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.

Dynamic Visual Acuity Results in Otolith Electrical Stimulation in Bilateral Vestibular Dysfunction.

(1) Background. Patients with bilateral vestibular disease (BVD) experience oscillopsia with a detriment to visual acuity (VA). This VA is driven mainly by the VOR that has two components: rotational and translational. VA can be tested by using dynamic visual acuity (DVA) on a treadmill because both systems are activated. The aim of this study is to compare VA before and after chronic electrical stimulation of the otolith organ. (2) Materials and Method. Five patients suffering from bilateral vestibular dysfunction (BVD), previously implanted with a new vestibular implant prototype, were included in this study with the aim to check VA with and without vestibular implant use (W and W/O) in static, 2 km/h and 4 km/h walking situations. DVAtreadmill was measured on a treadmill with a dynamic illegible E (DIE) test in static and dynamic conditions (while walking on the treadmill at 2 and 4 km/h). The DVA score was registered in a logarithm of the minimum angle of resolution (LogMAR) for each speed. In addition, every patient completed the oscillopsia severity questionnaire (OSQ) and video head impulse test (vHIT) before and after activation of the vestibular implant. (3) Results. The analysis shows a significant difference in OSQ scores and DVA with an improvement in dynamic conditions. Organized corrective saccades during the use of a vestibular implant with no changes in gain were also detected in the video head impulse tests (vHIT). (4) Conclusion. The vestibular implant with otolithic stimulation offers changes in the response of DVA, which makes this paper one of the first to address the possible restoration of it. It is not possible to rule out other contributing factors (presence of covert saccades, somatosensory system, …). More work seems necessary to understand the neurophysiological basis of these findings, but this implant is added as a therapeutic alternative for the improvement of oscillopsia.

Exploring the frontiers of vestibular migraine: A case series.

INTRODUCTION: Patients with episodic vestibular syndrome (EVS) whose symptoms resemble those of vestibular migraine (VM) but who do not meet the criteria for it are common. OBJECTIVE: To describe those patients suffering from EVS in whom defined etiologies have been ruled out in order to determine if their symptoms can be linked to VM. MATERIAL AND METHODS: Prospective multicenter study. The medical records of patients with VM and patients with EVS suggestive of VM but not meeting the criteria for it were examined. The characteristics of headache, the number and the length of attacks, the association of vestibular symptoms and headache, the intensity of symptoms and the response to treatment were recorded. RESULTS: 58 patients met the criteria for VM or probable VM; 30 did not. All of the symptoms improved significantly in the treated patients with VM or probable VM; in the rest of the treated patients, only the vestibular symptoms improved. CONCLUSION: A subgroup of patients that cannot be attributed to any known vestibulopathy according to present day VM criteria profited from migraine treatment, suggesting that their vestibular symptoms belong to the migraine spectrum; whereas some do not, yet our analysis could not identify distinctive features that allowed subgroup attribution.

Subjective benign paroxysmal positional vertigo in patients with osteoporosis or migraine / Vertigem posicional paroxística benigna subjetiva em pacientes com osteoporose ou migrânea

Abstract Introduction Subjective benign paroxysmal positional vertigo is a form of benign paroxysmal positional vertigo in which during the diagnostic positional maneuvers patients only present vertigo symptoms with no nystagmus. Objective To study the characteristics of subjects with subjective benign paroxysmal positional vertigo. Methods Prospective multicenter case-control study. All patients presenting with vertigo in the Dix-Hallpike test that presented to the participating hospitals were included. The patients were separated into two groups depending on whether nystagmus was present or not. An Epley Maneuver of the affected side was performed. In the follow-up visit, patients were checked to see if nystagmus and vertigo were present. Both groups of patients were compared to assess the success rate of the Epley maneuver and also to compare the presence of 19 variables. Results 259 patients were recruited, of which 64 belonged to the subjective group. Nystagmus was eliminated in 67.2% of the patients with benign paroxysmal positional vertigo. 89.1% of the patients with subjective benign paroxysmal positional vertigo remained unaffected by nystagmus, thus showing a significant difference (p = 0.001). Osteoporosis and migraine were the variables which reached the closest to the significance level. In those patients who were taking vestibular suppressors, the percentage of subjective benign paroxysmal positional vertigo was not significantly higher. Conclusions Subjective benign paroxysmal positional vertigo should be treated using the Epley maneuver. More studies are needed to establish a relationship between osteoporosis, migraine and subjective benign paroxysmal positional vertigo. The use of vestibular suppressants does not affect the detection of nystagmus.

