vascular imaging in the diagnosis and management of such cases.
A 54-year-old female patient presented with paroxysmal tinnitus and vertigo for 2 years, which have repeatedly occurred while rotating her neck, and lasted about 10 seconds. An anticonvulsant medication was prescribed with a diagnosis of microvascular compression syndrome on the 8th cranial nerve and audiovestibular evaluation and magnetic resonance imaging (MRI) scan were appointed a week later. In her next visit, she reported the disappearance of paroxysmal audiovestibular symptoms after medication. In the left ear, canal paresis and abnormal auditory brainstem response were observed. In MRI, a large meningioma in the cerebellopontine angle in the vicinity of the internal auditory canal orifice was detected, that was surgically resected by a neurosurgeon. After surgical removal of the tumor, she reported continuous dizziness due to vestibular nerve injury, but the paroxysmal attack of tinnitus and vertigo disappeared without anticonvulsant medication. This case suggests that an imaging study is mandatory when diagnosing microvascular compression syndrome on the 8th cranial nerve.
Sarcoidosis is a rare, multisystem granulomatous disease. Neurological complications occur in about 5% of patients and vestibulocochlear involvement is even rarer. A 27-year-old woman presented with acute spontaneous vertigo for 5 days. She was diagnosed with pulmonary sarcoidosis 4 months ago, but specific treatments have not yet started. She had preceding otologic symptoms including bilateral tinnitus and ear fullness in the right for 3 months without hearing loss. Initial bedside examinations revealed spontaneous right-beating nystagmus and abnormal catch-up saccades in the left during head impulse tests (HIT). After 2 weeks, video-oculography documented the direction of spontaneous nystagmus was changed into left-beating. Caloric test showed canal paresis in the left, and video HIT showed subtle covert saccades. After starting oral prednisolone, her symptoms improved rapidly. In our case, acute vestibular syndrome and otologic symptoms might be associated with sarcoidosis when considering clinical course and treatment response. Sarcoidosis may be considered as a cause in cases with audiovestibulopathy of unknown etiology.
Background and Objectives: Etiology of acute unilateral peripheral vestibulopathy
(AUPV) includes virus, ischemia, and autoimmune. As anatomical distribution is
similar between vasculature and innervation, AUPV with vascular risk factors
could be ischemic origin. We investigated the pattern of audiovestibular
dysfunction to explore the influence of risk factors on AUPV. Materials and Methods We collected records of 162 AUPV patients from 2011 to 2013 who
were admitted within 7 days from vertigo onset and diagnosed as AUPV by caloric
test and neuro-otologic examination. Vascular risk factors are stroke history,
hypertension, diabetes, body mass index >25, age >60, and vertebrobasilar
stenosis. Bedside examination includes spontaneous nystagmus grade, head
impulse test, head shaking test. Results of rotatory chair test (n=125), caloric test
(n=162), cervical (n=33) and ocular (n=23) vestibular evoked myogenic potential
(VEMP), subjective visual vertical (SVV) (n=91), and pure tone audiometry (PTA)
(n=62) are collected. Results: Abnormalities of PTA are found more in patients
with vascular risk factor than without any risk factor. Specifically, hypertension
(p=0.008) and old age (p=0.025) are associated with PTA abnormality (p=0.006).
Tilt angle of vertical is larger in risk factor group (p=0.019). The number of
vascular risk factor correlates with abnormalities of PTA (p=0.025) and tilt angle
of SVV. Results of bedside examination, rotatory chair test, caloric test, cervical
and ocular VEMP are not associated with vascular risk factors. Conclusion: AUPV
patients with vascular risk factors have more extensive involvement of
audiovestibular function. Ischemic etiology may contribute to pathogenesis of
extensive AUPV.
A case is reported of downbeat nystagmus associated with compression of the root of vestibulocochlear nerve by
vertebral arteries, which was revealed by magnetic resonance imaging. Chief complain of the patient was positional
vertigo, which aggrevated at left decubitus position. Downbeat nystagmus was increased in left Dix-hall pike test.
Tetsuo et al, reported downbeat nystagmus with compression of dolichoectatic vertebral arteries to the medulla
oblongata and surgical neurovascular decompression of the dolichoectasia reverses the progression of symptoms if
permanent neurologic damage has not already occurred.
Key Words : Vertical nystagmus, Vertebrobasilar dolichoectasia, Vestibulocochlear nerve