The Relationship between the Etiology of Profound Prelingual Sensorineural Hearing Loss and the Results of Vestibular-Evoked Myogenic Potentials

Abstract Introduction Cervical vestibular-evoked myogenic potentials (cVEMPs) are biphasic, short latency potentials, which represent the inhibition of the contraction of the sternocleidomastoid muscle (SCM) mediated by the saccule, the inferior vestibular nerve, the vestibular nuclei and the medial vestibular spinal tract. Objective To evaluate the response of cVEMPs in individuals with profound prelingual bilateral cochlear hearing loss. Methods A prospective case-control study. A total of 64 volunteers, divided into a study group (31 patients with profound prelingual sensorineural hearing loss) and a control group (33 subjectsmatched for age and gender with psychoacoustic thresholds of ≤ 25 dB HL between 500 and 8,000 Hz) were submitted to the cVEMP exam. The causes of hearing loss were grouped by etiology and the involved period. Results The subjects of the study group aremore likely to present changes in cVEMPs compared to the control group (35.5% versus 6.1% respectively; p = 0.003), with an odds ratio (OR) of 8.52 (p = 0.009). Itmeans that they had 8.52-fold higher propensity of presenting altered cVEMP results. There were no statistically significant differences between the latencies, the interamplitude and the asymmetry index. Regarding the etiology, there was a statistically significant difference when the cause was infectious, with an OR of 15.50 (p = 0.005), and when the impairment occurred in the prenatal period, with an OR of 9.86 (p = 0.009). Conclusion The present study showed abnormalities in the sacculocolic pathway in a considerable portion of individuals with profound prelingual sensorineural hearing loss due to infectious and congenital causes, as revealed by the cVEMP results. (AU)

Hipoacusia neurosensorial adquirida asociada a alteraciones vestibulares y / o control postural / Neurosensorial acquired hypoacusia associated with vestibular alterations and / or postural control

La otitis media aguda es una infección del oído medio con alta prevalencia en población pediátrica, las complicaciones pueden generar desde hipoacusia neurosensorial de diverso grado hasta alteración vestibular y/o control postural, aunque de ello no existen mayores reportes ni investigaciones en Chile. Por lo anterior, el objetivo fue asociar la hipoacusia neurosensorial a alteraciones vestibulares y/o de control postural. Se evaluó a un sujeto de sexo femenino, 13 años de edad, quien presentó múltiples cuadros de Otitis Media Aguda y fue diagnosticada con hipoacusia neurosensorial bilateral grado moderado. Antes del estudio, reportó desequilibrio y aumento de riesgo de caída. Se aplicaron test auditivos (timpanometría y audiometría), vestibulares (evaluación del VIII par craneal) y de control postural (posturógrafo y tests "Time up and go", Romberg y Romberg en tándem). Se encontraron alteradas la prueba de integración sensorial, con predominancia del hemicuerpo derecho, igualmente predominancia a alteraciones auditivas en el oído derecho ante pruebas que valoraron oído medio. Se observó una relación directa entre las alteraciones posturales y de equilibrio con el tipo y grado de pérdida auditiva que presenta el sujeto de estudio.

The acute otitis media is a middle ear infection with high prevalence in pediatric population, the complications could generate from sensorineural hearing loss to vestibular alteration and/or postural control, although, there aren´t report or researches of it in Chile. Therefore, the objective was to associate sensorineural hearing loss with vestibular alterations and/or postural control. We evaluated a female subject presenting multiple events of acute otitis media and she was diagnosed with sensorineural hearing loss middle grade. Before this study, she reported imbalance and falling risk. Hearing (tympanometry and audiometry), vestibular (evaluation of the VIII cranial nerve) and postural control tests were applied (posturography and "Time up and go", Romberg and Romberg in tandem test). It was found altered the integration sensorial test, with predominance to half body right and predominance of hearing impairment in the right ear to the middle ear evaluated evidence. It was observed a direct relation between postural alterations and balance with the hearing loss type from the subject of study.

Intraoperative Neurophysiological Monitoring during Microvascular Decompression Surgery for Hemifacial Spasm

Hemifacial spasm (HFS) is due to the vascular compression of the facial nerve at its root exit zone (REZ). Microvascular decompression (MVD) of the facial nerve near the REZ is an effective treatment for HFS. In MVD for HFS, intraoperative neurophysiological monitoring (INM) has two purposes. The first purpose is to prevent injury to neural structures such as the vestibulocochlear nerve and facial nerve during MVD surgery, which is possible through INM of brainstem auditory evoked potential and facial nerve electromyography (EMG). The second purpose is the unique feature of MVD for HFS, which is to assess and optimize the effectiveness of the vascular decompression. The purpose is achieved mainly through monitoring of abnormal facial nerve EMG that is called as lateral spread response (LSR) and is also partially possible through Z-L response, facial F-wave, and facial motor evoked potentials. Based on the information regarding INM mentioned above, MVD for HFS can be considered as a more safe and effective treatment.