Resumo Introdução A vertigem posicional paroxística benigna subjetiva é um tipo de vertigem posicional paroxística benigna na qual, durante as manobras posicionais diagnósticas, os pacientes apresentam apenas sintomas vertiginosos sem nistagmo. Objetivo Estudar as características de indivíduos com vertigem posicional paroxística benigna subjetiva. Método Estudo prospectivo multicêntrico de caso-controle. Foram incluídos todos os pacientes com vertigem no teste de Dix-Hallpike, que se apresentaram nos hospitais participantes. Os pacientes foram separados em dois grupos, dependeu da presença ou não do nistagmo. Uma manobra de Epley foi realizada no lado afetado. Na consulta de seguimento, os pacientes foram avaliados para verificar a presença ou não do nistagmo e da vertigem. Ambos os grupos de pacientes foram comparados para avaliar a taxa de sucesso da manobra de Epley e também para comparar a presença de 19 variáveis. Resultados Foram recrutados 259 pacientes, dos quais 64 pertenciam ao grupo subjetivo. O nistagmo foi eliminado em 67,2% dos pacientes com vertigem posicional paroxística benigna. Em 89,1% dos casos, os pacientes com vertigem posicional paroxística benigna subjetiva mantiveram-se não afetados pelo nistagmo, mostraram uma diferença significativa (p = 0,001). Osteoporose e enxaqueca foram as variáveis que atingiram o nível mais próximo ao de significância. Nos pacientes que tomavam supressores vestibulares, a porcentagem de vertigem posicional paroxística benigna subjetiva não foi significativamente maior. Conclusões A vertigem posicional paroxística benigna subjetiva deve ser tratada com a manobra de Epley. Mais estudos são necessários para estabelecer uma relação entre osteoporose, enxaqueca e vertigem posicional paroxística benigna subjetiva. O uso de supressores vestibulares não afeta a detecção do nistagmo.

Subjective benign paroxysmal positional vertigo in patients with osteoporosis or migraine.

INTRODUCTION: Subjective benign paroxysmal positional vertigo is a form of benign paroxysmal positional vertigo in which during the diagnostic positional maneuvers patients only present vertigo symptoms with no nystagmus. OBJECTIVE: To study the characteristics of subjects with subjective benign paroxysmal positional vertigo. METHODS: Prospective multicenter case-control study. All patients presenting with vertigo in the Dix-Hallpike test that presented to the participating hospitals were included. The patients were separated into two groups depending on whether nystagmus was present or not. An Epley Maneuver of the affected side was performed. In the follow-up visit, patients were checked to see if nystagmus and vertigo were present. Both groups of patients were compared to assess the success rate of the Epley maneuver and also to compare the presence of 19 variables. RESULTS: 259 patients were recruited, of which 64 belonged to the subjective group. Nystagmus was eliminated in 67.2% of the patients with benign paroxysmal positional vertigo. 89.1% of the patients with subjective benign paroxysmal positional vertigo remained unaffected by nystagmus, thus showing a significant difference (p=0.001). Osteoporosis and migraine were the variables which reached the closest to the significance level. In those patients who were taking vestibular suppressors, the percentage of subjective benign paroxysmal positional vertigo was not significantly higher. CONCLUSIONS: Subjective benign paroxysmal positional vertigo should be treated using the Epley maneuver. More studies are needed to establish a relationship between osteoporosis, migraine and subjective benign paroxysmal positional vertigo. The use of vestibular suppressants does not affect the detection of nystagmus.

Epidemiología de los trastornos vestibulares en la consulta de otoneurología / Epidemiology of vestibular disorders in the otoneurology unit

