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1.
Jpn J Radiol ; 2024 Apr 26.
Artículo en Inglés | MEDLINE | ID: mdl-38664364

RESUMEN

PURPOSE: The diploic veins have been suggested to be involved in the excretion of cerebrospinal fluid and intracranial waste products; however, to date, there have been no reports evaluating the space surrounding the diploic veins. Therefore, we aimed to visualize the distribution of gadolinium-based contrast agent (GBCA) in the space surrounding the diploic veins and to evaluate the spatial characteristics. MATERIALS AND METHODS: Ninety-eight participants (aged 14-84 years) were scanned 4 h after intravenous GBCA injection at Nagoya University Hospital between April 2021 and December 2022. The volume of the space surrounding the diploic veins where the GBCA was distributed was measured using contrast-enhanced T1-weighted images with the application of three-axis motion-sensitized driven equilibrium. The parasagittal dura (PSD) volume adjacent to the superior sagittal sinus was also measured using the same images. Both volumes were corrected for intracranial volume. The correlation between age and the corrected volume was examined using Spearman's rank correlation coefficient; the relationship between the corrected volume and sex was assessed using the Mann-Whitney U test. RESULTS: A significant weak negative correlation was observed between the volume of the space surrounding the diploic veins and age (r = -0.330, p < 0.001). Furthermore, there was a significant weak positive correlation between the PSD volume and age (r = 0.385, p < 0.001). Both volumes were significantly greater in men than in women. There was no correlation between the volume of the space surrounding the diploic veins and the volume of the PSD. CONCLUSION: The volume of the space surrounding the diploic veins was measurable and, in contrast to the volume of the PSD, was greater in younger participants. This space may be related to intracranial excretory mechanisms and immune responses during youth, requiring further research.

2.
Artículo en Inglés | MEDLINE | ID: mdl-38647077

RESUMEN

BACKGROUND: This study aimed to assess anxiety, depression and quality of life (QoL) in patients with head and neck cancer undergoing laryngectomy using comprehensive self-reported questionnaires for a period of up to 5 years. METHODS: This prospective observational study enrolled 150 consecutive patients with locally advanced head and neck cancer who underwent laryngectomy at Nagoya University Hospital between 2007 and 2020. Anxiety, depression and QoL were assessed at baseline (preoperative) and at 3, 6, 12, 24, 36, 48 and 60 months after surgery using two brief self-reported questionnaires, such as the eight-item Short Form Health Survey (SF-8) and the Hospital Anxiety and Depression Scale (HADS). RESULTS: The surgical procedures were total laryngectomy, pharyngo-laryngectomy and pharyngo-laryngo-oesophagectomy in 97 (65%), 41 (27%) and 12 (8%) patients, respectively. All eight items of the SF-8 were significantly worse than those of the normal population at baseline and at 3 months after surgery. However, general health, vitality, mental health and bodily pain improved to normal levels within 1 year after surgery and were maintained for 5 years. In this study, 35% of patients were categorised as potential cases of depression, and 35% were potential cases of anxiety. During the follow-up period, the proportion of patients with anxiety gradually decreased after surgery. Further analysis revealed that the SF-8 and HADS scores and trends in 89 patients without tumour recurrence were similar to those in the total enrolled 150 patients. CONCLUSION: Anxiety, depression and QoL in laryngectomised patients improved at 1 year after surgery and were maintained for up to 5 years. WHAT THIS PAPER ADDS: What is already known on the subject Laryngectomy is associated with prolonged functional and psychological effects and has a major impact on patient quality of life (QoL). Several prospective studies evaluating the QoL in laryngectomised patients have been reported, in which significant deterioration in social functioning was found even 1 year after surgery. What this paper adds to existing knowledge One year is not a sufficient period for laryngectomised patients to return to normal life and spend their time in a social community. A recent review showed that most studies on QoL in laryngectomised patients were conducted under 1 year after the procedure, and there were not enough studies of sufficient quality. This is the first long-term prospective observational study of Japanese patients with head and neck cancer who underwent laryngectomy up to 5 years after surgery. What are the potential or actual clinical implications of this work? Our long-term observational study showed that the scores for anxiety, depression and QoL in laryngectomised patients improved at 1 year after surgery and were maintained for up to 5 years. Clinicians should recognize the importance of psychosocial risk factors in their QoL and multidisciplinary management, including social and psychological support, is essential for long-term laryngectomised survivors.

