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The biallelic inheritance of an expanded intronic pentamer (AAGGG)A retrospective descriptive study from an ataxia database comprising 500 patients.The study was performed at the Otorhinolaryngology Department of a hospital in the north of Spain.Specific genetic testing for CANVAS was performed in 13 patients with clinical suspicion of complete or incomplete syndrome. The clinical diagnosis was supported by quantitative vestibular hypofunction, cerebellar atrophy, and abnormal sensory nerve conduction testing.Nine of 13 (69%) patients met clinical diagnostic criteria for definite CANVAS disease. The first manifestation of the syndrome was lower limb dysesthesia in 8 of 13 patients and gait imbalance in 5 of 13. Eleven of 13 (85%) patients were carriers of the biallelic (AAGGG)A genetic cause of CANVAS has recently been discovered. We propose genetic screening for biallelic expansions of the AAGGG pentamer of

Resumen Introduccion La hipoacusia neurosensorial (HNS) es el deficit sensorial mas prevalente en nuestro medio. La secuenciacion genomica de nueva generacion (NGS) permite obtener un diagnostico etiologico en un alto porcentaje de pacientes. Nuestro estudio piloto muestra los resultados de la aplicacion sistematica de la NGS en una Unidad de Hipoacusia Infantil, asi como sus implicaciones en el manejo clinico de los pacientes y sus familiares. Material y metodo Se incluyo a 27 pacientes diagnosticados de HNS entre 2014 y 2017 en los que se descarto una causa ambiental. El test genetico consistio en un panel de genes analizados mediante NGS (panel OTOgenicsTM). Este panel ha sido disenado para incluir genes asociados con hipoacusia neurosensorial o mixta, de inicio precoz o tardio, sindromica y no sindromica, independientemente de su patron de herencia. Resultados Se obtuvo un diagnostico genetico en el 56% (15/27) de los pacientes (62% en el caso de las HNS bilaterales); 5/27 (19%) presentaron variantes patogenicas en el gen GJB2 y el resto variantes patogenicas o probablemente patogenicas en otros genes asociados con HNS aislada (PR2X2, TECTA y STRC), con HNS sindromicas (CHD7, GATA3, COL4A5, MITF y SOX10) o con HNS sindromicas y no sindromicas (BSND, ACTG1 y CDH23). Discusion El diagnostico etiologico de la HNS supone un desafio en la practica clinica. Nuestra serie demuestra que es posible implementar el diagnostico genetico en la rutina asistencial y que esta informacion tiene implicaciones pronosticas y terapeuticas.

Introduction Sensorineural hearing loss (SNL) is the most prevalent sensory deficit in our environment. The next generation genomic sequencing (NGS) allows to obtain an etiological diagnosis in a high percentage of patients. Our pilot study shows the results of the systematic application of NGS in a Childhood Hearing Loss Unit, as well as its implications in the clinical management of patients and their families. Material and method We included 27 patients diagnosed with SNH between 2014 and 2017, in which an environmental cause was ruled out. The genetic test consisted of a panel of genes analyzed by NGS (OTOgenics™ panel). This panel has been designed to include genes associated with sensorineural or mixed hearing loss, early onset or late, syndromic and non-syndromic, regardless of their inheritance pattern. Results A genetic diagnosis was obtained in 56% (15/27) of the patients (62% in the case of bilateral HNS). 5/27 (19%) presented pathogenic variants in the GJB2 gene and the rest variants pathogenic and/or probably pathogenic in other genes associated with isolated HNS (PR2 × 2, TECTA and STRC), with syndromic HNS (CHD7, GATA3, COL4A5, MITF and SOX10) or with syndromic and non-syndromic HNS (BSND, ACTG1 and CDH23). Discussion The etiological diagnosis of SNL is a challenge in clinical practice. Our series demonstrates that it is possible to implement genetic diagnosis in the daily routine and that this information has prognostic and therapeutic implications.

Resumen La paralisis de Bell es la forma mas comun de paresia o paralisis facial. Sin embargo, no todos los pacientes con paralisis facial tienen una paralisis de Bell. Otras causas frecuentes incluyen las secuelas del tratamiento del neurinoma del viii par, el cancer de cabeza y cuello, la iatrogenia, el zoster otico y los traumatismos. El abordaje de cada una de estas situaciones es totalmente diferente. El objetivo de esta guia es servir de consejo para el tratamiento y el seguimiento de los pacientes con paralisis facial. Nuestra idea es que la guia sea practica, haciendo hincapie en recomendaciones efectivas y utiles en el manejo diario de los pacientes. Esta guia ha sido promovida por la Sociedad Espanola de ORL y escrita por medicos con experiencia en la enfermedad del nervio facial, incluyendo al menos un especialista de cada comunidad autonoma. Redactada en un formato de preguntas y respuestas, incluye 56 cuestiones relevantes relacionadas con el nervio facial.

Bell's palsy is the most common diagnosis associated with facial nerve weakness or paralysis. However, not all patients with facial paresis/paralysis have Bell's palsy. Other common causes include treatment of vestibular schwannoma, head and neck tumours, iatrogenic injuries, Herpes zoster, or trauma. The approach to each of these conditions varies widely. The purpose of this guideline is to provide clinicians with guidance on the treatment and monitoring of patients with different causes of facial paralysis. We intend to draft a practical guideline, focusing on operationalised recommendations deemed to be useful in the daily management of patients. This guideline was promoted by the Spanish Society of Otolaryngology and developed by a group of physicians with an interest in facial nerve disorders, including at least one physician from each Autonomous Community. In a question and answer format, it includes 56 relevant topics related to the facial nerve.

