Search

IntroductionIn 2017, the first adhesive bone conduction device (aBCD) was introduced. Since then, clinical studies have extensively compared adhesive bone conduction devices to conventional bone conduction systems on softbands. The aim of this study is to evaluate the audiological and subjective outcomes of patients suffering from conductive hearing loss (CHL) who used an aBCD for a trial period, comparing outcomes with their existing passive transcutaneous bone conduction implants (ptBCI), which was either the Sophono Alpha or the BAHA Attract.Material and methodsThis prospective study included 14 congenital aural atresia patients between 7 and 16 years old. Participants had been ptBCI users for at least 2 years and had bone conduction thresholds ≤ 25 dB HL. The aBCD trial was for 1 week. Average pure tone thresholds (PTA4) and word recognition scores (WRS) with disyllabic words at 65 dB SPL in quiet and in noise were measured for each device. Subjective outcomes were recorded using the aBCD mini questionnaire and the SSQ questionnaire for hearing satisfaction, which were filled in by parents.ResultsWe analyzed 16 ears. The mean sound field PTA4 was 52 dB HL unaided, 27 dB HL ptBCI-aided and 29 dB HL aBCD-aided. Mean WRS in quiet was 96% with ptBCI and 95% with BCD. In noise (+5 dB SNR) the mean WRS was 70% with ptBCI and 77% with aBCD. A questionnaire revealed easy handling and good acceptance of the aBCD.ConclusionsIn this group of patients, comparable audiological and subjective satisfaction results were achieved with a non-implantable adhesive bone conduction device. Despite differences in handling, the adhesive BCD presents itself as an alternative to transcutaneous bone conduction implants.

Background and objective Vestibular schwannoma is a benign tumour that originates in the eighth cranial nerve. It is termed intralabyrinthine schwannoma (ILS) when it develops in the inner ear, this being a rare origin. We present our experience in the management of three patients with ILS. Materials and methods The results of tumour excision and cochlear implantation were evaluated in three patients with ILS: two intracochlear schwannomas (ICS) and one intravestibular schwannoma (IVS). Results Prior to surgery, all patients presented progressive sensorineural hearing loss and tinnitus. Complete tumour resection and cochlear implantation was possible in all patients, with favourable hearing rehabilitation. Conclusions The therapeutic approach will depend on tumour size, growth rate, degree of hearing loss and presence of vestibular symptoms. Cochlear implantation (CI) in patients with ILS is possible when the cochlear nerve is present and functional. CI in patients, whether or not preceded by tumour excision, is an option with good hearing results in selected patients.

Resumen Introduccion y objetivo El schwannoma vestibular es un tumor benigno que se origina en el octavo par craneal. Se denomina schwannoma intralaberintico (SIL) cuando se origina en el oido interno, siendo este origen poco frecuente. Presentamos nuestra experiencia en el manejo de 3 pacientes con SIL. Material y metodos Los resultados de la exeresis tumoral e implantacion coclear fueron evaluados en 3 pacientes con SIL: 2 casos de schwannoma intracoclear y un caso de schwannoma intravestibular. Resultados Previo a la cirugia, todos los pacientes presentaban hipoacusia neurosensorial progresiva y acufeno ipsilateral. La reseccion tumoral completa y la colocacion de un implante coclear fue posible en todos los casos, con una rehabilitacion auditiva favorable. Conclusiones La actitud terapeutica dependera del tamano del tumor, el crecimiento, el grado de perdida auditiva y los sintomas vestibulares. La implantacion coclear en pacientes con SIL es posible cuando el nervio coclear esta presente y es funcionante. El IC en el SIL, ya sea con exeresis previa o no del tumor, es una opcion con buenos resultados auditivos en pacientes seleccionados.

Objective tinnitus is defined as a type of tinnitus perceived by both the patient and external observer. This paper presents two cases of objective tinnitus related to palatal tremor, along with a literature review. Palatal tremor is a condition characterized by soft palate involuntary contractions. Two types of palatal tremor have been described: symptomatic palatal tremor and essential palatal tremor, with different clinical manifestations. Diagnostic workup is based on medical history and physical examination, including direct oropharynx exploration and cavum visualization through nasopharyngoscopy. Brain MRI is mandatory in all cases. If a secondary origin is suspected, additional lab tests should be performed based on clinical suspicion. First-line treatment is botulinum toxin injection into the levator veli palatini and tensor veli palatini muscles, with velopharyngeal insufficiency being its main adverse effect. Other medications have not been shown to be effective.