3.
Magn Reson Med Sci ; 2024 Apr 03.
Artículo en Inglés | MEDLINE | ID: mdl-38569839

RESUMEN

PURPOSE: The endolymph of the inner ear, vital for balance and hearing, has long been considered impermeable to intravenously administered gadolinium-based contrast agents (GBCAs) due to the tight blood-endolymph barrier. However, anecdotal observations suggested potential GBCA entry in delayed heavily T2-weighted 3D-real inversion recovery (IR) MRI scans. This study systematically investigated GBCA distribution in the endolymph using this 3D-real IR sequence. METHODS: Forty-one patients suspected of endolymphatic hydrops (EHs) underwent pre-contrast, 4-h, and 24-h post-contrast 3D-real IR imaging. Signal intensity in cerebrospinal fluid (CSF), perilymph, and endolymph was measured and analyzed for temporal dynamics of GBCA uptake, correlations between compartments, and the influence of age and presence of EH. RESULTS: Endolymph showed a delayed peak GBCA uptake at 24h, contrasting with peaks in perilymph and CSF at 4h. Weak to moderate positive correlations between endolymph and CSF contrast effect were observed at both 4 (r = 0.483) and 24h (r = 0.585), suggesting possible inter-compartmental interactions. Neither the presence of EH nor age significantly influenced endolymph enhancement. However, both perilymph and CSF contrast effects significantly correlated with age at both time points. CONCLUSION: This study provides the first in vivo systematic confirmation of GBCA entering the endolymph following intravenous administration. Notably, endolymph uptake peaked at 24h, significantly later than perilymph and CSF. The lack of a link between endolymph contrast and both perilymph and age suggests distinct uptake mechanisms. These findings shed light on inner ear fluid dynamics and their potential implications in Ménière's disease and other inner ear disorders.

4.
Acta Otolaryngol ; 144(2): 107-111, 2024 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-38534204

RESUMEN

BACKGROUND: Posterior expansion of the utricular endolymphatic space (ES) is a finding occasionally observed, and often occurs in bilateral ears. The clinical significance of posterior expansion of the utricular ES is not clear. OBJECTIVES: To investigate the clinical significance of posterior expansion of the utricular ES detected on magnetic resonance imaging (MRI). MATERIALS AND METHODS: Among 957 patients who underwent contrast-enhanced MRI to investigate the presence of endolymphatic hydrops (EH), clinical findings in cases with posterior expansion of the utricular ES were evaluated and the significance of their existence is considered. RESULTS: Posterior expansion of the utricular ES was detected in 30 ears from 20 cases (7 males, 13 females; mean age 48.7 years). The ears with posterior expansion had a significantly lower incidence of vestibular EH than those without, and this tendency was more prominent in cases in bilateral ears. Some cases with posterior expansion had vestibular symptoms and were diagnosed with Ménière's disease, although EH was not detected in their vestibules. CONCLUSION AND SIGNIFICANCE: Posterior expansion of the utricular ES was associated with Ménière's disease without vestibular EH. Posterior expansion of the utricular ES might not be associated with the formation of EH but may occur independently.


