Paraneoplastic cochleovestibulopathy: clinical presentations, oncological and serological associations.

OBJECTIVE: Cochleovestibulopathy is a distinguishable paraneoplastic phenotype. In this study, we evaluate clinical presentation, serological/cancer associations and outcomes of paraneoplastic cochleovestibulopathy. METHODS: Retrospective chart review of patients with hearing impairment and/or vestibulopathy who underwent serological evaluations for paraneoplastic antibodies between January 2007 and February 2021 was performed. RESULTS: Twenty-six patients were identified (men, n=23; median age, 45 years, range: 28-70). Biomarkers detected included: KLHL11-IgG| |(n=20,| |77% (coexisting LUZP4-IgG, n=8)),| ||ANNA1-IgG| | |(n=3,| |12%),| |amphiphysin-IgG|| |(n=2,| |8%)| |and| |LUZP4-IgG|| |(n=1,| |4%). Most common neoplastic association was |testicular|/|extra-testicular| |seminoma| | (n=13,| |50%).|| Hearing| impairment (bilateral, 62%) was |present| |in| |all| |patients.| |Fifteen patients (58%) had cochleovestibular dysfunction as their initial presentation before rhombencephalitis/encephalomyelitis manifestations (hearing loss, four; acute vertigo, eight; both, three). |Brain| |MRI| |demonstrated| |internal| |auditory| |canal| |enhancement| |in| |four |patients.| Audiometry commonly revealed severe-profound bilateral sensorineural hearing loss. Most patients |had| a refractory course |despite| |immunotherapy| |and/or| |cancer| |treatment|. CONCLUSION: Cochleovestibulopathy commonly presents with rapidly progressive bilateral hearing loss and/or acute vertigo. However, in some patients, these symptoms present along with or following brainstem/cerebellar manifestations. KLHL11-IgG and seminoma are the most common serological and cancer associations, respectively. Recognition of this phenotype may aid in earlier diagnosis of paraneoplastic autoimmunity and associated cancer.

Internal auditory canal hypoplasia associated with bilateral vestibulocochlear nerve aplasia and deviant facial nerve course: A case report and MRI findings.

The evaluation of internal auditory canals and cochlea has gained significant importance due to the increasing number of cochlear implantations worldwide. This region's anatomical study is essential for cochlear implant surgery using magnetic resonance imaging as the method of choice. We report a case of a 6-year-old male patient diagnosed with a rare bilateral malformation of the internal auditory canals associated with an aberrant course of the facial nerve and vestibulocochlear nerve aplasia. This report raises the importance of identifying this rare malformation for appropriate management and reinforces awareness of possible complications.

Protective effect of lutein against acrolein-induced ototoxicity in rats.

BACKGROUND: Acrolein is a reactive aldehyde that forms during burning of wood and other fuels. It is also a product of lipid peroxidation (LPO) reactions and is present in cigarette smoke. Acrolein is known to cause oxidative stress and inflammatory nerve tissue damage. Lutein is a tetraterpenoid molecule with antioxidant and anti-inflammatory properties. There appear to be no studies on the effect of lutein on vestibulocochlear nerve damage induced by acrolein. The aim of this study was to investigate the effect of lutein on vestibulocochlear nerve damage induced by acrolein in rats using biochemical and histopathological methods. METHODS: The rats were divided into three groups (n = 6, for each group) a healthy control group (HG), an acrolein (ACR) group and a lutein and acrolein (LACR) group. In the LACR group, lutein was administered (1 mg/kg) via oral gavage. The ACR and HG groups received saline via oral gavage. Then, 1 h after the administration of lutein and saline, the LACR and ACR groups were treated with 3 mg/kg of acrolein via oral gavage. This procedure was repeated once a day for 30 days. RESULTS: The results of biochemical experiments showed that in the vestibulocochlear nerve tissues of the animals treated with acrolein, the levels of malondialdehyde, total oxidants, nuclear factor kappa b, tumor necrosis factor alpha and interleukin 1 beta significantly increased, whereas the levels of total glutathione and total antioxidants decreased as compared to those in the HG and LACR groups. In addition, severe histopathological damage was observed in vestibulocochlear nerve tissue of the acrolein group, whereas this damage was alleviated in the lutein group. CONCLUSION: Lutein protected vestibulocochlear nerve tissue from acrolein-associated oxidative and proinflammatory damage. This suggests that lutein might be useful in preventing or treating acrolein-induced ototoxicity.

