Clinical practice guideline on the management of vestibular schwannoma.

INTRODUCTION: Vestibular schwannoma (VS) is the most common tumour of the cerebellopontine angle. The greater accessibility to radiological tests has increased its diagnosis. Taking into account the characteristics of the tumour, the symptoms and the age of the patient, three therapeutic strategies have been proposed: observation, surgery or radiotherapy. Choosing the most appropriate for each patient is a frequent source of controversy. MATERIAL AND METHODS: This paper includes an exhaustive literature review of issues related to VS that can serve as a clinical guide in the management of patients with these lesions. The presentation has been oriented in the form of questions that the clinician usually asks himself and the answers have been written and/or reviewed by a panel of national and international experts consulted by the Otology Commission of the SEORL-CCC. RESULTS: A list has been compiled containing the 13 most controversial thematic blocks on the management of VS in the form of 50 questions, and answers to all of them have been sought through a systematic literature review (articles published on PubMed and Cochrane Library between 1992 and 2023 related to each thematic area). Thirty-three experts, led by the Otology Committee of SEORL-CCC, have analyzed and discussed all the answers. In Annex 1, 14 additional questions divided into 4 thematic areas can be found. CONCLUSIONS: This clinical practice guideline on the management of VS offers agreed answers to the most common questions that are asked about this tumour. The absence of sufficient prospective studies means that the levels of evidence on the subject are generally medium or low. This fact increases the interest of this type of clinical practice guidelines prepared by experts.

Early Diagnosis of Central Disorders Mimicking Horizontal Canal Cupulolithiasis.

BACKGROUND: Horizontal Canal Cupulolithiasis (hc-BPPV-cu) can mimic a pathology of central origin, so a careful examination is essential to prevent misdiagnosis. METHODS: Retrospective cross-sectional cohort study of 45 patients suffering from suspected hc-BPPV-cu. We recorded whether patients first presented through an ENT Emergency Department (ED) or through an Outpatient Otolaryngology Clinic (OC). RESULTS: We found statistically significant differences (p < 0.05) between the OC versus the ED in relation to the time between symptom onset and first assessment (79.7 vs. 3.6 days, respectively), the number of therapeutic maneuvers (one maneuver in 62.5% vs. 75.9%, and more than one in 25.1% vs. 13.7%), and multi-canal BPPV rate (43.8% vs. 3.4%). hc-BPPV-cu did not resolve in 2 patients (12.5%) from the OC and in 3 (10.3%) from de ED, all of which showed central pathology. DISCUSSION: There are no prior studies that analyze the approach to hc-BPPV-cu in the ED. The benefits of early specialist input are early identification of central positional nystagmus, a decrease in symptom duration, reduced number of therapeutic maneuvers required for symptom resolution, and lower rates of iatrogenic multi-canal BPPV. CONCLUSION: A comprehensive approach to hc-BPPV-cu in the ED allows both more effective treatment and early identification of central disorder mimics.

Prevention of Severe Vestibular Hypofunction after Systemic Gentamicin.

The importance of early evaluation by a neurotologist in patients with infective endocarditis treated with systemic gentamicin and its impact on the patients' quality of life was evaluated. This is a longitudinal retrospective cohort study of 29 patients who received intravenous gentamicin for the treatment of infective endocarditis. Patients were classified into two groups: group A, before a neurotologist was included in the treatment protocol, and group B, after the inclusion of a neurotologist. The frequency of the different symptoms in each group was measured, and the gain of the vestibulo-ocular reflex (VOR) and its relationship with the presence of oscillopsia. In total, 13 and 16 patients were assigned to groups A and B, respectively. The mean gain of the VOR measured using the video head impulse test in group A was 0.44 in the best side and 0.39 in the worst side. In group B, the mean gain was 0.71 (best side) and 0.64 (worst side) (p < 0.0001). The patients who complained about oscillopsia had a main gain of 0.41 in the best side and 0.35 in the worst side. Evaluation of vestibular function should be included in the infective endocarditis treatment protocol, including the adverse effects of systemic gentamicin.

The map of dizziness in vestibular schwannoma.