Asunto(s)
Hidropesía Endolinfática , Imagen por Resonancia Magnética , Enfermedad de Meniere , Sáculo y Utrículo , Humanos , Femenino , Masculino , Persona de Mediana Edad , Adulto , Enfermedad de Meniere/diagnóstico por imagen , Hidropesía Endolinfática/diagnóstico por imagen , Sáculo y Utrículo/diagnóstico por imagen , Anciano , Adulto Joven , Estudios Retrospectivos , Relevancia Clínica
5.
Laryngoscope Investig Otolaryngol ; 9(1): e1210, 2024 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-38362179

RESUMEN

Objective: The pathophysiology and symptoms underlying Meniere's disease (MD) manifest as endolymphatic hydrops (EH), potentially impacting acoustic power absorbance in vestibular EH. The longitudinal effects of middle ear pressure therapy (MEPT) and conservative therapies for EH by magnetic resonance imaging (MRI) and on acoustic power absorbance on wideband acoustic immittance (WAI) were evaluated, and their changes were compared with clinical symptoms. Methods: Eleven patients with definite MD or delayed endolymphatic hydrops (DEH), resistant to conservative therapies and who continued MEPT for 1 year, were included. Vertigo scores, hearing levels, acoustic power absorbance on WAI, and degrees of EH on 3-T MRI were evaluated and compared before and after the treatments. Results: One year after the start of MEPT, all cases showed symptomatic improvement in vertigo score; however, the degrees of EH showed no improvements except in one case. In the affected ears with EH, their absorbances on WAI improved, particularly at 1580-1905 or 2400-2953 Hz (p < .05). Conclusion: Alleviation of vestibular symptoms with the therapy of MD was not necessarily associated with improved EH. Vestibular symptoms could be related to the change in the impedance of inner ear pressure, which was proven by the normalization of acoustic power absorbance. Assessments of acoustic power absorbance may provide useful information for physiological conditions and causative factors of vertigo in ears with EH. Level of evidence: 4.

6.
Magn Reson Med Sci ; 23(1): 80-91, 2024 Jan 01.
Artículo en Inglés | MEDLINE | ID: mdl-36653154

RESUMEN

PURPOSE: To investigate the characteristics of the putative meningeal lymphatics located at the posterior wall of the sigmoid sinus (PML-PSS) in human subjects imaged before and after intravenous administration (IV) of a gadolinium-based contrast agent (GBCA). The appearance of the PML-PSS and the enhancement of the perivascular space of the basal ganglia (PVS-BG) were analyzed for an association with gender, age, and clearance of the GBCA from the cerebrospinal fluid (CSF). METHODS: Forty-two patients with suspected endolymphatic hydrops were included. Heavily T2-weighted 3D-fluid attenuated inversion recovery (hT2w-3D-FLAIR) and 3D-real inversion recovery (IR) images were obtained at pre-administration, immediately post-administration, and at 4 and 24 hours after IV-GBCA. The appearance of the PML-PSS and the presence of enhancement in the PVS-BG were analyzed for a relationship with age, gender, contrast enhancement of the CSF at 4 hours after IV-GBCA, and the washout ratio of the GBCA in the CSF from 4 to 24 hours after IV-GBCA. RESULTS: The PML-PSS and PVS-BG were seen in 23 of 42 and 21 of 42 cases, respectively, at 4 hours after IV-GBCA. In all PML-PSS positive cases, hT2w-3D-FLAIR signal enhancement was highest at 4 hours after IV-GBCA. A multivariate analysis between gender, age, CSF signal elevation at 4 hours, and washout ratio indicated that only the washout ratio was independently associated with the enhancement of the PML-PSS or PVS-BG. The odds ratios (95% CIs; P value) were 4.09 × 10-5 (2.39 × 10-8 - 0.07; 0.0078) for the PML-PSS and 1.7 × 10-4 (1.66 × 10-7 - 0.174; 0.014) for the PVS-BG. CONCLUSION: The PML-PSS had the highest signal enhancement at 4 hours after IV-GBCA. When the PML-PSS was seen, there was also often enhancement of the PVS-BG at 4 hours after IV-GBCA. Both observed enhancements were associated with delayed GBCA excretion from the CSF.


