A giant pedunculated tumor (Fibrolipoma) of oesophagus – a rare case

A giant pedunculate tumour of oesophagus is reported in this study for its rareness. A mate patient aged 48 years was admitted at Jorhat Christian Medical Centre with the complaint of swelling of the neck and dysphagia for a period of 4 months. Clinically the neck swelling looks like swelling of left lobe of thyroid with intrathoracic extension, ENT examination was normal The barium Swallow X-ray of oesophagus showed surprisingly huge dilatation of upper half of the oesophagus. The oesophagoscopy snowed a large mobile infra oesophageal tumour extending from the upper end of the oesophagus upto the mid thoracic region. The neck is explored by collar incission and the cervical oesophagus is opened by longitudinal incission. The tumour is pulled out from the oesophagus and is removed completely by cutting the peduncle which was attached to the tight wall of the oesophagus just below the cricopharynx. The peduncle was about I em in diameter., The length of the tumour was about 3–4 cm at the thoracic part. Post Operative recovery was uneventful. The histopathology report skewed it to be a ease of FIBROLIPOMA.     

Molecular and pharmacological characteristics of the gerbil α(1a)-adrenergic receptor

The spiral modiolar artery supplies blood and essential nutrients to the cochlea. Our previous functional study indicates the α(1A)-adrenergic receptor subtype mediates vasoconstriction of the gerbil spiral modiolar artery. Although the gerbil cochlea is often used as a model in hearing research, the molecular and pharmacological characteristics of the cloned gerbil α(1a)-adrenergic receptor have not been determined. Thus we cloned, expressed and characterized the gerbil α(1a)-adrenergic receptor and then compared its molecular and pharmacological properties to those of other mammalian α(1a)-adrenergic receptors. The cDNA clone contained 1404 nucleotides, which encoded a 467 amino acid peptide with a deduced sequence having 96.8, 96.4 and 91.6% identity to rat, mouse and human α(1a)-receptors, respectively. We transiently transfected the α(1a)-adrenergic receptor into COS-1 cells and determined its pharmacological characteristics by [(3)H]prazosin binding. Unlabeled prazosin had a K(i) of 0.89±0.1nM. The α(1A)-adrenergic receptor-selective antagonists, 5-methylurapidil and WB-4101, bound with high affinity and had K(i) values of 4.9±1 and 1.0±0.1nM, respectively. BMY-7378, an α(1D)-adrenergic receptor-selective antagonist, bound with low affinity (260±60nM). The 91.6% amino acid sequence identity and K(i)s of the cloned gerbil α(1a)-adrenergic receptor are similar to those of the human α(1a)-adrenergic receptor clone. These results show that the gerbil α(1a)-adrenergic receptor is representative of the human α(1a)-adrenergic receptor, lending validity to the use of the gerbil spiral modiolar artery as a model in studies of vascular disorders of the cochlea.     

When is a biopsy justified in a case of relapsing polychondritis?

Relapsing polychondritis (RP) is a relatively rare rheumatic condition of unknown aetiology. It is characterized by recurrent episodic inflammation of cartilaginous structures (nose, ear and trachea). The clinical diagnosis of polychondritis can frequently be made with confidence in the absence of histological confirmation. A 61-year-old diabetic man, with bilateral relapsing aural inflammation, left ear deafness with tinnitus and pain at the sternocostal junctions is reported. After clinical diagnosis of relapsing polychondritis steroid therapy was started. An ear cartilage biopsy was performed confirming the clinical diagnosis. Subsequently soft tissue infection occurred at the operation site. The abscess was drained and oral ciprofloxacin was given with complete resolution of the infection over 30 days. As the infection is the main cause of death in these patients, we analyse whether biopsy is absolutely necessary for the diagnosis of RP in some patients.     

Is hearing loss a feature of Joubert syndrome, a ciliopathy?

Objective

To assess if hearing loss is a feature of Joubert syndrome (JBS), one of the ciliopathies and therefore possibly associated with hearing loss.

Design

Retrospective case series.

Setting

University Children's Hospital.

Patients

Dutch patients with JBS.

Main outcome measures

Audiological data.