INTRODUCTION: Dizziness is a frequent complaint in patients with vestibular schwannoma (VS). An abnormal vestibulo-ocular reflex (VOR) can explain this dizziness in patients with VS. The video Head impulse test (vHIT) offers a chance to describe specifically the VOR findings in such patients. STUDY DESIGN: Retrospective cases series study in a tertiary referral hospital. METHODS: Fifty consecutive patients with VS were classified in accordance with the morphology of the VOR; gain, covert saccade, and overt saccade were analyzed both in the affected side and in the healthy side. For all patients, caloric tests were performed. All patients were tested before surgery. RESULTS: Caloric response was normal in 31 of 50 patients. The video Head impulse test was abnormal in 45 of 50 patients. For the affected side, low horizontal VOR gain was found in 27 of 50 patients, covert saccade was observed in 37 of 50, and overt saccade was observed in 26 of 50. In the healthy side, vHIT was abnormal in 29 of 50 patients, with a low gain in four of 50, covert saccade in seven of 50, and overt saccade in 23 of 50. In VS, gain for the affected side is not associated with caloric response, but gain for the affected side is associated with gain in the healthy side. Covert and overt saccade for the affected side is associated with gain for the affected side. In the healthy side, overt saccade is associated with low gain for the affected side. CONCLUSIONS: Video head impulse test improves the vestibular testing before surgery in patients with VS and should be included in the usual clinical tests for these patients. LEVEL OF EVIDENCE: 4.

[Peripheral vertigo versus central vertigo. Application of the HINTS protocol]. / Vértigo periférico frente a vértigo central. Aplicación del protocolo HINTS.

INTRODUCTION: One of the most important dilemmas concerning vertigo in emergency departments is its differential diagnosis. There are highly sensitive warning signs in the examination that can put us on the path towards finding ourselves before a case of central vertigo. AIM: To determine how effective the application of the HINTS protocol is in the diagnosis of cerebrovascular accidents that mimics peripheral vertigo. PATIENTS AND METHODS: We conducted a descriptive observation-based study on patients admitted to hospital with a diagnosis of acute vestibular syndrome in the emergency department. All the patients were monitored on a day-to-day basis until their symptoms improved, with information about nystagmus, the oculocephalic manoeuvre and the skew test. The results from the magnetic resonance imaging study were compared with the alteration of any of those three signs during the time the patient was hospitalised. RESULTS: Altogether 91 patients were examined, with a mean age of 55.8 years. A cerebrovascular accident was observed in eight cases. Of these (mean age: 71 years), in seven of them there were alterations in some of the HINTS signs, and in one case the study was normal (sensitivity: 0.88; specificity: 0.96). All of them had some vascular risk factor. CONCLUSIONS: Faced with a patient who visits the emergency department with an acute vestibular syndrome, a suitably directed examination is essential to be able to establish the differential diagnosis between peripheral and central pathology, since some cerebrovascular accidents can present with the appearance of acute vertigo. Applying a protocol like HINTS makes it possible to suspect the central pathology with a high degree of sensitivity and specificity.

TITLE: Vertigo periferico frente a vertigo central. Aplicacion del protocolo HINTS.Introduccion. Uno de los dilemas mas importantes concernientes al vertigo en urgencias es su diagnostico diferencial. Existen signos de alarma de gran sensibilidad en la exploracion que pueden ponernos en la pista de encontrarnos ante un vertigo central. Objetivo. Determinar la eficacia de la aplicacion del protocolo HINTS en el diagnostico del accidente cerebrovascular que simula un vertigo periferico. Pacientes y metodos. Estudio observacional descriptivo sobre pacientes ingresados con diagnostico de sindrome vestibular agudo en urgencias. Todos los pacientes fueron objeto de un seguimiento diario hasta la mejoria de sus sintomas con informacion del nistagmo, la maniobra de impulso oculocefalico y el test de skew. Se comparan los resultados del estudio de resonancia magnetica con la alteracion en alguno de esos tres signos a lo largo del ingreso del enfermo. Resultados. Se reunio a 91 pacientes, con una edad media de 55,8 años. Se objetivo un accidente cerebrovascular en ocho de ellos. De estos (edad media: 71 años), en siete existia una alteracion en alguno de los signos HINTS y en uno el estudio fue normal (sensibilidad: 0,88; especificidad: 0,96). Todos ellos tenian algun factor de riesgo vascular. Conclusiones. Una exploracion adecuada y dirigida ante un paciente que acude a urgencias con un sindrome vestibular agudo resulta de vital importancia para establecer el diagnostico diferencial entre la patologia periferica y la central, ya que algunos accidentes cerebrovasculares se pueden presentar bajo la apariencia de un vertigo agudo. Aplicar un protocolo como HINTS permite sospechar la patologia central con una gran sensibilidad y especificidad.

NF2 genetic alterations in sporadic vestibular schwannomas: clinical implications.