Introducción y objetivos: El conjunto de síntomas relacionados con los trastornos del sistema de equilibrio son comunes en la población general. Sin embargo, hay pocos estudios que cuantifiquen la frecuencia de inicio de los diversos trastornos vestibulares que se presentan en unidades especializadas de otoneurología. El objetivo de este estudio es establecer la epidemiología de los trastornos vestibulares, La forma de presentación y la carga de atención esperada en una clínica especialista en otoneurología. Material y métodos: Estudio descriptivo observacional retrospectivo sobre los pacientes remitidos a la consulta de Otoneurología de un centro hospitalario de tercer nivel entre el 1/1/2015 y el 31/12/2015. Resultados: Fueron valorados 174 pacientes (121 mujeres y 53 hombres) con una media de edad de 5 3± 17 años. Durante el periodo de estudio se valoraron 43 individuos por cada 100.000 habitantes. Los pacientes fueron remitidos en su mayoría desde consultas externas de Otorrinolaringología (36,8%) y desde Atención Primaria (28,7%). El motivo de consulta más frecuente fue inestabilidad (40,2%), seguido de vértigo (31%). Los diagnósticos más frecuentes fueron vértigo posicional paroxístico benigno (28,2%) y migraña vestibular (28,2%) -definida (59%) o probable (41%)-, seguidos de enfermedad de Ménière (13.8%), trastornos de origen vascular (5.7%), mareo subjetivo crónico (4,6%) y neuritis vestibular (4%). Conclusiones: Disponer de información precisa acerca de la prevalencia e impacto de los trastornos vestibulares es importante para la correcta planificación de los servicios sanitarios con el objetivo de responder satisfactoriamente a la demanda asistencial esperada en la comunidad. El trastorno más frecuentemente diagnosticado en una consulta de Otoneurología es el vértigo posicional paroxístico benigno y la migraña vestibular. Para la comparación de resultados es indispensable la utilización de terminología sistemática (AU)

Introduction and objectives: The set of symptoms relating to disorders of the balance system are common in the general population. However, there are few studies quantifying the frequency of onset of the various vestibular disorders that present in specialist otoneurology units in the Spanish population. The aim of this study is to establish the epidemiology of vestibular disorders, their form of presentation, and the expected care burden in a specialist otoneurology clinic. Material and methods: A retrospective, descriptive, observational study of patients referred to the otoneurology unit of a third level hospital between 1/1/2015 and 31/12/2015. Results: One hundred and seventy-four patients were assessed (121 women and 53 men) with a mean age of 53 ± 17 years. Forty-three individuals per 100,000 inhabitants were assessed over the study period. The patients were referred in the majority from external ENT (36.8%) and primary care (28.7%) clinics. The most frequent reason for consultation was dizziness (40.2%) followed by vertigo (31%). The most frequent diagnoses were benign paroxysmal positional vertigo (28.2%) and vestibular migraine (28.2%) -defined (59%) or probable (41%)-, followed by Ménière's disease (13.8%), vascular disorders (5.7%), chronic subjective dizziness (4.6%) and vestibular neuritis (4%). Conclusions: The availability of precise information on the prevalence and impact of vestibular disorders is important to enable the health services to plan an appropriate response to the expected care demand in the community. Benign paroxysmal positional vertigo and vestibular migraine are the most commonly diagnosed disorders in otoneurology clinics. Systematic terminology is essential for the comparison of results (AU)

Siringocistoadenoma papilífero del conducto auditivo externo. Serie de casos y revisión bibliográfica / Syringocystadenoma papilliferum of the external auditory canal. Case report and literature review

No disponible

Epidemiology of vestibular disorders in the otoneurology unit. / Epidemiología de los trastornos vestibulares en la consulta de otoneurología.

INTRODUCTION AND OBJECTIVES: The set of symptoms relating to disorders of the balance system are common in the general population. However, there are few studies quantifying the frequency of onset of the various vestibular disorders that present in specialist otoneurology units in the Spanish population. The aim of this study is to establish the epidemiology of vestibular disorders, their form of presentation, and the expected care burden in a specialist otoneurology clinic. MATERIAL AND METHODS: A retrospective, descriptive, observational study of patients referred to the otoneurology unit of a third level hospital between 1/1/2015 and 31/12/2015. RESULTS: One hundred and seventy-four patients were assessed (121 women and 53 men) with a mean age of 53±17 years. Forty-three individuals per 100,000 inhabitants were assessed over the study period. The patients were referred in the majority from external ENT (36.8%) and primary care (28.7%) clinics. The most frequent reason for consultation was dizziness (40.2%) followed by vertigo (31%). The most frequent diagnoses were benign paroxysmal positional vertigo (28.2%) and vestibular migraine (28.2%) -defined (59%) or probable (41%)-, followed by Ménière's disease (13.8%), vascular disorders (5.7%), chronic subjective dizziness (4.6%) and vestibular neuritis (4%). CONCLUSIONS: The availability of precise information on the prevalence and impact of vestibular disorders is important to enable the health services to plan an appropriate response to the expected care demand in the community. Benign paroxysmal positional vertigo and vestibular migraine are the most commonly diagnosed disorders in otoneurology clinics. Systematic terminology is essential for the comparison of results.