Resumen Antecedentes y objetivo El riesgo de meningitis bacteriana aumenta en los pacientes con implante coclear. Por ello, se indica la vacunacion antineumococica, antigripal y frente a Haemophilus influenzae tipo b en este grupo. El objetivo del presente estudio es conocer el cumplimiento del calendario vacunal en los pacientes implantados en un hospital de referencia. Materiales y metodos Se incluyeron los pacientes con implante coclear intervenidos entre 2005 y 2015. Se evaluaron las coberturas vacunales frente a gripe estacional, Haemophilus influenzae tipo b, neumococo conjugada de 13 serotipos y neumococo polisacarida de 23 serotipos. Se dividio la muestra en 2 grupos por edad ( Resultados De los 153 pacientes estudiados (28,01% 0-13 anos y 71,9% ≥ 14), solo 2 (5,71%) tuvieron un 100% de adherencia al calendario vacunal, mientras que el 65,71% registro un cumplimiento del 50% o menor. Globalmente, la cobertura de vacunacion frente a la pauta secuencial de neumococo fue del 48,57%. La poblacion pediatrica supero el 90% de cobertura para la vacuna frente a Haemophilus influenzae tipo b y neumococo conjugada de 13 serotipos, mientras que en los mayores de 14 anos apenas supero el 50%. La cobertura frente a gripe estacional fue inferior al 40%. Se obtuvo una correlacion inversa entre la edad y el cumplimiento, aunque no estadisticamente significativa. Conclusiones Las coberturas de vacunacion en los pacientes con implante coclear evaluados son mas bajas de lo esperado. Se propone la colaboracion estrecha entre los servicios de Otorrinolaringologia y las Unidades de Vacunas como principal estrategia para la mejora.

The risk of bacterial meningitis increases in cochlear implant patients. Therefore, pneumococcal, influenza and Haemophilus influenzae type b vaccination is indicated in this group. The aim of this study was to determine compliance with the vaccination calendar in patients implanted in a referral hospital.Patients with cochlear implant operated between 2005 and 2015 were included. Vaccine coverage for seasonal influenza, Haemophilus influenzae type b and pneumococcal conjugate 13-serotypes and pneumococcal polysaccharide 23-serotypes was evaluated. The sample was divided into 2 age groups (14 years and≥14 years). A univariate and bivariate analysis was performed.Of the 153 patients studied (28.01% 0-13 years old and 71.9%≥14), only 2 (5.71%) had 100% adherence to the vaccination schedule, while 65.71% had compliance of 50% or less. Overall, vaccination coverage against the sequential pneumococcal pattern was 48.57%. The paediatric population exceeded 90% coverage for the vaccine against Haemophilus influenzae type b and pneumococcal conjugate 13-serotypes while in those over 14 years of age it barely exceeded 50%. Influenza coverage was less than 40%. An inverse correlation was obtained between age and compliance, although not statistically significant.Vaccination coverage in patients with cochlear implant is lower than expected. Close collaboration between Otolaryngology departments and the Vaccination Units is proposed as the main strategy for improvement.

Resumen Introduccion y objetivos La inadecuada retroalimentacion auditiva en los ninos con hipoacusia prelocutiva altera la articulacion de consonantes y vocales. El proposito de esta investigacion es comparar la produccion de vocales en ninos hipoacusicos hispanohablantes con implante coclear o adaptacion audioprotesica con ninos normooyentes por medio del analisis acustico de las frecuencias formanticas y el espacio vocal. Metodo Un total de 56 ninos con hipoacusia prelocutiva (25 con implante coclear y 31 con audifonos) y 47 ninos normooyentes participaron en el estudio. Los 2 primeros formantes (F1 y F2) de las 5 vocales del idioma espanol se midieron utilizando el programa Praat. Para analizar las diferencias entre los 3 grupos se aplico el metodo ANOVA de analisis de la varianza y el test de Scheffe. Tambien se calculo el area del espacio vocal. Resultados El valor medio del F1 en todas las vocales no mostro diferencias significativas en los 3 grupos de ninos. Para las vocales /i/, /o/ y /u/ el valor medio del F2 fue significativamente diferente entre los 2 grupos de ninos hipoacusicos y el grupo de ninos normooyentes. Conclusion Ambos grupos de ninos con hipoacusia prelocutiva tienden a presentar sutiles desviaciones en la articulacion de las vocales que pueden ser analizadas mediante un programa de analisis acustico.

Introduction Contralateral sensorineural hearing loss (CSNHL) after vestibular schwannoma (VS) is a severe complication, especially in those cases in which hearing preservation in the operated side was not possible. There are several theories that attempt to explain this issue, but there is no established guideline of treatment. Material and methods We report 4 patients treated in our institution who developed a severe CSNHL after surgery. Results Of the 185 cases of VS treated with surgery, 4 patients (2.2%) developed a CSNHL after VS surgery. After medical treatment, partial recovery of hearing occurred in one patient the other 3 patients presented a well-established severe SNHL. Conclusions Established treatment guidelines do not exist, but the use of high doses of corticosteroids has been recommended and cochlear implant in cases with no recovery and complete hearing loss may be useful.