Cervical and ocular vestibular evoked myogenic potentials in determining nerve division involvement in patients with a tumor located in the internal auditory canal.

OBJECTIVES: The study aimed at the analysis of the parameters of acoustic cervical and ocular vestibular evoked myogenic potentials (AC-cVEMP and AC-oVEMP) response in patients with a confirmed tumor located in the internal auditory canal. It also aimed to assess to what degree a combination of these tests may be of benefit in the preoperative indication of the affected nerve division via preoperative determination whether the tumor originated from the superior or inferior division of the vestibular nerve, both divisions, or if it originated from a different nerve in the internal auditory canal. METHODS: The study group included 50 patients. Preoperative MRI scans were used to measure tumor diameter. AC-cVEMP and AC-oVEMP testing were performed before tumor resection. The surgeon was asked for a detailed description of the tumor origin. RESULTS: The corrected amplitude of cVEMP was significantly lower on the tumor side than on the non-affected side and in the control group. The corrected Asymmetry Ratio (AR) of cVEMPs in patients with the tumor was significantly elevated above the reference values with the mean being 58.29% and the mean AR of oVEMPs in patients the tumor was 71.78% which made both results significantly higher than in the control group. Neither cVEMP nor oVEMP latency was significantly correlated with tumor size. Data obtained from cVEMP and oVEMP tests was an effective indicator of tumor origin in 74% of patients showing which division (or both divisions) of the VIIIth nerve was affected in comparison with information obtained from the surgeon. CONCLUSIONS: The combined use of AC-cVEMP and AC-oVEMP tests may be useful in surgical planning in patients the tumor located in the internal auditory canal, providing a highly probable determination of the division of the affected nerve. Such information is valuable for the surgeon as it offers additional knowledge about the tumor before the procedure. cVEMP and oVEMP results may not be used as the basis for the calculation of tumor size in patients.

Cranial Nerve Disorders Associated With Immune Checkpoint Inhibitors.

OBJECTIVE: To describe the spectrum, treatment, and outcome of cranial nerve disorders associated with immune checkpoint inhibitor (Cn-ICI). METHODS: This nationwide retrospective cohort study on Cn-ICI (2015-2019) was conducted using the database of the French Refence Center. In addition, a systematic review of the literature (MEDLINE, Scopus, and Web of Science) for records published between 2010 and 2019 was performed following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines using the search terms cranial nerve or neuropathy or palsy and immune checkpoint inhibitors. RESULTS: Among 67 cases with ICI-related neurologic toxicities diagnosed in our reference center, 9 patients with Cn-ICI were identified (7 men, 78%, median age 62 years [range 26-82 years]). Patients were receiving a combination of anti-cytotoxic T-lymphocyte antigen 4 and anti-programmed cell death 1 (PD-1)/PD-1 ligand (n = 5, 56%) or anti-PD-1 antibodies alone (n = 4, 44%). Cn-ICI involved optic (n = 3), vestibulocochlear (n = 3), abducens (n = 2), facial (n = 2), and oculomotor (n = 1) nerves. Two patients had involvement of 2 different cranial nerves. Treatment comprised corticosteroids (n = 8, 89%), ICI permanent discontinuation (n = 7, 78%), plasma exchange (n = 2, 22%), and IV immunoglobulin (n = 1, 11%). Median follow-up was 11 months (range 1-41 months). In 3 cases (33%), neurologic deficit persisted/worsened despite treatment: 2 optic and 1 vestibulocochlear. Among cases from the literature and the present series combined (n = 39), the most commonly affected cranial nerves were facial (n = 13, 33%), vestibulocochlear (n = 8, 21%), optic (n = 7, 18%), and abducens (n = 4, 10%). Trigeminal, oculomotor, and glossopharyngeal nerves were less frequently affected (total n = 7). CONCLUSION: Cranial nerve disorders can complicate treatment with ICIs. Approximately one-third of the patients had persisting deficits, most frequently involving hearing and vision loss.