Cochlear Implant Evaluation: Prognosis Estimation by Data Mining System.

OBJECTIVE: Prediction of speech recognition (SR) and quality of life (QoL) outcomes after cochlear implantation is one of the most important challenges for otologists. By sifting through very large amounts of data, data mining reveals trends, patterns, and relationships that might otherwise have remained undetected. There are identifiable pre-implantational factors that condition the cochlear implantation outcome. Our objective is to design a data mining system to predict and classify cochlear implant (CI) predictable benefits in terms of SR and QoL in each patient. MATERIALS AND METHODS: This is an observational study of CI users for at least one year. Audiological benefits and its relation to QoL are analyzed using the Glasgow Benefit Inventory (GBI) and the Specific Questionnaire (SQ). Sociodemographic and medical variables are processed in SPSS Statistics 19.0, MatLab® and Weka®. Classifiers are designed using the nearest neighbour and decision tree algorithms. Estimators are created by linear logistic regression. RESULTS: A total of 29 patients (mean age, 55.3 years; 52% female and 48% male) including 48% unilateral CI users and 51% bimodal CI users were included in the study. GBI improved by 36 points and SQ by 1.7 (p<0.05). Using Nearest Neighbour (IB1) algorithm for classifiers, interesting attributes were identified for SR and SQ result classification (success rate: 80.7%). Decision tree algorithm (J48) showed influencing variables for GBI (success rate: 81%). Estimators by linear logistic regression analysis disclosed a precision of 85%, 68%, and 71% for SR, GBI, and SQ, respectively. CONCLUSION: Our study proposes a systematized system to classify and estimate SR and QoL improvement based on our initial evaluation to complement decision making and patients' information.

Reducción de la ganancia del canal semicircular posterior en el test del impulso cefálico con la edad. Estudio observacional / Reduction in posterior semicircular canal gain by age in video head impulse testing. Observational study

Introducción: y objetivos: La evaluación detallada del reflejo vestíbulo-ocular con los nuevos sistemas de vídeo disponibles, proporciona información sobre algunas características no identificadas previamente en pacientes con vestibulopatía periférica. El objetivo de este trabajo es cuantificar la frecuencia de aparición de ganancias patológicas, uni- o bilaterales, del canal semicircular posterior (CSP) en la prueba de impulso cefálico vídeo asistida (vHIT) con preservación de la ganancia en el resto de los canales semicirculares, y analizar su relación con la edad y/o el diagnóstico del paciente. Métodos: Estudio prospectivo observacional en adultos entre junio de 2012 y febrero de 2014. Se analizó la edad, género, antecedentes patológicos, historia previa de patología vestibular, diagnóstico, tiempo de evolución del cuadro clínico y sus características. Se completó un examen otoneurológico completo, un vHIT (Otometrics®), y según la sospecha, potenciales evocados vestibulares miogénicos cervicales (cVEMP), audiometría tonal, logoaudiometría así como una evaluación por Neurología y/o un estudio de imagen. Resultados: Trescientos sesenta y tres pacientes fueron evaluados: 57 (16%) pacientes (33 varones, 24 mujeres, edad media 57 ± 15 años) mostraron ganancias patológicas del CSP, 46 (81%) unilateral y 11 (19%) bilateral. El diagnóstico más frecuente fue la enfermedad de Ménière, seguido del VPPB (27%). Los pacientes con afectación unilateral son más jóvenes que los pacientes con afectación bilateral (55 vs. 68 años; p = 0,03). En los pacientes con afectación bilateral, la ganancia del CSP es simétrica en ambos oídos (0,7; p = 0,05) y se relaciona inversamente con la edad (-0,7; 0,04) y el valor de ganancia. La ganancia de los canales anteriores y horizontales tiende a disminuir con la edad, pero sin significación estadística en nuestra muestra. Conclusiones: El 16% de los pacientes remitidos a una consulta de otoneurología muestran ganancias patológicas del CSP, unilateral en el 81% y bilateral en el 19% de los casos. Esta afectación de la ganancia es atribuible a la edad cuando es bilateral y simétrica (AU)