HYPOTHESIS: NF2 gene alterations may have a clinical impact in non-NF2 vestibular schwannomas (VSs). BACKGROUND: It has been suggested that NF2 mutations might correlate with clinical expression of VS in NF2 patients. The aim of this study was to analyze the impact of genetic alterations in the NF2 gene on epidemiologic, clinical, and radiologic features of patients with sporadic VS. The association between cigarette consumption and the molecular genetic findings was also studied. METHODS: The study group consisted of 51 patients who underwent surgery for removal of vestibular schwannoma in our institution between January 2006 and December 2010. Five highly polymorphic microsatellite DNA markers were used to observe the frequency of loss of heterozygosity (LOH) in chromosome 22. The NF2 gene mutations were detected using polymerase chain reaction amplification and denaturing high-performance liquid chromatography analysis (PCR/dHPLC), and direct sequencing of NF2. Multiplex ligation-dependent probe amplification (MLPA) of the NF2 gene was also performed. RESULTS: An NF2 mutation was identified in 49%, 22q LOH in 57%, and MLPA alterations in 13.7% of the cases. One mutational hit was present in 27%, and 2 hits were present in 45% of the tumors. No association was found between the type of NF2 mutation and relevant clinical parameters. The presence of NF2 mutations detected by PCR/dHPLC was associated with no complaint of hearing loss at the time of diagnosis (p = 0.023), with subjective aural fullness (p = 0.022) and with an absence of tumor involvement of the internal auditory canal (p = 0.029). Patients with NF2 mutations had lower mean corrected PTA thresholds compared with those with no NF2 mutation (p = 0.037). Inactivation of the NF2 gene by mutation, MLPA, or LOH was more frequent in smokers when compared with never smokers (p = 0.048). CONCLUSION: NF2 mutations may play a role in the pathophysiology of hearing loss as well as in the pattern of growth of VS. Cigarette smoking in patients with VS seems to play a role in both the risk of developing the tumor and also in its genetic profile. More studies are needed to corroborate these results and, more broadly, to establish links between molecular and clinical data.

Vertigo as the first sign of chronic myeloid leukemia: a case report and literature review.

Acute vestibular deficit as the first sign of leukemia is extremely rare. The literature shows some cases of sudden hearing loss accompanied by instability and associated with hyperviscosity syndrome. We present the case of a patient who presents a harmonic vestibular deficit of the right ear. The complementary studies revealed an abnormally high level of leukocytes. A peripheral blood cytogenetic analysis is performed due to a high suspicion of leukemia, and the results show BCR/ABL fusion gene with a cut point in the M-BCR region, which confirms the diagnosis of chronic myeloid leukemia. In this case we detail the importance of taking hematological disorders into consideration in the differential diagnosis of patients with otoneurological symptoms, and we also review the etiopathogenic mechanisms, symptoms, diagnosis, and therapeutic options for chronic myeloid leukemia with sudden hearing loss and vertigo.

Is benign paroxysmal vertigo of childhood a migraine precursor?

INTRODUCTION: Benign Paroxysmal Vertigo of Childhood (BPVC) is a common cause of vertigo in children and it is characterized by recurrent attacks of vertigo without warning resolving spontaneously after minutes to hours. It has been considered the equivalent of migraine in childhood. METHODS: Twenty-seven patients diagnosed with BPVC were recruited between 1991 and 1997 with a follow up of at least 15 years. The incidence of migraine, inner ear disorders and family medical history is analyzed. RESULTS: The average age for the onset of the attacks of BPVC was 3 years and 11 months, and for spontaneous resolution, it was around 5 years and 7 months. The average age for follow up was 18 years. Nine patients developed migraine during adulthood. Nineteen of them had a family history of migraine. CONCLUSION: We have observed that the prevalence of migraine in patients that had been diagnosed with BPVC is higher than in the general population, which leads us to propose BPVC as a precursor of migraine during childhood.

[Treatment of severe to profound mixed hearing loss with the BAHA Cordelle II]. / Tratamiento de las hipoacusias mixtas severas a profundas con el BAHA Cordelle II.