Asunto(s)
Hidropesía Endolinfática , Gadolinio , Humanos , Medios de Contraste , Ganglios Basales/patología , Hidropesía Endolinfática/patología , Administración Intravenosa , Imagen por Resonancia Magnética/métodos
7.
Nagoya J Med Sci ; 85(4): 866-874, 2023 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-38155617

RESUMEN

Sporadic inclusion body myositis (s-IBM) is an acquired degenerative inflammatory myopathy that leads to slowly progressive muscle weakness and atrophy of the limbs, face, and pharynx. Owing to the slow progression of the disease, the indications for surgical intervention remain unclear. Herein, we retrospectively reviewed the records of four patients with s-IBM who had undergone cricopharyngeal myotomy for severe dysphagia at our institution between 2016 and 2021. Among these, one patient underwent transcervical cricopharyngeal myotomy and laryngeal suspension, as videofluoroscopic examination of swallowing revealed poor laryngeal elevation. The remaining three patients underwent endoscopic cricopharyngeal myotomy using a curved rigid laryngoscope. Preoperatively, the mean Hyodo score was 8 points (range: 6-10) using a flexible endoscope. The mean surgical duration was 104 min, and no severe complications were observed. Postoperatively, all patients achieved improvement in swallowing function and food intake. Moreover, swallowing function was maintained in all four patients even 6-12 months postoperatively. Cricopharyngeal myotomy may be a safe surgical procedure with the potential to improve swallowing function, and a Hyodo score of 6 may be considered a surgical indication for cricopharyngeal myotomy in patients with s-IBM.


Asunto(s)
Laringoscopios , Miositis por Cuerpos de Inclusión , Miotomía , Humanos , Miositis por Cuerpos de Inclusión/cirugía , Miositis por Cuerpos de Inclusión/complicaciones , Estudios Retrospectivos , Endoscopía/métodos , Miotomía/métodos
8.
Otol Neurotol ; 44(8): e560-e565, 2023 09 01.
Artículo en Inglés | MEDLINE | ID: mdl-37525397

RESUMEN

OBJECTIVE: To evaluate the listening conditions of bilateral cochlear implant (CI) users in their daily living environment. STUDY DESIGN: Retrospective study. SETTING: University hospital. PATIENTS: Thirty-one adult CI users 16 years or older; 18 underwent sequential surgery, and 13 underwent simultaneous surgery. Inclusion criteria included the availability of CI data logging features and a minimum duration of binaural use of 6 months. INTERVENTION: Retrospective analysis of data obtained from the automatic scene classifier data logging system. MAIN OUTCOME MEASURE: Comparison of data logging and maximum speech discrimination scores of the two surgery groups (sequential vs. simultaneous) to investigate the potential influence of these factors on the listening conditions of CI users. RESULTS: The maximum speech discrimination score of the second CI in the sequential group was significantly worse than that of any other CI in the sequential and simultaneous CI groups. Additionally, the longer the interval between surgeries, the more significant the difference in "time on air" between the first and the second CIs. The second CI in sequential CI surgery had a shorter "time on air" than the first or the bilateral simultaneous CIs; the second CI was also used more frequently in noisy and speech with background noise environments. CONCLUSIONS: A second CI may be more frequently used in challenging listening environments because of its binaural auditory effect, despite its lower speech discrimination performance. The timing of sequential implantation and the potential impact of binaural hearing should be considered when developing rehabilitation strategies for individuals with bilateral CIs.