Results

Data from 22 Dutch Joubert syndrome (JBS) cases (17 males, 5 females) aged 3-40 years were available. Audiological tests were successfully performed in 14 cases. Three cases (aged 17-26 years) showed very mild sensorineural hearing loss (SNHL) at different frequencies. Conductive hearing loss due to middle ear infections occurred frequently in young JBS children (6 out of 22 cases). In three cases (aged 3-13 years) the parents reported the child was hypersensitive to sound.

Conclusion

We found no evidence for significant hearing loss in Joubert syndrome patients. However, given the compromised speech development in JBS, conductive hearing loss due to middle ear infections should be treated vigorously. SNHL at a later age cannot be excluded on the basis of our data, given the sample size. Three of the older cases showed discretely increased hearing thresholds. Analogous to the ciliopathy Bardet-Biedl syndrome, where hearing thresholds were reported to be subclinically increased in a group of adolescents patients, we recommend follow-up of JBS patients in view of the possibility of progressive, late-onset SNHL.     

Long-term prophylactic treatment of attacks of vertigo in Menière's disease--comparison of a high with a low dosage of betahistine in an open trial

CONCLUSION: Despite the considerable limitations of an open, non-masked trial, particularly in Menière's disease (MD), a higher dosage of betahistine-dihydrochloride and a long-term treatment seems to be more effective than a low dosage and short-term treatment. OBJECTIVE: To evaluate the prophylactic effects of a low versus high dosage long-term treatment with betahistine-dihydrochloride on the number of attacks in MD. PATIENTS AND METHODS: We performed an open, non-masked trial, in which patients with MD received either a low dosage of betahistine-dihydrochloride (16 or 24 mg tid) or a higher dosage of 48 mg tid for at least 12 months. The outcome measure was the number of attacks per month during a 3-month period. Non-parametric tests and a random effects model were used for statistical analysis. RESULTS: A total of 112 patients were included in the analysis: 50 received betahistine-dihydrochloride in a low dosage (16 mg tid, n=21, 24 mg, n=29) and 62 received 48 mg tid. Follow-up examination every 3 months showed that the number of attacks per month decreased in both groups over time. For instance, after 12 months the mean (median) number of attacks dropped from 7.6 (4.5) to 4.4 (2.0) (p<0.0001) in the low-dosage group, and from 8.8 (5.5) to 1.0 (0.0) (p<0.0001) in the high dosage group. The number of attacks after 12 months was significantly lower in the high dosage group than in the low dosage group (p(12M)=0.0002). The treatment was well tolerated in both groups.     

Neurovascular compression syndrome with Meniere's disease—a report of the treatment of a patient with microvascular decompression

Neurovascular compression syndrome(NVCS) is currently regarded as one of the causes of disabling positional vertigo (DPV). A 59-year-old man visited our hospital on December 15, 1993. The neuro-otological examination suggested that the patient had Meniere's disease and he was treated with conservative medication. However, the patient continued to suffer definite vertigo attacks, as well as a fluctuation in his hearing. A consultation and discussion lead us to believe the patient was also suffering from NVCS. We planned the microvascular decompression surgery. After surgery, the patient stopped suffering from vertigo attacks but his audiogram showed severe fluctuation in sensorineural hearing levels. It was concluded that the present patient had been suffering from NVCS combined with Meniere’s disease.     

Temperature-controlled Radiofrequency Tissue Volume Reduction of the Soft Palate (Somnoplasty®) in the Treatment of Habitual Snoring: Results of a European Multicenter Trial

Temperature-controlled radiofrequency tissue volume reduction of the soft palate has been introduced as a minimally invasive, outpatient procedure for the treatment of habitual snoring and mild obstructive sleep apnea. A prospective, non-randomized multi-center European clinical study was conducted to investigate the efficacy of Somnoplasty^®     

Is a redefinition of the growth criteria of vestibular schwannomas needed?

Purpose

The natural history of vestibular schwannomas is poorly understood. Knowledge of growth rate and growth pattern is essential because the treatment strategy is based upon these. The purpose of this study was to determine the inter- and intraobserver variability in measuring VS size.