Vestibular mapping in patients with unilateral peripheral-vestibular deficits.

OBJECTIVE: To test the hypothesis that patterns of semicircular canal (SCC) and otolith impairment in unilateral vestibular loss depend on the underlying disorders, we analyzed peripheral-vestibular function of all 5 vestibular sensors. METHODS: For this retrospective case series, we screened the hospital video-head-impulse test database (n = 4,983) for patients with unilaterally impaired SCC function who also received ocular vestibular-evoked myogenic potentials and cervical vestibular-evoked myogenic potentials (n = 302). Frequency of impairment of vestibular end organs (horizontal/anterior/posterior SCC, utriculus/sacculus) was analyzed with hierarchical cluster analysis and correlated with the underlying etiology. RESULTS: Acute vestibular neuropathy (AVN) (37.4%, 113 of 302), vestibular schwannoma (18.2%, 55 of 302), and acute cochleovestibular neuropathy (6.6%, 20 of 302) were most frequent. Horizontal SCC impairment (87.4%, 264 of 302) was more frequent (p < 0.001) than posterior (47.4%, 143 of 302) and anterior (37.8%, 114 of 302) SCC impairment. Utricular damage (58%, 175 of 302) was noted more often (p = 0.003) than saccular impairment (32%, 98 of 302). On average, 2.6 (95% confidence interval 2.48-2.78) vestibular sensors were deficient, with higher numbers (p ≤ 0.017) for acute cochleovestibular neuropathy and vestibular schwannoma than for AVN, Menière disease, and episodic vestibular syndrome. In hierarchical cluster analysis, early mergers (posterior SCC/sacculus; anterior SCC/utriculus) pointed to closer pathophysiologic association of these sensors, whereas the late merger of the horizontal canal indicated a more distinct state. CONCLUSIONS: While the extent and pattern of vestibular impairment critically depended on the underlying disorder, more limited damage in AVN and Menière disease was noted, emphasizing the individual range of loss of function and the value of vestibular mapping. Likely, both the anatomic properties of the different vestibular end organs and their vulnerability to external factors contribute to the relative sparing of the vertical canals and the sacculus.

Quantitative Analysis of Saccade Gain in Video Head Impulse Testing.

OBJECTIVE: To quantitatively analyze corrective saccade (CS) gain and further characterize the specific relationship between vestibulo-ocular reflex (VOR) gain and CS gain in patients with vestibular loss and healthy controls. STUDY DESIGN: Prospective combined with retrospective study. SETTING: Affiliated Sixth People's Hospital, Shanghai Jiao Tong University. SUBJECTS AND METHODS: Forty patients with unilateral vestibular loss and 40 participants with normal vestibular function were subjected to video head impulse testing (vHIT). The analysis of the horizontal semicircular canal VOR and CS gains was based on individual head impulses. RESULTS: The patient group had significantly higher CS gain and lower VOR gain than the control group (P < .001). While there was no significant correlation between VOR and CS gains in the control group after adjusting for age and sex (P = .689), VOR gain negatively correlated with CS gain in the patient group (r = -0.853, P < .001). The specific relationship between VOR and CS gains was characterized as y = -1.17x + 1.12 (x: VOR gain, y: CS gain; r2 = 0.732, P < .001) in the patient group. CONCLUSIONS: In healthy participants, CS was not correlated with VOR gain, suggesting that CS is not due to VOR hypofunction. In patients with unilateral vestibular loss, CS was closely associated with VOR gain and can almost correct gaze position errors required for visual stabilization. CS gain could be an important indicator to diagnose vestibular loss and help physicians identify abnormal vHIT curves caused by artifacts and irregular practices.