Cuba implemented policies mandating social distancing on March 11, 2020, which were still in place at the time of this study. During such periods of isolation, people with psychoactive substance-related disorders and other addictions may be tempted to reduce tension, stress, uncertainty and possible distress by increasing the use of substances or practices they have abused. This can mean relapses and setbacks for patients undergoing treatment. A multidisciplinary team of health professionals specializing in addiction at the Center for Academic Development in Drug Addiction, in Havana, Cuba, cares for people with these disorders and followed their evolution during the initial period of COVID-19 social isolation. With the aim of characterizing strategies employed by patients undergoing treatment for substance abuse and addictions, we conducted a qualitative study from April 2020 through May 2020, using a convenience sample of 37 patients (all students) who had been progressing towards recovery from addictive behaviors when face-to-face encounters were suspended due to COVID-19 restrictions. Contact was maintained through information and communication technologies. The research used telepsychology and focused on understanding patient life experiences. Patients were interviewed using a semi-structured survey, which was then transcribed and coded thematically using a grounded-theory approach. We found that patients' ability to cope successfully with challenges presented by COVID-19 were influenced by: 1) the individual's own methods for maintaining self-control (commitment to studies, projects, and work with therapists) that aided them in their goals concerning abstinence; 2) difficulties faced in addressing specific events and situations (doubts, uncertainties, disagreements, isolation and time use); 3) perpetuation and revivification of myths related to substances and addictive activities (exacerbation of supposed benefits of tobacco, alcohol, marijuana, overuse of social networks); and 4) tendencies toward irrationality and lack of emotional control (fear, sadness, anger, constant worry and self-imposed demands). Our findings suggest that despite the potential negative psychological impact of preventive social isolation during the COVID-19 pandemic, individual coping mechanisms developed by these patients, aiming at improved self-control, allowed most to avoid setbacks that could have affected their recovery. Nevertheless, patients faced challenges to their recovery that were compounded by difficulties in specific situations, myths related to substances and addictive activities, and tendencies toward irrationality or lack of emotional control.

Introduction: Ototoxicity occurs in different percentages in patients after treatment with platinum-based chemotherapy or cranial radiation therapy. The aim of this study was to present our experience in ototoxicity monitoring. Material and methods: A review was made of the registry of paediatric cancer patients referred to the Children’s Hearing Loss Unit from 1999 to 2019. Results: Of the 46 patients referred to this unit, 41 had received platinum as part of their treatment, 17 patients underwent neurosurgery, and 18 patients received cranial radiation therapy. An anamnesis and otoscopy were performed on all of them, and the monitoring was carried out with tone-verbal audiometry and/or distortion products. Hearing loss was observed in eight patients (21.05% of patients referred for audiological follow-up) as a consequence of the treatment. It was impossible to determine the audiological situation in eight patients at the end of treatment. Hearing aid adaption was necessary in two patients. In coordination with Paediatric Oncology, a change from cisplatin to carboplatin due to bilateral grade two ototoxicity was considered appropriate during treatment in one patient. Conclusion: Adequate coordination with Paediatric Oncology is essential to carry out active surveillance for ototoxicity and to modify, if possible, the dosage or type of chemotherapy in case hearing is affected. In our experience, and following current recommendations, a pre-treatment assessment is usually performed, as well as monitoring during treatment, at the end of treatment, and annually thereafter due to the risk of a later development of hearing loss. Resumen: Introducción: La ototoxicidad se presenta en diversos porcentajes según estudios tras el tratamiento con quimioterapia basada en platino y/o radioterapia craneal. El objetivo es mostrar nuestra experiencia en la monitorización de la ototoxicidad. Material y métodos: Se realizó una revisión del 1999 al 2019 en el registro de pacientes oncológicos pediátricos de nuestro hospital y remitidos a la Unidad de Hipoacusia Infantil. Resultados: 46 pacientes fueron remitidos a nuestra unidad. 41 pacientes recibieron platinos como parte de su tratamiento, 17 pacientes fueron sometidos a una intervención neuroquirúrgica y 18 pacientes recibieron radioterapia craneal. A todos se les realizó una anamnesis y otoscopia, y la monitorización se llevó a cabo con una audiometría tono-verbal y/o productos de distorsión. Se objetivó una hipoacusia como secuela del tratamiento en ocho pacientes (21,05% de los pacientes remitidos para seguimiento audiológico). Fue imposible determinar la situación audiológica al finalizar el tratamiento en ocho pacientes. La adaptación audioprotésica fue necesaria en dos pacientes. En la coordinación con Oncología Pediátrica, se consideró oportuno el cambio de cisplatino por carboplatino por ototoxicidad importante durante el tratamiento en un único paciente. Conclusión: Es imprescindible una adecuada coordinación con Oncología Pediátrica para realizar una vigilancia activa de la ototoxicidad y modificar, si es posible, la dosificación o el tipo de quimioterápico en caso de verse afectada la audición. En nuestra experiencia, y siguiendo las recomendaciones actuales, realizamos una valoración pretratamiento, una monitorización durante el tratamiento, al finalizarlo y después de forma anual por el riesgo de desarrollo diferido de una hipoacusia

First bite syndrome is a potential complication of surgery involving the infratemporal fossa, deep lobe of the parotid gland and parapharyngeal space. It is described as an acute and intense pain in the parotid region caused with the first bite of each meal. It is related to damage to sympathetic innervation of the parotid gland. Parasympathetic hyperactivation is believed to stimulate an exaggerated myoepithelial cell contraction causing pain. Usual analgesic treatments have poor results. Botulinum toxin type A causes parasympathetic nerve paralysis of the parotid gland and this fact would minimize salivation and decrease first bite syndrome. The aim of this study is to show the details of the technique and our outcomes in 5 patients treated with botulinum toxin type A.

