Interaction effect of tegmen tympani and superior semicircular canal statuses on the thickness of the roof of the glenoid fossa: a cross-sectional descriptive study.

PURPOSE: Homogeneous development of temporal bone structures is explained by their ontogenic origin; tegmen tympani (TT) and superior semicircular canal (SSC) are related with the glenoid fossa at the temporomandibular joint (TMJ). Therefore, our objective was to determine a possible relationship between TT status (dehiscence or integrity) and the roof of the glenoid fossa (RGF) thickness; SSC status has also been considered. METHODS: This cross-sectional descriptive study was conducted in two tertiary hospitals on 95 patients (109 ears) presenting hypoacusia, facial palsy, vertigo, tinnitus, and other single or combined symptoms, and submitted to a thin-section multidetector-row computed axial tomography (CT) scan. RESULTS: A significant interaction effect of TT × SSC statuses on RGF thickness was found (p = 0.049). A significant difference in RGF thickness was found only for SSC integrity status between TT integrity and TT dehiscence (p = 0.004). The TT dehiscence increased the risk for RGF dehiscence 12.047 times (p = 0.002). CONCLUSIONS: There is an interaction effect of the statuses of both TT and SSC on the thickness of the RGF, instead of an independent effect of the TT status. When RGF dehiscence is found, TT and SSC statuses should be assessed, to discard associated dehiscences.

Proinflammatory cytokines and response to molds in mononuclear cells of patients with Meniere disease.

Epidemiological studies have found a higher prevalence of allergic symptoms and positive prick tests in patients with Meniere's disease (MD); however the effect of allergenic extracts in MD has not been established. Thus, this study aims to determine the effect of Aspergillus and Penicillium stimulation in cytokine release and gene expression profile in MD. Patients with MD showed higher basal levels of IL-1ß, IL-1RA, IL-6 and TNF-α when compared to healthy controls. We observed that IL-1ß levels had a bimodal distribution suggesting two different subgroups of patients, with low and high basal levels of cytokines. Gene expression profile in peripheral blood mononuclear cells (PBMC) showed significant differences in patients with high and low basal levels of IL-1ß. We found that both mold extracts triggered a significant release of TNF-α in MD patients, which were not found in controls. Moreover, after mold stimulation, MD patients showed a different gene expression profile in PBMC, according to the basal levels of IL-1ß. The results indicate that a subset of MD patients have higher basal levels of proinflammatory cytokines and the exposure to Aspergillus and Penicillium extracts may trigger additional TNF-α release and contribute to exacerbate inflammation.

Influence of Sex and Age on Posterior Semicircular Canal Thickness.

OBJECTIVE: The aim of this study is to determine whether sex and age influence posterior semicircular canal (PSC) thickness. METHODS: This observational study was conducted in 3 tertiary hospitals. The minimal distance between the PSC and the posterior cranial fossa (PSC thickness) was estimated by thin-section multidetector row computed axial tomography (CAT) scan of the temporal bones. Nonselected consecutive patients of all ages (607 temporal bones) were considered. RESULTS: A significant effect was only detected for sex (F = 5.418, p = 0.020); PSC thickness showed a higher mean value in women (mean difference ± SE: 0.224 ± 0.096 mm). A significant and negative r value was detected for males aged >45 years (-0.173, p = 0.026); in that group of patients, PSC thickness decreased as age increased (0.018 ± 0.008 mm/year). For females aged ≤45 years, a significant and positive r value was found (0.198, p = 0.022); in that group, PSC thickness increased as age increased (0.020 ± 0.008 mm/year). CONCLUSIONS: PSC thickness did not significantly evolve with age in young males (≤45 years) but it decreased from age 45 years onwards. On the other hand, PCS thickness increased with age in women until the age of 45 years and it did not significantly change in older females.

Clinical Subgroups in Bilateral Meniere Disease.

