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Biomedical subjects

L Feenstra

Publications and source records attributed to L Feenstra.

At least 55 records · Page 3Linked to original sources

Non-syndromic dominant sensorineural hearing loss: from a few phenotypes to many genotypes.

Sensorineural hearing loss affects approximately 1 in 2 persons at about 80 years of age and 1 in 750 in childhood. The best known forms of hearing loss with an autosomal dominant pattern of inheritance are the syndromic-mediated ones. At present, the non-syndromic autosomal dominant inherited forms can only be distinguished by the shape of the tone-audiogram. Based on gene linkage studies twelve different genotypes for autosomal dominant hereditary non-syndromic forms of sensorineural hearing loss have been recognized in a period of almost 2 years. In view of the great diversity of types that have been recognized in such a short period, it can be expected that over the next 10 years, several dozens genetically-mediated forms of autosomal dominant inherited sensorineural hearing loss will be detected. Similar developments are taking place in the non-syndromic autosomal recessive hereditary forms of sensorineural hearing loss and deafness. The above indicates clearly that before too long, new genetic investigation techniques will enable us to distinguish between forms of sensorineural hearing loss that could not be distinguished in the past.

Chromosome Aberrations↗

Vocal cord paralysis and cystic kidney disease in Hajdu-Cheney syndrome.

In this report we describe the clinical history and symptoms in a 36-year-old male with Hajdu-Cheney syndrome, an autosomal dominant condition with dissolution of the terminal phalanges (acro-osteolysis), characteristic craniofacial dysmorphism, and musculoskeletal alterations. He was admitted at that age because of progressive respiratory problems, with Cheyne-Stokes respiration and bilateral vocal cord paralysis. Terminal renal failure with cystic renal disease was diagnosed at the age of 14 years. The findings in the present patient illustrate the risk of progressive neurologic degeneration with involvement of the cranial nerves in patients with Hajdu-Cheney syndrome. Moreover, we confirm that cystic renal changes are an integral part of Hajdu-Cheney syndrome, and agree that Hajdu-Cheney syndrome and Serpentine fibula syndrome are probably variant examples of the same disease.

Adult↗

Use of a composite fascial carrier for laryngotracheal reconstruction.

A vascularized fascia flap may be used as a vascular carrier for free mucosal and cartilage grafts. This composite reconstruction technique has been performed in clinical situations with complex laryngeal or tracheal defects. This paper describes the technique and the rationale for applying this concept to some particular laryngotracheal defects. Morphologic and histologic aspects of this composite reconstruction technique using a transferable recipient bed are discussed.

Humans↗

The management of tinnitus with or without Menière's disease.

More than 95% of tinnitus patients do not require anything but careful examination and explanation, i.e. directive counselling. The first and principal therapy consists of history taking and routine ear, nose and throat (ENT) examination, including a thorough audiometric assessment with tinnitus analysis. The best management of the patient appears to be a behavioural approach centred on the complaint, including information provision and explanation, discussion of treatment options, and an informed selection from the alternatives available.

Humans↗

Experiences of a Belgian multidisciplinary breath odour clinic.

Experiences of a Belgian multidisciplinary breath odour clinic. Oral malodour is a frequent problem, which can benefit from a multidisciplinary approach (periodontology, ENT, internal medicine, psychiatry). An objective measurement of the amount of volatile sulphur compounds in the patient is very useful and easy to perform. Tongue coating is the most frequent cause. Initial treatment strategy should focus on oral hygiene. In a few cases, tonsillectomy or endoscopic sinus surgery may be necessary.

Adolescent↗

Quality of life in head and neck cancer patients.

In recent years the number of Quality of Life (QOL) studies has increased rapidly. When evaluating outcomes of clinical trials in patients, one should take into consideration QOL aspects in addition to the well-known standard biomedical endpoints. The difficult question is how to select the best instrument from the flood of papers. A review and a few reflections are presented.

Attitude to Health↗

Labyrinthine fistulae: a retrospective analysis.

A retrospective analysis has been conducted of 57 labyrinthine fistulae found in 375 cholesteatoma cases, primarily treated by removal of the matrix of the cholesteatoma and covering the fistula with a mixture of bone paste and fibrin glue. CT-scan with slices of 1 mm demonstrated the fistula in almost 90% of the cases. Eighteen percent of the ears were pre-operatively totally deaf. Large fistulae are riskier than smaller ones for post-operative perceptive losses, but even in very large and multiple fistulae the hearing may be preserved in most cases.

Bone Cements↗

Osteoma of the internal auditory canal.

A case of an osteoma of the internal auditory canal in a patient with severe hearing loss is presented. Osteomata of the internal auditory canal have only rarely been diagnosed. Symptoms consist in sensorineural hearing loss, vertigo and/or tinnitus. CT scan is an appropriate imaging modality. In selected cases surgery can be beneficial. A review of the literature is presented.

Adolescent↗

Mucociliary clearance following segmental tracheal reversal.

Ciliated tracheal epithelium is arranged in a polarized pattern oriented according to the inferior-superior axis of the trachea and is responsible for the transport of mucus toward the larynx. In this study, ciliary beat orientation and the influence of external factors on mucociliary clearance direction were studied in rabbit inverted cervical tracheas. The animals displayed normal respiration postoperatively. After 16 weeks, airway clearance was studied by observation of the movement of silicone particles placed in the inverted segment and in normal parts of the ciliated epithelium. Cilia exhibited unidirectional and coordinated movement within inverted tracheal segments. As shown by the direction of effective flow produced by beating cilia and by scanning electron microscopy, the cilia in the inverted segment beat in the opposite direction from the cilia in the remainder of the trachea. This study demonstrated that ciliary orientation is irreversibly determined, but the reversal of ciliary beating within the cervical trachea had no adverse effects on the survival of the animals.