Resumen Introduccion y objetivos En la ultima decada se han producido numerosos y relevantes avances en el tratamiento de la hipoacusia transmisiva y mixta que han desembocado en una ampliacion de las indicaciones de los implantes de conduccion de via osea y la aparicion de nuevos dispositivos. La Comision Cientifica de Audiologia de la Sociedad Espanola de Otorrinolaringologia y Cirugia de Cabeza y Cuello (SEORL-CCC), junto con las comisiones de Otologia y Otoneurologia, ha llevado a cabo una revision del estado actual de los implantes de via osea con la finalidad de ofrecer a los especialistas de Otorrinolaringologia, a los profesionales de la sanidad, a las autoridades sanitarias y a la sociedad en general una guia clinica sobre implantes de conduccion de via osea. Metodos Esta guia clinica sobre implantes de conduccion osea contiene informacion sobre los siguientes temas: 1) definicion y descripcion de los implantes auditivos de via osea; 2) indicaciones actuales y emergentes de los implantes de via osea; compatibilidad y resonancia magnetica, y 3) requisitos organizativos para un programa de implantes de via osea. Resultado y conclusiones La finalidad de esta guia es describir los diferentes sistemas de conduccion osea, sus particularidades e indicaciones, con el objeto de aportar unas coordenadas que ayuden a todos estos agentes en las tomas de decisiones que deban asumir en los diferentes ambitos de responsabilidad en los que estan enmarcados en sus areas de trabajo.

Langerhans cell histiocytosis (LCH) is a rare disease of unknown etiology with a heterogeneous and unspecific clinical presentation. Any organ or system may be involved but the most frequent is the skeleton. The diagnostic gold standard is done through histopathology combined with immunohistochemistry in the correct clinical setting. Treatment is still controversial. The objective of this study is to present a series of pediatric patients in order to contribute to the diagnosis and treatment of LCH.A longitudinal, descriptive and retrospective study was done in 20 pediatric patients histologically diagnosed of LCH int the last 10 years, in a tertiary center. A review of the medical history was done and data collection included: epidemiological data, clinical manifestations and classification, lesion characteristics, treatment employed, follow-up and evolution.The mean age presentation was 6.5 years (2-12). The most frequent disease presentation was a palpable skull tumor followed by headache. Other otorhinolaryngologic presenting symptoms were bloody otorrhea and post auricular swelling. Single-systems LCH with skull tumors was the most frequent classification. Treatment options (surgery, chemotherapy and corticosteroid infiltration) were indicated according to disease classification.LCH should be suspected in pediatric patients with a palpable cranial vault tumor, as well as in patients with chronic otorrhea with granulation tissue at the external ear canal. Management of LCH fundamentally includes chemotherapy and surgery, or the combination of both. High-risk organs lesions, including temporal bone lesions, and multi-system LCH are predictors of recurrence and should have a long-term follow-up.

Tinnitus is defined as a tinnitus perceived both by the patient and external observers. This category represents 1.5% of the total amount of tinnitus evaluated in tertiary healthcare institutions.

A cohort of 128 patients from 118 families diagnosed with non-syndromic or syndromic hearing loss (HL) underwent an exhaustive clinical evaluation. Molecular analysis was performed using targeted next-generation sequencing (NGS) with a custom panel that included 59 genes associated with non-syndromic HL or syndromic HL. Variants were prioritized according to the minimum allele frequency and classified according to the American College of Medical Genetics and Genomics guidelines. Variant(s) responsible for the disease were detected in a 40% of families including autosomal recessive (AR), autosomal dominant (AD) and X-linked patterns of inheritance. We identified pathogenic or likely pathogenic variants in 26 different genes, 15 with AR inheritance pattern, 9 with AD and 2 that are X-linked. Fourteen of the found variants are novel. This study highlights the clinical utility of targeted NGS for sensorineural hearing loss. The optimal panel for HL must be designed according to the spectrum of the most represented genes in a given population and the laboratory capabilities considering the pressure on healthcare.