GOALS: Evaluation of the audiological outcome and subjective satisfaction of BAHA Cordelle II in the treatment of patients with severe to profound bilateral mixed hearing loss. MATERIAL AND METHOD: Retrospective study of 12 patients suffering a severe to profound bilateral sensorineural hearing loss, using pure tone audiometry (PTA), speech audiometry and subjective evaluation before and after the implantation of a BAHA Cordelle II (Cochlear(®)). RESULTS: The average gain in conversational frequencies (0.5 to 4kHz) with BAHA in free field was 43, 51, 47 and 44dB, respectively. We observed a GAP over closure in 10 of the 12 patients. Speech audiometry improved from 85% at 83dB of maximum discrimination to 96% at 62dB. The subjective evaluation questionnaires showed great satisfaction with a slight decrease in noisy or windy environments. The great majority of our patients used the BAHA device throughout the entire day. CONCLUSIONS: The BAHA Cordelle II (Cochlear(®)) is a good option in the treatment of severe to profound bilateral mixed hearing loss. Its best advantages are a low risk of labyrinthization, high result predictability, easy and step-by-step surgery, no need for general anaesthesia, and the GAP over closure in all frequencies. Active middle ear devices represent another alternative, but specific indications have not been defined yet because of low universal experience. When the intelligibility of the patient is poor, cochlear implantation should be considered.

[PANDAS Syndrome: a new tonsillectomy indication?]. / Síndrome PANDAS: ouna nueva indicación de amigdalectomía?

PANDAS Syndrome (Paediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcus) is a rare disease described in 1998. In this disease, there is a relationship between group A beta haemolytic streptococcal tonsil infections and the exacerbation of neuropsychiatric disorders. A case report of a 9-year-old child with PANDAS syndrome is presented. This child has had no further symptoms after tonsillectomy. The understanding about PANDAS syndrome and tonsillectomy is reviewed.

[Facial paralysis of infectious origin in patients receiving cochlear implants]. / Parálisis facial de origen infeccioso en paciente intervenido de implante coclear.

Peripheral facial paralysis during cochlear implant surgery appears in 0.43 % of adults and 0.39 % of children. Peripheral facial paralysis secondary to acute otitis media is very rare in adults, while the incidence in children remains between 0.19 % and 0.45 %. We present 3 cases of patients who underwent cochlear implant surgery at our department, and who presented peripheral facial paralysis secondary to acute otitis media. The procedure is similar to facial paralysis secondary to acute otitis media in children. Treatment consists of parenteral antibiotic and corticosteroid treatment. Prognosis is favourable, with a total recovery of facial function in 1 or more months.

[Aorto-oesophageal fistula in patient with Montgomery salivary bypass tube]. / Fístula aortoesofágica en paciente con tubo de derivación salival de Montgomery.

Aorto-oesophageal fistula is a well-reported pathology with several known causes. The co-existence of this pathology associated with the use of a Montgomery salivary bypass tube (MSBT) is exceptional and only one case is described in the literature. We present here a case report about an 81-year-old patient with an MSBT who died because of a massive upper gastrointestinal bleeding caused by an aorto-oesophageal fistula at the site of the MSBT. The literature on this pathology will also be reviewed.

[Phobic orthostatic insecurity]. / Inseguridad fóbica ortostática.

BACKGROUND AND OBJECTIVES: The authors attempt to expand knowledge about a subjective balance disorder they have called phobic orthostatic insecurity, a condition representing the second cause of medical visits (22.3 %) to their ENT and neuro-otology clinic, and attempt to identify relationships with similar conditions described in psychiatry (agoraphobia, somatoform vertigo, and space-phobia) and in neurology (phobic postural vertigo). They also propose a simple diagnostic method and present their therapies and results. PATIENTS AND METHOD: A total of 151 patients with an indefinite symptomatology of "dizziness" "vertigo" or "insecurity" were evaluated (from 1999 to 2005) by means of a full medical history and an appropriate neurological examination, pharmacological treatments with anxiolytics-antidepressives, a measurement of the degree of depression with the Beck test (a kind of psychiatric benchmark) and with a specific standardized test. RESULTS: Three symptoms and one exploratory condition, among others, were found in all 151 patients studied; these constitute the four bases for a positive diagnosis. This is confirmed if the treatment achieves total remission (this occurred in 69.53 % of all patients) or a sub-total remission (24.49 %), according to valuation scale for insecurity in all situations. CONCLUSIONS: The statistical analysis showed a symptomatic concordance within the group analyzed, a syndromic equivalence between patients and satisfactory results with the antidepressive treatments (94 %), thus confirming the diagnostic and aetiopathogenic hypotheses for the disorder and, later, providing a logical method for diagnosis. The authors propose to assimilate this diagnostic protocol (and therapeutic when no specialist psychotherapy teams are available) to most of the psychogenic insecurity syndromes described.