以耳部症状首发、合并鼻咽占位的肉芽肿性多血管炎1例并文献复习

目的 探讨1例临床少见的以耳部症状首发、合并鼻咽部占位的肉芽肿性多血管炎(GPA)的临床特征、实验室检查、病理表现及诊疗过程。方法 回顾性分析患者的病例资料,总结病例特点并回顾国内外GPA相关文献。结果 患者以中耳炎、迷路炎症状为首发表现,合并鼻咽部占位,病程中逐渐出现面瘫,三叉神经刺激症状加重。多次留取耳及鼻咽部活检示急慢性炎症细胞浸润。升级抗生素,同时为避免中耳炎侵犯岩骨及颅内行乳突开放术。中耳局部炎症改善后其耳痛、面瘫等仍不缓解,但激素治疗有效,遂进一步完善自免病相关检查并再次行鼻咽部活检,最终确诊为GPA,予激素及免疫抑制剂治疗得以控制症状。术后3个月暂无显著肺部及肾脏受累表现。结论 临床上发现不典型的中耳炎或常规治疗反复不愈,且逐渐进展出现内耳、颅神经侵犯表现如眩晕发作、面神经麻痹等,同时激素治疗有效,且合并鼻咽部占位、鼻窦炎影像学表现,或累及其他器官如肺、肾脏时,均应考虑到GPA的可能。当反复留取病理活检未能取得特异性确诊依据时,动态监测抗中性粒细胞胞浆抗体、红细胞沉降率、尿潜血、胸部CT、血肌酐等也具有重要的提示意义。     

Decreased miRNA-320e correlates with allergy in children with otitis media with effusion

ObjectiveOtitis media with effusion (OME) is a common childhood disease and the main cause of conductive hearing loss in this age group. Many factors predispose to OME but allergy is still widely disputed. The answer may lay in the molecular mechanisms of ear exudate formation and the recent studies showed miRNAs might take part in it. MiRNAs are also potent regulators of allergic response. As miRNAs are present in the middle ear, we hypothesized their expression differs between allergic and non-allergic patients and reflects the difference in pathomechanism of effusion formation between these two groups.Materials and methodsThis study aimed to establish the expression of 5 different miRNAs (miR-223-3p, miR-451a, miR-16-5p, miR-320e, miR-25-3p) in ear exudates in children diagnosed with OME. The allergy group consisted of 18 patients whereas the non-allergic group had 36 patients. MicroRNA was isolated from the middle ear fluid collected during myringotomy and transcribed into cDNA. MiRNA expression was measured with TaqMan™ MicroRNA Assays and analyzed with DataAssist software. The comparative CT method was used for calculating the relative quantification of gene expression based on the endogenous control gene expression (U6 snRNA-001973).ResultsMiR-320e expression was significantly decreased in allergic children with OME. Other studied miRNAs also showed reduced expression in allergic children, but the decrease was not significant.ConclusionsMiRNA expression differs between children with and without allergy in the course of OME, but further studies are needed to explain the exact role of miR-320e and its target genes in OME pathology in allergic patients.     

Experience with vertical partial laryngectomy with special reference to laryngeal reconstruction with cervical fascia

In this paper we report our experience of vertical partial laryngectomy using the superficial cervical fascia; we describe the technique and present the functional and oncological results of this method of treatment. A total of 42 patients with squamous cell carcinoma of the true vocal folds, in stage T(1) (n = 28) or T(2) (n = 14), were treated in our department using vertical partial laryngectomy during the decade 1987-1997. Nine patients had post-operative radiotherapy. The shortest follow-up time was three years. There were six recurrences in all, four in the larynx and two in the neck. All four of the laryngeal recurrences were treated with total laryngectomy and are doing well. Both the patients with neck metastases, who were treated with neck dissection, died. Permanent tracheotomy was necessary in one patient. There were no problems with aspiration. The recurrence rate was 14 per cent, the three-year survival index was 95.2 per cent and the three-year larynx preservation index was 90 per cent. According to our experience, vertical partial laryngectomy, using the method we describe, has a good functional and oncological result for stage T(1) and T(2) tumours.     

Cytokines in children with otitis media with effusion

We assayed 38 middle ear effusions from 23 children aged 4–13 years (mean 7) undergoing tympanostomy tube placements. All fluid was assayed for tumor necrosis factor (TNF) α, interleukin (IL) 1β, IL-8, and IL-10. Cytokine concentrations were measured by means of an enzyme-linked immunosorbent assay. Detectable levels of IL-1β, IL-8, and IL-10 were found in all of the effusions. TNF-α was detected in 18 of the middle ear effusions (47.4%). The mean concentration of TNF-α, IL-1β, IL-8, and IL-10 was, respectively, 0.423 ± 1.39, 30.58 ± 68.7, 7001.9 ± 6743, and 56 ± 58.7 pg/ml. There was a strong, statistically significant correlation between the concentrations of TNF-α and IL-1β (r = 0.87, P = 0.001) and between IL-1β and IL-8 (r = 0.53, P = 0.001). There was no correlation between the concentrations of IL-10 and other cytokines examined or between tympanic membrane pathology and the concentrations of TNF-α, IL-1β, IL-8, or IL-10. The presence of IL-10 in middle ear effusions may be one of the causes of a lack of clinical features of acute inflammation and may lead to a chronic inflammatory state. Received: 25 August 1999 / Accepted: 5 January 2000     