Meniere disease (MD) is a heterogeneous clinical condition characterized by sensorineural hearing loss, episodic vestibular symptoms, and tinnitus associated with several comorbidities, such as migraine or autoimmune disorders (AD). The frequency of bilateral involvement may range from 5 to 50%, and it depends on the duration of the disease. We have performed a two-step cluster analysis in 398 patients with bilateral MD (BMD) to identify the best predictors to define clinical subgroups with a potential different etiology to improve the phenotyping of BMD and to develop new treatments. We have defined five clinical variants in BMD. Group 1 is the most frequently found, includes 46% of patients, and is defined by metachronic hearing loss without migraine and without AD. Group 2 is found in 17% of patients, and it is defined by synchronic hearing loss without migraine or AD. Group 3, with 13% of patients, is characterized by familial MD, while group 4, that includes 12% of patients, is associated by the presence of migraine in all cases. Group 5 is found in 11% of patients and is defined by AD. This approach can be helpful in selecting patients for genetic and clinical research. However, further studies will be required to improve the phenotyping in these clinical variants for a better understanding of the diverse etiological factors contributing to BMD.

Association Between Tegmen Tympani Status and Superior Semicircular Canal Pattern.

OBJECTIVE: Detecting and quantifying the possible association between tegmen tympani (TT) status and superior semicircular canal (SSC) pattern. DESIGN: Observational study. SETTING: Study conducted in three tertiary Spanish hospitals. PATIENTS: Nonselected consecutive patients of all ages (607 temporal bones). INTERVENTIONS: Thin-section multidetector row computed axial tomography (CAT scan) of the temporal bones. MAIN OUTCOME MEASURE: Thickness of SSC bone coverture adjacent to the middle fossa, and TT status as a dichotomous variable: dehiscence (TTD) or integrity (TTI). RESULTS: The observed SSC patterns were dehiscence (3.79%), papyraceous or thin (11.20%), normal (76.77%), thick (4.94%), and pneumatized (3.29%). The observed TT statuses were TTD (10.87%) and TTI (89.13%). TTD was associated with SSCD and papyraceous patterns, and TTI percentages were higher in normal and thick patterns (χ2 = 11.102; p = 0.001). The TTD probability was estimated as a function of SSC pattern and age by a multivariate binary logistics regression model (χ2 = 45.939; p < 0.001). CONCLUSION: SSC pattern was significantly associated with TT status. Age influenced this association. The risk for TTD increased by 4.1% per each year of increasing age, did not differ significantly for normal and thick patterns, and increased 12 times and 20 times for papyraceous and SSCD patterns, respectively.

Intronic variants in the NFKB1 gene may influence hearing forecast in patients with unilateral sensorineural hearing loss in Meniere's disease.

Meniere's disease is an episodic vestibular syndrome associated with sensorineural hearing loss (SNHL) and tinnitus. Patients with MD have an elevated prevalence of several autoimmune diseases (rheumatoid arthritis, systemic lupus erythematosus, ankylosing spondylitis and psoriasis), which suggests a shared autoimmune background. Functional variants of several genes involved in the NF-κB pathway, such as REL, TNFAIP3, NFKB1 and TNIP1, have been associated with two or more immune-mediated diseases and allelic variations in the TLR10 gene may influence bilateral affectation and clinical course in MD. We have genotyped 716 cases of MD and 1628 controls by using the ImmunoChip, a high-density genotyping array containing 186 autoimmune loci, to explore the association of immune system related-loci with sporadic MD. Although no single nucleotide polymorphism (SNP) reached a genome-wide significant association (p<10(-8)), we selected allelic variants in the NF-kB pathway for further analyses to evaluate the impact of these SNPs in the clinical outcome of MD in our cohort. None of the selected SNPs increased susceptibility for MD in patients with uni or bilateral SNHL. However, two potential regulatory variants in the NFKB1 gene (rs3774937 and rs4648011) were associated with a faster hearing loss progression in patients with unilateral SNHL. So, individuals with unilateral MD carrying the C allele in rs3774937 or G allele in rs4648011 had a shorter mean time to reach hearing stage 3 (>40 dB HL) (log-rank test, corrected p values were p = 0.009 for rs3774937 and p = 0.003 for rs4648011, respectively). No variants influenced hearing in bilateral MD. Our data support that the allelic variants rs3774937 and rs4648011 can modify hearing outcome in patients with MD and unilateral SNHL.