First bite syndrome is a potential complication of surgery involving the infratemporal fossa, deep lobe of the parotid gland and parapharyngeal space. It is described as an acute and intense pain in the parotid region caused with the first bite of each meal. It is related to damage to sympathetic innervation of the parotid gland. Parasympathetic hyperactivation is believed to stimulate an exaggerated myoepithelial cell contraction causing pain. Usual analgesic treatments have poor results. Botulinum toxin type A causes parasympathetic nerve paralysis of the parotid gland and this fact would minimize salivation and decrease first bite syndrome. The aim of this study is to show the details of the technique and our outcomes in 5 patients treated with botulinum toxin type A.

Background: Sensorineural hearing loss (SNHL) is the most common sensory impairment. Comprehensive next-generation sequencing (NGS) has become the standard for the etiological diagnosis of early-onset SNHL. However, accurate selection of target genomic regions (gene panel/exome/genome), analytical performance and variant interpretation remain relevant difficulties for its clinical implementation. Methods: We developed a novel NGS panel with 199 genes associated with non-syndromic and/or syndromic SNHL. We evaluated the analytical sensitivity and specificity of the panel on 1624 known single nucleotide variants (SNVs) and indels on a mixture of genomic DNA from 10 previously characterized lymphoblastoid cell lines, and analyzed 50 Spanish patients with presumed hereditary SNHL not caused by GJB2/GJB6, OTOF nor MT-RNR1 mutations. Results: The analytical sensitivity of the test to detect SNVs and indels on the DNA mixture from the cell lines was > 99.5%, with a specificity > 99.9%. The diagnostic yield on the SNHL patients was 42% (21/50): 47.6% (10/21) with autosomal recessive inheritance pattern (BSND, CDH23, MYO15A, STRC [n = 2], USH2A [n = 3], RDX, SLC26A4); 38.1% (8/21) autosomal dominant (ACTG1 [n = 3; 2 de novo], CHD7, GATA3 [de novo], MITF, P2RX2, SOX10), and 14.3% (3/21) X-linked (COL4A5 [de novo], POU3F4, PRPS1). 46.9% of causative variants (15/32) were not in the databases. 28.6% of genetically diagnosed cases (6/21) had previously undetected syndromes (Barakat, Usher type 2A [n = 3] and Waardenburg [n = 2]). 19% of genetic diagnoses (4/21) were attributable to large deletions/duplications (STRC deletion [n = 2]; partial CDH23 duplication; RDX exon 2 deletion). Conclusions: In the era of precision medicine, obtaining an etiologic diagnosis of SNHL is imperative. Here, we contribute to show that, with the right methodology, NGS can be transferred to the clinical practice, boosting the yield of SNHL genetic diagnosis to 50-60% (including GJB2/GJB6 alterations), improving diagnostic/prognostic accuracy, refining genetic and reproductive counseling and revealing clinically relevant undiagnosed syndromes., Work performed at IMOMA for this project was partially supported by a grant from Fundacion Maria Cristina Masaveu Peterson. Work performed at DREAMgenics was partially supported by University of Oviedo Foundation grants (D.C., P.C.P.).

Introduction and objectives Inadequate auditory feedback in prelingually deaf children alters the articulation of consonants and vowels. The purpose of this investigation was to compare vowel production in Spanish-speaking deaf children with cochlear implantation, and with hearing-aids with normal-hearing children by means of acoustic analysis of formant frequencies and vowel space. Methods A total of 56 prelingually deaf children (25 with cochlear implants and 31 wearing hearing-aids) and 47 normal-hearing children participated. The first 2 formants (F1 and F2) of the five Spanish vowels were measured using Praat software. One-way analysis of variance (ANOVA) and post hoc Scheffe test were applied to analyze the differences between the 3 groups. The surface area of the vowel space was also calculated. Results The mean value of F1 in all vowels was not significantly different between the 3 groups. For vowels /i/, /o/ and /u/, the mean value of F2 was significantly different between the 2 groups of deaf children and their normal-hearing peers. Conclusion Both prelingually hearing-impaired groups tended toward subtle deviations in the articulation of vowels that could be analyzed using an objective acoustic analysis program.

Resumen Introduccion y objetivos La tecnica quirurgica clasica de los implantes oseointegrados tiene como principio fundamental la extirpacion del tejido celular subcutaneo hasta la capa periostica. Recientemente se han disenado pilares de mayor longitud, lo que ha permitido el desarrollo de la tecnica de preservacion de tejido, es decir, sin eliminacion de los tejidos blandos. Este estudio evalua las complicaciones cutaneas postquirurgicas a corto, medio y largo plazo, comparando la tecnica clasica con la tecnica de preservacion de tejido. Material y metodos Estudio prospectivo de pacientes a los que se les ha colocado un implante oseointegrado desde febrero de 2009 hasta febrero de 2013. Se realizaron un total de 29 cirugias, 14 segun la tecnica clasica y 15 con cirugia de preservacion de tejido. Las complicaciones cutaneas fueron evaluadas segun la clasificacion de Holgers en revisiones a la semana, al mes, y al ano. Resultados En ningun paciente se registraron complicaciones cutaneas de grado 4 que precisaran la retirada del implante oseointegrado o cirugia de revision. Aunque la puntuacion en la clasificacion de Holgers fue siempre mas desfavorable en la tecnica clasica, sobre todo a corto plazo (a la semana reaccion de grado 3 en el 28% de los pacientes versus 7% con tecnica de preservacion), no se alcanzaron diferencias estadisticamente significativas entre los 2 grupos a la semana (p = 0,233), al mes (p = 0,470) o al ano (p = 0,401). Conclusion En nuestra experiencia, la tecnica minimamente invasiva sin reduccion de tejido es la tecnica de eleccion para la cirugia de los implantes oseointegrados por ser mas sencilla, mas rapida, poder realizarse con anestesia local y resultados postoperatorios iguales.

