Los pioneros en el conocimiento y tratamiento de los paragangliomas carotídeos / Pioneers in the knowledge and treatment of carotid paragangliomas

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Extended phenotype and clinical subgroups in unilateral Meniere disease: A cross-sectional study with cluster analysis.

OBJECTIVES: To define clinical subgroups by cluster analysis in patients with unilateral Meniere disease (MD) and to compare them with the clinical subgroups found in bilateral MD. DESIGN: A cross-sectional study with a two-step cluster analysis. SETTINGS: A tertiary referral multicenter study. PARTICIPANTS: Nine hundred and eighty-eight adult patients with unilateral MD. MAIN OUTCOME MEASURES: best predictors to define clinical subgroups with potential different aetiologies. RESULTS: We established five clusters in unilateral MD. Group 1 is the most frequently found, includes 53% of patients, and it is defined as the sporadic, classic MD without migraine and without autoimmune disorder (AD). Group 2 is found in 8% of patients, and it is defined by hearing loss, which antedates the vertigo episodes by months or years (delayed MD), without migraine or AD in most of cases. Group 3 involves 13% of patients, and it is considered familial MD, while group 4, which includes 15% of patients, is linked to the presence of migraine in all cases. Group 5 is found in 11% of patients and is defined by a comorbid AD. We found significant differences in the distribution of AD in clusters 3, 4 and 5 between patients with uni- and bilateral MD. CONCLUSIONS: Cluster analysis defines clinical subgroups in MD, and it extends the phenotype beyond audiovestibular symptoms. This classification will help to improve the phenotyping in MD and facilitate the selection of patients for randomised clinical trials.

Afectación vestibular en el CHARGE. Ver lo invisible / Vestibular impairment in CHARGE syndrome. To see the invisible

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Familial clustering and genetic heterogeneity in Meniere's disease.

The aims of this study were to estimate the prevalence of familial cases in patients with Meniere's disease (MD) and to identify clinical differences between sporadic and familial MD. We recruited 1375 patients with definite MD according to the American Academy of Otolaryngology-Head and Neck Surgery criteria, obtaining the familial history of hearing loss or episodic vertigo by direct interview or a postal survey in 1245 cases in a multicenter study. Familial clustering was estimated by the recurrence risk ratio in siblings (λs ) and offspring (λo ) using intermediate and high prevalence values for MD in European population. A total of 431 patients (34%) reported a familial history of hearing loss or recurrent vertigo and 133 patients had a relative with possible MD. After clinical reevaluation, 93 relatives in 76 families were diagnosed of definite MD (8.4%), including three pairs of monozygotic twins. λs and λo were 16-48 and 4-12, respectively. We observed genetic heterogeneity, but most families had an autosomal dominant inheritance with anticipation. No clinical differences were found between sporadic and familial MD, except for an early onset in familial cases. We may conclude that MD has a strong familial aggregation and that sporadic and familial MDs are clinically identical.

Hearing and vestibular disorders in patients with systemic lupus erythematosus.

OBJECTIVES: Systemic lupus erythematosus (SLE) is associated with several comorbidities, including hearing and vestibular disorders. We recently described an increase of SLE prevalence in patients with Menierés disease (MD). The aim of this study is to explore if a subset of SLE patients may have a common inner ear disorder and determine the potential relationship with migraine. METHODS; Eighty-nine patients with SLE (according to the American College of Rheumatology criteria for the diagnosis of SLE) were evaluated for audiovestibular symptoms by a telephone interview carried out by two experienced otoneurologists. Twenty-one patients with SLE were referred to the otoneurology clinic for a detailed clinical history for criteria assessment for MD and a complete audiovestibular examination (audiogram, oculomotor, and caloric testing with video-oculography). RESULTS: Recurrent headache was found in 50/89 patients, and 26 of them had diagnostic criteria for migraine. Twenty-four percent of patients reported sensorineural hearing loss (SNHL) or episodic vertigo. Among the eight patients (9%) with episodic vertigo, one had criteria for definite MD and another two patients had criteria for possible MD. SNHL was found to be associated with a history of episodic vertigo (Fisher's test, p=0.02), but not with headache or migraine. SLE and episodic vertigo were not associated with tinnitus, migraine, lupus nephritis, antinuclear antibodies or antiphospholipid syndrome. CONCLUSIONS: Migraine, SNHL and episodic vertigo are comorbid conditions in patients with SLE, but migraine is not associated with SNHL or vertigo in these patients. However, SNHL and vertigo are associated conditions in SLE, suggesting a common audiovestibular dysfunction.