Resumen Introduccion En la ultima decada son numerosos los hospitales que han iniciado su actividad en pacientes candidatos a un implante coclear (IC), y se han producido numerosos y relevantes avances para el tratamiento de la hipoacusia neurosensorial que han desembocado en una ampliacion de las indicaciones de los IC. Objetivos Ofrecer a los especialistas de otorrinolaringologia, de otras especialidades medicas, autoridades sanitarias y a la sociedad en general una guia clinica sobre implantes cocleares. Metodos Las comisiones cientificas de otologia, otoneurologia y audiologia de la Sociedad Espanola de Otorrinolaringologia y Cirugia de Cabeza y Cuello (SEORL-CCC), de manera coordinada y consensuada, han llevado a cabo una revision del estado actual de los IC basandose en las reglamentaciones existentes y en las publicaciones cientificas que se referencian en la bibliografia del documento elaborado. Resultados La guia clinica sobre implantes cocleares aporta informacion sobre: a) definicion y descripcion sobre IC; b) indicaciones de los IC; y c) requisitos organizativos para un programa de IC. Conclusiones Se ha elaborado por un comite de expertos de la SEORL-CCC una Guia clinica sobre implantes cocleares que aporta coordenadas de actuacion para todos aquellos agentes de la sanidad en la toma de decisiones en el ambito de los IC como forma de tratamiento de la discapacidad auditiva.

Resumen Objetivo Revision y descripcion de la afectacion otoaudiologica en el seguimiento de 23 ninos con diagnostico de mucopolisacaridosis (MPS) tipo I , II , III y IV . Metodos Estudio retrospectivo de los hallazgos clinicos, audiologicos y tratamiento (medico y/o quirurgico) de 23 ninos con diagnostico de MPS tipo I , II , III o IV en seguimiento en un hospital terciario entre 1997 y 2015. Resultados Seis casos de MPS I , 8 de MPS II , 4 de MPS III y 5 de MPS IV fueron revisados. Al inicio del seguimiento el 71,2% de los pacientes presentaban otitis media serosa (OMS) y el 54% de los casos presentaban algun tipo de hipoacusia. El comportamiento de la hipoacusia fue fluctuante en cada uno de los subgrupos de MPS, encontrando mayor afectacion y variabilidad en los tipos I y II . Conclusiones Los ninos afectos de MPS tienen un alto riesgo de hipoacusia, siendo MPS tipo I y II los casos con mayor porcentaje de afectacion audiologica y con un comportamiento menos homogeneo, mostrando un importante porcentaje de hipoacusias transmisivas que progresan a componentes mixtos o neurosensoriales. Se requiere un seguimiento periodico dada la importante repercusion de esta patologia en la calidad de vida y en el desarrollo de estos pacientes.

Objective The aim of our study is to reflect hearing impairment of 23 children diagnosed with mucopolysaccharidosis (MPS) type I, II, III and IV. Methods Retrospective study of the clinical, audiological and treatment (medical vs surgical) findings of 23 children diagnosed with MPS type I, II, III and IV followed at a Tertiary Referral Hospital between 1997 and 2015. Results Six cases of MPS I, 8 of MPS II, 4 of MPS III and 5 of MPS IV were reviewed. 71.2% of patients had secretory otitis media (SOM) and 54% of patients had some type of hearing loss (HL). The behaviour of hearing loss was variable in each of the subgroups of MPS, finding greater involvement and variability in types I and II. Conclusions Children with MPS have a high risk of hearing loss. A significant percentage of transmissive HL progressing to mixed or sensorineural HL was observed. This was more common in types I and II. Periodic follow up of these patients is mandatory because of hearing impairment and consequences for their development and quality of life.

Introduction and objectives Prevalence of congenital sensorineural hearing loss (SNHL) is approximately 1.5–6 in every 1000 newborns. Dysfunction of the auditory nerve (auditory neuropathy) may be involved in up to 1%–10% of cases; hearing losses because of vestibulocochlear nerve (VCN) aplasia are less frequent. The objectives of this study were to describe clinical manifestations, hearing thresholds and aetiology of children with SNHL and VCN aplasia. Methodology We present 34 children (mean age 20 months) with auditory nerve malformation and profound HL taken from a sample of 385 children implanted in a 10-year period. We studied demographic characteristics, hearing, genetics, risk factors and associated malformations (Casselman's and Sennaroglu's classifications). Data were processed using a bivariate descriptive statistical analysis ( P Results Of all the cases, 58.8% were bilateral (IIa/IIa and I/I were the most common). Of the unilateral cases, IIb was the most frequent. Auditory screening showed a sensitivity of 77.4%. A relationship among bilateral cases and systemic pathology was observed. We found a statistically significant difference when comparing hearing loss impairment and patients with different types of aplasia as defined by Casselman's classification. Computed tomography (CT) scan yielded a sensitivity of 46.3% and a specificity of 85.7%. However, magnetic resonance imaging (MRI) was the most sensitive imaging test. Conclusions Ten percent of the children in a cochlear implant study had aplasia or hypoplasia of the auditory nerve. The degree of auditory loss was directly related to the different types of aplasia (Casselman's classification) Although CT scan and MRI are complementary, the MRI is the test of choice for detecting auditory nerve malformation.