Asunto(s)
Implantación Coclear , Implantes Cocleares , Percepción del Habla , Adulto , Humanos , Estudios Retrospectivos , Percepción Auditiva , Audición
9.
Audiol Res ; 13(4): 563-572, 2023 Jul 27.
Artículo en Inglés | MEDLINE | ID: mdl-37622925

RESUMEN

Clinical findings on cartilage conduction hearing aids (CCHAs) have gradually become clear; however, few reports include a large number of cases. This study included 91 ears from 69 patients who underwent CCHA fitting in our hospital. Their ears were divided into six groups (i.e., bilateral aural atresia or severe canal stenosis, unilateral aural atresia or severe canal stenosis, chronic otitis media or chronic otitis externa with otorrhea, sensorineural hearing loss, mixed hearing loss, and conductive hearing loss) according to their clinical diagnosis and type of hearing loss. Most clinical diagnoses were aural atresia or meatal stenosis (bilateral, 21.8%; unilateral, 39.6%). The purchase rate of CCHAs was higher in the closed-ear group (bilateral, 77.3%; unilateral, 62.5%). In the bilateral closed-ear group, air conduction thresholds at 1000, 2000, and 4000 Hz and aided thresholds with CCHAs at 4000 Hz were significantly lower in the purchase group than the non-purchase group. No significant difference was observed between the purchase and non-purchase groups in the unilateral closed-ear group. In the bilateral closed-ear group, air conduction thresholds and aided thresholds were associated with the purchase rate of CCHAs. In the unilateral closed-ear group, factors other than hearing might have affected the purchase rate of CCHAs.

12.
Nagoya J Med Sci ; 85(2): 375-379, 2023 May.
Artículo en Inglés | MEDLINE | ID: mdl-37346840

RESUMEN

The potential mechanism of augmented response on cervical vestibular evoked myogenic potential (cVEMP) testing and its decrease following treatment in a patient with Meniere's disease (MD) are discussed. Changes of static posturography and cVEMP testing before and after a glycerol drip in a 69-year-old man with unilateral MD, in which significant endolymphatic hydrops (EH) was confirmed on magnetic resonance imaging (MRI) on the diseased side, were evaluated. Values of total locus lengths, areas of postural sway, and their Romberg ratios were decreased after the glycerol drip. On cVEMP testing, the diseased ear demonstrated a 375% larger amplitude than the contralateral ear before treatment, but both ears showed almost the same responses after treatment. An augmented response on cVEMP testing and a decrease following treatment for MD reflect the diversity of clinical findings in MD. Responses on cVEMP testing may relate not only to the degree of EH, but also be due to abnormal acoustic energy absorbance transmitted into the saccule.


Asunto(s)
Hidropesía Endolinfática , Enfermedad de Meniere , Potenciales Vestibulares Miogénicos Evocados , Masculino , Humanos , Anciano , Enfermedad de Meniere/diagnóstico , Potenciales Vestibulares Miogénicos Evocados/fisiología , Glicerol , Hidropesía Endolinfática/diagnóstico , Imagen por Resonancia Magnética/métodos
13.
Front Public Health ; 11: 1124404, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-37151589

RESUMEN

Introduction: Sensory dysfunctions and cognitive impairments are related to each other. Although a relationship between tinnitus and subjective olfactory dysfunction has been reported, there have been no reports investigating the relationship between tinnitus and olfactory test results. Methods: To investigate the relationship between tinnitus and olfactory test results, we conducted sensory tests, including hearing and visual examinations. The subjects included 510 community-dwelling individuals (295 women and 215 men) who attended a health checkup in Yakumo, Japan. The age of the subjects ranged from 40 to 91 years (mean ± standard deviation, 63.8 ± 9.9 years). The participants completed a self-reported questionnaire on subjective tinnitus, olfactory function, and hearing function, as well as their lifestyle. The health checkup included smell, hearing, vision, and blood examinations. Results: After adjusting for age and sex, the presence of tinnitus was significantly associated with subjective olfactory dysfunction, poor olfactory test results, hearing deterioration, vertigo, and headache. Additionally, high serum calcium levels and a low albumin/globulin ratio were significantly associated with low physical activity and nutrition. Women scored higher than men in olfactory and hearing examinations, but there was no gender difference in vision examinations. Conclusion: Subjective smell dysfunction and poor smell test results were significantly associated with tinnitus complaints. Hearing and vision were associated even after adjusting for age and sex. These findings suggest that evaluating the mutual relationships among sensory organs is important when evaluating the influence of sensory dysfunctions on cognitive function.