MRI surveillance following concurrent cochlear implantation in cases of vestibular schwannoma resection.

OBJECTIVE: Cochlear nerve preserving translabyrinthine vestibular schwannoma (VS) resection enables concurrent cochlear implantation. Implantation in patients with VS raises important concerns including the ability to undergo postoperative magnetic resonance imaging (MRI) monitoring of residual tumor growth or tumor recurrence, specifically with a retained magnet. We aim to assess the feasibility of MRI monitoring and the impact on image quality with retained cochlear implant (CI) magnets. METHODS: Retrospective review of post-operative head MRI scans in CI recipients with a retained CI magnet, after cochlear nerve preserving translabyrinthine excision of VS. The ability to visualize the ipsilateral and contralateral internal auditory canal (IAC) and cerebellopontine angle (CPA) were assessed. RESULTS: A total of eight surveillance head MRI were performed in six patients. In one case, in which the receiver was positioned lower, the view of the ipsilateral IAC and CPA was distorted. In all other cases, the views of both the ipsilateral and contralateral IAC and CPA were overall unimpaired. DISCUSSION: Imaging artifact only very rarely impedes adequate visualization of the ipsilateral IAC or CPA in CI recipients. In anticipation of the need for further IAC and CPA imaging, it would be advisable to place the receiver in an exaggerated superior-posterior position to further decrease obscuring artifact. Thus, serial monitoring of VS tumors can be performed safely with preservation of image quality with a retained receiver magnet. CONCLUSIONS: When placing the CI receiver-stimulator farther posterior-superiorly, excellent visualization of the IAC and CPA can be accomplished without significantly impairing the image quality.

Clinical long-term observation of the keyhole microvascular decompression with local anesthesia on diagnosis and treatment of vestibular paroxysmia.

Background: The pathophysiology and etiology of vestibular paroxysmia (VP) remains unclear, moreover, due to the lack of reliable diagnostic features for VP, the clinical diagnosis will be made mainly by exclusion.Aims/objectives: To evaluate the diagnostic value and curative effect of keyhole microvascular decompression with local anesthesia for VP.Material and methods: 54 patients with trigeminal neuralgia, hemifacial spasm and glossopharyngeal neuralgia underwent keyhole microvascular decompression with local anesthesia, twelve of whom were coexistent with VP. The evaluation of the vertigo after operation was performed with symptom report card for 12 patients with VP and the mean follow-up period was 116 months (range 114-118 months).Results: The cochleovestibular neurovascular compression at the root zone of vestibular nerve was found in 12 patients with VP, of whom 11 patients had the neurovascular compressive vertigo induced intra-operatively and the vertigo disappeared postoperatively, moreover, one patient had no neurovascular compressive vertigo induced intra-operatively and the vertigo was not improved significantly after operation. Of 12 patients with VP during the mean 116-month follow-up, 11 patients had no recurrence of neurovascular compressive vertigo and the effective control rate of vertigo was 91.7%.Conclusions and significance: Keyhole microvascular decompression with local anesthesia is not only an effective method for treating VP and controlling neurovascular compressive vertigo, but also has definite clinical significance in the diagnosis of VP.

Clinical Profile and Results Obtained in Patients Treated by Auditory Brainstem Implants. / Perfil clínico y resultados obtenidos en los pacientes tratados mediante implante auditivo del tronco del encéfalo.