Background Primary inverted papillomas of the middle ear are extremely rare tumors, with only few cases published in the literature. They have a characteristic epithelium surface inversion to the inside of the stroma. Inverted papillomas are benign lesions but have a high propensity of recurrence, local aggressiveness, and association with malignancy. Methods We present a patient with an inverted papilloma originating in the middle ear producing chronic otorrhea and facial nerve palsy treated with a subtotal petrosectomy. Results During the follow-up, the patient developed an intracranial invasion of the tumor causing his death. Conclusion Primary inverted papillomas of the middle ear can have an extremely aggressive behavior despite their benign characteristics; therefore, an aggressive treatment must be performed in order to minimize the possibility of recurrence. © 2015 Wiley Periodicals, Inc. Head Neck, 2015

Schwannomas are benign tumours, rare among tumours of the larynx. They normally present as supraglottic masses (because they may arise from the internal branch of the superior laryngeal nerve), most commonly involving aryepiglottic folds or false vocal folds. Most patients present with a globus sensation, dysphagia or hoarseness. Conservative surgery is the treatment of choice. We report a case of a laryngeal asymptomatic neuroma that was diagnosed accidentally in an imaging test. Complete excision of the tumour was performed through a transoral CO2 laser microsurgery without resorting to a tracheotomy. We discuss the clinical, pathologic and imaging findings and the management of this neoplasm. We also try to update the knowledge on the management of these tumours.

Schwannoma of the larynx. An infrequent laryngeal tumour Abstract Schwannomas are benign tumours, rare among tumours of the larynx. They normally present as supraglottic masses (because they may arise from the internal branch of the supe- rior laryngeal nerve), most commonly involving aryepiglottic folds or false vocal folds. Most patients present with a globus sensation, dysphagia or hoarseness. Conservative surgery is the treatment of choice. We report a case of a laryngeal asymptomatic neuroma that was diagno- sed accidentally in an imaging test. Complete excision of the tumour was performed through a transoral CO2 laser microsurgery without resorting to a tracheotomy. We discuss the clinical, pathologic and imaging findings and the management of this neoplasm. We also try to update the knowledge on the management of these tumours. © 2011 Elsevier Espana, S.L. All rights reserved.

Resumen El sindrome de Grisel es una complicacion infrecuente de las infecciones que afectan al area otorrinolaringologica. Consiste en una subluxacion atloaxoidea no traumatica, tras un proceso infeccioso. Su sintoma caracteristico es la torticolis, dolorosa y persistente, a pesar de haber solucionado la infeccion. El conocimiento de esta afeccion ayuda al diagnostico y tratamiento precoz de la misma. Queremos, por lo tanto, presentar el caso de un paciente con un sindrome de Grisel como secuela de una mastoiditis aguda complicada con un absceso de Bezold, a fin de favorecer el conocimiento de este tipo de proceso.

The objective of this study was to expose our results in the treatment of petrous bone cholesteatomas (PBC), paying attention to diagnosis, surgical strategy, facial management, results, and recurrences. The main objective is to compare the results of obliterative and open techniques in their management concerning the recurrence rate, due to the controversy elicited on obliterative or closed techniques in large cholesteatomas. A retrospective study was performed from July 1977 to September 2007 at the Tertiary referral cranial base center. Thirty-five patients were treated for PBC through different surgical approaches, and in 25 cases (72%) the surgical cavity was obliterated with a muscle flap. Four patients (11%) had a long-term recurrence. These patients received an open technique and after surgical re-exploration using a closed technique they had no recurrence. There were no recurrences in patients who underwent an obliterative technique and they received periodic MRI controls. The facial function after surgery was acceptable (71% of patients had House-Brackmann grades I to III). PBC is a complex pathology and presents difficulties in its diagnosis and treatment. Surgical technique should be suitable for removing the pathology and preventing damage to structures such as the facial nerve or great vessels. Obliterative techniques, where possible, are at least as safe as open cavity procedures and they have fewer postoperative complications; however, regular follow-up with CT and MRI is mandatory.