Asunto(s)
Trastornos del Olfato , Acúfeno , Masculino , Humanos , Femenino , Adulto , Persona de Mediana Edad , Anciano , Anciano de 80 o más Años , Olfato , Audición , Trastornos del Olfato/epidemiología , Encuestas y Cuestionarios
14.
Front Neurol ; 14: 1193104, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-37153681

RESUMEN

Objective: To summarize the pathophysiological analysis of idiopathic sudden sensorineural hearing loss (ISSNHL) by magnetic resonance imaging (MRI), focusing on the findings of high signal or endolymphatic hydrops (EH) in the inner ear. Methods: We summarize the published studies of our research group regarding the pathophysiological analysis of ISSNHL on MRI and review related clinical articles that have reported significantly high signal or the existence of EH in ears with ISSNHL. Results: Pre-contrast high signal on MRI may indicate minor hemorrhage or increased permeability of surrounding vessels to the perilymph, whereas post-contrast high signal indicates breakdown of the blood-labyrinth barrier, in which irreversible changes would lead to poor prognosis. In some cases of ISSNHL, primary EH could be pre-existing and may be a risk factor for the onset of ISSNHL. Conclusion: Analysis of ISSNHL by cutting-edge MRI evaluation could provide useful information for elucidating its pathophysiology and for predicting prognosis in this disease.

15.
Invest New Drugs ; 41(3): 512-521, 2023 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-37171720

RESUMEN

Immune-related sclerosing cholangitis (irSC) is relatively rare and its clinical characteristics are not well known. In this study, we aimed to summarize the clinical features of irSC. Clinical data were collected retrospectively from 1,393 patients with advanced malignancy treated with immune-checkpoint inhibitors (ICIs) between August 2014 and October 2021. We analyzed patients with immune-related adverse events of liver injury (liver-irAEs) and compared irSC and non-irSC groups. Sixty-seven patients (4.8%) had a liver-irAE (≥ grade 3) during the follow-up period (median, 262 days). Among these, irSC was observed in eight patients (11.9%). All patients in the irSC group were treated with anti-PD-1/PD-L1 antibodies. Compared with the non-irSC group, the irSC group showed mainly non-hepatocellular liver injury (87.5 % vs 50.8 %, P = 0.065), and had elevated serum inflammatory markers (e.g., CRP and NLR) and biliary enzymes (e.g., GGTP and ALP) at the onset of liver-irAEs. Furthermore, most patients with irSC had abdominal pain. In the non-irSC group, the liver injury of 23 patients improved only with the discontinuation of ICIs, and 22 patients improved with medication including prednisolone (PSL). Conversely, almost all patients (n=7) in the irSC group were treated with PSL, but only two patients experienced an improvement in liver injury. We found that irSC is characterized by a non-hepatocellular type of liver injury with abdominal pain and a high inflammatory response and is refractory to treatment. Further examination by imaging is recommended to detect intractable irSC in cases with these characteristics.


Asunto(s)
Antineoplásicos Inmunológicos , Colangitis Esclerosante , Humanos , Inhibidores de Puntos de Control Inmunológico/efectos adversos , Colangitis Esclerosante/inducido químicamente , Colangitis Esclerosante/tratamiento farmacológico , Antineoplásicos Inmunológicos/uso terapéutico , Estudios Retrospectivos , Dolor Abdominal/inducido químicamente , Dolor Abdominal/tratamiento farmacológico
16.
Acta Otolaryngol ; 143(2): 163-169, 2023 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-36855795