Resumen Introduccion y objetivos La prevalencia de la hipoacusia neurosensorial (HNS) congenita es aproximadamente de 1,5-6 de cada 1.000 nacimientos. La disfuncion del nervio auditivo (neuropatia auditiva) puede estar presente hasta en el 1-10% de los casos de HNS en ninos, siendo menos frecuentes aquellas perdidas debidas a una aplasia o hipoplasia del nervio auditivo. Los objetivos del estudio son describir la clinica, umbrales auditivos y etiologia en una muestra de ninos con HNS y aplasia o hipoplasia del octavo par. Metodologia Presentamos 34 ninos (edad media 20 meses) con malformacion del nervio auditivo e HNS profunda de una muestra de 385 ninos implantados en los ultimos 10 anos. Estudiamos las caracteristicas demograficas, clinicas y malformaciones asociadas (clasificaciones de Casselman y Sennaroglu). Los datos fueron procesados usando un analisis estadistico descriptivo bivariante (p Resultados Un 58,8% fueron bilaterales ( II a/ II a y I / I fueron los mas frecuentes). De los unilaterales el II b fue el mas frecuente. La sensibilidad del cribado auditivo fue de un 77,4%. Encontramos diferencias estadisticamente significativas entre el grado de hipoacusia y los distintos tipos de aplasia (clasificacion de Casselman). La sensibilidad de la TC fue del 46,3% y la especificidad del 85,7%. La RNM fue la prueba de imagen mas sensible. Conclusiones Un 10% de los ninos en estudio para un implante coclear tienen una aplasia o hipoplasia del nervio auditivo. El grado de perdida auditiva esta directamente relacionado con los distintos tipos de aplasia (clasificacion de Casselman). Aunque la TC y la RNM son complementarias, la RNM es el test de eleccion para la deteccion de malformaciones del nervio auditivo.

OBJECTIVES: Electrically evoked compound action potentials (eCAP) recordings are widely used in functional evaluation and fitting of cochlear implants (CI) in clinics. We compared the results from two eCAP recording approaches (StandardART and FineGrain, MED-EL, Austria). The FineGrain method is more advanced than the Auditory Nerve Response Telemetry (StandardART) method in terms of the stimulation and algorithm for the eCAP threshold detection. To understand the benefits of these alterations, we compared the two methods on a larger scale in pediatric CI users alongside evoked auditory brainstem responses (eABR). MATERIALS and METHODS: We collected the eCAP recordings obtained with both methods from a population of pediatric subjects with CI, either intra-or post-operatively. The eABR recordings were only collected post-operatively. For comparability reasons, we used the same stimulation rate and similar amplitude levels for all three approaches. RESULTS: Our results demonstrate that, although the success rates are similar, the FineGrain method outperforms traditional StandardART in terms of robustness and measurement duration. The eCAP recordings in general outperform the eABR in terms of speed. CONCLUSION: We conclude that the eCAP recordings are the method of choice for measuring the auditory neural activity, and FineGrain outperforms StandardART. From the three investigated approaches, we conclude that FineGrain performed best and should be the first-choice method in pediatric patients.

