The vestibular calyceal junction is dismantled following subchronic streptomycin in rats and sensory epithelium stress in humans.

Hair cell (HC) loss by epithelial extrusion has been described to occur in the rodent vestibular system during chronic 3,3'-iminodipropionitrile (IDPN) ototoxicity. This is preceded by dismantlement of the calyceal junction in the contact between type I HC (HCI) and calyx afferent terminals. Here, we evaluated whether these phenomena have wider significance. First, we studied rats receiving seven different doses of streptomycin, ranging from 100 to 800 mg/kg/day, for 3-8 weeks. Streptomycin caused loss of vestibular function associated with partial loss of HCI and decreased expression of contactin-associated protein (CASPR1), denoting calyceal junction dismantlement, in the calyces encasing the surviving HCI. Additional molecular and ultrastructural data supported the conclusion that HC-calyx detachment precede HCI loss by extrusion. Animals allowed to survive after the treatment showed functional recuperation and rebuilding of the calyceal junction. Second, we evaluated human sensory epithelia obtained during therapeutic labyrinthectomies and trans-labyrinthine tumour excisions. Some samples showed abnormal CASPR1 label strongly suggestive of calyceal junction dismantlement. Therefore, reversible dismantlement of the vestibular calyceal junction may be a common response triggered by chronic stress, including ototoxic stress, before HCI loss. This may partly explain clinical observations of reversion in function loss after aminoglycoside exposure.

Pre- and intra-operative prognostic factors of facial nerve function in cerebellopontine angle surgery.

PURPOSE: The study assesses whether pre- and intraoperative factors linked to electromyography and direct electrical stimulation (DES) of facial nerve can predict facial nerve function in the short- (12 days) and long-term (1 year) after cerebellopontine angle (CPA) tumor resection. METHODS: 157 patients who underwent surgical resection of CPA tumors with facial nerve monitoring. Pre-operative factors (age, tumor size, pure tone average), surgical time and intra-operative parameters regarding facial function, minimum stimulation threshold (MST), compound muscle action potential (CMAP) and the difference between proximal and distal CMAP (DPDC) were evaluated. RESULTS: A correlation between tumor size, MST, CMAP and facial function in both short and long term was found. A higher grade of immediate facial paralysis corresponded to a higher risk of poor outcome after one year. A postoperative House-Brackmann (HB) score of V or VI was correlated with poor outcome in 88.8% and 93.8% of cases. A risk of HB 3 or more, in the long term, was correlated with a tumor size of 20.2 mm. Using an MST of 0.1 mA, for long-term predictions, sensitivity and specificity were 0.62 (95% CI 0.46-0.75) and 0.73 (95% CI 0.61-0.82), respectively. With a CMAP cut-off < 200 µV, for long-term prediction, sensitivity was 0.73 (95% CI 0.53-0.87) and specificity 0.73 (95% CI 0.55-0.85). CONCLUSION: The assessment based on the cut-offs described increases the ability to predict facial function. Improving predictive accuracy enables surgeons to address patients' expectations and to establish an intervention timeline for planning facial reanimation.

Adaptation and initial validation of the Spanish version of the chronic otitis media questionnaire-12 (COMQ-12) and chronic otitis media benefit inventory (COMBI)

Background and objectives: The chronic otitis media questionnaire-12 (COMQ-12) and chronic otitis media benefit inventory (COMBI) aim to assess the quality of life related to chronic otitis media or its surgical treatment. The objective of this study was to validate the Spanish adaptation of the COMQ-12 and COMBI.Patients and methodsMulticentre prospective instrument validation study. guidelines for the cross-cultural adaptation process were followed. The psychometric properties of the Spanish versions (“COMQ-12-Spa” for “COMQ-12-Spanish” and “COMBI-Spa” for “COMBI-Spanish”) were assessed in 25 consecutive adults undergoing tympanoplasty and in a separate cohort of 25 unaffected adults.ResultsCOMQ-12-Spa: test–retest reliability, r=0.98; internal consistency reliability, α=0.92. Item-subscale and item-total coefficients, ≥0.40. Between-group discrimination, p<0.0001. COMBI-Spa: internal consistency, α=0.96. A high-intensity relationship between the variables was shown, ≥0.40. Correlation between the COMBI-Spa and the Spanish Glasgow Benefit Inventory (GBI), r=0.89; p<0.01.ConclusionPsychometric testing of the Spanish versions of the COMQ-12-Spa and COMBI-Spa yielded satisfactory results, thus allowing pre- and postintervention assessment of the subjective severity of chronic otitis media in adult patients. (AU)