Resumen Introduccion y objetivos Los tumores originados en el espacio parafaringeo son poco frecuentes, y representan el 0,5 % de la enfermedad oncologica de cabeza y cuello. El objetivo de este estudio es presentar nuestra experiencia en el diagnostico y el tratamiento de estos tumores. Pacientes y metodo Realizamos un estudio retrospectivo de 51 pacientes, diagnosticados y tratados quirurgicamente de una tumoracion parafaringea, durante el periodo comprendido entre 1984 y 2006. Se excluyeron los tumores originados en el lobulo profundo de la parotida. Resultados El 70 % de las neoplasias del espacio parafaringeo fueron de naturaleza benigna y el 30 %, maligna. El adenoma pleomorfo fue el tumor mas frecuente (37 %), seguido por un grupo de tumores de origen miscelaneo (34 %), los paragangliomas (21 %) y los tumores de origen neurogenico (8 %). En todos los casos el tratamiento fue quirurgico. Se realizo un tratamiento transcervical en 32 pacientes, un tratamiento cervical-transparotideo en 13 pacientes, un tratamiento transmandibular en 4 pacientes (solo se preciso de mandibulectomia en un caso), un caso de tratamiento transoral y un caso de tratamiento infratemporal tipo A. Las complicaciones mas frecuentes fueron las derivadas de lesiones de estructuras nerviosas. Conclusiones La mayoria de los tumores localizados en el espacio parafaringeo son subsidiarios de tratarse de forma quirurgica, con una tasa baja de complicaciones y recurrencias. El mas utilizado es el tratamiento transcervical.

Introduccion La procalcitonina (PCT) es el precursor de la hormona calcitonina. Las concentraciones de PCT en plasma aumentan en relacion con las manifestaciones sistemicas de la reaccion inflamatoria, especialmente cuando estan causadas por una infeccion bacteriana. El objetivo de este estudio es conocer y validar las concentraciones basales de PCT alcanzadas tras diferentes tipos de cirugia de base de craneo por si pudiese utilizarse como marcador precoz de complicaciones infecciosas, fundamentalmente meningitis. Material y metodo Se realiza un estudio prospectivo sobre 16 pacientes intervenidos de base de craneo. Se estudiaron las concentraciones de PCT, proteina C reactiva, leucocitos en sangre y sintomas clinicos en el preoperatorio y el primero, el segundo y el quinto dia postoperatorios. Resultados Todos los pacientes tuvieron un postoperatorio sin signos infecciosos generales ni fiebre. Las concentraciones de PCT no aumentaron en ningun caso por encima de los valores normales. Conclusiones Dado que los fenomenos inflamatorios propios de las intervenciones no complicadas de base de craneo no elevan de forma resenable las concentraciones de PCT, se podria utilizarla para estudiar por tecnicas no invasivas los casos que si presenten complicaciones infecciosas intercurrentes.

Objetivo Averiguar la fraccion de pacientes intervenidos de un neurinoma del acustico que recuperan la funcion normal del nervio facial tras una paralisis facial completa postoperatoria, atendiendo a las complicaciones oftalmologicas asociadas a la falta de recuperacion y sus opciones terapeuticas. Material y metodo Se realiza un estudio retrospectivo de 30 pacientes intervenidos de neurinomas del acustico en nuestro servicio que, con preservacion anatomica del nervio facial, presentaron una paralisis facial completa (grado VI de House-Brackmann [HB]) postoperatoria. Se analiza la evolucion de la paralisis en funcion del tamano tumoral, la edad del paciente, el abordaje quirurgico y la localizacion del neurinoma. Tambien se estudian las complicaciones oftalmologicas surgidas y su tratamiento. Resultados De los 30 pacientes estudiados, solo 5 (16,6%) recuperaron la funcion normal facial (grado I de HB). Se observo una tendencia a una peor recuperacion en los casos con tamano tumoral mayor de 2 cm, de sexo masculino, edad mayor de 65 anos y abordados por via translaberintica. Solo un paciente presento complicaciones oftalmologicas graves. Conclusiones Nuestro analisis revela que un escaso porcentaje de pacientes recupera de forma completa su funcion facial, y que es necesario estar alerta ante posibles complicaciones oculares en este espectro de pacientes.

Objective To evaluate the fraction of patients recovering to normal function after complete facial nerve paralysis secondary to acoustic neuroma surgery, and also to address the ophthalmologic complications associated with it and the therapeutic options. Material and method We performed a retrospective review of 30 cases operated on in our department for acoustic neuroma and who, despite anatomical preservation of the facial nerve, developed a complete postoperative facial nerve paralysis (grade VI of House-Brackmann [HB]). We analyzed the evolution of the facial paralysis in relation to the tumour size, patient age, surgical approach, and localization of the tumour. In addition, we studied the ophthalmologic complications and their treatment. Results Only 5 of the 30 cases studied (16.6%) recovered to normal facial nerve function (HB grade I). We observed a tendency for a poor recovery of the cases with tumour size bigger than 2 cm, males, those older than 65 years and lesions resected by the translabyrinthine approach. Only 1 patient presented serious ophthalmologic complications. Conclusions Our study reveals that only a small percentage of patients achieve total recovery of facial function. We have to be on the alert to ocular complications in this kind of patient.

Introduction Procalcitonin (PCT) is the precursor of the calcitonin hormone. Plasma concentrations of procalcitonin increase in systemic inflammation, especially when they are caused by bacterial infections. The aim of this study is to analyze and validate the baseline PCT concentrations after different types of skull base surgery and to test the hypothesis that PCT could be useful as an early marker of post-operative infectious complications, especially meningitis. Material and method This is a prospective study of sixteen patients who underwent skull base surgery. PCT and C-reactive protein plasma levels, leukocytes and clinical symptoms were registered pre-operatively and 1, 2, and 5 days post-operatively. Results All patients had a normal post-operative course without clinical signs of infection. PCT concentrations did not increase above the normal range in any case. Conclusions As PCT plasma concentration does not notably increase despite the inflammatory phenomena associated with uncomplicated skull base surgery, it may be possible to use PCT as an early marker for non-invasive study of post-operative infectious complications.