OBJECTIVES: Auditory brainstem potentials can be elicited by electrical stimulation of the round window (RW). In this technique, extracochlear stimulation is objectively used in the selection of cochlear implant (CI) candidates to avoid cochlear damage. However, until now, its use is limited due to the large artifacts generated by electrical stimulation. Our objective was to obtain reliable and reproducible electrically evoked auditory brainstem responses (eFARRs) using a new method of stimulation MATERIALS and METHODS: This was a prospective study including subjects who under went electrical stimulation on RW during CI surgery between 2013 and 2016. A "Stimulator Box;" which produces electric stimuli identical to those provided by a CI, and an evoked potential recording equipment were used. The results obtained with monopolar and bipolar electrodes were compared. RESULTS: RW eEABR recordings of 49 subjects (mean age, 34 years) were characterized by their stability and by having wave V between 3 and 5.5 ms. A higher percentage of responses wee obtained on increasing the phase duration instead of the pulse amplitude. A significantly greater percentage of positive responses were obtained using bipolar stimulation than using monopolar stimulation (p

Introduction and goals During the last decade there have been multiple and relevant advances in conduction and mixed hearing loss treatment. These advances and the appearance of new devices have extended the indications for bone-conduction implants. The Scientific Committee of Audiology of the Sociedad Espanola de Otorrinolaringologia y Cirugia de Cabeza y Cuello SEORL-CCC (Spanish Society of Otolaryngology and Head and Neck Surgery), together with the Otology and Otoneurology Committees, have undertaken a review of the current state of bone-conduction devices with updated information, to provide a clinical guideline on bone-conduction implants for otorhinolaryngology specialists, health professionals, health authorities and society in general. Methods This clinical guideline on bone-conduction implants contains information on the following: (1) Definition and description of bone-conduction devices; (2) Current and upcoming indications for bone conduction devices: Magnetic resonance compatibility; (3) Organisation requirements for a bone-conduction implant programme. Results and conclusions The purpose of this guideline is to describe the different bone-conduction implants, their characteristics and their indications, and to provide coordinated instructions for all the above-mentioned agents for decision making within their specific work areas.

Introduction In the last decade numerous hospitals have started to work with patients who are candidates for a cochlear implant (CI) and there have been numerous and relevant advances in the treatment of sensorineural hearing loss that extended the indications for cochlear implants. Objectives To provide a guideline on cochlear implants to specialists in otorhinolaryngology, other medical specialities, health authorities and society in general. Methods The Scientific Committees of Otology, Otoneurology and Audiology from the Spanish Society of Otolaryngology and Head and Neck Surgery (SEORL-CCC), in a coordinated and agreed way, performed a review of the current state of CI based on the existing regulations and in the scientific publications referenced in the bibliography of the document drafted. Results The clinical guideline on cochlear implants provides information on: (a) definition and description of Cochlear Implant; (b) indications for cochlear implants; (c) organisational requirements for a cochlear implant programme. Conclusions A clinical guideline on cochlear implants has been developed by a Committee of Experts of the SEORL-CCC, to help and guide all the health professionals involved in this field of CI in decision-making to treathearing impairment.

Resumen Introduccion y objetivo El neurinoma del acustico es un tumor benigno que suele afectar a la porcion vestibular del viii par craneal. Representan el 8% de todos los tumores intracraneales y el 80% de los que surgen a nivel del angulo pontocerebeloso. Existen 3 opciones terapeuticas: la microcirugia, que es la tecnica de eleccion, la radiocirugia y la observacion. El objetivo del estudio ha sido valorar los resultados obtenidos con la radiocirugia en el tratamiento del neurinoma del acustico, asi como los efectos secundarios derivados del mismo. Material y metodos Hemos realizado en nuestro hospital una revision de todos los pacientes tratados con radiocirugia (Gamma Knife y LINAC) a dosis de 1.200-1.300 cGy por neurinoma del acustico unilateral, entre enero de 1999 y enero del 2010. En todos los pacientes se valoraron el estado general, la tasa de control de crecimiento tumoral, entendiendose como tal que no cambia de tamano o que se reduce, la afectacion del v y vii par craneal, asi como alteraciones del sistema nervioso central. Tambien el tiempo de seguimiento y las variaciones de los umbrales auditivos tras la radiocirugia. Resultados De un total de 35 pacientes estudiados, con una edad media de 58,29 anos y sin diferencias estadisticamente significativas en cuanto al sexo, la tasa de control de crecimiento tumoral fue superior al 90%. El principal motivo de consulta (65,71%) fue la hipoacusia unilateral y progresiva. Un 34,28% de los pacientes tratados presentaron empeoramiento en su nivel de audicion postratamiento. La afectacion de los pares craneales ( v - vii ) en el 100% de los casos fue transitoria. La radiocirugia tipo Gamma Knife fue administrada en la mayoria de los pacientes (82,85%). Conclusion Aunque la microcirugia sigue siendo el tratamiento de eleccion para los neurinomas del acustico, consideramos la radiocirugia como una alternativa valida en pacientes seleccionados (edad avanzada, comorbilidad asociada, pequeno tamano e hipoacusia neurosensorial, entre otros).