Polymyositis with dysphagia treated with endoscopic balloon dilatation

Polymyositis is characterized by non-specific inflammatory disease associated with an autoimmune disorder involving muscles of the limbs and neck. We report a case of an 80-year-old man who was referred to our clinic with a chief complaint of dysphagia and muscle weakness in all four limbs. The patient was diagnosed with polymyositis based on pathological findings, muscle weakness, electromyogram findings, and an elevated creatine phosphokinase level. The patient was also positive for HLA-DR3. Intravenous predonine administration was initiated, but dysphagia was not improved. We considered a cricopharyngeal myotomy, but this could not be performed because of heart failure. Endoscopic balloon dilation was performed and dysphagia improved on the same day. Therefore, we suggest that this method is a safe and effective approach for polymyositis with dysphagia.     

Otoacoustic emissions in children with otitis media with effusion

OBJECTIVES: Otoacoustic emissions (OAE) are transmitted from the cochlea to the ear canal via the middle ear and the transmission properties of the middle ear directly influence OAE characteristics. The purpose of this study was to establish the mechanisms of changes occurred in middle ear by tympanometric, audiometric and OAE examination. METHODS: Audiometric and tympanometric examination were performed and otoacoustic emissions were recorded from 22 normal ears and 52 ears with middle ear effusions and repeated 3 months later. RESULTS: Results of the air conduction in study group were significantly different from the control group and we found significant recovery in 3 months. When we analysed the DPOAE evaluation results in our study, some of the DPOAE parameters were found to be different between the control and the study group at low frequencies. Changes in the amplitude, especially at low frequencies, were statistically significant after 3 months. CONCLUSION: The results of this study revealed that measurement of otoacoustic emissions, especially distortion product otoacoustic emissions, is helpful in evaluating the condition of middle ear during the treatment.     

Hearing acuity of children with otitis media with effusion

Hearing levels are reported for a cohort of 222 infants (aged 7 to 24 months) and 540 older children (aged 2 to 12 years) with otitis media with effusion (OME). The infants had an average speech awareness threshold of 24.6 dB hearing level (HL). The older group had mean bone conduction thresholds less than 10 dB HL, and air conduction thresholds averaged 27 dB HL; however, acuity was 7 dB less impaired at 2,000 Hz. The mean three-frequency pure tone average and speech reception threshold were 24.5 and 22.7 dB, respectively. Hearing acuity was not significantly related to age or previous duration of OME. The otoscopic observation of an air-fluid level or bubbles was associated with less hearing impairment; however, a predictive relationship between hearing levels and tympanogram characteristics could not be demonstrated.     

Speech improvement with velopharyngoplasty in patients with lip-jaw-palate clefts

BACKGROUND: Despite successful closure of the hard and soft palate and intensive speech therapy a velopharyngeal insufficiency is not completely avoidable in each case of cleft palate. An improvement by velopharyngoplasty should be possible. PATIENTS AND METHODS: Two hundred and ninety patients suffering from cleft palate were examined before and one year after velopharyngoplasty according to Sanvenero-Rosselli. Four main symptoms of the affected speech were assessed: changes of the resonance, inappropriate nasal air emission, articulary disorders (deviations from articulation areas), and non-physiological facial expression during speaking, each divided into three grades. Afterwards, the complete speech quality was classified. RESULTS: Assessing the symptoms separately and summarised the therapy including velopharyngoplasty was suitable to improve the speech quality in 83.1% of the cases, resulting in a widely unaffected speech. The improvement was greater in younger patients undergoing operation (Gamma-test, p < 0.001). But the final results were independent from age due to poorer initial situation in younger patients (Gamma-test, p < 0.001). CONCLUSIONS: The velopharyngoplasty is an important method for repair of velopharyngeal insufficiency in patients with cleft palate. In each individual case it is necessary to consider carefully if and when this operation should be performed. An intensive interdisciplinary co-operation of all specialists involved in the treatment is indispensable.     

Clinical experiments with hearing aids with amplitude compression.