Duplicated Internal Auditory Canal: High-Resolution CT and MRI Findings

OBJECTIVE: To summarize the high-resolution computed tomography (HRCT) and magnetic resonance imaging (HRMRI) features of duplicated internal auditory canals (DIACs). MATERIALS AND METHODS: Ear HRCT data of 64813 patients with sensorineural hearing loss (SNHL), obtained between August 2009 and November 2017, were reviewed. Among these patients, 12 (13 ears) were found to have DIACs, 9 of whom underwent HRMRI. Their images were evaluated by two otoradiologists. RESULTS: The rate of occurrence of DIAC among SNHL patients was 0.019% (12/64813). The internal auditory canals of 13 ears were divided into double canals by complete (n = 6) and incomplete (n = 7) bony septa, with varied orientations ranging from horizontal to approximately vertical. All of the anterosuperior canals extended into the facial nerve (FN) canal, except for 1, which also extended to the vestibule. The posteroinferior canals ended in the cochlea and vestibule, except for 2, which also connected to the FN canals. Magnetic resonance images revealed that 77.8% (7/9) and 22.2% (2/9) of vestibulocochlear nerves (VCNs) were aplastic and hypoplastic, respectively. Furthermore, 88.9% (8/9) of FNs were normal, except for 1, which was hypoplastic. All of the affected ears also had other ear anomalies: a narrow, bony cochlear nerve canal was the most common other anomaly, accounting for 92.3% (12/13). Malformations of other systems were not found. CONCLUSION: Double-canal appearance is a characteristic finding of DIAC on HRCT, and it is usually accompanied by other ear anomalies. The VCN usually appears aplastic, with a normal FN, on HRMRI.

Intraoperative Neurophysiological Monitoring during Microvascular Decompression Surgery for Hemifacial Spasm

Hemifacial spasm (HFS) is due to the vascular compression of the facial nerve at its root exit zone (REZ). Microvascular decompression (MVD) of the facial nerve near the REZ is an effective treatment for HFS. In MVD for HFS, intraoperative neurophysiological monitoring (INM) has two purposes. The first purpose is to prevent injury to neural structures such as the vestibulocochlear nerve and facial nerve during MVD surgery, which is possible through INM of brainstem auditory evoked potential and facial nerve electromyography (EMG). The second purpose is the unique feature of MVD for HFS, which is to assess and optimize the effectiveness of the vascular decompression. The purpose is achieved mainly through monitoring of abnormal facial nerve EMG that is called as lateral spread response (LSR) and is also partially possible through Z-L response, facial F-wave, and facial motor evoked potentials. Based on the information regarding INM mentioned above, MVD for HFS can be considered as a more safe and effective treatment.

Vértigo como presentación clínica de asa vascular de la arteria cerebelosa antero inferior / Vertigo as a clinical presentation of a vascular loop of the anterior inferior cerebellar artery

Resumen El vértigo es un frecuente motivo de consulta cuyo origen puede ser periférico o central; causas poco frecuentes de este último son las asas vasculares que afectan el VIII par craneal llevando a acúfenos, pérdida de la audición y mareo. Se presenta una mujer de 47 años de edad, quien consulta por un cuadro de mareo, náuseas, pérdida del tono postural, cefalea y parestesia facial, cuyo examen físico revela signos de vértigo periférico, iniciándose tratamiento con antieméticos, anticinetósicos y vasodilatadores que llevan a empeoramiento del cuadro, por lo que se sospecha de patología a nivel de sistema nervioso central (SNC) que ejerce compresión con la vasodilatación. Se realiza una resonancia magnética que muestra un asa vascular en la arteria cerebelosa inferior anterior (AICA) que ingresa al conducto auditivo interno (CAI) explicando la sintomatología y cuyo efecto compresivo de los nervios centrales genera un efecto paradójico con la toma de vasodilatadores. (Acta Med Colomb 2018; 43: 226-229).

