Vestibular Hypofunction Secondary to Topical Use of Aminoglycosides in ears with perforated tympanic membrane.

INTRODUCTION: The objective of this study was to identify and clinically characterize patients treated in an Otoneurology Unit who experienced vestibular ototoxicity as a result of using aminoglycoside ear drops during outbreaks of superinfection in chronic otitis media. MATERIAL AND METHODS: An observational retrospective study was conducted, including patients with perforated eardrums who developed vestibular ototoxicity within the past ten years following the application of topical ear aminoglycosides in a tertiary referral center. The study encompassed the assessment of the clinical presentation, treatment, quality of life, and evolution after treatment of the identified individuals. RESULTS: During the study period, six patients, aged between 33 and 71 years, developed vestibular ototoxicity following the use of topical aminoglycoside drops due infection flares in chronic otitis media. All cases involved the use of gentamicin. Two cases were unilateral, and four were unilateral. The onset of symptoms occurred within one to four weeks of using the drops, resulting in all patients experiencing instability without vertigo attacks. After discontinuing the drops and undergoing vestibular rehabilitation, four patients experienced sequelae, with two patients (both with bilateral vestibular hypofunction) suffering significant impairment in their quality of life. CONCLUSIONS: Conclusion: Vestibular ototoxicity due to the topical application of aminoglycosides during acute exacerbations of chronic otitis media is a rare occurrence. However, given its potential for severe consequences and the fact that we are still encountering patients with this condition, healthcare professionals should explore alternative antibacterial agents that offer similar efficacy.

Sudden hearing loss secondary to syphilis.

BACKGROUND: Syphilis is a sexually transmitted disease caused by the spirochete Treponema pallidum, whose incidence has increased significantly in recent years. Some patients may develop sudden hearing loss (SHL) against the background of otosyphilis. OBJECTIVES: The objective of our study was to determine whether routine lues serology is useful in patients presenting with sudden hearing loss. METHODS: For this purpose, all cases of SHL treated in our hospital during a period of 6 years were propectively collected. The frequency of positivity for syphilis in these patients, the treatment received, and their evolution were determined. RESULTS: Of the total number of patients evaluated during that period, 71 underwent serological screening for syphilis, of whom 2 (2.8 %) presented positive screening antibodies. In one of them, the RPR was normal and had been treated with lues a few years before. After treatment there was no improvement. The other patient, diagnosed with otosyphilis with unconfirmed suspected neurological disease, showed normalization of hearing after specific treatment. CONCLUSIONS: Since it is a potentially curable disease, despite the low overall frequency of syphilis in patients with SHL it is advisable to perform serological screening for syphilis in high risk patients (e.g., incarceration, multiple recent sexual partners, men who have sex with men) or atypical clinical presentation (e.g., concurrent neuropathies).

Sudden Hearing Loss as an Initial Symptom of Vestibular Schwannoma.

OBJECTIVE: This study aims to determine the prevalence of vestibular schwannoma (VS) among patients presenting with sudden hearing loss (SHL) and to characterize the clinical features of individuals diagnosed with both VS and SHL. METHODS: We conducted an observational retrospective review at a tertiary referral center, spanning a 30-year period, focusing on patients diagnosed with SHL where VS was confirmed as the underlying cause. We included patients meeting these criteria while excluding those lacking imaging or with a pre-existing diagnosis of VS. We evaluated the audiological characteristics at the time of diagnosis and assessed clinical outcomes following treatment. RESULTS: Among the 403 patients presenting with SHL during the study period, 9 (2.2%) were diagnosed with VS, aged between 25 and 72 years. Although audiometric profiles varied, high-frequency hearing loss predominated, mostly categorized as mild to moderate. Six patients (66%) had Koos grade I-II schwannomas. Only 2 patients achieved complete hearing recovery post-treatment, while 4 showed no improvement. CONCLUSIONS: VS is a rare etiology of SHL, accounting for slightly over 2% of cases. Its symptomatology, severity, and audiometric patterns do not significantly differ from SHL caused by other factors. Tumor size does not correlate with hearing characteristics. Treatment modalities resemble those for other SHL cases, and hearing improvement does not obviate the necessity for follow-up magnetic resonance imaging (MRI) scans.

