Sudden hearing loss secondary to syphilis Rodríguez-Martín, Minerva; Cantón-Benito, Elena; Méndez-Legaza, José Manuel ...
American journal of otolaryngology,
May-June 2024, 2024 May-Jun, 2024-05-00, 20240501, Volume:
45, Issue:
3
Journal Article
Peer reviewed
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.
The objective of our study was to determine whether routine lues serology is useful in patients presenting with sudden hearing loss.
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.
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.
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).
Adult genetic sensorineural hearing loss (SNHL) may be underestimated.
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.
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.
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).
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.
Over the last few years, oral and pharyngeal signs and symptoms due to oral sex have increased significantly. However, no review articles related to this subject have been found in the medical ...literature. The objective of our study was to identify otorhinolaryngological manifestations associated with orogenital/oroanal contact, both in adults and children, in the context of consensual sex or sexual abuse.
We performed a review of the medical literature on otorhinolaryngological pathology associated with oral sex published in the last 20 years in the PubMed database.
Otorhinolaryngological manifestations secondary to oral sex practice in adults can be infectious, tumoral or secondary to trauma. The more common signs and symptoms found in the literature were human papillomavirus infection (above all, condyloma acuminata and papilloma/condyloma), oral or pharyngeal syphilis, gonococcal pharyngitis, herpes simplex virus infection and pharyngitis from Chlamydia trachomatis. The incidence of human papillomavirus -induced oropharyngeal carcinoma has dramatically increased. In children past the neonatal period, the presence of condyloma acuminatus, syphilis, gonorrhoea or palatal ecchymosis (the last one, unless justified by other causes) should make us suspect sexual abuse.
Sexual habits have changed in the last decades, resulting in the appearance of otorhinolaryngological pathology that was rarely seen previously. For this reason, it is important for primary care physicians to have knowledge about the subject to perform correct diagnosis and posterior treatment. Some sexual abuse cases in children may also be suspected based on the knowledge of the characteristic oropharyngeal manifestations secondary to them.
Introducción y objetivo: La hipoacusia súbita neurosensorial unilateral (HSN) plantea un diagnóstico diferencial con múltiples patologías. El schwannoma vestibular (SV) es una de ellas en el 1-5% de ...los casos. Describimos 3 casos clínicos diagnosticados en nuestro centro.Método: Se realizó un estudio retrospectivo de los pacientes diagnosticados de hipoacusia súbita en nuestro centro durante 17 años y se describió los casos que finalmente presentaba schwannoma vestibular tras completar pruebas de imagen. Resultados: Se registraron un total de 230 pacientes, de los cuales tan solo 3 fueron posteriormente diagnosticados de SV. Todos ellos recibieron tratamiento con corticoides orales y cámara hiperbárica sin mejoría. Entorno al mes del episodio se realizó resonancia magnética (RMN) que confirmó el diagnóstico. El primer paciente fue un varón de 61 años con antecedente de hipoacusia neurosensorial moderada que presentaba clínica de vértigo por hipofunción vestibular y cofosis derecha y precisó tratamiento quirúrgico. El segundo fue un varón de 49 años con clínica de hipoacusia derecha moderada en agudos, sin afectación vestibular, que fue diagnosticado de SV, grado Koos 1 según RMN, y recibió tratamiento con radiocirugía, sin crecimiento posterior. El último paciente fue un varón de 25 años con hipoacusia neurosensorial derecha moderada en agudos que presentaba SV grado Koos 3 según RMN y recibió tratamiento quirúrgico. Discusión: La hipoacusia súbita neurosensorial puede ser el síntoma de debut, e incluso el único síntoma que presenten, pacientes con schwannoma vestibular. Los avances en las pruebas de imagen han hecho posible que hoy día la prueba definitiva para el diagnóstico de SV sea la RMN, especialmente la secuencia T1 con contraste de gadolinio, siendo posible objetivar incluso los más pequeños; es por ello, que en las guías actuales para el manejo de la hipoacusia súbita se aconseja la realización de esta prueba de forma preferente para evitar retraso diagnóstico pese a la buena respuesta con tratamiento corticoideo. Aunque no es el caso de nuestros pacientes, debemos considerar, que al contrario de lo que inicialmente se puede pensar, la mejoría tras tratamiento no excluye el diagnóstico de SV. Conclusiones: La hipoacusia súbita neurosensorial puede ser el síntoma principal de manifestación del SV hasta en el 1,3% de casos de HSN en nuestro estudio. Debe tratarse como urgencia otológica y realizar tratamiento con corticoides así como RMN de forma preferente.
Hypertrophic pachymeningitis is an infrequent inflammatory disease resulting in thickening and fibrosis of the dura mater. In most cases, the cause in unknown and is called idiopathic hypertrophic ...pachymeningitis (IHP). Audiovestibular symptoms are infrequent and the pathogenesis is still unclear.
A systematic literature review of cases with IHP and vestibular symptoms from 2000 to February 2020 was performed. PRISMA Checklist was followed and PubMED database, Web of Science and Cochrane library were searched. We report a case of an adolescent with a diagnosis of vestibular neuritis in the context of IHP attended in our clinic.
We reviewed 5 articles related to IHP and vestibular disorders. A total of 7 cases (5 women and 2 men), with ages between 27 and 68 years with IHP were found. They all had audiovestibular symptoms. In contrast to our patient, uni or bilateral neurosensorial hearing loss was reported in all cases. Furthermore, there is no other case report published describing the association between IHP and vestibular neuritis. High dose steroids improved symptoms in 85.7% of the patients.
Vestibular symptoms in IHP are uncommon and the pathogenesis is still debatable. Entrapment of nerves in the internal auditory canal and secondary neuronal damage could be suspected as the main cause of hearing and vestibular loss.
La paquimeningitis hipertrófica es un proceso inflamatorio fibrosante infrecuente de la duramadre intracraneal. En la mayor parte de los casos la causa es desconocida y se denomina paquimeningitis hipertrófica idiopática (PHI). La sintomatología audiovestibular asociada es poco frecuente y su patogenia no está aclarada.
Se realizó una revisión sistemática de literatura médica de los casos publicados de PHI asociados a sintomatología audiovestibular desde el año 2000 a febrero 2020. Se siguieron los criterios PRISMA para revisiones sistemáticas evaluando las bases de datos electrónicas PubMED, Web of Science y Cochrane. Se complementa con el caso clínico de una adolescente que debutó con neuritis vestibular en el contexto de PHI atendida en nuestro servicio.
Se han revisado 5 artículos con casos publicados de PHI que asociaban sintomatología audiovestibular. Se encontraron un total de 7 casos (5 mujeres y 2 varones), con edades comprendidas entre los 27 y 68 años, con diagnóstico de PHI. El 100% presentaron sintomatología audiovestibular. A diferencia de nuestra paciente, todos presentaron hipoacusia neurosensorial uni o bilateral. No se ha encontrado ningún otro caso descrito de asociación entre PHI y neuritis vestibular. El tratamiento empleado fueron los corticoides consiguiendo mejoría en el 87,5% de los pacientes.
Las manifestaciones vestibulares en el contexto de una PHI son muy poco fre-cuentes. La patogénesis no está clara. La principal causa de la hipoacusia y la sintomatologíavestibular podría ser el atrapamiento de los nervios en el conducto auditivo interno con dañoneuronal secundario. <q16.
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