Antecedentes y objetivo: El Cuestionario de otitis media crónica-12 (COMQ-12) y el Inventario de beneficios en otitis media crónica (COMBI) valoran la calidad de vida relacionada con la otitis media crónica o su tratamiento quirúrgico. El objetivo del estudio fue validar la adaptación española de los originales británicos.Pacientes y métodosEstudio multicéntrico prospectivo de validación de instrumento. Se siguieron las pautas para el proceso de adaptación transcultural. Las propiedades psicométricas de las versiones españolas, COMQ-12-Spa y COMBI-Spa, fueron evaluadas en 25 adultos consecutivos intervenidos de timpanoplastia y en una cohorte separada de 25 adultos sanos.ResultadosCOMQ-12-Spa: fiabilidad test-retest, r=0,98; consistencia interna, α=0,92; coeficientes ítem-subescala e ítem-total, ≥0,40; discriminación entre grupos, p<0,0001. COMBI-Spa: consistencia interna, α=0.96. Se encontró una relación de intensidad elevada entre las variables, ≥0,40. Correlación entre COMBI-Spa y Glasgow Benefit Inventory español (GBI), r=0,89; p<0,01.ConclusionesLas pruebas psicométricas de las versiones españolas COMQ-12-Spa y COMBI-Spa ofrecieron resultados satisfactorios, lo que permite la valoración pre y postintervención de la severidad subjetiva de la otitis media crónica en pacientes adultos. (AU)

Adaptation and initial validation of the Spanish version of the chronic otitis media questionnaire-12 (COMQ-12) and chronic otitis media benefit inventory (COMBI).

BACKGROUND AND OBJECTIVES: The chronic otitis media questionnaire-12 (COMQ-12) and chronic otitis media benefit inventory (COMBI) aim to assess the quality of life related to chronic otitis media or its surgical treatment. The objective of this study was to validate the Spanish adaptation of the COMQ-12 and COMBI. PATIENTS AND METHODS: Multicentre prospective instrument validation study. guidelines for the cross-cultural adaptation process were followed. The psychometric properties of the Spanish versions ("COMQ-12-Spa" for "COMQ-12-Spanish" and "COMBI-Spa" for "COMBI-Spanish") were assessed in 25 consecutive adults undergoing tympanoplasty and in a separate cohort of 25 unaffected adults. RESULTS: COMQ-12-Spa: test-retest reliability, r=0.98; internal consistency reliability, α=0.92. Item-subscale and item-total coefficients, ≥0.40. Between-group discrimination, p<0.0001. COMBI-Spa: internal consistency, α=0.96. A high-intensity relationship between the variables was shown, ≥0.40. Correlation between the COMBI-Spa and the Spanish Glasgow Benefit Inventory (GBI), r=0.89; p<0.01. CONCLUSION: Psychometric testing of the Spanish versions of the COMQ-12-Spa and COMBI-Spa yielded satisfactory results, thus allowing pre- and postintervention assessment of the subjective severity of chronic otitis media in adult patients.

CT Assessment of Otic Capsule Bone Density in Paget's Disease of the Temporal Bone and Its Relationship With Hearing Loss.