Manifestaciones otorrinolaringológicas del Síndrome VEXAS. Revisión sistemática / Ear, nose, and throat manifestations of VEXAS Syndrome. Systematic review

Introducción y objetivo: El síndrome VEXAS (SV) es una entidad de descripción reciente que afecta fundamentalmente a varones, y se debe a una mutación somática en el gen UBA1. Puede cursar con múltiples manifestaciones sistémicas, siendo la afectación de cabeza y cuello muy frecuente. El objetivo de este estudio fue describir las manifestaciones otorrinolaringológicas del SV, que contribuyan a un diagnóstico y tratamiento temprano de la enfermedad. Método: Se realizó una revisión de la literatura médica, utilizando los criterios PRISMA adaptados al tipo de estudio, de las manifestaciones otorrinolaringológicas del SV, utilizando la base de datos PubMed. Resultados: Fueron incluidos en nuestro trabajo 81 artículos que cumplían los criterios de inclusión del mismo, los cuales describían 133 casos. Los resultados mostraron que el SV se produce, sobre todo, en varones mayores de 50 años de edad, presentando en más de la mitad de los casos, manifestaciones de cabeza y cuello, entre las que destacan la CA, la condritis nasal y el edema periorbitario. Conclusiones: Con frecuencia el SV es confundido, en los pacientes con manifestaciones otorrinolaringológicas, con la policondritis recurrente. El conocimiento por parte del otorrinolaringólogo de las manifestaciones de cabeza y cuello asociadas al mismo, puede contribuir a un diagnóstico y tratamiento temprano mejorando el pronóstico de la enfermedad. (AU)

Introduction and objectives: VEXAS syndrome (VS) is a recently described clinical entity that mainly affects males, and is due to a somatic mutation in the UBA1 gene. It can present with multiple systemic manifestations, especially hematological and dermatological, being the affectation of the head and neck very frequent, especially, auricular chondritis (AC). The objective of this study was to describe the otorhinolaryngological manifestations of VS, knowledge of which by the otolaryngologist could contribute to early diagnosis and treatment of the disease. Methods: A review of the medical literature was carried out, using the PRISMA criteria adapted to the type of study, of the otorhinolaryngological manifestations of VS, from its description in the year 2020 to December 2022, using the Pubmed database. Results: 81 articles that met the inclusion criteria were included in our work, which described 133 cases in detail. The results showed that VS occurs mainly in men over 50 years of age, presenting in more than half of the cases head and neck manifestations, among which AC, nasal chondritis and periorbital edema stand out. Conclusions: Since VS can affect any part of the body, mimicking the clinical pictures of other diseases, it has often been confused, in patients with otorhinolaryngological manifestations, especially with recurrent polychondritis. Knowledge by the otolaryngologist of the head and neck manifestations associated with it can contribute to early diagnosis and treatment, improving the prognosis of the disease. (AU)

Manifestaciones otológicas de la enfermedad relacionada con IgG4 / Otologic manifestations of IgG4-related disease

La enfermedad relacionada con IgG4 (ER-IgG4) constituye un proceso fibroinflamatorio autoinmune sistémico caracterizado por la infiltración multiorgánica de células plasmáticas positivas para IgG4, fibrosis y vasculopatía que determinan la disfunción del órgano afectado. La presente revisión tiene por objetivo caracterizar las manifestaciones otológicas de la enfermedad.Se realizó una revisión sistemática de la literatura biomédica publicada describiendo los casos de ER-IgG4 con manifestaciones otológicas. Para la búsqueda utilizamos las siguientes bases de datos: Pubmed, Web of Science y Scopus. Seleccionamos 48 casos de los que se extrajeron diferentes variables para caracterizar las manifestaciones otológicas de la misma.Alrededor del 52% de los pacientes eran varones con edades comprendidas entre los 19 y los 79 años. La forma más habitual de presentación fue lesiones seudotumorales con presentación clínica similar a una otitis seromucosa, con lesión coclear asociada o una hipoacusia neurosensorial (uni o bilateral). Presentaciones menos frecuentes fueron un síndrome vertiginoso, condritis auricular, otitis eosinofílica o paquimeningitis hipertrófica con afectación cocleovestibular. En 32 pacientes (67%) la manifestación otológica constituyó la forma de presentación de la ER-IgG4.Dieciséis pacientes (33%) fueron tratados mediante cirugías agresivas como consecuencia del retraso diagnóstico y el desconocimiento de esta entidad clínica. Dos pacientes precisaron un implante coclear.Si bien las manifestaciones óticas de la ER-IgG4 son infrecuentes, es importante que el otorrinolaringólogo conozca las formas de presentación otológicas de esta entidad, ya que pueden ser la primera manifestación de la enfermedad, permitiendo un diagnóstico precoz y un tratamiento adecuado, evitando secuelas permanentes posteriores. (AU)