INTRODUCTION: Cochlear implants have been able to treat some types of hearing loss, but those related to cochlear nerve impairment made it necessary to find new ways to manage these deficits; leading to auditory brainstem implants (ABI). AIM: Our objective is to present the clinical profile of patients treated through an ABI and the results obtained from 1997 to 2017. MATERIAL AND METHODS: On the one hand, patients with statoacoustic nerve tumours (VIIIcranial nerve) were selected, and on the other hand, patients withoutVIII tumours with congenital malformations of the inner ear. Before and after the placement of the ABI, hearing was assessed through tonal audiometry, from which the PTA (Pure Tone Average) and the CAP (Categories of Auditory Performance) scale were obtained. RESULTS: A total of 20 patients undergoing ABI surgery were included. Eight were of tumour cause (40%) and 12 non-tumour (60%). In 15 subjects (75%) a suboccipital approach was performed and in 5 (25%) translabyrinthine. The mean of active electrodes before the implantation of Cochlear® (Nucleus ABI24) was 13/21 (61.90%) versus 8.5/12 (70.83%) of the Med-el® (ABI Med-el). An improvement in the mean PTA of 118.49dB was found against 46.55dB at 2years. On the CAP scale, values of1 were obtained in the preimplantation and of 2.57 (1-5) in the 2-year revision. CONCLUSION: The ABI is a safe option, and with good hearing results when the indication is made correctly.

Microvascular Decompression and Transposition of the 8th Cranial Nerve Using a Fenestrated Clip.

Neurovascular compression is a potential, yet rare cause of vertigo and unilateral tinnitus. Despite the high success rate of microvascular decompression (MVD) in other vascular compression syndromes, the outcome reported after MVD of the vestibulocochlear nerve is variable.1,2 The presence of combined symptoms of tinnitus and vertigo treated by MVD has demonstrated a higher predictive value for success.3 In this video, we present the case of a 68-year-old male who presented with vertigo and tinnitus refractory to medical management and vestibular therapy. Audiologic evaluation was normal apart from mild sensorineural hearing loss. Vestibular testing was suggestive of uncompensated right peripheral vestibulopathy. Magnetic resonance imaging with fast imaging employing steady-state acquisition sequences revealed vascular compression by the right anteroinferior cerebellar artery (AICA) at the cisternal component of vestibulocochlear nerve. After a multidisciplinary discussion, a microvascular decompression was performed through a right retrosigmoid craniotomy. The AICA was mobilized off the vestibulocochlear nerve and was secured to the petrous dura using a fenestrated clip. Indocyanine green angiography with Glow-800 was conducted before and after AICA transposition to confirm adequate flow through the mobilized vessel. Postoperatively, the patient's vestibular symptoms improved significantly. This case demonstrates that microvascular decompression can provide a satisfactory outcome in patients with unilateral tinnitus and vertigo associated with vascular compression in appropriately selected cases (Video 1).

Sixteen-and-a-half syndrome with metastatic pons tumor: A case report.

RATIONALE: One-and-a-half syndrome (OAAH) is characterized as the combination of ipsilateral horizontal gaze palsy and internuclear ophthalmoplegia. OAAH syndrome accompanied with 7th and 8th cranial nerve palsy is called 16-and-a-half syndrome. We aimed to report the case of 16-and-a-half syndrome with metastatic pons tumor. PATIENT CONCERNS: A 57-year-old male diagnosed with nonsmall-cell lung cancer (NSCLC) with brain metastasis occurring 15 months ago was referred to our clinic with the chief complaint of horizontal diplopia and right gaze palsy. DIAGNOSIS: According to the patient symptom, ocular examination, and radiographic findings, he was diagnosed as 16-and-a-half syndrome which was caused by brain tumor metastasis from NSCLC. INTERVENTIONS: We referred him to hemato-oncology department and he was treated with radiation and supportive therapy. OUTCOMES: Unfortunately, the patient passed away 1 month later without improvement of ophthalmoplegia. LESSONS: The clinical findings of our case indicate 16-and-a-half syndrome caused by brain tumor metastasis from NSCLC, which to our knowledge has not been previously reported. The case highlights a rare cause of OAAH spectrum disease and the importance of a systemic work-up including associated neurologic symptoms and brain imaging in patients with horizontal gaze palsy.