OBJECTIVES: To study computed tomography findings in Paget's disease of temporal bone (PDTB) and analyze the relations between otic capsule bone mineral density values measured in Hounsfield Units (HU) and hearing loss (HL). STUDY DESIGN: Observational case-control study. SETTING: Tertiary referral center. PATIENTS: Radiographically confirmed PDTB cases and control group. INTERVENTION: Diagnostic. MAIN OUTCOME MEASURES: Hearing thresholds and computed tomography bone density values. RESULTS: Twenty-three ears in the case group (PDTB) and 27 control ears were included. In the PDTB group, HL was found in 87% of the ears (43% mixed) and an air-conduction threshold of 50.7 dB (SD = 19.8). In the control group, 48% of the ears showed HL (7% mixed) and an air-conduction threshold of 34.5 dB (SD = 20.6) was found; the difference was statistically significant (p < 0.05). Measurements of bone density (HU) in the otic capsule (regions of interest [ROI] 1 and 2) and in the petrous bone (ROI 3) were significantly lower (p < 0.05) in the PDTB group than in controls.The PDTB group presented a significant association between otic capsule bone density in ROI 1 and mean otic capsule density with air and bone-conduction thresholds (p < 0.05). In controls, no association was observed between any density value and audiometric thresholds. CONCLUSION: PDTB patients showed more frequent HL, lower thresholds, and a higher proportion of mixed HL than controls. Bone density (HU) was decreased in all ROIs in PDTB patients in comparison with controls. Bone density in the otic capsule was associated with HL in PDTB patients, but no association was observed between bone density and HL in controls.

La audición en la enfermedad ósea de Paget / Hearing in Paget's disease of bone

Introducción y objetivos: La enfermedad ósea de Paget (EOP) puede cursar con hipoacusia. Con el objetivo de cuantificar, caracterizar y determinar los factores de riesgo de hipoacusia en un grupo de pacientes con EOP se realiza el presente estudio. Métodos: Se realizó un estudio observacional, transversal del tipo casos y controles que incluyó una cohorte de 76 sujetos con diagnóstico de EOP en el grupo caso y un grupo control de 134 sujetos. Se analiza la información clínica, demográfica y audiométrica de los sujetos incluidos. Resultados: El análisis comparativo entre el grupo de sujetos con EOP y el grupo control determinó que el grupo caso presentaba un umbral medio auditivo mayor (39,51dB) que el grupo control (37,28 dB) (p = 0,069) y que presentaba hipoacusia transmisiva con mayor frecuencia (22,76%) que el grupo control (12,05%) (p = 0,0062). El análisis de los factores de riesgo de hipoacusia determinó que la afectación craneal en la gammagrafía ósea, la edad y la HTA, entre otros, constituían factores de riesgo de mayor pérdida auditiva en la EOP. Conclusiones: Los sujetos con EOP presentaron una pérdida auditiva más severa y con mayor frecuencia de tipo transmisivo que el grupo control. Los sujetos con afectación de la calota craneal por EOP presentaron mayor pérdida auditiva que los sujetos sin afectación craneal. La afectación de la calota craneal por la EOP y la edad constituyeron factores de riesgo de hipoacusia

Introduction and objectives: Paget's disease of bone (PDB) may lead to hearing loss. The present study was conducted with the aim of measuring, characterizing and determining the risk factors for hearing loss in a group of subjects with PDB. Methods: An observational, transversal, case-control study was conducted, a cohort of 76 subjects diagnosed with PDB in the case group and a control group of 134 subjects were included. Clinical, demographic and audiometric data were analysed. Results: The comparative analysis between the subjects in the PDB group and the control group found that the case group showed higher hearing thresholds (39,51 dB) compared with the control group (37.28 dB) (P=.069) and presented a greater rate of conductive hearing loss (22.76%) than the control group (12.05%) (P=.0062). The study of risk factors for hearing loss found that skull involvement in bone scintigraphy, age and high blood pressure were risk factors for higher impairment in PDB. Conclusions: The subjects with PDB showed more profound and a higher proportion of conductive hearing loss than the control group. The patients with PDB and skull involvement presented a more severe hearing loss compared with the subjects without skull involvement. Skull involvement and age were found to be risk factors for hearing loss

Hearing in Paget's disease of bone. / La audición en la enfermedad ósea de Paget.