Introduction: and objectives: In-depth assessment of the vestibulo-ocular reflex with the new video systems available provides information on various previously-unidentified characteristics in patients with peripheral vestibular disorder. The aim of this work is to quantify how often pathological gain (uni- or bilateral) in the posterior semicircular canal (PSC) gain appears in video head-impulse testing (vHIT) with preservation of the gain in the rest of the semicircular canals, and to analyse the relation of this frequency with patient age and/or diagnosis. Methods: This was a prospective observational study on adults referred to our unit between June 2012 and February 2014. Age, sex, pathological antecedents, prior history of vestibular pathology, diagnosis and time of evolution of the clinical picture and its characteristics were recorded. Each patient underwent a complete otoneurological examination, including vHIT (Otometrics®). Depending on suspected diagnosis, patients also received cervical vestibular evoked myogenic potential (VEMP) testing, tonal audiometry and speech test, as well as assessment by Neurology and/or nuclear magnetic resonance or computed axial tomography scan imaging study. Results: In all, 363 patients were assessed; 57 (16%) patients (33 males and 24 females; mean age, 57 ± 15 years) showed pathological PSC gains, 46 (81%) unilateral and 11 (19%) bilateral. Ménière's Disease was the most frequent diagnosis, followed by benign paroxysmal positional vertigo (27%). Patients with unilateral involvement were younger than those with bilateral (55 vs. 68 years; p = 0.03). In the patients with bilateral involvement, PSC gain and age were related (-0.7; 0.04) and the gain value was symmetrical in both ears (0.7; p = 0.05). The gain in the anterior and horizontal canals tended to decrease with age, but without statistical significance in our sample. Conclusions: Of the patients referred for an otoneurological consultation, 16% showed pathological PSC gain (unilateral in 81% and bilateral in 19% of the cases). This gain involvement was attributable to age when the gain was bilateral and symmetrical (AU)

Reduction in posterior semicircular canal gain by age in video head impulse testing. Observational study.

INTRODUCTION: and objectives: In-depth assessment of the vestibulo-ocular reflex with the new video systems available provides information on various previously-unidentified characteristics in patients with peripheral vestibular disorder. The aim of this work is to quantify how often pathological gain (uni- or bilateral) in the posterior semicircular canal (PSC) gain appears in video head-impulse testing (vHIT) with preservation of the gain in the rest of the semicircular canals, and to analyse the relation of this frequency with patient age and/or diagnosis. METHODS: This was a prospective observational study on adults referred to our unit between June 2012 and February 2014. Age, sex, pathological antecedents, prior history of vestibular pathology, diagnosis and time of evolution of the clinical picture and its characteristics were recorded. Each patient underwent a complete otoneurological examination, including vHIT (Otometrics®). Depending on suspected diagnosis, patients also received cervical vestibular evoked myogenic potential (VEMP) testing, tonal audiometry and speech test, as well as assessment by Neurology and/or nuclear magnetic resonance or computed axial tomography scan imaging study. RESULTS: In all, 363 patients were assessed; 57 (16%) patients (33 males and 24 females; mean age, 57 ± 15 years) showed pathological PSC gains, 46 (81%) unilateral and 11 (19%) bilateral. Ménière's Disease was the most frequent diagnosis, followed by benign paroxysmal positional vertigo (27%). Patients with unilateral involvement were younger than those with bilateral (55 vs. 68 years; p=0.03). In the patients with bilateral involvement, PSC gain and age were related (-0.7; 0.04) and the gain value was symmetrical in both ears (0.7; p=0.05). The gain in the anterior and horizontal canals tended to decrease with age, but without statistical significance in our sample. CONCLUSIONS: Of the patients referred for an otoneurological consultation, 16% showed pathological PSC gain (unilateral in 81% and bilateral in 19% of the cases). This gain involvement was attributable to age when the gain was bilateral and symmetrical.