Endoglin-based assessment of neoangiogenesis in sporadic VIII cranial nerve schwannoma.

Although the diagnosis and treatment of sporadic vestibular schwannoma has improved in recent years, no factors capable of predicting its growth have been identified as yet. Endoglin (CD105) is a proliferation-associated protein expressed in angiogenic endothelial cells, and a potential prognostic indicator for several solid tumors. The aim of the present study was primarily to investigate the expression and role of CD105 in a series of sporadic vestibular nerve schwannomas. In 71 consecutive cases of vestibular schwannoma, vessel cross-sectional area and density were calculated from immunohistochemically assessed CD105 expression using image analysis to correlate them with: (i) tumor dimensions; and (ii) tumor growth rate measured on high-resolution contrast-enhanced MRI (ceMRI). Based on assessments of CD105 expression, a significant positive correlation was identified between vessel cross-sectional area and tumor size at the time of surgery (p = 0.0024), and between vessel density and tumor size (p = 0.013). Vessel cross-sectional area (p = 0.0006) and vessel density (p = 0.003) were significantly greater in tumors measuring ≥10 mm in size than in those <10 mm. Conversely, when tumor growth rate could be calculated from two or more ceMRI (38 cases), there was no significant correlation between tumor growth rate and cross-sectional vessel area or vessel density as assessed with CD105. Further investigations are needed to ascertain the feasibility of: (i) using circulating endoglin assay to monitor tumor growth; and (ii) targeting neoangiogenesis with anti-endoglin antibodies in sporadic vestibular schwannoma.

Diffuse large B-cell lymphoma recurrence presenting as multiple, progressive cranial neuropathies.

A 58-year-old man with a history of rheumatoid arthritis and stage IV diffuse large B-cell lymphoma, in complete remission with no evidence of residual disease on positron emission tomography/CT after completing six cycles of rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone chemotherapy, presented with acute onset of dysphagia to solids and liquids. On further evaluation, his dysphagia was attributed to a vagus nerve palsy, and later during his admission, he developed rapidly progressing left facial and vestibulocochlear nerve palsies. Imaging studies displayed pathological enhancement of bilateral seventh and eighth cranial nerves, concerning for leptomeningeal recurrence of lymphoma. Cerebrospinal fluid analysis and flow cytometry were confirmatory, revealing markedly atypical monotypic CD19 positive B cells.

Hemifacial Spasm Caused by Small Epidermoid Tumor, Misinterpreted as Delayed Secondary Hemifacial Spasm Caused by Vestibular Schwannoma Treated with Gamma Knife Surgery.