INTRODUCTION AND OBJECTIVES: Paget's disease of bone (PDB) may lead to hearing loss. The present study was conducted with the aim of measuring, characterizing and determining the risk factors for hearing loss in a group of subjects with PDB. METHODS: An observational, transversal, case-control study was conducted, a cohort of 76 subjects diagnosed with PDB in the case group and a control group of 134 subjects were included. Clinical, demographic and audiometric data were analysed. RESULTS: The comparative analysis between the subjects in the PDB group and the control group found that the case group showed higher hearing thresholds (39,51dB) compared with the control group (37.28dB) (P=.069) and presented a greater rate of conductive hearing loss (22.76%) than the control group (12.05%) (P=.0062). The study of risk factors for hearing loss found that skull involvement in bone scintigraphy, age and high blood pressure were risk factors for higher impairment in PDB. CONCLUSIONS: The subjects with PDB showed more profound and a higher proportion of conductive hearing loss than the control group. The patients with PDB and skull involvement presented a more severe hearing loss compared with the subjects without skull involvement. Skull involvement and age were found to be risk factors for hearing loss.

Nuestra experiencia con la monitorización del nervio facial en cirugía del schwannoma del vestibular bajo bloqueo neuromuscular parcial / Our experience with facial nerve monitoring in vestibular schwannoma surgery under partial neuromuscular blockade

Introducción y objetivos: La monitorización del nervio facial es fundamental en la preservación del nervio facial en la cirugía del schwannoma del nervio vestibular. Nuestro objetivo es analizar la utilidad de la monitorización facial bajo bloqueo neuromuscular parcial. Métodos: Análisis retrospectivo de 69 pacientes operados en un hospital de nivel terciario. Resultados: En el 100% de los casos se pudo realizar monitorización electromiográfica. Se obtuvo respuesta electromiográfica tras la escisión tumoral en el 75% de los casos. En 17 casos no hubo respuesta electromiográfica. En 15 hubo lesión de continuidad del nervio facial y en 2 hubo lesión pero se logró mantener la continuidad anatómica del nervio. La presencia de parálisis facial preoperatoria (29 vs. 7%; p = 0,0349), el mayor tamaño tumoral (88 vs. 38%; p = 0,0276) y una audición no funcional (88 vs. 51%; p = 0,0276) son factores significativamente más prevalentes en el grupo en que no se pudo obtener una respuesta electromiográfica. Conclusiones: La monitorización del nervio facial bajo bloqueo neuromuscular moderado es factible y segura en pacientes sin lesión facial preoperatoria. Los pacientes que obtienen respuesta electromiográfica tras la extirpación del tumor tienen mejor función facial en el postoperatorio y al año de seguimiento (AU)

Introduction and objectives: Facial nerve monitoring is fundamental in the preservation of the facial nerve in vestibular schwannoma surgery. Our objective was to analyse the usefulness of facial nerve monitoring under partial neuromuscular blockade. Methods: This was a retrospective analysis of 69 patients operated in a tertiary hospital. Results: We monitored 100% of the cases. In 75% of the cases, we could measure an electromyographic response after tumour resection. In 17 cases, there was an absence of electromyographic response. Fifteen of them had an anatomic lesion with loss of continuity of the facial nerve and, in 2 cases, there was a lesion with preservation of the nerve. Preoperative facial palsy (29% 7%; P=.0349), large tumour size (88 vs. 38%; P=.0276), and a non-functional audition (88 vs. 51%; P=.0276) were significantly related with an absence of electromyographic response. Conclusions: Facial nerve monitoring under neuromuscular blockade is possible and safe in patients without previous facial palsy. If the patient had an electromyographic response after tumour excision, they developed better facial function in the postoperative period and after a year of follow up (AU)

Our experience with facial nerve monitoring in vestibular schwannoma surgery under partial neuromuscular blockade.