[History and epidemiology]. / Recuerdo histórico y epidemiología.

The development of knowledge of paragangliomas has been inextricably linked to the development of all fields of medicine. Throughout history, both knowledge of diseases and technical improvements in diagnosis have served to understand these tumors, although unanswered questions remain. From the XVIII century to the XXI century events sometimes unexpected and other times intensely sought have occurred that have helped to identify the characteristics of these tumors. These events have ranged from description of paragangliomas to their genetic diagnosis. The incidence of paragangliomas is not high. Nevertheless, the variability of these tumors in terms of their localization, forms of presentation, possibilities of metastasis or their hereditary component has been well characterized.

Recuerdo histórico y epidemiología / History and epidemiology

El desarrollo del conocimiento de los paragangliomas ha ido inexorablemente unido al desarrollo de la medicina en todos sus campos. Tanto el propio conocimiento de las enfermedades como la mejora técnica en el diagnóstico han servido, a lo largo de la historia, para llegar a entender esta patología, que presenta, por otra parte, interrogantes sin resolver. Desde el siglo XVIII hasta el siglo XXI se han ido sucediendo acontecimientos, a veces ocasionales y a veces profundamente buscados, que han ido revelando cómo es esta patología, desde la descripción de la enfermedad hasta el diagnóstico genético. Su incidencia no es elevada pero, a pesar de ello, su variabilidad en cuanto a localización, formas de presentación, posibilidad de metastatizar o su componente hereditario está perfectamente estudiada (AU)

The development of knowledge of paragangliomas has been inextricably linked to the development of all fields of medicine. Throughout history, both knowledge of diseases and technical improvements in diagnosis have served to understand these tumors, although unanswered questions remain. From the XVIII century to the XXI century events–sometimes unexpected and other times intensely sought–have occurred that have helped to identify the characteristics of these tumors. These events have ranged from description of paragangliomas to their genetic diagnosis. The incidence of paragangliomas is not high. Nevertheless, the variability of these tumors in terms of their localization, forms of presentation, possibilities of metastasis or their hereditary component has been well characterized (AU)

Linfangiomas de cavidad oral en edad pediátrica / Lymphangiomas of the oral cavity in childhood

Los linfangiomas son tumores raros y benignos producidos por el desarrollo anómalo de los vasos linfáticos. Presentamos un caso de linfangioma quístico asintomático localizado en paladar duro; se trata de una localización atípica, puesto que la mayoría de esas lesiones se localizan en la lengua a nivel de cavidad oral. Se realizó una resección con láser de CO2, sin que se evidenciase recidiva tras dos años de seguimiento. Actualmente se acepta adoptar una actitud expectante o el tratamiento con agentes esclerosantes que reduzcan su tamaño o los eliminen (AU)

Lymphangiomas are rare, benign tumors produced by the abnormal development of the lymphatic vessels. We present a case of asymptomatic cystic lymphangioma localized in the hard palate. This is an uncommon location since most of these lesions are found in the tongue at the level of the oral cavity. Resection was performed with CO laser, and there was no evidence of recurrence after two years of follow-up. At the present time, the approaches most widely accepted are expectant treatment or administration of sclerosing agents that reduce the size of the lesions or eliminate them (AU)