BACKGROUND: Hemifacial spasm (HFS), one of the most common hyperactive cranial rhizopathies, is a disorder characterized by spontaneous, intermittent, and repetitive contraction of unilateral facial muscle. The most common cause of HFS is a mechanical compression of the facial nerve at the root exit zone (REZ) by blood vessels located in the brainstem. The incidence of cerebellopontine angle (CPA) tumor-induced HFS ranges from 0.3%-2.5% of all patients with HFS, and the incidence of epidermoid cyst among such tumors is extremely rare (0.2% to 0.25%). We present a patient manifesting HFS induced by a small epidermoid cyst who underwent Gamma Knife surgery (GKS) for ipsilateral vestibular schwannoma (VS) more than 10 years ago. CLINICAL DESCRIPTION: A 59-year-old female was admitted to our hospital with HFS. Ten years before admission, the patient underwent GKS for ipsilateral VS. Enhanced brain magnetic resonance imaging demonstrated that the volume of VS was not increased. Also, no definitive mechanical compression of the facial nerve REZ by blood vessels was detected. We concluded that HFS was secondary to the VS treatment by GKS and decided to operate to remove the tumor and identify REZ. A standardized lateral suboccipital retrosigmoid approach was performed. The cochlear nerve was encased by a tumor mass, and the posterior inferior cerebellar artery (PICA) was displaced by the mass. During tumor excision, we ensured that the VS and displaced PICA did not directly compress the REZ. Interestingly, an unexpected lesion was found with a yellowish viscous mass located between the sixth nerve and ventral side of the seventh nerve REZ. After the tumor removal, a small piece of Teflon was inserted between the PICA and REZ of the facial nerve. Postoperatively, the patient experienced no HFS or facial nerve dysfunction. Histopathologic examination of the lesion revealed an epidermoid cyst. CONCLUSIONS: We should consider various possibilities for the cause of HFS and review brain imaging meticulously if patients were treated for CPA tumor with GKS a long time ago and the delayed HFS occurred without changes in tumor volume. HFS induced by epidermoid cyst is rare. Therefore it is critical for the neurosurgeon to ensure the absence of any abnormal contact with the REZ of the facial nerve during surgery for HFS induced by CPA tumors.

Influence of socioeconomic factors on the perception of cochlear-vestibular symptoms and adherence to the treatment of congenital hypothyroidism / Influência dos fatores socioeconômicos na percepção de sintomas cócleo-vestibulares e na adesão ao tratamento do hipotireoidismo congênito

Abstract Objectives: to verify if there is an association between socioeconomic factors and adherence to treatment in congenital hypothyroidism and to verify if there is an association between socioeconomic factors and vestibulocochlear symptoms noticed by parents/caregivers of children diagnosed with congenital hypothyroidism. Methods: a cross-sectional, exploratory and descriptive study, with a convenience sample. The sample consisted of 108 children with clinical and laboratory diagnosis of congenital hypothyroidism, of both sexes, aged ≥ 5 years. The researchers applied a structured questionnaire to parents/caregivers, consisting of closed and objective questions about the presence or absence of tinnitus, hearing loss and dizziness/vertigo in children with congenital hypothyroidism. Results: There was no association between socioeconomic factors and adherence to treatment or perception of cochlear-vestibular symptoms. Conclusions: socioeconomic factors did not influence treatment adherence or perceived cochlear-vestibular symptoms by caregivers of children with congenital hypothyroidism.

Resumo Objetivos: verificar se existe associação entre fatores socioeconômicos e adesão ao tratamento no hipotireoidismo congênito e verificar se existe associação entre fatores socioeconômicos e sintomas vestibulococleares percebidos pelos pais / cuidadores de crianças diagnosticadas com hipotireoidismo congênito. Métodos: estudo transversal, exploratório e descritivo, com amostra de conveniência. A casuística foi composta por 108 crianças com diagnóstico clínico e laboratorial de hipotireoidismo congênito, de ambos os sexos com idade ≥ 5 anos. Foi aplicado um questionário estruturado para os pais/cuidadores, formado por questões fechadas e objetivas sobre a presença ou ausência de zumbido, hipoacusia e tontura/vertigem nas crianças com hipotireoidismo congênito. Resultados: não houve associação entre fatores socioeconômicos e adesão ao tratamento ou percepção dos sintomas cócleo-vestibulares. Conclusões: os fatores socioeconômicos não influenciaram na adesão ao tratamento nem na percepção de sintomas cócleo-vestibulares pelos cuidadores de crianças com hipotireoidismo congênito.

Management of jugular bulb injury during drilling of the internal auditory canal (ICA) for vestibular schwannoma surgery.