INTRODUCTION AND OBJECTIVES: Facial nerve monitoring is fundamental in the preservation of the facial nerve in vestibular schwannoma surgery. Our objective was to analyse the usefulness of facial nerve monitoring under partial neuromuscular blockade. METHODS: This was a retrospective analysis of 69 patients operated in a tertiary hospital. RESULTS: We monitored 100% of the cases. In 75% of the cases, we could measure an electromyographic response after tumour resection. In 17 cases, there was an absence of electromyographic response. Fifteen of them had an anatomic lesion with loss of continuity of the facial nerve and, in 2 cases, there was a lesion with preservation of the nerve. Preoperative facial palsy (29% 7%; P=.0349), large tumour size (88 vs. 38%; P=.0276), and a non-functional audition (88 vs. 51%; P=.0276) were significantly related with an absence of electromyographic response. CONCLUSIONS: Facial nerve monitoring under neuromuscular blockade is possible and safe in patients without previous facial palsy. If the patient had an electromyographic response after tumour excision, they developed better facial function in the postoperative period and after a year of follow up.

Neurofibromatosis tipo 2 / Neurofibromatosis type 2

La neurofibromatosis tipo 2 es una enfermedad invalidante que se hereda de forma autosómica dominante. A menudo se ha confundido con la neurofibromatosis tipo 1, aunque son patologías distintas. Todos los sujetos que hereden una mutación en el gen de la neurofibromatosis tipo 2 (NF2), desarrollarán dicha enfermedad, caracterizada por el crecimiento de schwanomas, habitualmente vestibulares y de forma bilateral, así como meningiomas u otros tumores benignos del sistema nervioso central, antes de los 30 años de edad. Actualmente, podemos identificar la mutación del NF2 en la mayoría de las familias afectas. Hasta un 20% de los pacientes afectos de NF2 sin historia familiar, aparentemente casos esporádicos, son en realidad individuos con mosaicismo para esa mutación. La morbilidad de esos tumores es en gran medida debida a su tratamiento, que es principalmente quirúrgico. Cuando son pequeños, los schwanomas vestibulares se pueden resecar completamente con preservación tanto de la función auditiva como facial. En caso de tumores grandes se puede colocar un implante coclear o bien de tronco cerebral durante el mismo acto quirúrgico. Los principales factores pronósticos son: la edad media al diagnóstico, la presencia de meningiomas intracraneales y si el paciente fue tratado o no en un centro especializado (AU)

Type 2 neurofibromatosis (NF2) is an invalidating, inherited, dominant, autosomal disease. It is commonly confused with type 1 neurofibromatosis, although the two disorders are different. All subjects who inherit a mutated NF2 gene will develop the disease, which is characterised by the growth of schwannomas, generally affecting the vestibular nerve bilaterally, as well as meningiomas and other benign central nervous system tumours, before their third decade of life. It is currently possible to identify the NF2 mutation in most affected families. Up to about 20% of NF2 patients with no family history, apparently sporadic cases, are actually individuals with mosaicism for this mutation. Much of the morbidity from these tumours results from their treatment, which is primarily surgical. Small vestibular schwannomas can often be completely resected with preservation of both hearing and facial function. In case of large tumours it is possible to place a cochlear or brain stem implant during the schwannoma surgery. Age at diagnosis, the presence of intracranial meningiomas, and whether the patient was treated at a specialty centre or not, have been cited as the strongest prognostic factors (AU)

[Neurofibromatosis type 2]. / Neurofibromatosis tipo 2.

Type 2 neurofibromatosis (NF2) is an invalidating, inherited, dominant, autosomal disease. It is commonly confused with type 1 neurofibromatosis, although the two disorders are different. All subjects who inherit a mutated NF2 gene will develop the disease, which is characterised by the growth of schwannomas, generally affecting the vestibular nerve bilaterally, as well as meningiomas and other benign central nervous system tumours, before their third decade of life. It is currently possible to identify the NF2 mutation in most affected families. Up to about 20% of NF2 patients with no family history, apparently sporadic cases, are actually individuals with mosaicism for this mutation. Much of the morbidity from these tumours results from their treatment, which is primarily surgical. Small vestibular schwannomas can often be completely resected with preservation of both hearing and facial function. In case of large tumours it is possible to place a cochlear or brain stem implant during the schwannoma surgery. Age at diagnosis, the presence of intracranial meningiomas, and whether the patient was treated at a specialty centre or not, have been cited as the strongest prognostic factors.