Objetivo Evaluar las complicaciones y secuelas de la cirugia del schwannoma vestibular, en virtud del tamano tumoral. Pacientes y metodo Se realiza un estudio retrospectivo de 120 pacientes afectos de neurinomas del acustico, a los que se practico reseccion microquirurgica de la lesion entre noviembre de 1994 y septiembre de 2006. Se recogen datos del tamano tumoral, la extension de la reseccion, la preservacion de los nervios facial y coclear, las complicaciones y las secuelas vestibulares. Se determina el grado de preservacion de la audicion segun la clasificacion de Gardner-Robertson. Resultados Entre las citadas fechas se intervinieron en 39 neurinomas pequenos ( 3 cm). La reseccion total de la lesion se consiguio en 106 (88,3 %) de los 120 casos. Se alcanzo una conservacion anatomica del nervio facial y functional en el seguimiento a largo plazo en 103 (85,4 %). La funcion del nervio coclear se conservo (grados 1 y 2 de la clasificacion de Gardner-Robertson) en el 54,4 % de los schwannomas de menos de 1,5 cm de diametro con audicion valida. Fallecieron 2 pacientes por complicaciones postoperatorias (tasa de mortalidad, 1,6 %) y en 15 (12,5%) se desarrollo una fistula de liquido cefalorraquideo. Conclusiones Pese a la notable mejora en los resultados de la cirugia de los neurinomas del acustico, todavia presenta una tasa apreciable de complicaciones y secuelas. Por ello debe sopesarse en cada caso particular su realizacion en funcion de las circunstancias concurrentes y valorar la posibilidad de otras alternativas como la conducta expectante o la radiocirugia.

Resumen La agenesia de la arteria carotida interna (ACI) es una entidad infrecuente. Suele ser diagnosticada de forma casual en pacientes sometidos a una exploracion radiologica por otro motivo. El diagnostico de esta anomalia es importante ya que puede asociarse a otras malformaciones potencialmente graves, y debe ser tenido en cuenta si se va a realizar una cirugia sobre el eje carotideo del lado sano. Presentamos el caso de un varon de 64 anos en el que se objetivo incidentalmente una agenesia de ACI izquierda.

Unilateral agenesis of the internal carotid artery (ICA) is an extremely rare anomaly. Diagnosis is often incidental in a radiological examination. Recognition of this anomaly has important clinical implications because other life-threatening conditions can be associated and it must be taken into account when planning carotid surgery. We report a case of 64-year-old man with agenesis of the ICA.

El objetivo de este estudio es mostrar las formas inusuales de presentacion con que pueden manifestarse inicialmente los neurinomas del acustico. Se realiza un estudio retrospectivo de los pacientes intervenidos de neurinomas del acustico en nuestro servicio; se incluye en el analisis solo los casos cuyo sintoma inicial no se encuadraba en la hipoacusia unilateral. Se recogen datos de tamano tumoral, localizacion, forma de presentacion y edad del paciente. Encontramos 9 pacientes con sintomas atipicos y sin hipoacusia, entre los que la queja mas comun (22,2 %) fueron las parestesias faciales. Ninguno de ellos refirio otra clinica otologica acompanante. Un grupo significativo de los pacientes de nuestro estudio no se presento con los sintomas clasicos de los neurinomas del acustico. Que los clinicos conozcan este tipo de sintomas puede llevar al diagnostico precoz de esta afeccion.

Background Although appropriate perioperative antibiotic prophylaxis has significantly reduced wound infection rates in clean-contaminated head and neck surgical procedures, controversy still remains regarding the optimal antibiotic regimen. Methods In this prospective, double-blind clinical trial, 159 patients were randomized to receive amoxicillin-clavulanate, clindamycin plus gentamicin, or cefazolin intravenously up to ½ hour before surgery and at 8-hour intervals for an additional three doses. Results An overall wound infection rate of 23% was observed. Thirteen (22.8%) infections occurred in the amoxicillin-clavulanate-treated group, 11 (21.2%) in the clindamycin plus gentamicin-treated group, and 13 (26%) in the cefazolin-treated group, which was not statistically significant. Only prior medical illnesses, such as chronic obstructive pulmonary disease and diabetes mellitus, correlated with an increased wound infection rate (p = 0.018). Conclusions Amoxicillin-clavulanate, clindamycin plus gentamicin, and cefazolin seem to have similar efficacy when administered prophylactically in clean-contaminated head and neck surgical procedures. © 1997 John Wiley & Sons, Inc. Head Neck 19: 188–193, 1997.

INTRODUCTION: Smoking and alcohol use (beyond social norms) by health sciences students are behaviors contradictory to the social function they will perform as health promoters in their eventual professions. OBJECTIVE: Identify prevalence of tobacco and alcohol use in health sciences students in Mexico and Cuba, in order to support educational interventions to promote healthy lifestyles and development of professional competencies to help reduce the harmful impact of these legal drugs in both countries. METHODS: A descriptive cross-sectional study was conducted using quantitative and qualitative techniques. Data were collected from health sciences students on a voluntary basis in both countries using the same anonymous self-administered questionnaire, followed by an in-depth interview. RESULTS: Prevalence of tobacco use was 56.4% among Mexican students and 37% among Cuban. It was higher among men in both cases, but substantial levels were observed in women as well. The majority of both groups were regularly exposed to environmental tobacco smoke. Prevalence of alcohol use was 76.9% in Mexican students, among whom 44.4% were classified as at-risk users. Prevalence of alcohol use in Cuban students was 74.1%, with 3.7% classified as at risk. CONCLUSIONS: The high prevalence of tobacco and alcohol use in these health sciences students is cause for concern, with consequences not only for their individual health, but also for their professional effectiveness in helping reduce these drugs' impact in both countries.