The acoustic neuroma is a benign tumour that usually affects the vestibular portion of the vestibulocochlear nerve. It represents 8% of all intracranial tumours and 80% of those arising at the cerebellopontine angle. There are 3 treatment options: microsurgery (the technique of choice), radiosurgery and observation. The objective of the study was to evaluate the results and side effects obtained using radiosurgery as treatment for acoustic neuroma.We performed a review of all patients treated with radiosurgery (Gamma Knife and linear accelerator) at doses of 1200-1300 cGy for unilateral acoustic neuroma in our hospital from January 1999 until January 2010. In all patients we evaluated the overall state, tumour growth control rate (tumour smaller or remaining the same size), the involvement of v and vii cranial nerves and central nervous system disorders. We also assessed follow-up time and changes in hearing thresholds after radiosurgery.From a total of 35 patients studied, with a mean age of 58.29 years and lacking statistically significant differences in gender, the tumour growth control rate was over 90%. The main reason for visit (65.71%) was unilateral and progressive hearing loss. After treatment, 34.28% of patients had hearing loss. The involvement of the cranial nerves (v-vii) was transitory in 100% of cases. Gamma Knife radiosurgery was administered in 82.85% of patients.Although microsurgery is the treatment of choice for acoustic neuroma, we consider radiosurgery as a valid alternative in selected patients (elderly, comorbidity, small tumour size and sensorineural hearing loss, among others).

Resumen Introduccion y objetivos El diagnostico del colesteatoma se basa en los hallazgos clinicos y en la tomografia computarizada. Actualmente, con las nuevas tecnicas de resonancia magnetica potenciada en difusion no ecoplanares, sin necesidad de contraste intravenoso, es posible diferenciar entre colesteatoma y tejido de granulacion o inflamatorio. Por ello, esta tecnica muestra su maxima utilidad en la valoracion de recidivas de colesteatoma tras timpanoplastias, sobre todo en tecnicas cerradas, ya que puede evitar un alto porcentaje de cirugias de revision. Otras indicaciones de la tecnica son los casos de diagnostico complejo y el colesteatoma congenito. El objetivo de este estudio es valorar la validez (sensibilidad y especificidad) y la seguridad (valor predictivo positivo y valor predictivo negativo) de la secuencia de difusion PROPELLER, una de las tecnicas potenciada en difusion no ecoplanar en el diagnostico del colesteatoma. Metodos Estudio prospectivo de 52 pacientes con sospecha de colesteatoma en el que se correlacionan hallazgos clinicos y quirurgicos con los obtenidos del estudio de resonancia magnetica, que incluia una secuencia potenciada en difusion no ecoplanar (PROPELLER) de oidos. Resultados La sensibilidad de la prueba para el grupo fue del 92,85%, la especificidad del 92,30%, el valor predictivo positivo del 92,85% y el valor predictivo negativo del 92,30%. Conclusiones La resonancia magnetica con imagen potenciada en difusion no ecoplanar utilizando la secuencia PROPELLER, es una tecnica eficaz en el control del colesteatoma, permitiendo diagnosticar lesiones mayores de 2 mm.

Introduction and objectives The diagnosis of cholesteatoma is based on clinical evaluation and computed tomography. New non-echo-planar diffusion-weighted magnetic resonance imaging (MRI) techniques, without intravenous contrast, are capable of differentiating cholesteatoma from inflammatory tissue, cholesterol granuloma and granulation tissues. The technique is very helpful in differential diagnosis of cholesteatoma, mainly after canal wall-up tympanoplasty surgery, to avoid routine second-look surgery in these patients. Congenital cholesteatoma and difficult cases can be detected and correctly diagnosed as well. The aim of this study was to evaluate sensitivity, specificity, positive predictive value and negative predictive value of the diffusion-weighted PROPELLER MRI in cholesteatoma diagnosis. Methods A prospective study was performed on 52 patients. Clinical and surgical findings were correlated with diffusion-weighted PROPELLER MRI results. Results Sensitivity, specificity and positive and negative predictive values were 92.85%, 92.30%, 92.85% and 92.30%, respectively. Conclusions Diffusion-weighted PROPELLER imaging is an effective technique in cholesteatoma diagnosis. It is capable of detecting lesions larger than 2 mm.