Materials and methods

Two consultant neuroradiologists independently made three linear measurements (d1, d2, d3) using digital MRI scans. MRI scans from 72 patients diagnosed between 2002 and 2010 with VS were obtained. These patients had a total of 223 MRI scans. d1 (medio-lateral diameter) was made perpendicular to d2. d2 was made parallel to the posterior border of the petrous ridge, and d3 was a measure of the cranio-caudal height of the tumor.

Results

Limits of Agreement ranges are larger for interobserver reliability compared to intraobserver reliability. Measurement error for all diameters (except d1, intraobserver) is greater than 2 mm. d1 measurements had the least variability and d3 measurements the highest variability, both for intra and interobserver measurements.

Conclusions

The optimal method of estimating VS size needs further investigation, and measurements need to be standardized and clearly defined. d3 seems to be the most difficult diameter to measure reliably. Interobserver measurement error for all diameters is greater than 2 mm. The current VS growth criterion of more than 1–2 mm, used to triage patients to surgery, lies within this error range, and thus is problematic as a guide for clinical practice. We therefore suggest that the growth criterion for VS be redefined.     

Teratoma of the middle ear: a real entity or a non-entity?

Space-occupying lesions of the middle ear range from wholly benign developmental anomalies to highly virulent and aggressive malignancies. Amongst the benign entities classed with the family of developmental anomalies, the middle ear choristoma is well known. But what of the middle ear teratoma? Recent reports suggest that, while rare, teratomas of the middle ear do exist. Middle ear teratomas are defined as tumors consisting of benign tissues derived from all three embryologic layers: endoderm, mesoderm and ectoderm. They are lesions of the neonatal period and early infancy. While complete surgical excision will be curative, a thorough histologic examination of the specimen is recommended to exclude the possibility of immature and/or frankly malignant elements being admixed with the benign teratomatous elements.     

Evaluation of neural response telemetry (NRT™) with focus on long-term rate adaptation over a wide range of stimulation rates

Abstract

Custom Sound EP? (CSEP) is an advanced flexible software tool dedicated to recording of electrically evoked compound action potentials (ECAPs) in Nucleus^® recipients using Neural Response Telemetry? (NRT?). European multi-centre studies of the Freedom? cochlear implant system confirmed that CSEP offers tools to effectively record ECAP thresholds, amplitude growth functions, recovery functions, spread of excitation functions, and rate adaptation functions and an automated algorithm (AutoNRT?) to measure threshold profiles. This paper reports on rate adaptation measurements. Rate adaptation of ECAP amplitudes can successfully be measured up to rates of 495 pulses per second (pps) by repeating conventional ECAP measurements and over a wide range of rates up to 8000 pps using the masked response extraction technique. Rate adaptation did not show a predictable relationship with speech perception and coding strategy channel rate preference. The masked response extraction method offers opportunities to study long-term rate adaptation with well-defined and controlled stimulation paradigms.     

Is the Trachea a Marker of the Type of Environmental Pollution?

OBJECTIVES/HYPOTHESIS: The differentiated character of changes in the mucous relief of the trachea as induced by air containing pollutants from the wastes of nickel-, mercury-, and cement-producing plants and by Candida albicans occurring in the waste disposal site of a large town are identified. The trachea was chosen because it is the entrance gate for the penetration of polluted air into the lungs. Changes on the trachea influence the character and extent of changes in lungs. STUDY DESIGN: Histological study with Viennese grey strain laboratory rabbits and rats caught directly on an investigated site. METHODS: We present new results of the functional morphology of the respiratory system as the results of scanning and transmission electron microscopy studies that can reveal character and range of damage of mucosal relief of trachea relevant to the functional dynamics of mucociliary clearance. Under physiological conditions this mechanism allows that only respirable dust particles enter the deep respiratory tract. RESULTS: In case of a damaged tracheal relief because of exposure to various aerosols, the functional morphology is changed, which aids in understanding the mechanisms damaging to mucociliary clearance induced by living in heavily polluted areas. CONCLUSION: Understanding of these morphological changes on base of detailed electron microscopic findings sheds new light on elaborating novel diagnostic methods for clinical practice.     