RESUMEN

BACKGROUND: Listening difficulties (LiD) present difficulties in listening and paying attention to spoken information despite normal pure tone audiometry. Endolymphatic hydrops (EH) is a common inner ear condition associated with Ménière's disease but may also be present in the asymptomatic ear. OBJECTIVES: Using magnetic resonance imaging, we investigated EH in patients with LiD and assessed whether the severity of EH was related to the results of auditory processing tests (APTs). MATERIALS AND METHODS: 111 patients with no abnormalities on pure tone audiometry, but displaying difficulties in listening, underwent evaluation through APTs and questionnaires. Upon obtaining informed consent, the inner ears of 20 consenting patients were evaluated utilizing a 3-Tesla magnetic resonance imaging. RESULTS: A higher percentage of patients diagnosed with LiD by APTs had significant EH in the cochlea and vestibule than in previously reported control cases. The percentage of correct answers in the speech-in-noise test was significantly lower in patients with than in those without significant EH. CONCLUSION AND SIGNIFICANCE: In this study, significant EH of the cochlea was associated with poor listening to noise. The presence of EH affects the functioning of the auditory processing system, even in ears that test normally on standard audiometric tests.


Asunto(s)
Hidropesía Endolinfática , Vestíbulo del Laberinto , Humanos , Hidropesía Endolinfática/complicaciones , Hidropesía Endolinfática/diagnóstico por imagen , Vestíbulo del Laberinto/patología , Audiometría de Tonos Puros , Percepción Auditiva , Audición , Imagen por Resonancia Magnética/métodos
17.
Nagoya J Med Sci ; 85(1): 150-156, 2023 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-36923612

RESUMEN

Erdheim-Chester disease is characterized by the infiltration of foamy histiocytes in tissues. Lesional tissue biopsy is recommended to confirm diagnosis and establish the BRAF mutational status. A 52-year-old man presented to our hospital with hydronephrosis. Computed tomography showed enhancement of soft shadows around the left renal pelvis transition area and the aorta. He was treated with prednisolone 0.2 mg/kg for 1 year; however, no improvement was observed. 18Fluorodeoxyglucose-positron emission tomography/computed tomography revealed increased fluorodeoxyglucose uptake in various body parts, including the maxillary sinuses, indicative of Erdheim-Chester disease. He refused further examination, and the maxillary sinus lesions were treated with antibiotics and intranasal steroids, but no improvement was observed. Two years later, he underwent biopsy with endoscopic sinus surgery of the maxillary sinus, which showed the highest increase in fluorodeoxyglucose uptake on repeat 18fluorodeoxyglucose-positron emission tomography/computed tomography. Endoscopic findings showed only nonspecific inflammatory findings, but pathological findings revealed the proliferation of cells with abundant foamy cytoplasms. Sufficient tumor volume was available to perform PCR for BRAF V600E mutation analysis, which was positive and resulted in a diagnosis of Erdheim-Chester disease with the BRAF V600E mutation. This is the first case of a patient with Erdheim-Chester disease with the BRAF V600E mutation identified in a sinus lesion. Endoscopic sinus surgery biopsy of the paranasal sinuses was considered to contribute to the histological and genetic diagnosis of Erdheim-Chester disease, particularly following the notable increase in fluorodeoxyglucose uptake.


Asunto(s)
Enfermedad de Erdheim-Chester , Masculino , Humanos , Persona de Mediana Edad , Enfermedad de Erdheim-Chester/diagnóstico , Enfermedad de Erdheim-Chester/genética , Enfermedad de Erdheim-Chester/cirugía , Proteínas Proto-Oncogénicas B-raf/genética , Mutación , Aorta/patología , Fluorodesoxiglucosa F18
18.
Laryngoscope Investig Otolaryngol ; 8(1): 262-268, 2023 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-36846415