Objetivo Valorar la relacion de la hipoacusia con la arthritis reumatoide (AR). Pacientes y metodo Estudio comparativo de casos y controles equiparados en edad y sexo, de 194 pacientes con AR y 107 sujetos sanos. Se efectuo en todos valoracion mediante audiometria tonal y determinacion de parametros inflamatorios y Western blot de anticuerpos anticocleares. Resultados El 42,7 % de los pacientes con AR presentaron hipoacusia (el 15,9 % de los controles; p Western blot positivo. No se detecto ninguno en los controles. Conclusiones Hay una predisposicion a la hipoacusia en los sujetos con AR, principalmente neurosensorial. Esta prevalencia obliga a la revision audiologica de este grupo e intentar definir si este trastorno tiene un componente inmunomediado que permitiera un tratamiento modificador del curso de la hipoacusia.

Objetivo Valorar los resultados obtenidos, asi como los efectos secundarios derivados del tratamiento con radiocirugia del neurinoma del acustico. Material y metodo Entre los anos 1999 y 2004, hemos tratado a 30 pacientes afectos de neurinoma del acustico (NA) unilateral, con una media de edad de 54 (31-76) anos, un tiempo medio de seguimiento de 34 (12-54) meses y un tamano que oscilo en 4-38 (media, 16,5) mm. Se practico radiocirugia a todos ellos (24 Gamma Knife y 6 LINAC) a dosis de 1.200-1.300 cGy. Resultados La tasa de control del crecimiento tumoral fue del 93 % (28/30). Un 36,6% (11/30) presento empeoramiento de su nivel de audicion postratamiento. La afeccion del V par (3/30), asi como la del VII (2/30), fue transitoria. Conclusiones Aunque la microcirugia sigue siendo el tratamiento de eleccion para los NA, consideramos la radiocirugia como una alternativa valida en pacientes seleccionados (edad avanzada, comorbilidad asociada, pequeno tamano, hipoacusia contralateral, entre otros).

Resumen Presentamos el caso de un paciente varon de 17 meses con sindrome de Duane tipo 1 bilateral asociado a displasia cocleovestibular unilateral, fistula perilinfatica y meningitis de repeticion. El diagnostico se realizo mediante TC de penascos y RM cerebral. Se describe su manejo y tratamiento, asi como la evolucion postoperatoria. Debido a la poca frecuencia de este sindrome, asi como a la de sus complicaciones de tipo otoneurologico, consideramos que se trata de un caso excepcional.

Resumen La histiocitosis de celulas de Langerhans es una enfermedad de causa desconocida con compromiso focal o diseminado y que afecta con frecuencia a la cabeza y el cuello; el compromiso craneal es una de las manifestaciones mas comunes en ninos mayores de 5 anos. Presentamos el caso de una nina de 3 anos, sin antecedentes otologicos de interes remitida por sospecha de absceso subperiostico y diagnosticada posteriormente de granuloma eosinofilico. Se describe el manejo y su evolucion.

Objective tinnitus can have many different etiologies, palatal myoclonus being one of the less frequent. This type of tinnitus is generated by involuntary rhythmic contraction of the soft palate, which generates an audible click for the patient and for the explorer. Botulinum toxin achieves temporary muscle paralysis through presynaptic inhibition of the acetylcholine level at the neuromuscular union. We present a patient with long-term objective tinnitus, along with this patient's response to botulinum toxin injection.

Langerhans cell histiocytosis is a disease of unknown aetiology which may be isolated or affect multiple organs and which frequently affects the head and neck, with cranial compromise being one of the most common manifestations in children over five years. We present the case of a three year old girl with no otologic history who came to our hospital with a clinic of subperiostic abscess, subsequently diagnosed as eosinophilic granuloma. We describe the treatment and clinical evolution of the case.

To evaluate the characteristics of hearing loss (HL) in patients with rheumatoid arthritis (RA).A comparative case-control study was performed with 194 patients and 107 healthy subjects. All of them were submitted to pure-tone audiometry and detection of inflammatory parameters and Western blot for anticochlear antibodies.HL was detected in 42.7 % of patients with RA (15.9 % in controls; P.001). This was sensorineural in 38.6 %. Three or more altered blood parameters appeared in 28.9 % of patients with RA (17.6 % in controls; P.01). We observed positive Western blot in 12 % of patients with RA and HL and none among healthy controls.There is a predisposition to HL, mainly sensorineural, in RA. In view of this prevalence, audiologic reviews must be performed to try to determine if this disorder shows an immunomediated aspect so that a therapeutic alternative could modify the course of HL.