The retrosigmoid approach for vestibular schwannoma surgery has remained the standard approach by most neurosurgeons. Drilling the posterior wall of the internal auditory meatus (IAM) is an essential step in removing the intrameatal tumor. During IAM drilling, three anatomical structures can be encountered, including the posterior semicircular canal, vestibular aqueduct, and jugular bulb. Any of these can be injured during drilling, especially if the jugular bulb lies above the inferior edge of the IAM. Although IAM drilling is performed in most vestibular schwannoma surgeries, information on how to manage complications such as jugular bulb injury is lacking. Here we use an intraoperative video to demonstrate how to manage the inadvertent injury to the jugular bulb in order to avoid massive blood loss. We present a case of a 39-year-old woman with hearing loss, diagnosed with a cerebellopontine angle mass extending into the IAM. Surgery was required due to tumor progression. We used the retrosigmoid approach to access the tumor. During IAM drilling, the jugular bulb was injured. A thin layer of bone wax was applied under continuous suction. The margins of the wax were then gently compressed with a dissector; great care was taken to avoid pushing the wax into the jugular bulb. Excess bone wax was removed (video 1). A small diamond drill (2 mm) was used for further drilling. Our instructional video shows the surgical approach, microsurgical anatomy, and technical aspects of managing massive bleeding from jugular bulb injury. It should therefore be helpful for young neurosurgeons.

Quality of life related to symptomatic outcomes in patients with vestibular schwannomas: A Canadian Centre perspective.

Patients with vestibular schwannomas (VS) typically present with hearing loss and tinnitus as well as variable cranial nerve dysfunctions. Surgical resection, stereotactic radiotherapy and/or conservative management employing serial magnetic resonance or computed tomography imaging serve as the main treatment options. Quality of life (QoL) may be impacted by the extent of tumour burden and exacerbated or relieved by treatment. Subjective assessment and quality of life inventories provide valuable information in client centered approaches with important implications for treatment. The intention of QoL measurements affecting VS patients within a clinical setting is to facilitate discussions regarding treatment options and objectively evaluate patient- centered clinical outcomes in a naturalistic setting.

Acute vertigo and sensorineural hearing loss from infarction of the vestibulocochlear nerve: A case report.

RATIONALE: Acute unilateral audiovestibulopathy is a common neurotological syndrome. Differential diagnoses of acute unilateral audiovestibulopathy include viral infection, vascular insults, and tumors. Regarding vascular causes, ischemic stroke in the anterior inferior cerebellar artery (AICA) territory is known to be the leading cause of acute audiovestibular loss. Previous reports of AICA infarction with audiovestibulopathy failed to demonstrate magnetic resonance imaging (MRI)-positive vestibulocochlear infarctions. Only 1 report demonstrated acute infarction involving the vestibulocochlear nerve on diffusion weighted imaging (DWI)-MRI. PATIENT CONCERNS: A 67 year old man complained of sudden left hearing loss and vertigo. The patient showed left horizontal gaze-evoked nystagmus (GEN) and the head impulse test (HIT) was positive on the left side. Videonystagmography revealed spontaneous rebound nystagmus toward the right side; head-shaking nystagmus toward the right side. The patient presented with left caloric paresis (20.1%). Pure tone audiometry (PTA) revealed severe sensorineural hearing loss on the left side. DIAGNOSIS: MRI of temporal bone showed multifocal acute infarctions in the left inferior cerebellum. Moreover, images revealed tiny infarctions along the left vestibulocochlear nerve and the cochlea, implying acute vestibulocochlear nerve and labyrinthine infarction. There was no evidence of steno-occlusion of major cerebral vessels on MR angiography. INTERVENTIONS: Immediate stroke management was done. OUTCOMES: Neurological symptoms gradually improved after 3 to 5 days. LESSONS: We present a case illustrating a rare but significant finding of vestibulocochlear nerve infarction revealed by DWI-MRI. Prompt imaging protocol enabled the detection of significant findings in this patient with acute unilateral audiovestibulopathy. Clinicians should be aware of the vestibulocochlear nerve and labyrinth on MRI in patients with cerebellar stroke.