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Biomedical subjects

P Van de Heyning

Publications and source records attributed to P Van de Heyning.

At least 19 recordsLinked to original sources

Relevance of subglottic pressure in normal and dysphonic subjects.

The purpose of this study was to determine the clinical value of subglottic pressure (SGP) in normal and dysphonic subjects. "The airway interrupted method" was used to measure the intra-oral pressure. Voice samples from 60 healthy subjects and 79 dysphonic patients were recorded under normal conditions of pitch and intensity. In normal subjects, age and gender have no significant impact on mean and peak SGP. The peak SGP measurements are significantly higher in dysphonic patients and could be included in the basic clinical set of objective voice parameters. No differences could be shown between pre- and postoperative assessments or between groups of dysphonic patients.

Adolescent↗

Evolution of vocal fold nodules from childhood to adolescence.

SUMMARY: Bilateral (quasi) symmetrical lesions of the anterior third of the vocal folds, commonly called vocal fold nodules (VFNs) are the most frequent vocal fold lesions in childhood caused by vocal abuse and hyperfunction. This study evaluates their long-term genesis with or without surgery and voice therapy. A group of 91 postmutational adolescents (mean age, 16 years), in whom VFNs were diagnosed in childhood, were questioned to analyze the evolution of their complaints. Thirty four of them could be clinically reexamined by means of the European Laryngological Society-protocol, including a complete laryngological investigation and voice assessment. A total of 21% of the questioned group (n=91) had voice complaints persisting into postpubescence with a statistically significant difference (P <or= 0.001) between boys (8%) and girls (37%). VFNs were still present in 47% of the girls and 7% of the boys of the clinically evaluated group (n=34). Analysis of the data before and after puberty shows that the variables gender, allergy, and degree of dysphonia ("G") in childhood enable a fairly correct prediction of persisting voice complaints in adolescence (sensitivity of 89% and specificity of 67%). The results of this study show a clearly different evolution for both sexes, with significant higher long-term risks for dysphonic girls with allergy.

Adolescent↗

Frequency specific hearing improvement in microvascular decompression of the cochlear nerve.

BACKGROUND: Microvascular compressions of the cochlear nerve can lead to hearing loss. Due to the tonotopic organization of the cochlear nerve any focal compression of the cochlear nerve will result in a frequency specific hearing loss. Decompressing the cochlear nerve could result in a frequency specific hearing improvement, without improving overall hearing. METHOD: Thirty one patients underwent microvascular decompression operations of the vestibulocochlear nerve for vertigo or tinnitus. Preoperative audiograms were substracted from postoperative audiograms obtained 2 years after microvascular decompression. The frequencies of maximal hearing improvement postoperatively were determined. FINDINGS: Of the 31 patients studied, 19 had improvements of 5 dB or more at one or more frequencies postoperatively, and 15 patients had improvements of 10 dB or more. Three patients had improvements of 25 dB or more postoperatively. The postoperative hearing improvement was frequency-specific and related to the anatomical location of the vascular contact on the auditory nerve. The improvement of hearing becomes diluted when the difference between pre- and postoperative hearing thresholds are averaged over all audiometric frequencies. We therefore present results for each frequency that was tested. CONCLUSIONS: Microvascular decompression of the cochlear nerve can improve hearing in selected patients. The improvement seems too small to justify decompressive surgery for the sole purpose of hearing improvement, but it could be considered if associated short vertigo spells, ipsilateral tinnitus, otalgia and cryptogenic hemifacial spasm are present. Decompression should be performed early, before BAEP changes become noticeable. 3D-MRI could become a valuable tool for selecting good surgical candidates.

Adult↗

Chronic suppurative otitis media and related complications at the University Clinic of Kinshasa.

During the five-year period from 1998 to 2002, the ENT department of the University Clinic of Kinshasa treated 343 patients suffering from chronic suppurative otitis media (CSOM) and complications. Two hundred and seven patients had simple, 28 had cholesteatomatous form of CSOM, and 108 patients presented with intratemporal or intracranial complications. Roughly half of the patient group consisted of children younger than 10 years. The simple form of CSOM affects men and women equally, but the cholesteatomatous form was more prevalent among men. Otorrhoea and hearing loss were the major presenting symptoms. Hearing loss was moderate to severe in 2/3 and slight in 1/3 of the audiometrically tested ears. A mastoidectomy was performed on 55% of the patients. Roughly 1/3 of the patients presented with complications, the majority of which (69) were subperiostal abscesses. Complications other than subperiostal abscesses were associated with a high morbitidy and a few patients even died. Our data suggest that the prevalence of CSOM in the Kinshasa area is at least 4% and, in fact, has not declined over the last 20 years.