Objective To evaluate the results and side effects obtained using radiosurgery to treat acoustic neuromas. Material and method Between 1999 and 2004 we treated 30 patients with unilateral acoustic neuromas with a mean age of 54 years old (31–76), a mean follow-up of 34 months (12–54), and tumour size between 4 and 38 mm (mean, 16.5). All of them were treated with radiosurgery (24 Gamma Knife and 6 LINAC) with doses of 1200–1300 cGy. Results Tumour growth control rate (smaller or same size) was 93% (28/30). In 11 patients there was a post-treatment hearing loss (36.6%). Trigeminal (3/30) and facial (2/30) neuropathy was transient. Conclusions Although microsurgery is the treatment of choice for acoustic neuromas, we consider radiosurgical treatment a valid alternative for selected patients (the elderly, co-morbid conditions, small size, contralateral hearing loss…).

La hipoacusia es uno de los motivos mas frecuentes de consulta en otorrinolaringologia. Ante la sospecha, es fundamental realizar un estudio audiologico completo y complementarlo con tecnicas de imagen adecuadas. La tomografia computarizada (TC) tiene una elevada sensibilidad para evaluar el conducto auditivo interno (CAI), mientras que la resonancia magnetica nuclear (RMN) aporta informacion mucho mas detallada para conocer su contenido, ya que un CAI de diametro normal en la TC podria presentar una ausencia del VIII par craneal, siendo la RMN el unico medio para detectarlo. Presentamos el caso clinico de un nino de 11 anos remitido a nuestro servicio por sospecha de hipoacusia de oido izquierdo (OI), detectada de forma casual por sus padres. No presentaba antecedentes familiares ni factores de riesgo de hipoacusia. Lenguaje normal y adaptacion escolar adecuada a su edad. No antecedentes de otitis medias de repeticion ni sintomas vestibulares. La otoscopia es normal bilateral. Figura 2

La hipoacusia es uno de los motivos mas frecuentes de consulta en otorrinolaringologia. Ante la sospecha, es fundamental realizar un estudio audiologico completo y complementarlo con tecnicas de imagen adecuadas. La tomografia computarizada (TC) tiene una elevada sensibilidad para evaluar el conducto auditivo interno (CAI), mientras que la resonancia magnetica nuclear (RMN) aporta informacion mucho mas detallada para conocer su contenido, ya que un CAI de diametro normal en la TC podria presentar una ausencia del VIII par craneal, siendo la RMN el unico medio para detectarlo. Presentamos el caso clinico de un nino de 11 anos remitido a nuestro servicio por sospecha de hipoacusia de oido izquierdo (OI), detectada de forma casual por sus padres. No presentaba antecedentes familiares ni factores de riesgo de hipoacusia. Lenguaje normal y adaptacion escolar adecuada a su edad. No antecedentes de otitis medias de repeticion ni sintomas vestibulares. La otoscopia es normal bilateral. En la audiometria tonal liminar se muestra una normoacusia del oido derecho (OD) y una cofosis del OI. Las otoemisiones acusticas transitorias fueron positivas en OD y negativas en OI. Se solicita una RMN donde aparecen unos aparatos vestibulo-cocleares normoconformados con permeabilidad de laberintos membranosos, CAI de calibre normal, pero se detecta una ausencia de visualizacion del nervio coclear (NC) en el interior del CAI izquierdo, tanto en cortes coronales (Figura 1) como axiales (Figura 2), lo que se corresponde a un estadio IIb de la clasificacion de Casselman. Figura 1. Figura 2. Actualmente, la RMN se ha impuesto a...

Resumen El acufeno objetivo o somatoacufeno puede presentar multiples etiologias, siendo una de las mas infrecuentes la mioclonia palatal generada por la contraccion ritmica e involuntaria del paladar blando que genera un click audible por el enfermo y el explorador. La toxina botulinica genera una paralisis temporal a nivel muscular mediante la inhibicion presinaptica de la acetilcolina a nivel de la union neuromuscular. Presentamos el caso de una paciente con un acufeno objetivo de larga evolucion y su respuesta a la inyeccion con toxina botulinica.