El absceso retrofaríngeo en la edad infantil / Childhood retropharyngeal abscess

Introducción: Las infecciones del espacio retrofaríngeo tienen una baja incidencia. Suceden entre los 2 y los 5 años de edad normalmente. Son infecciones polimicrobianas, aunque dominan los estreptococos, estafilococos y gérmenes anaerobios. Producen dolor faríngeo, fiebre y disfagia, asociada a rigidez cervical. En la exploración se observa un abombamiento de la pared posterior faríngea. Caso clínico: Presentamos el caso de un niño de 2 años que presentó un absceso de 11 cm3 de volumen, y otro de una niña de 4 años con un absceso de 8 cm3, por lo que se procede a la incisión y el drenaje del absceso por vía transoral de entrada. Discusión: La tomografía computarizada (TC) cervical es la prueba diagnóstica de elección; permite realizar el diagnóstico en estadio temprano, así como la diferenciación entre celulitis y absceso. El absceso retrofaríngeo rara vez se resuelve espontáneamente, por lo que es necesario administrar antibioterapia intravenosa y, si es mayor de 3 mL, un drenaje quirúrgico por vía transoral o externa mediante cervicotomía lateral. Conclusión: La prueba estándar es la TC. Las complicaciones pueden ser muy graves(AU)

Introduction: The incidence of retropharyngeal infection is low. The usually occur in children between the ages of 2 and 5 years. They are often polymicrobial infections, although Streptococcus, Staphylococcus, and anaerobic organisms are especially predominant. The clinical symptoms are sore throat, fever, dysphagia and stiff neck. Physical exploration reveals a bulging of the posterior pharyngeal wall. Case report: We report the cases of a two-year-old boy with and abscess of 11 cm3 and a four-year-old girl with an abscess of 8 cm3 that required transoral surgical drainage. Discussion: Computed tomography of the neck is the diagnostic technique of choice as it enables the early detection of abscesses and makes it possible to distinguish between and abscess and cellulitis. Retropharyngeal abscesses rarely resolve spontaneously and, thus, require intravenous antibiotic therapy and, when the volume is greater thar 3 cc., transoral surgical drainage or external drainage via lateral neck incision. Conclusion: Computed tomography is the gold standard for the diagnosis of this entity. Very severe complications can develop(AU)

Malformaciones arteriovenosas durales en el ámbito de la ORL / Arterioveinous dural malformation in ENT

La Malformación Arteriovenosa Dural (MAVD) es una entidad rara dentro de las anomalías vasculares intracraneales, con etiología nobien conocida y variabilidad en sus tratamientos. Presentamos once casos de MAVDs de entre los cuales 5 presentaban el acúfeno como síntoma de aparición. La importancia de esta patología hace necesario descartarla ante un paciente que consulta por un acúfeno pulsátil con otoscopia normal

The arterioveinous dural malformation (MAVD) is a rare entity between the vascular craneal anomalies with a not well nown ethiology and variability in treatments. We present eleven cases of MAVDs, between them five presented tinnitus as symptom of aparition. The importance of this pathology makes necessary to discard it before a patient consulting because of pulsaltil tinnitus with normal otoscopy

[Fistula of the first branchial arch]. / Fístula de primer arco branquial.

The complexity of the embriologic development of the neck and the possible abnomarlies in this process, with the consequent posterior manifestations make necessary the knowledge of the embriologic anatomy of the neck. In the possible manifestations that can be of the anomalies of the development of the branchial archs are the abnomarlies of the first branchial archs. They are, in the anomalies of the development of the branchial archs infrequent and are intimately related with the facial nerve. We present a clinic case of a seven years old boy affected by this pathology and revise its more important aspects.