Animals↗

Functional endoscopic sinus surgery under local anaesthesia: possibilities and limitations.

Functional endoscopic sinus surgery under local anaesthesia with one single intramuscular injection of systemic premedication (pethidine and promethazine) and careful local preparation (decongestive nose drops, cocaïne application and lidocaine infiltration). It is well tolerated and accepted in 95% of the patients. Blood loss is minimal and pain during the operation is rare. Major and minor orbital and intracranial complications were not seen. Local anaesthesia with this kind of surgery offers many advantages and is preferable to general anaesthesia when possible.

Adjuvants, Anesthesia↗

Hearing in hospitalised geriatric patients. A descriptive study.

A descriptive audiometric study was performed on 394 patients hospitalised in the geriatric department of our hospital. The mean age of these patients was 82 years. No significant correlation could be found between hearing thresholds and age of the patients. Eighty-six percent of our patients had a socially handicapping hearing loss.

Aged↗

Tinnitus maskers in the treatment of tinnitus. The MICROTEK 321Q.

Traditional tinnitus maskers are generators that deliver a grossly inaccurate broad band high energy noise which most tinnitus patients find an excessive noise intrusion, unacceptable and intolerable. Audiometric analysis defines tinnitus in terms of frequency and intensity. The 321Q-minimum energy narrow band microtek maskers (Mikrotek, Medical, Inc. Columbus) deliver a variable width and a low energy noise that is programmable to any frequency within the audible spectrum. We studied one hundred consecutive tinnitus patients. All patients underwent thorough ENT-analysis and audiometry. Only eight of them could be provided with the masking device and only four found some relief with its use. No correlation in the parameters could be found to discriminate between satisfied and unsatisfied patients.

Acoustic Stimulation↗

Spiral CT of the temporal bone: value of image reconstruction at submillimetric table increments.

Axial spiral CT of the temporal bones with a nominal slice thickness of 1 mm and 180 degrees linear interpolation was performed in 13 patients. In 18 temporal bones, the spiral data set was used to reconstruct overlapping axial images with a table increment of 0.1 mm. These images gave additional information in four cases: in two by examining the heavily overlapping axial images themselves, and in two by obtaining supplementary information from secondary image reconstructions. In two cases less information was obtained than by using the conventional incremental images. This study shows that reconstructing overlapping slices can be useful, even if the temporal bone is scanned at 1 mm nominal slice thickness.

Humans↗

Experimental tracheal allograft revascularization and transplantation.

The feasibility of tracheal allotransplantation with a fascial vascular carrier was examined in three groups with varied dose sequences of immunosuppression. A control group (group 1) received no medication. The three experimental groups were given daily cyclosporine intramuscular doses of 5 mg/kg (group 2), 5 mg/kg plus 3 mg/kg methylprednisolone (Solu-Medrol) (group 3), and 10 mg/kg (group 4) for 6 weeks or until death. Grafts were assessed by silicone dye infusion of the artery of the fascial flap to examine their microcirculation and by quantitative histologic study. Group 1 evidenced complete rejection after a heterotopic revascularization period of 14 days. The allografts of the experimental groups remained viable after 14 days of revascularization and could be transplanted orthotopically after this period. After transplantation, the viability of group 2 tracheas was unpredictable with changes ranging from mild to complete rejection. Group 3 evidenced well-preserved transplant viability with infection-induced necrosis at the anastomoses caused by the corticosteroid component. All group 4 animals survived the follow-up period with normal allograft viability. Cyclosporine in a dosage of 10 mg/kg per day can effectively suppress the immune response after transplantation of vascularized tracheal allografts. This experimental model will allow future studies to examine airway wall immunogenicity.

Acute Disease↗

Three-dimensional CT-imaging of the ossicular chain.

In eight patients, with a known air-conduction hearing loss, three-dimensional images of the ossicular chain were constructed based on heavily overlapping high resolution CT-slices, obtained from a spiral CT data set. High quality three-dimensional images could be obtained in all eight patients. Supplementary information was derived from these three-dimensional images in three patients; in one, this supplementary information had therapeutic importance. The slight image quality loss in spiral-CT of the temporal bone, compared with conventional CT, is in some cases largely compensated by the possibility of rendering high quality secondary reconstructions.

Adult↗

Correlation between functional, radiological and anatomical abnormalities in upper airway obstruction (UAO) due to tracheal stenosis.

Few data are available on the relative sensitivity of different lung function tests in upper airway obstruction (UAO) and on the correlation of these tests with radiological estimations of tracheal stenosis. This paper is based on patients with a goiter or tracheal tumor and in healthy subjects breathing through rigid resistors, as well as on literature data. Lung function abnormalities present a specific pattern in UAO: this is characterised by a typical shape of the maximal flow-volume curve, a marked reduction in peakflow (PEF) and only minor changes in routine spirometry (e.g. FEV1, which thus tends to underestimate the stenosis). The clinical relevance of the lung function abnormalities in UAO can be estimated from their relationship with the exercise limitation: a moderate exercise limitation to about 60% pred is found if PEF is reduced to about 40% pred, and Raw is increased to about 600% pred (in which instance FEV1 is still 75% pred). This limitation grossly corresponds to a UAO of about 6 mm diameter. No good correlation is found between lung function abnormalities and the radiological stenosis (CT-scan or X-ray): CT-scan or X-ray are only moderately correlated with each other. Moreover, stenosis can be visualised through X-ray in only about 60% of the cases.

Adult↗