Manifestaciones audiovestibulares en la malformación de Chiari tipo I. Serie de casos y revisión bibliográfica / Audio-vestibular signs and symptoms in Chiari malformation type I. Case series and literature review

Introducción: La malformación de Chiari es una alteración de la base del cráneo, en la que se produce herniación del cerebelo y del tronco cerebral a través del foramen magnum. Aunque su forma de presentación más frecuente es la cefalea occipitonucal, no es rara la asociación de síntomas audiovestibulares. El objetivo de nuestro estudio fue conocer las manifestaciones audiovestibulares en la malformación de Chiari tipo i (MCH-I). Material y métodos: Se realizó un estudio retrospectivo observacional de los pacientes remitidos a nuestra unidad en los últimos 5 años, así como una revisión bibliográfica de las manifestaciones audiovestibulares de esta enfermedad. Resultados: Se presentan 9 pacientes (2 varones y 7 mujeres) de 42,8 años de edad media. Cinco de los pacientes consultaron con un síndrome menieriforme, 2 casos como vértigo recurrente de características periféricas, otro como hipoacusia súbita y el último caso como hipoacusia neurosensorial de inicio en la infancia. La manifestación audiovestibular más frecuentemente descrita en la literatura en pacientes es la inestabilidad (49%), seguida de vértigo (18%), nistagmo (15%) e hipoacusia (15%). Dentro del nistagmo, el más frecuente es el nistagmo horizontal (74%) seguido del vertical hacia abajo (18%). Otras manifestaciones audiovestibulares son acúfenos (11%), plenitud ótica (10%) e hiperacusia (1%). La cefalea occipitonucal que aumenta con las maniobras de Valsalva y las parestesias en las manos son muy sugestivos de esta enfermedad. Conclusiones: La aparición de síntomas audiovestibulares en la MCH-I hace que sea relativamente frecuente su derivación al otoneurólogo. Estos pacientes presentan inestabilidad, síndrome vertiginoso e hipoacusia neurosensorial. No es rara la presencia de nistagmo, sobre todo horizonal y vertical hacia abajo. Es importante la familiarización de los otoneurólogos con la sintomatología de esta enfermedad de cara a su diagnóstico diferencial (AU)

Introduction: Chiari malformation is an alteration of the base of the skull with herniation through the foramen magnum of the brain stem and cerebellum. Although the most common presentation is occipital headache, the association of audio-vestibular symptoms is not rare. The aim of our study was to describe audio-vestibular signs and symptoms in Chiari malformation type i (CM-I). Materials and methods: We performed a retrospective observational study of patients referred to our unit during the last 5 years. We also carried out a literature review of audio-vestibular signs and symptoms in this disease. Results: There were 9 patients (2 males and 7 females), with an average age of 42.8 years. Five patients presented a Ménière-like syndrome; 2 cases, a recurrent vertigo with peripheral features; one patient showed a sudden hearing loss; and one case suffered a sensorineural hearing loss with early childhood onset. The most common audio-vestibular symptom indicated in the literature in patients with CM-I is unsteadiness (49%), followed by dizziness (18%), nystagmus (15%) and hearing loss (15%). Nystagmus is frequently horizontal (74%) or down-beating (18%). Other audio-vestibular signs and symptoms are tinnitus (11%), aural fullness (10%) and hyperacusis (1%). Occipital headache that increases with Valsalva manoeuvres and hand paresthesias are very suggestive symptoms. Conclusions: The appearance of audio-vestibular manifestations in CM-I makes it common to refer these patients to neurotologists. Unsteadiness, vertiginous syndromes and sensorineural hearing loss are frequent. Nystagmus, especially horizontal and down-beating, is not rare. It is important for neurotologists to familiarise themselves with CM-I symptoms to be able to consider it in differential diagnosis (AU)

Audio-vestibular signs and symptoms in Chiari malformation type i. Case series and literature review.

INTRODUCTION: Chiari malformation is an alteration of the base of the skull with herniation through the foramen magnum of the brain stem and cerebellum. Although the most common presentation is occipital headache, the association of audio-vestibular symptoms is not rare. The aim of our study was to describe audio-vestibular signs and symptoms in Chiari malformation type i (CM-I). MATERIALS AND METHODS: We performed a retrospective observational study of patients referred to our unit during the last 5 years. We also carried out a literature review of audio-vestibular signs and symptoms in this disease. RESULTS: There were 9 patients (2 males and 7 females), with an average age of 42.8 years. Five patients presented a Ménière-like syndrome; 2 cases, a recurrent vertigo with peripheral features; one patient showed a sudden hearing loss; and one case suffered a sensorineural hearing loss with early childhood onset. The most common audio-vestibular symptom indicated in the literature in patients with CM-I is unsteadiness (49%), followed by dizziness (18%), nystagmus (15%) and hearing loss (15%). Nystagmus is frequently horizontal (74%) or down-beating (18%). Other audio-vestibular signs and symptoms are tinnitus (11%), aural fullness (10%) and hyperacusis (1%). Occipital headache that increases with Valsalva manoeuvres and hand paresthesias are very suggestive symptoms. CONCLUSIONS: The appearance of audio-vestibular manifestations in CM-I makes it common to refer these patients to neurotologists. Unsteadiness, vertiginous syndromes and sensorineural hearing loss are frequent. Nystagmus, especially horizontal and down-beating, is not rare. It is important for neurotologists to familiarise themselves with CM-I symptoms to be able to consider it in differential diagnosis.