Abstract Vertigo is a frequent reason for consultation whose origin can be peripheral or central; infrequent causes of the latter are the vascular loops that affect the VIII cranial nerve leading to tinnitus, hearing loss and dizziness. The case of a 47-year-old woman who consulted for a picture of dizziness, nausea, loss of postural tone, headache and facial paraesthesia, and whose physical examination revealed signs of peripheral vertigo, is presented. Treatment was started with antiemetics, anticinetics and vasodilators that lead to worsening of the condition, for which pathology exerting compression due to vasodilation at the central nervous system (CNS) level, is suspected. An MRI is performed showing a vascular loop in the Anterior Lower Cerebellar Artery (AICA) that enters the Internal Auditory Canal (IAC) explaining the symptomatology and whose compressive effect of the central nerves generates a paradoxical effect with the taking of vasodilators. (Acta Med Colomb 2018; 43: 226-229).

Abducens Nerve Palsy Associated with Ramsay-Hunt Syndrome / 대한평형의학회지

Ramsay-Hunt syndrome is an infectious disease caused by the varicella zoster virus. It is usually associated with facial and vestibulocochlear nerve palsy, but other cranial nerve dysfunction can be accompanied. We present a 68-year-old woman with abducens nerve palsy associated with Ramsay-Hunt syndrome. She showed abduction limitation of left eye with peripheral facial palsy and vestibulopathy of the left side. Varicella zoster virus polymerase chain reaction of cerebrospinal fluid was positive and internal auditory canal magnetic resonance imaging was revealed enhancement of labyrinthine segment of left facial nerve. Although abducens nerve palsy is uncommon feature of Ramsay-Hunt syndrome, but it can be developed by several different mechanisms.

Is the Auditory Brainstem Response Diagnostic for Vestibular Paroxysmia? / 대한평형의학회지

OBJECTIVES: Vestibular paroxysmia (VP) of the eighth cranial nerve is characterized by recurrent auditory and vestibular disturbances when a proximal part of the eighth cranial nerve is continuously pressed by a vessel. A detailed history and several ancillary diagnostic tools, such as tinnitogram, caloric test, auditory brainstem response (ABR) and magnetic resonance imaging, are used for diagnosis of VP. Among them, although Møller criteria using ABR is a simple method, the previous study is insufficient. Therefore, this study aimed to evaluate ABR's diagnostic value of VP. METHODS: ABR records of the 14 patients (patient group) who were diagnosed with VP and 45 patients (as control) who were diagnosed with only tinnitus were reviewed retrospectively. We analyzed the differences in Møller criteria between 2 groups. RESULTS: Mean age of the patient group was 52.9 years old and the control group was 55.4 years old. As compared with the control group, there were no significant differences of Møller 3 criteria contents (peak II wave amplitude < 33% [35.7% vs. 15.5%, p=0.133], interpeak latency I–III ≥2.3 msec [42.8% vs. 35.5%, p=0.622]), Contralateral interpeak latency III–V ≥2.2 msec (0% vs. 4.4%, p=1.000) in patient group. CONCLUSION: There was no significant difference of ABR parameters according to the Møller criteria between patient and control groups.

Cranial Polyneuropathy in Ramsay Hunt Syndrome Manifesting Severe Pharyngeal Dysphagia: a Case Report and Literature Review

Ramsay Hunt syndrome (RHS) is characterized by herpes zoster oticus, facial nerve palsy, and vestibulocochlear symptoms. Dysphagia caused by the involvement of multiple cranial nerves (CNs) in RHS is very rare. We encountered a rare case presented with severe dysphagia due to cranial polyneuropathy involving trigeminal nerve (CN V), facial nerve (CN VII), vestibulocochlear nerve (CN VIII), glossopharyngeal nerve (CN IX), vagus nerve (CN X), and hypoglossal nerve (CN XII) in RHS. This case report suggested that the prognosis for dysphagia in RHS seems favorable, and swallowing rehabilitation therapy could be beneficial in expediting tube removal.

Microvascular decompression for hemifacial spasm induced by vertebral artery dissecting aneurysm: one case report / 浙江大学学报·医学版

A 61-year-old female presented with 4 years history of left-sided hemifacial spasm. Head MRI and angiography indicated left vertebral artery dissecting aneurysm which compressed ipsilateral cranial nerves Ⅶ and Ⅷ. Microvascular decompression was performed. The dissecting aneurysm was pushed apart and the distal part of the parent artery was adhered to the dura on the petrosum. The compressed nerves were totally decompressed. The symptom of facial spasm was completely resolved immediately after surgery and did not recur during 6 months of follow up.