IgG4-related disease (IgG4-RD) is a systemic autoimmune fibroinflammatory disease characterized by multiorgan infiltration of IgG4-positive plasma cells, fibrosis and vasculopathy that determine dysfunction of the affected organ. This review aims to characterize the otologic manifestations of the disease.We have conducted a systematic review of the biomedical literature published describing cases of IgG4-RD with otologic manifestations. For the review, the bibliographic databases utilized were Pubmed, Web of Science and Scopus. We selected 48 cases from which we extracted several data collections.About 52% of the patients were male between the ages of 19 and 79 years. Otologic findings were characterized by pseudotumoral lesions that most often caused a clinical presentation similar to otitis media with effusion, with cochlear involvement or sensorineural hearing loss (uni or bilateral). Less frequent presentations included auricular chondritis, eosinophilic otitis, or hypertrophic pachymeningitis. In 32 patients (67%) the otologic manifestation was the first symptom of the ER-IgG4.Sixteen patients (33%) were treated with mastoidectomies as a result of delayed diagnosis and lack of knowledge about this entity. Two patients needed a cochlear implant.Although the otologic manifestations of IgG4-RD are unusual, it is important for the otorhinolaryngologist to know the otologic manifestations of this entity as it can be the onset of the disease, in order to allow early diagnosis and adequate treatment, avoiding permanent sequelae.

Otologic manifestations of IgG4-related disease.

IgG4-related disease (IgG4-RD) is a systemic autoimmune fibroinflammatory disease characterized by multiorgan infiltration of IgG4-positive plasma cells, fibrosis and vasculopathy that determine dysfunction of the affected organ. This review aims to characterize the otologic manifestations of the disease. We have conducted a systematic review of the biomedical literature published describing cases of IgG4-RD with otologic manifestations. For the review, the bibliographic databases utilized were Pubmed, Web of Science and Scopus. We selected 48 cases from which we extracted several data collections. About 52% of the patients were male between the ages of 19 and 79 years. Otologic findings were characterized by pseudotumoral lesions that most often caused a clinical presentation similar to otitis media with effusion, with cochlear involvement or sensorineural hearing loss (uni- o bilateral). Less frequent presentations included auricular chondritis, eosinophilic otitis, or hypertrophic pachymeningitis. In 32 patients (67%) the otologic manifestation was the first symptom of the ER-IgG4. Sixteen patients (33%) were treated with mastoidectomies as a result of delayed diagnosis and lack of knowledge about this entity. Two patients needed a cochlear implant. Although the otologic manifestations of IgG4-RD are unusual, it is important for the otorhinolaryngologist to know the otologic manifestations of this entity as it can be the onset of the disease, in order to allow early diagnosis and adequate treatment, avoiding permanent sequelae.

Clinical Impact of Genetic Diagnosis of Sensorineural Hearing Loss in Adults.

HYPOTHESIS: Adult genetic sensorineural hearing loss (SNHL) may be underestimated. BACKGROUND: The diagnosis of genetic hearing loss is challenging, given its extreme genetic and phenotypic heterogeneity, particularly in adulthood. This study evaluated the utility of next-generation sequencing (NGS) in the etiological diagnosis of adult-onset SNHL. MATERIALS AND METHODS: Adults (>16 yr old) with SNHL were recruited at the Otolaryngology Department at Marqués de Valdecilla University Hospital (Spain). Environmental factors, acoustic trauma, endolymphatic hydrops, and age-related hearing loss were excluding criteria. An NGS gene panel was used, including 196 genes (OTOgenics v3) or 229 genes (OTOgenics v4) related to syndromic and nonsyndromic hearing loss. RESULTS: Sixty-five patients were included in the study (average age at the onset of SNHL, 41 yr). Fifteen pathogenic/likely pathogenic variants considered to be causative were found in 15 patients (23% diagnostic yield) in TECTA (4), KCNQ4 (3), GJB2 (2), ACTG1 (1), COL2A1 (1), COCH (1), COCH/COL2A1 (1), STRC (1), and ABHD12 (1). Three patients had syndromic associations (20% of patients with genetic diagnosis) that had not been previously diagnosed (two Stickler type I and one polyneuropathy, hearing loss, ataxia, retinitis pigmentosa, cataract syndrome). Seven variants of unknown significance were found in COL11A1 (1), GSMDE (2), DNTM1 (1), SOX10 (1), EYA4 (1), and TECTA (1). CONCLUSION: NGS gene panels can provide diagnostic yields greater than 20% for adult SNHL, with a significant proportion of variant of unknown significance that could potentially contribute to increasing diagnostic output. Identifying a genetic cause enables genetic counseling, provides prognostic information and can reveal unrecognized syndromes contributing to an accurate management of their associated manifestations.