A twenty year (1971–1990) review of tracheostomies in a major paediatric hospital

Changing trends in the indications for paediatric tracheostomies, with decreasing numbers of tracheostomies being performed, have been reported in the literature. In a retrospective analysis of the period 1971 to 1990 the experience of tracheostomies in children under the age of 15 at Our Lady's Hospital (Dublin) is reviewed. Only 29 tracheostomies were performed during this time with an increase in numbers (90%) performed during the second 10 year period. The major underlying indication for tracheostomy in both 10 year periods was for the management of an airway problem secondary to congenital abnormalities (65%). In 14 children the operation was performed during the first year of life. However, while 90% of the children were under the age of one in the period 1971–1980 this fell to 26% during 1981–1990. Complications occurred in 41% overall, however, in the under 1 year old group 64% developed complications. There were no deaths as a direct result of the tracheostomy or its complications, but six children died because of the severity of the underlying disease. The average length of time before decannulation was 2.1 years, with decannulation difficulties occurring infrequently (11%).     

Kimura’s Disease — report of a case masquerading as a parotid tumor

Kimura’s Disease is a part of an ill- defined spectrum of vascular lesions of the subcutis. It is a benign chronic inflammatory condition producing subcutaneous tumour like nodules chiefly in the head and neck region with predilection for periauricular areas. It is characterised histologically by dense fibrosis, lymphoid infiltration, vascular proliferation and eosinophilia. The condition has a preponderance in the for eastern countries. It is extremely uncommon in Indian subcontinent. There has been no reported case from the Indian subcontinent in the English Literature. We report such a rare case in which the condition presents as parotid masses. We also present its CT, MRI & histopathology features and a brief review of literature.     

diagnosis and management of first branchial fistula: a study of 12 cases

To report authors' experiences in the diagnosis and treatments of congenital first branchial fistula (congenital auriculocervical fistula). Materials and Methods Twelve cases of congenital first branchial fistula were reviewed. Of these, 8 underwent fistulectomy with facial nerve dissection and partial parotidectomy and 4 underwent simple fistulectomy. Results The inner openings (upper opening) of fistulae lay in the following sites: inferioposterior wall at the junction of cartilaginous and bony segments of the auricular canal and inferior wall of cartilaginous auricular canal. The outer openings(lower opening) lay along the anterior border of upper sternocleidomastoid muscle, at the mastoid tip and posterior to the mandibular angle. Complete fistulae resection was achieved in all but one case. Eleven cases were followed for 5 year with no recurrence. Recurrence occurred in 1 case 6 months after the primary surgery and revision surgery was performed. Conclusions Pre-operative radiography for the location and course of the fistula is crucial for successful fistula resection, especially in cases with past infections. Facial nerve dissection should be done routinely for deeply located fistulae.     

Identification of early-stage Meniere’s disease as a cause of unilateral tinnitus

Introduction:Meniere’s disease(MD)is characterized by episodic symptoms,including vertigo,hearing loss,and tinnitus.Objectives:in this study,cases of unilateral tinnitus were investigated for MD.Method:Twenty-two patients who presented with chronic unilateral tinnitus on puretone audiograms showing an average threshold better than 25 dB HL and thresholds in the lower frequencies worse than those in the higher frequencies by more than 10 dB were suspected to have early-stage MD and underwent electrocochleography(ECochG).Patients showing ECochG findings conclusive for MD were compared to a control group of patients presenting with chronic unilateral tinnitus on pure-tone audiograms showing an average threshold better than 25 dB and thresholds in the higher frequencies worse than those in the lower frequencies by more than 10 dB.Results:Eighteen of the 22 patients included in this study showed elevated summating potential amplitude to action potential amplitude ratios in ECochG(suggestive of endolymphatic hydrops due to MD)and were followed up for 2 months;14 of them experienced at least two attacks of vertigo or unsteadiness.In contrast,only one patient in the control group reported two or more attacks of vertigo or unsteadiness in the 2 month observation period.The incidence of this finding in the two groups was significantly different.Conclusion:Patients with early-stage MD can present with only unilateral tinnitus.Thus,the addition of“pure-tone audiograms showing lower-frequency thresholds worse than higher-frequency thresholds”to the probable MD category in the globally.