Vestibular Paroxysmia Mimicking Benign Parxysmal Positional Vertigo / 대한평형의학회지

Vestibular paroxysmia is the name given to the syndrome caused by vascular compression of the vestibulocochlear nerve. The main symptoms of vestibular paroxysmia are recurrent, spontaneous, brief attacks of spinning, non-spinning vertigo or positional vertigo that generally last less than one minute, with or without ear symptoms (tinnitus and hypoacusis). Prior to attributing a patient's symptoms to vestibular paroxysmia, however, clinicians must exclude common conditions like benign paroxysmal positional vertigo, Menière's disease, vestibular neuritis and vestibular migraine. This is usually possible with a thorough history and bedside vestibular/ocular motor examination. Herein, we describe a patient with vestibular paroxysmia that mimicked resolved BPPV with a literature review.

Microvascular Decompression for Tinnitus / 한양의대학술지

Tinnitus had been considered a surgically incurable disease, given the failure of several treatment methods. Jannetta reported that tinnitus is one of the hyperactive diseases of the cranial nerve along with hemifacial spasm and trigeminal neuralgia (TGN). Microvascular decompression (MVD) of the eighth cranial nerve was introduced to treat medically intractable tinnitus. Intraoperative monitoring of brainstem auditory evoked potential (BAEP) was able to reduce postoperative complication rates. Less than 1 ms of latency delay and a 40% decrease in amplitude of wave V of the brainstem evoked potential is a landmark of monitoring during surgery. Less than 6 years of duration of tinnitus, normal BAEP before surgery, no accompanying dizziness before surgery and a loop-type offending artery are effective factors for good surgical results of MVD.

Ruptured Total Intrameatal Anterior Inferior Cerebellar Artery Aneurysm

Among the distal anterior inferior cerebellar artery (AICA) aneurysms, a unique aneurysm at the meatal loop inside the internal auditory meatus is extremely rare. The authors report a case of surgically treated total intrameatal AICA aneurysm. A 62-year-old female patient presenting with sudden bursting headache and neck pain was transferred to our department. Computed tomography and digital subtraction angiography showed subarachnoid hemorrhage at the basal, prepontine cistern and an aneurysm of the distal anterior inferior cerebellar artery inside the internal auditory meatus. Surgery was performed by retrosigmoid craniotomy with unroofing of the internal auditory meatus. The aneurysm was identified between the seventh and eighth cranial nerve in the meatus and was removed from the canal and clipped with a small straight Sugita clip. After operation the patient experienced transient facial paresis and tinnitus but improved during follow up.

A Case of Recurrent and Multiple Schwannomas in the Caudal Septum

Schwannomas are benign neoplasms arising from the sheath of myelinated nerve fibers and may occur in any part of the body. They mostly occur in the head and neck region, accounting for about 25% to 45% of all cases. The eighth cranial nerve is the most common site of origin. About 4% of all head and neck schwannomas originate in the nasal cavity and paranasal sinuses. The best treatment of schwannomas is surgical excision. Since it is an encapsulated tumor, difficultly is rarely encountered in its complete removal, and recurrence is unlikely. We present a unique and rare case of a 71-year-old man with a recurrent septal mass, finally diagnosed as a schwannoma, with a review of the literature.

Neurovascular Compression Syndrome of the Eighth Cranial Nerve: Clinical Features and Medical Treatment / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Neurovascular compression syndrome of the eighth cranial nerve is characterized by recurrent auditory and vestibular symptoms. A detailed history and laboratory findings are important in the differential diagnosis of other diseases, such as Meniere's disease, vestibular neuritis, or vestibular migraine. This study reviewed its clinical features and the efficacy of medical treatment. SUBJECTS AND METHOD: The medical records of seven patients with a diagnosis of neurovascular compression syndrome of the eighth cranial nerve were reviewed retrospectively. RESULTS: Their ages at the time of disease onset ranged from 30 to 67 years. Six of the patients had unilateral typewriter tinnitus, like Morse code, and five had vertigo. The duration of these symptoms was up to 20 seconds. Three of the five patients with vertigo had canal paresis. All patients responded completely to carbamazepine or oxcarbazepine. CONCLUSION: Medical treatment was very successful for treating the symptoms of neurovascular compression syndrome of the eighth cranial nerve. The characteristics of the audiovestibular symptom and laboratory results are important for differentiating other diseases.