Cirugía endoscópica endonasal durante la pandemia COVID-19: protocolo de actuación / Endoscopic endonasal surgery during COVID-19 pandemic: Management guideline

La actual pandemia por coronavirus SARS-CoV-2 está planteando una serie de desafíos al modo en que ejercemos la actividad médica y quirúrgica. En concreto, dentro de la neurocirugía se ha visto que los abordajes endoscópicos endonasales suponen un elevado riesgo de contagio para el personal sanitario que interviene en la misma, por lo que, inicialmente, la recomendación fue evitar dichas cirugías. Dado que la pandemia se ha extendido en el tiempo y desconocemos cuándo se podrá controlar, se deben proponer nuevas soluciones para continuar con la realización de dichos abordajes de manera segura. Ante la falta de protocolos establecidos, planteamos el siguiente, en el que se establecen, de modo conciso, las medidas a tomar tanto en cirugía urgente como programada, además de la descripción de un nuevo dispositivo de protección-aspirado (Maskpirator) AU)

Current SARS-CoV-2 coronavirus pandemic is challenging medical and surgical activities. Specifically, within neurosurgery, endoscopic endonasal approaches pose a high risk of contagion for healthcare personnel involved in it. Initially, the recommendation was to avoid such surgeries. However, the pandemic has dragged on and new solutions must be proposed to continue carrying out these approaches safely. Given the lack of established protocols, we propose the following one, which concisely establishes the measures to be taken in both urgent and scheduled surgery. In addition, a new protecti (AU)n-aspiration device (Maskpirator) is described.

Gb3/cd77 Is a Predictive Marker and Promising Therapeutic Target for Head and Neck Cancer.

Head and neck squamous cell carcinoma is the sixth leading cancer in the world. This cancer is difficult to treat and is characterized by recurrences that are often fatal. This cancer is generally removed surgically, but it often regrows from the edges of the lesion from where most recurrences reappear. In this study, we have investigated if the expression of GB3 in human cell lines, tissues from patient biopsies, and a murine animal model could be used as an early and determinant marker of HNC. We found that in all the investigated systems, this marker appears in neoplastic cells from the very early stages of their malignant transformation. Our conclusions support the hypothesis that GB3 is a reliable and independent target for HNC identification and selective delivery of treatments. Furthermore, we show that the level of expression of this marker correlates with the degree of malignancy of the tumor. These studies suggest that GB3 may provide the basis for the early identification and new targeted therapies for head and neck cancer.

Manifestaciones laríngeas de la enfermedad relacionada con IgG4. Revisión sistemática / Laryngeal manifestations of IgG4-related disease. Systematic review

Introducción y objetivos: La enfermedad relacionada con IgG4 (ER-IgG4) es un trastorno sistémico autoinmune caracterizado por la infiltración tisular por células plasmáticas positivas para IgG4, aparición de fibrosis y disfunción orgánica. Puede dar afectación a muchos niveles del organismo. El objetivo de este trabajo es identificar las posibles manifestaciones de esta enfermedad a nivel laríngeo. Material y métodos: Se realizó una revisión sistemática de la literatura médica publicada entre 2003 y abril de 2021, en busca de los casos descritos de ER-IgG4 con afectación laríngea. La búsqueda se realizó a través de las bases de datos Pubmed, Web of Science y Scopus. Se seleccionaron 23 casos, todos ellos con demostración histológica de la enfermedad a ese nivel. Resultados: La mayoría de los pacientes eran varones, con edades comprendidas entre los 41 y los 60 años. Las lesiones a nivel de la laringe mayoritarimaente tenían un aspecto pseudotumor. La zona de aparición preferente de estas fue la supraglotis. Con tratamiento corticoideo los pacientes suelen evolucionar favorablemente. Sin embargo, gran parte de estos sufrió importantes retrasos en el diagnóstico, así como cirugías agresivas. Conclusiones: La afectación laríngea es infrecuente en la ER-IgG4. Se debe tener en mente esta entidad ante lesiones pseudotumorales con biopsias negativas para malingnidad, dado que un diagnóstico temprano y un tratamiento adecuado son efectivos para prevenir secuelas importantes a largo plazo. (AU)