Neurofibromatosis tipo II y sus manifestaciones en cabeza y cuello: revisión bibliográfica y estudio poblacional en la Comunidad de Cantabria y la provincia de Las Palmas / Neurofibromatosis type 2 and its head and neck manifestations: Literature review and population study in the Community of Cantabria and the province of Las Palmas

Introducción y objetivos: La neurofibromatosis tipo II (NFII) es una enfermedad infrecuente de herencia autosómica dominante que se caracteriza por la aparición de schwanomas del VIII par, alteraciones oculares y meningiomas. Se estima una incidencia de NFII de 1:25.000 y una prevalencia mayor de 1:80.000. Los objetivos de nuestro estudio fueron determinar la prevalencia puntual de NFII en la Comunidad de Cantabria y la provincia de Las Palmas, así como caracterizar sus manifestaciones de cabeza y cuello. Material y métodos: Se trata de un estudio poblacional, retrospectivo, en 3 hospitales de tercer nivel. Resultados: El estudio poblacional mostró una prevalencia puntual de 1:600.000 en la Comunidad de Cantabria y 1:280.000 en la provincia de Las Palmas. El tumor más frecuentemente diagnosticado fue el neurinoma del acústico (n = 15), seguido del neurinoma del trigémino (n = 2) y del vago (n = 1). Conclusiones: La NF II en Cantabria y Las Palmas es infrecuente, menor a la descrita en la literatura. El tumor de cabeza y cuello más frecuentemente descrito en la literatura es el neurinoma del acústico seguido del schwanoma del v y del x par. Están descritos aisladamente otros tumores, como el schwanoma nasal, laríngeo, de chorda timpanae o del VII par. La manifestación ORL más frecuente es la hipoacusia, sobre todo unilateral, seguida de masa cervical, acúfenos y cefalea. Un diagnóstico precoz y el manejo multidisciplinar en centros especializados podrían mejorar la esperanza y calidad de vida en estos pacientes (AU)

Introduction and objectives: Neurofibromatosis type 2 (NF2) is an infrequent autosomal dominant disease characterised by the appearance of VIII nerve schwannomas, meningiomas and ocular abnormalities. Incidence of 1:25,000 and prevalence above 1:80,000 are estimated in general. The objectives of our study were to determine current prevalence of NF2 in the Community of Cantabria and the province of Las Palmas, and its head and neck manifestations. Material and methods: This was a population-based, retrospective study in 3 tertiary hospitals. Results: The study population showed prevalence of 1:600,000 in the Community of Cantabria and 1:280,000 in the province of Las Palmas. The most frequently diagnosed tumour was acoustic neuroma (n = 15), followed by trigeminal neurinoma (n = 2) and vagus (n = 1). Conclusions: Cases of NF2 are infrequent in Cantabria and Las Palmas, lower than that reported in the literature. The most frequently described head and neck tumour in the literature is acoustic neuroma, followed by schwannoma of cranial nerves v and x. Other tumours such as nasal, laryngeal, chorda tympani or cranial nerve vii schwannomas are also described. The most frequent ENT manifestation is hearing loss, especially unilateral, followed by cervical mass, tinnitus and headache. Early diagnosis and multidisciplinary management in specialised centres could improve life expectancy and quality of life for these patients (AU)

Trastorno congénito de la glucosilación: experiencia en el primer paciente hipoacúsico tratado con implante coclear / Congenital disorders of glycosylation: first deaf patient treated with a cochlear implant

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[Neurofibromatosis type 2 and its head and neck manifestations: literature review and population study in the Community of Cantabria and the province of Las Palmas]. / Neurofibromatosis tipo ii y sus manifestaciones en cabeza y cuello: revisión bibliográfica y estudio poblacional en la Comunidad de Cantabria y la provincia de Las Palmas.