A retrospective review of 302 previously untreated patients with primary supraglottic carcinoma was undertaken to ascertain the efficacy of postoperative radiotherapy. The 302 patients underwent a total of 383 functional neck dissections and 45 classic radical neck dissections. Pathological examination revealed nodal involvement in 117 patients (39%). In the histologically N0 group, the incidence of contralateral neck recurrence when a unilateral neck dissection and postoperative radiotherapy were carried out was 8%, rising 21% when no postoperative radiation was given. There was no difference in the ipsilateral recurrence rate in the N0 group, radiated patients (3%) and nonradiated patients (2%). Similar figures were found in the N1 group. Bilateral functional neck dissection in histologically N0, N1, and N2 necks had similar recurrence rates with or without radiotherapy. Distant metastasis appeared in 10% of N0 patients and in 35% of N3 patients. Patients who received postoperative radiotherapy showed a significantly higher rate of distant metastasis (21%) than patients who did not (8%). The number of involved lymph nodes had no relevance both in neck recurrence and distant metastasis.

Introduction and objectives Tumours of the parapharyngeal space are rare, representing only 0.5% of head and neck oncology. The aim of this study is to present our experience with the diagnosis and therapies for these tumours. Patients and method This study is a retrospective review of 51 patients diagnosed as having tumours of the parapharyngeal space and treated surgically between 1984 and 2006. Tumours originating in the deep lobe of parotid gland were excluded. Results Seventy percent of the parapharyngeal space neoplasms were benign and thirty percent malignant. Pleomorphic adenoma was the most common neoplasm (37%), followed by miscellaneous benign tumours (34%), paraganglioma (21%), and neurogenic tumours (8%). Surgery was chosen in all cases, with the transcervical approach used in 32 cases, the cervical-transparotid approach in 13 cases, the transmandibular approach in 4 cases (with mandibulectomy in 1 patient), the transoral approach in 1 case, and the infratemporal fossa approach in 1 case. The most common complications were those deriving from damage to nerve structures. Conclusions Most parapharyngeal space tumours can be removed surgically with a low rate of complications and recurrence. The transcervical approach is the most frequently used.

The aim of this study is evaluate the unusual ways of initial presentation of the vestibular schwannomas. We performed a retrospective study of the patients who underwent resection of acoustic neuromas on our service, including for analysis only the cases which initial symptom was not the hearing loss. Tumor size, localization, clinical presentation, and age of the patients were considered. Nine patients present with atypical symptoms. The most common complain in this group were facial paresthesias (22,2 %). None of them complained about other otological symptoms. A significant group of patients did not present with the otological symptoms classically associated with vestibular schwannoma. Clinical knowledge of these kinds of symptoms may lead to earlier detection of these lesions.

Giant lymph node hyperplasia (Castleman's disease) is usually reported as a solitary mediastinal tumour, although involvement of other anatomical sites and a multicentric form have been reported. We describe a rare case of Castleman's disease due to its localisation (the left infratemporal fossa) and histology (plasma-cell variant). A brief review of the main clinico-histological characteristics of Castleman's disease is also presented.

Objectives/Hypothesis: The objective was to determine the characteristics of horizontal head-shaking nystagmus of peripheral origin and its relationship to vestibular dysfunction. Study Design: Retrospective case series. Methods: Eighty-three patients met the inclusion criteria of having peripheral and unilateral vestibular disease. Patients were tested with video nystagmography. Head-shaking nystagmus was performed in the horizontal plane during 30 cycles at a frequency of approximately 3 Hz. Head-shaking nystagmus was classified as monophasic or biphasic and, based on the pathological ear, as ipsilateral or contralateral related to nystagmus fast phases. The two-tailed t test, ANOVA, Mann-Whitney and X 2 tests, and lineal and polynomial regression tests were used for statistical analysis. Results: Twenty-three patients showed a positive head-shaking nystagmus. All cases of head-shaking nystagmus observed were horizontal. There were four biphasic and 19 monophasic cases of head-shaking nystagmus. First phases of biphasic head-shaking nystagmus beat toward the normal ear. Eleven of the monophasic cases of head-shaking nystagmus were ipsilateral, and nine were contralateral. There was a statistically significant correlation between caloric weakness and head-shaking nystagmus. Ipsilateral head-shaking nystagmus corresponded to lower caloric asymmetries, and contralateral and biphasic head-shaking nystagmus corresponded to greater caloric weakness (P

Malignant fibrous histiocytomas are uncommon in the head and neck, the sinonasal tract being the most common location. This report describes 5 cases in this area: two in the nasal cavity, two in the maxillary sinus, and one in the frontal sinus. Four were primary cases and one was secondary to previous irradiation. All patients received surgical treatment, one of them with postoperative irradiation. All five patients experienced local recurrences and three also experienced distant metastases. Only one of these recurrences was successfully salvaged and the patient is alive and free of disease 3 years after resection. A summary of knowledge about the entity is reviewed.