Introduction and objectives: IgG4-Related disease (IgG4-RD) is a systemic autoimmune disorder characterized by tissue infiltration with IgG4-secreting plasma cells, fibrosis and organic disfunc-tion. It can involve multiple sites. The objectives in this article are to identify the laryngeal involvement of this disease. Methods: A systematic review was conducted by collecting the literature published between 2003 and February 2021, seeking for case reports of IgG4-RD with laryngeal involvement. The bibliographic databases utilized were Pubmed, Web of Science and Scopus. 22 cases were selected, all of them including histological findings in the larynx. Results: Most of the patients were male, between 41 and 60 years old. The most common laryngeal manifestations were pseudotumoral neoformations. The supraglottis was the area were most of these lesions appeared. In this disease the standard treatment involves glucocorticoids, often with an optimal clinical response. However, a great part of the patients included in the review got a delayed diagnosis, some of them leading to aggressive surgical procedures. Conclusions: Laryngeal mani-festations in the IgG4-Related disease are unusual. That ́s why it should be considered when considering the differential diagnosis of malignant tumors. This is an illness that needs to be suspected in some cases, to ensure a prompt diagnosis and a proper treatment, thus avoiding long-term sequelae. (AU)

Manifestaciones de cabeza y cuello secundarias al uso de cocaína. Revisión bibliográfica / Head and neck manifestations of cocaine abuse. Review

Introducción y objetivo: El consumo de cocaína se ha asociado a patología a múltiples niveles, pero ésta es especialmente relevante a nivel de cabeza y cuello, por ser la vía intranasal una de sus más frecuentes vías de consumo. Este trabajo describe las principales manifestaciones clínicas del consumo de cocaína en el área otorrinolaringológica, y se analizan algunos de los diagnósticos diferenciales que se han de descartar. Método: Se realizó una revisión bibliográfica vía, PubMed, Cochrane y Google Schoolar con artículos publicados entre 1999 y 2019 con la palabra cocaine junto con diferentes términos de búsqueda, siguiendo los criterios PRISMA para la selección de artículos. Resultados: La búsqueda inicial redujo los artículos a 641, finalizando la selección en 63 artículos, a los cuales se añadieron otros 66 desde otras fuentes. Finalmente, 129 artículos fueron incluidos en la presente revisión sistemática. Conclusiones: La necrosis de la línea media y la perforación septal son las manifestaciones más frecuentemente descritas asociadas al abuso de cocaína. Sin embargo, esta sustancia puede originar otras muchas lesiones en cabeza y cuello, generalmente de difícil diagnóstico, sobre todo cuando el paciente niega el abuso de sustancias. (AU)

Introduction and objective: Cocaine abuse has been linked to pathology in multiple levels, but especially relevant are the ones located in the head and neck area, since one of the most used routes of administration is precisely through the nose (snorted). This paper describes the main clinical manifestations of cocaine use in the otolaryngologic area, and analyses some of the differential diagnoses that must be ruled out. Method: A systematic review was conducted via PubMed, Cochrane and Google Schoolar with articles published between 1999 and 2019 using the word «cocaine » together with some other search terms, following the PRISMA criteria for the selection of articles. Results: The initial search reduced the articles to 641, from which 63 articles were selected based on their content. In addition to those, another 66 were added from different sources. Finally, 129 articles were included in this systematic review. Conclusions: Midline necrosis and septal perforation are the most common manifestations described as due to cocaine abuse. However, this substance can cause many other head and neck lesions, usually difficult to diagnose, especially when the patient denies substance abuse. (AU)

[Endoscopic endonasal surgery during COVID-19 pandemic: Management guideline]. / Cirugía endoscópica endonasal durante la pandemia COVID-19: protocolo de actuación.

Current SARS-CoV-2 coronavirus pandemic is challenging medical and surgical activities. Specifically, within neurosurgery, endoscopic endonasal approaches pose a high risk of contagion for healthcare personnel involved in it. Initially, the recommendation was to avoid such surgeries. However, the pandemic has dragged on and new solutions must be proposed to continue carrying out these approaches safely. Given the lack of established protocols, we propose the following one, which concisely establishes the measures to be taken in both urgent and scheduled surgery. In addition, a new protection-aspiration device (Maskpirator) is described.

Endoscopic endonasal surgery during COVID-19 pandemic: Management guideline.

Current SARS-CoV-2 coronavirus pandemic is challenging medical and surgical activities. Specifically, within neurosurgery, endoscopic endonasal approaches pose a high risk of contagion for healthcare personnel involved in it. Initially, the recommendation was to avoid such surgeries. However, the pandemic has dragged on and new solutions must be proposed to continue carrying out these approaches safely. Given the lack of established protocols, we propose the following one, which concisely establishes the measures to be taken in both urgent and scheduled surgery. In addition, a new protection-aspiration device (Maskpirator) is described.