The model for audiological rehabilitation in Denmark is brief-described. Clinical experiments have not demonstrated a general superiority of the compression hearing aids compared to the conventional hearing aids. In situations with negligible background noise the compression hearing aid tends to provide the best comprehension of speech. However, in situations with background noise the conventional hearing aid is generally preferred by hearing impaired persons. The attitude with regard to compression hearing aids differs among Danish hearing clinics. One clinic with a very positive attitude delivers compression hearing aids to 27% of the total clientele. In several clinics it has been observed that patients with a perceptive impairment who are using the hearing aid for the first time may benefit from compression during the early stages of hearing aid adjustment. In many cases, however, these patients prefer less compression as they continue to use their hearing aids in daily life. It is pointed out, that in connection with compression hearing aids it is especially important to provide careful instruction about the possibilities and limitations of the hearing aid.     

Vestibular rehabilitation with biofeedback in patients with central imbalance

Central Nervous System disorders may cause important functional unbalance in the maintenance of balance and posture. There is no effective rehabilitation for these symptoms until now.ObjectiveThe aim of this paper is to evaluate the use of tongue electrotactile stimulation on patients with central imbalance using BrainPort.Materials and MethodsThis is a prospective case series study. We evaluated 8 patients with central imbalance, 6 men and 2 women, with mean age of 67.75 years. The patients were submitted to Computed Dynamic Posturography (CDP) and then received 18 sessions of electrotactile stimulation by BrainPort® device for 20 minutes, twice a day. Then they were submitted to a new CDP test and to a self-perception scale to assess symptom remission, partial improvement and no improvement at all.Results75% of the patients reported being more stable. There was no improvement in the balance control of the mass center in these patients.ConclusionThe patients were able to use the electrotactile stimulus to improve their balance control.     

Multifrequency tympanometry in adults with otitis media with effusion

We compared the diagnostic values of multifrequency tympanometry (MFT) and conventional 226-Hz tympanometry in adults with otitis media with effusion (OME) and discuss whether the resonant frequency (RF) can be used as a reliable method in adults with OME. Prospective study was designed to compare the normal hearing group and the group with OME. In the OME group (n = 85), conductive or mixed hearing loss was found, air-bone gap was more than 10 dBHL, and acoustic reflex was not elicited. In the normal hearing group (n = 36), pure tone threshold was less than or equal to 15 dBHL and air-bone gap was less than 10 dBHL. Levene's test was used to compare the difference between the OME group and the normal hearing group on day1, day15, day30, day90, respectively. The relationship among multifrequency tympanometry, 226-Hz tympanometry and acoustic reflex test in ears recovering from OME was also investigated. A statistically significant decrease in RF value was found in ears with OME compared to normative data. In follow-up visits, both the RF values and the percentage of type A tympanograms increased while the percentage of type B and C tympanograms decreased. A high agreement between middle ear resonant frequency test and acoustic reflex test in ears recovering from OME was found. The resonant frequency test provides more detailed information than the 226-Hz tympanometry. Multifrequency tympanometry may be a more sensitive and objective diagnostic tool in adults with OME.     

Giant ethmoid osteoma with orbital extension presenting with epiphora

Paranasal sinus osteoma is a slow-growing, benign, encapsulated bony tumour that may be commonly asymptomatic, being detected incidentally in 1% of plain sinus radiographs or in 3% sinus computerized tomographic scans. In a patient presenting with epiphora and nasal obstruction, computed tomography disclosed a giant osteoma of the ethmoid. In this article, one patient having giant ethmoidal osteoma with orbital extension is described, and epidemiology, diagnosis, treatment, and pathological findings in paranasal sinus osteoma are reviewed.     

Air-bone gap estimated with multiple auditory steady-state response in young children with otitis media with effusion

Objective

Multiple auditory steady-state responses (ASSRs) to air- and bone-conduction stimuli were recorded in young children with otitis media with effusion (OME). After treatment for OME, differences between pre-treatment bone-conduction ASSR levels and post-treatment conditioned orientation reflex (COR) or air-conduction ASSR levels were examined, and compared with ASSR-estimated air-bone gap (ABG) before treatment.

Methods

Navigator Pro with Master was used to assess the threshold of air- and bone-conduction ASSR in both ears at 500 Hz, 1000 Hz, 2000 Hz and 4000 Hz. For bone-conduction ASSR, RadioEar B-71 bone-vibrator placed on the mastoid was used with white-noise masking on the contralateral ear.

Results

After ventilation tube placement, the thresholds of COR got closer to those of pre-treatment bone-conduction ASSR in young children with OME. Moreover, post-treatment air-conduction ASSR thresholds also got closer to those of pre-treatment bone-conduction ASSR. The differences between pre-treatment bone-conduction ASSR thresholds and post-treatment COR or air-conduction ASSR thresholds became much smaller than ASSR-estimated ABG before treatment.

Conclusion

These findings suggest that bone-conduction ASSR can assess the normal or near normal cochlear sensitivity in young children with conductive hearing loss. It is also suggested that ASSR-estimated ABG can be used clinically to predict their accurate ABG.