RESUMEN

Objective: The increased endolymph volume affects a shift in the organ of Corti and basilar membrane in ears with endolymphatic hydrops (EH), which might affect distortion-product otoacoustic emissions (DPOAE) by altering the operating point of the outer hair cells. We investigated how changes in DPOAE are related to the distribution site of EH. Study Design: Prospective study. Methods: Among 403 patients with hearing or vestibular symptoms who underwent contrast-enhanced magnetic resonance imaging (MRI) for the diagnosis of EH and subsequent DPOAE testing, subjects whose hearing levels on pure tone audiometry were ≤35 dB at all frequencies were included in this study. In patients with EH on MRI, the presence and amplitude of DPOAE were evaluated between groups with hearing levels of ≤25 dB at all frequencies versus hearing levels of >25 dB at one or more frequencies. Results: There were no differences in the distribution of EH between groups. The amplitude of DPOAE had no clear correlation with the presence of EH. However, in both groups, there was a significantly higher probability of the presence of a DPOAE response from 1001 to 6006 Hz in cases with EH in the cochlea. Conclusion: Among patients whose hearing levels were ≤35 dB at all frequencies, better responses on DPOAE testing were found in subjects with EH in the cochlea. Alteration of DPOAEs in the early stages of hearing impairment could indicate morphological changes in the inner ear with altered basilar membrane compliance due to EH. Level of Evidence: 4.

19.
J Neurol Surg B Skull Base ; 84(1): 69-78, 2023 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-36743718

RESUMEN

Objective Surgical indications for advanced-stage squamous cell carcinoma (SCC) of the external auditory canal (EAC) are highly dependent on the skull base surgery team. The aim of this study was to evaluate the surgical outcomes in patients with SCC of the EAC and to clarify the surgical indication of far advanced cases using the T4 subclassification. Methods Patients with SCC of the EAC who underwent curative treatment from 2002 to 2021 at our hospital were retrospectively reviewed. Clinical and surgical results, including operative data, overall survival (OS), and disease-specific survival (DSS), were analyzed. To clarify the surgical indication for advanced-stage tumors, we proposed the T4 subclassification. Results In the 46 patients included in the study, 8 patients had T1 tumors, 10 had T2 tumor, 5 had T3 tumors, and 23 had T4 tumors. The 5-year DSS with T1, T2, T3, and T4 tumors were 100, 85.7, 100, and 61.7%, respectively. No prognostic impacts for margin status were found between the 5-year OS and DSS ( p = 0.23 and 0.13, respectively). Patients with far-advanced-stage (T4b) tumors were significantly associated with shorter DSS than those with early-stage (T1/T2) and advanced-stage (T3/T4a) tumors ( p = 0.007 and 0.03, respectively). Conclusion The present study focused on patients with SCC of the EAC at a university hospital over a period of 20 years, especially with skull base involvement, and a T4 subclassification was proposed. Complete tumor resection in an en bloc fashion could help achieve a good survival rate even in patients with locally advanced tumors.

20.
Mol Clin Oncol ; 18(2): 10, 2023 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-36761390

RESUMEN

Endoscopic cricopharyngeal myotomy (ECPM) is a safe and minimally invasive technique that is used to treat patients whose esophageal inlet fails to open because of specific diseases, such as Wallenberg's syndrome and neuromuscular diseases. The present study described the performance of a new, safe and simple ECPM using a curved rigid laryngoscope, which is used for endoscopic laryngopharyngeal surgery for patients with dysphagia due to pharyngeal residue after swallowing. The patient was an 80-year-old woman with laryngeal palsy caused by lower cranial nerve palsy after cranial base schwannoma surgery. ECPM was performed with a curved rigid laryngoscope. The postoperative course was good; postoperative rehabilitation eliminated the residue after swallowing a thickened solution and mealtimes were shorter than before surgery. This procedure allows the hypopharynx to be widely expanded and it is possible to develop a wider surgical field than when using a direct laryngoscope. In addition, this procedure appears to be relatively easy to perform if the surgeon is familiar with the curved rigid laryngoscope technique.

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