INTRODUCTION AND OBJECTIVES: Neurofibromatosis type 2 (NF2) is an infrequent autosomal dominant disease characterised by the appearance of viii nerve schwannomas, meningiomas and ocular abnormalities. Incidence of 1:25,000 and prevalence above 1:80,000 are estimated in general. The objectives of our study were to determine current prevalence of NF2 in the Community of Cantabria and the province of Las Palmas, and its head and neck manifestations. MATERIAL AND METHODS: This was a population-based, retrospective study in 3 tertiary hospitals. RESULTS: The study population showed prevalence of 1:600,000 in the Community of Cantabria and 1:280,000 in the province of Las Palmas. The most frequently diagnosed tumour was acoustic neuroma (n=15), followed by trigeminal neurinoma (n=2) and vagus (n=1). CONCLUSIONS: Cases of NF2 are infrequent in Cantabria and Las Palmas, lower than that reported in the literature. The most frequently described head and neck tumour in the literature is acoustic neuroma, followed by schwannoma of cranial nerves v and x. Other tumours such as nasal, laryngeal, chorda tympani or cranial nerve vii schwannomas are also described. The most frequent ENT manifestation is hearing loss, especially unilateral, followed by cervical mass, tinnitus and headache. Early diagnosis and multidisciplinary management in specialised centres could improve life expectancy and quality of life for these patients.

Beneficios comunicativos de la implantación coclear bilateral. Estudio retrospectivo en niños de 12 años / Communication benefits of bilateral cochlear implantation. Retrospective study in 12-year-old children

Introducción y objetivos: Algunos estudios sugieren que la implantación coclear (IC) simultánea o secuencial en un corto periodo de tiempo aporta beneficios adicionales. Existe controversia acerca de la existencia de una edad límite a partir de la cual la segunda implantación aporta un beneficio menor en la adquisición de habilidades comunicativas. Los objetivos de nuestro estudio consisten en corroborar que la implantación coclear secuencial aporta beneficios sobre la unilateral, y estudiar si existen a los 12 años diferencias significativas según la edad en el momento de la segunda implantación. Métodos: Se trata de un estudio observacional descriptivo sobre una población de 12 años portadores de implante coclear (n = 69). Para evaluar los beneficios audiológicos se llevó a cabo una audiometría tonal liminar y un test de discriminación verbal a campo libre (bisílabos, frases cotidianas en contexto abierto, con y sin ruido). Resultados: Los resultados en términos de discriminación verbal fueron mejores en los pacientes implantados antes de los 2 años de edad, sin embargo no existen diferencias estadísticamente significativas (p > 0,5). Los usuarios de IC bilateral implantados antes de los 2 años y con un periodo entre interimplante inferior a 4 años muestran mejores porcentajes de discriminación verbal (p < 0,05). Conclusiones: La implantación coclear precoz y con un corto periodo interimplante demuestra en nuestra muestra proveer a los pacientes beneficios significativos en términos de inteligibilidad. Parece haber una edad y un periodo interimplante concreto a partir de la cual se reduce el beneficio auditivo sobre el primer implante (AU)

Introduction and objectives: Some studies suggest that simultaneous or sequential cochlear implantation in a short period of time offers additional benefits. There is controversy regarding the existence of an age limit after which a second implantation offers less benefit for the acquisition of communication skills. The objectives of this study were to confirm that sequential cochlear implantation offers benefits compared to unilateral implantation and to study whether, at 12 years of age, there are significant differences regarding the age at the time of the second implantation. Methods: Descriptive and observational study of a population of 12-year-old children carrying cochlear implants (n=69). A liminal pure tone audiometry and an open-field verbal discrimination test (disyllables, common phrases in an open context, with and without noise) were conducted to evaluate audiological benefits. Results: Verbal discrimination results were better among patients who had been implanted before the age of 2 years, although the differences were not statistically significant (P>0 . 5). Children who had received bilateral cochlear implants before the age of 2 years and with a period less than 4 years between both implants presented better verbal discrimination percentages (P<0 . 05). Conclusions: In our sample, early cochlear implantation with a short period between both implants provided significant benefits regarding intelligibility. There seem to be a specific age and interimplant period, after which the auditory benefit on the first implant becomes reduced (AU)