Fístula del primer arco branquial / Fistula of the first branchial arch

La complejidad del desarrollo embriológico del cuello y las posibles del cuello y las posibles anomalías en este proceso, con las consiguientes manifestaciones posteriores hacen necesario el conocimiento de la anatomía embriológica del cuello. Dentro de las posibles manifestaciones que pueden darse de las anomlías del desarrollo de los arcos branquiales se encuentran las anomalías del primer arco branquial. Son dentro de las anomalías del desarrollo de los arcos branquiales, infrecuentes y están íntimamente relacionadas con el nervio facial. Presentamos el caso clínico correspondiente a un niño de 7 años con esta patología y repasamos los aspectos más importantes de la misma

The complexity of the embryologic development of the neck and the possible abnormalies in thi process, with the consequent posterior manifestations make necessary the knowledge of the embryologic anatomy of the neck. In the possible manifestations that can be of the abnormalies of the development of the branchial archs are the abnormalies of the first branchial archs. The are, in the anomalies of the development of the branchial archs infrequent and are intimately related with the facial nerve. We present a clinic case of a seven years old boy affected by this pathology and revise its more important aspects

[Arterioveinous dural malformation in ENT]. / Malformaciones arteriovenosas durales en el ambito de la ORL.

The arterioveinous dural malformation (MAVD) is a rare entity between the vascular craneal anomalies with a not well nown ethiology and variability in treatments. We present eleven cases of MAVDs, between them five presented tinnitus as symptom of aparition. The importance of this pathology makes necessary to discard it before a patient consulting because of pulsaltil tinnitus with normal otoscopy.

Tortícolis y vértigo paroxístico / Tortícolis and vértigo paroxístico

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[Persistence of the third branchial arch]. / Persistencia de tercer arco branquial.

The third branquial arch anomalies represent less than 1% of this deformites. The clinical manifestation can be varied althouth the formation of cysts is the commonest one and may appear at any time in life. We report the clinical case of a 7 year old boy with an neck mass. This mass was diagnosed as a branquial cyst. The patologist found a "timic reminescences". We carry out a review of the literature for this pathology.

Persistencia de tercer arco branquial / Persistence of the third branchial arch

Las anomalías derivadas del desarrollo del tercer arco y bolsa branquiales representan menos del 1% del total de estas malformaciones. Su forma de manifestación puede ser variada, aunque la más frecuente es la forma quística y pueden aparecer en cualquier etapa de la vida. Presentamos el caso clínico correspondiente a un paciente de 7 años con una tumoración laterocervical derecha de un año de evolución, diagnosticado de quiste branquial y con un estudio anatomopatológico tras la extirpación de restos tímicos. De igual modo realizamos una revisión de la literatura de estos casos

The third branquial arch anomalies represent less than 1% of this deformites. The clinical manifestation can be varied althouth the formation of cysts is the commonest one and may appear at any time in life. We report the clinical case of a 7 year old boy with an neck mass. This mass was diagnosed as a branquial cyst. The patologist found a "timic reminescences". We carry out a review of the literature for this pathology

[Cochlear implants in obliterated or ossified cochleas]. / Implantes cocleares en cócleas obliteradas o semiosificadas.

Our last cases of cochlear implants in obliterated or ossified cochleas are presented, and we had described the surgical technique for double-array or compressed system. In our opinion the best surgical method is the combined approach (endoaural with aticotomy view and posterior timpanotomy) for increased promontory view. This technique provides the advantage that it always preserve the posterior wall of the external ear canal, and the array is positioned easily.

Implantes cocleares en cócleas obliteradas o semiosificadas / Cochlear implants in obliterated or ossified cochleas

Presentamos los últimos casos de IC en cócleas obliteradas u osificadas, describiendo la técnica quirúrgica más apropiada para el empleo de los portaelectrodos bífidos o comprimidos, destacando la importancia de la vía combinada (endoaural con aticotomía y timpanotomía posterior) para lograr la mejor visualización del promontorio. Este método tiene la ventaja de respetar la pared posterior del CAE en todos los casos y lograr con facilidad la colocación del implante

Our last cases of cochlear implants in obliterated or ossified cochleas are presented, and we had described the surgical technique for double-array or compressed system. In our opinion the best surgical method is the combined approach (endoaural with aticotomy view and posterior timpanotomy) for increased promontory view. This technique provides the advantage that it always preserve the posterior wall of the external ear canal, and the array is positioned easily