Peculiaridades en el desarrollo del canal semicircular superior / Peculiarities in the development of the superior semicircular canal

Objetivos: Realizar un estudio sobre la ontogénesis del canal semicircular superior con el fin de describir sus peculiaridades. Métodos: Para ello se han analizado 76 series embriológicas humanas de edades comprendidas entre los 32 días (6 mm) y recién nacidos. Las preparaciones estaban cortadas en serie y teñidas con la técnica de tricrómico de Martins. Resultados: En el desarrollo del canal semicircular hemos observado una serie de peculiaridades, como: secuencia cronológica definida de su osteogénesis con un ritmo de osificación variable, cada núcleo de osificación interviene en la formación de una de sus cubiertas, el superior de la superficial y el inferior de la profunda; la aparición de una dehiscencia transitoria, y el cierre del canal por hueso de tipo laminar con un grosor mínimo de 0,1 mm. Conclusión: Las peculiaridades en el desarrollo del canal podrían explicar las causas del origen de la dehiscencia patológica del mismo, ya sean congénitas o adquiridas (AU)

Objective: Our objective was to study the ontogeny of the superior semicircular canal in order to describe its peculiarities. Methods: We analyzed 76 series of human embryos aged between 32 days (6 mm) and newborns. The samples were cut serially and stained using Martin's trichrome technique. Results: In semicircular canal development there were a number of peculiarities, such as: a defined chronological sequence of osteogenesis with a variable rate of ossification; the fact that each nucleus of ossification was involved in the formation of one of its covers (the upper in the superficial and the lower in the deep); the appearance of transitory dehiscence; and canal closure by means of bone with laminar pattern, with a minimum thickness of 0.1 mm. Conclusion: The peculiarities in canal development could explain the origin of pathological dehiscence in the canal, whether congenital or acquired (AU)

[Peculiarities in the development of the superior semicircular canal]. / Peculiaridades en el desarrollo del canal semicircular superior.

OBJECTIVE: Our objective was to study the ontogeny of the superior semicircular canal in order to describe its peculiarities. METHODS: We analyzed 76 series of human embryos aged between 32 days (6mm) and newborns. The samples were cut serially and stained using Martin's trichrome technique. RESULTS: In semicircular canal development there were a number of peculiarities, such as: a defined chronological sequence of osteogenesis with a variable rate of ossification; the fact that each nucleus of ossification was involved in the formation of one of its covers (the upper in the superficial and the lower in the deep); the appearance of transitory dehiscence; and canal closure by means of bone with laminar pattern, with a minimum thickness of 0.1mm. CONCLUSION: The peculiarities in canal development could explain the origin of pathological dehiscence in the canal, whether congenital or acquired.

Síndrome branquio-oto-renal y colesteatoma congénito

Presentamos un caso del inusual síndrome branquio-oto-renal (BOR) asociado a colesteatoma congénito unilateral, anomalía en el trayecto del nervio facial y microdontia. Tales hechos son excepcionales, estando descrita la asociación síndrome BOR-colesteatoma congénito hasta ahora sólo en tres ocasiones y la anomalía en el trayecto del nervio facial en dos. Realizamos una revisión de este síndrome en cuanto a formas de manifestación, etiopatogenia y diagnóstico diferencial (AU)

Postabortal haemorrhage and disseminated intravascular coagulation due to placenta accreta.

We describe the case of a second trimester placenta accreta presenting as postabortal haemorrhage complicated by disseminated intravascular coagulation, requiring hysterectomy.