Acute Vestibular Neuritis Associated with Herpes Zoster Ophthalmicus / 대한평형의학회지

Vestibular neuritis, one of common causes of acute spontaneous vertigo, is characterized by a sudden onset of vertigo with horizontal-torsional spontaneous nystagmus and unsteadiness with a falling tendency. Herpes zoster is a common infection caused by varicella-zoster virus (VZV), and herpes zoster ophthalmicus (HZO) occurs when this virus is reactivated in the ophthalmic branch of the trigeminal nerve. VZV can cause vestibular neuritis with cochlear dysfunction as a form of herpes zoster oticus, also known as Ramsay-Hunt syndrome. However, to our knowledge, isolated vestibular neuritis associated with HZO has been rarely reported, because of distance between the trigeminal nerve and the vestibulocochlear nerve. We present an unusual case of vestibular neuritis complicated by the HZO.

Vestibular Paroxysmia in a 7-Year-Old Child / 대한평형의학회지

Neurovascular cross-compression of the eighth cranial nerve is characterized by brief attacks of vertigo, unilateral audiologic symptoms such as tinnitus, ear fullness and hearing disturbance and relatively rare disease, in particular, in children. We report a 7-year-old female patient who presented with recurrent spontaneous vertigo, lasting 15 seconds and occuring up to 40 times per day and often associated with physical activity. Her symptoms were developed by hyperventilation. Associated aural symptoms are not founded. Magnetic resonance image showed the eighth cranial nerve compression caused by the vascular loop. She was treated with oxcarbazepine and showed improving symptoms. Therefore we report our clinical experience with a brief review of literature.

Meningovascular and Spinal form of Neurosyphilis Presenting as Multiple Cranial Nerve Palsy, Cerebral Infarction and Meningomyelitis in a Human Immunodeficiency Virus Negative-Patient: MR Imaging Features

Neurosyphilis is a rare infection of the brain and spinal cord caused by a spirochete named Treponema pallidum. We describe the magnetic resonance imaging of a 53-year-old man with syphilis who manifested as both meningovascular, and spinal meningomyelitic types, which involved the optic, trigeminal, facial and vestibulocochlear nerves, both middle and left posterior cerebral arteries, thoracic spinal cord and meninges of the lumbar spine. This case report suggests that neurosyphilis should be considered as a possible diagnosis in patients showing complex brain and spinal imaging features. These features include enhancing meningeal lesions with multiple cranial nerve involvement, stenoses in large to medium size cerebral arteries, and intramedullary and meningeal lesions of spine.

Clinical Application of 3D-FIESTA Image in Patients with Unilateral Inner Ear Symptom

BACKGROUND AND OBJECTIVES: Unilateral auditory dysfunction such as tinnitus and hearing loss could be a warning sign of a retrocochlear lesion. Auditory brainstem response (ABR) and internal auditory canal magnetic resonance image (MRI) are suggested as novel diagnostic tools for retrocochlear lesions. However, the high cost of MRI and the low sensitivity of the ABR test could be an obstacle when assessing patients with unilateral ear symptoms. The purpose of this study was to introduce the clinical usefulness of three-dimensional fast imaging employing steady-state acquisition (3D-FIESTA) MRI in patients with unilateral ear symptoms. SUBJECTS AND METHODS: Two hundred and fifty-three patients with unilateral tinnitus or unilateral hearing loss who underwent 3D-FIESTA temporal bone MRI as a screening test were enrolled. We reviewed the abnormal findings in the 3D-FIESTA images and ear symptoms using the medical records. RESULTS: In patients with unilateral ear symptoms, 51.0% of the patients had tinnitus and 32.8% patients were assessed to have sudden sensory neural hearing loss. With 3D-FIESTA imaging, twelve patients were diagnosed with acoustic neuroma, four with enlarged vestibular aqueduct syndrome, and two with posterior inferior cerebellar artery aneurysm. Inner ear anomalies and vestibulocochlear nerve aplasia could be diagnosed with 3D-FIESTA imaging. CONCLUSIONS: 3D-FIESTA imaging is a highly sensitive method for the diagnosis of cochlear or retrocochlear lesions. 3D-FIESTA imaging is a useful screening tool for patients with unilateral ear symptoms.

Recurrent Vertigo in Vestibular Schwannoma Responsive to Oxcarbazepine / 대한평형의학회지

Vestibular schwannoma (VS) are benign neoplasms that arise from Schwann cells of the eighth cranial nerve. Although progressive unilateral hearing loss with dizziness or disequilibrium provides a high suspicion index of VS, vertigo is the symptom causing the most pronounced negative effect on quality of life in patients with VS. We report a 55-year-old woman with recurrent paroxysmal vertigo and hyperventilation-induced nystagmus due to VS, which improved by oxcarbazepine treatment. We suggest that episodic vertigo in VS may be ascribed to the ectopic paroxysmal neuronal discharge from the partially demyelinated vestibular nerve due to tumor compression.