Academic Medical Centers↗

Genetic testing for hearing impairment.

For some patients, genetic testing can reveal the etiology of their hearing impairment, and can provide evidence for a medical diagnosis. However, a gap between fundamental genetic research on hereditary deafness and clinical otology emerges because of the steadily increasing number of discovered genes for hereditary hearing impairment (HHI) and the comparably low clinical differentiation of the HHIs. In an attempt to keep up with the scientific progress, this article enumerates the indications of genetic testing for HHI from a clinical point of view and describes the most frequently encountered HHIs in Belgium. Domains of recent scientific interest, molecular biological aspects, and some pitfalls with HHIs are highlighted. The overview comprises bilateral congenital hearing loss, late-onset progressive high frequency hearing loss, progressive bilateral cochleo-vestibular deficit, and progressive low frequency hearing loss. Also, several syndromal forms of HHI are summarized, and the availability of genetic tests mentioned. Finally, the requirements for successful linkage analysis, an important genetic research tool for localizing the potential genes of a trait on a chromosome, are briefly described.

Belgium↗

Epidemiology of hearing impairment at three Flemish Institutes for Deaf and Speech Defective Children.

A retrospective analysis of 190 records of hearing impaired children up to the age of 14, all educated at one of three Flemish Institutes for Deaf and Speech Defective Children in Belgium, was performed. Until 1999, the well-known behavioral test (Ewing test) was used in the Flemish national screening program for hearing losses. Because it presented a lot of disadvantages, it has been replaced by Automated Auditory Brainstem Responses (AABR), enabling the Flemish national neonatal screening program to commence. This study is an extension of the preliminary results of a recently performed retrospective analysis in one Flemish institute of the hearing loss of patients that were diagnosed in the pre-AABR era. The authors analyzed the following data: etiology, risk factors of congenital hearing impairment, the patient's history from the moment of the first suspicion to diagnosis and treatment. The median age of the children was 8.5 years (2-14 year). In 66.5% the parents and/or grandparents were the first to raise suspicion, this was at a median age of approximately 9 months. The diagnosis was often made late, at a median age of 15 months (0-88 months). The etiology was unknown in 32.6% of the cases. The only cast-iron certain diagnoses were pre- and perinatal infections, syndromal and genetic hearing loss, and acquired infections (meningitis and measles). No risk factors of hearing loss, as they are stated by the Joint Committee on Infant Hearing, were found in more than 50% of the cases. Audiometry was performed in all cases, often supplemented with Auditory Brainstem Responses and/or click-evoked otoacoustic emissions, while other diagnostic investigations (imaging, genetics, etc.) were only variably performed. Finally, the authors confirm the need for universal neonatal screening, which only recently started in Belgium, and suggest that a detailed protocol should be established to pursue a coherent diagnostic policy.

Adolescent↗

Aortic root diameter and nasal intermittent positive airway pressure treatment in Marfan's syndrome.

We report on a patient with Marfan's syndrome, with coexistent obstructive sleep hypopnea (OSH) and restrictive lung disease, complicated by respiratory insufficiency, who was successfully treated with nasal intermittent positive airway pressure (NIPPV) and oxygen. NIPPV therapy turned out to be effective on arterial gas exchange and well tolerated. Moreover, progressive dilatation of the aortic root was attenuated during NIPPV, but could, however, not be reversed. We hypothetized that (at least partially) a decrease of nocturnal intrathoracic pressures could explain this.

Adult↗

Intermittent positive airway pressure by nasal mask as a treatment for respiratory insufficiency in a patient with syringomyelia.

We report a case of a 73-year-old woman with chronic respiratory failure in association with syringohydromyelia and sleep apnea. She was initially intubated and mechanically ventilated. Weaning from the ventilator was only successful after nasal intermittent positive airway pressure therapy was started. A normalization of arterial blood gas exchange could be achieved.

Aged↗

Short-term CPAP does not influence the increased CO2 drive in idiopathic central sleep apnea.

Continuous positive airway pressure (CPAP) improves idiopathic central sleep apnea (ICSA), but the mechanisms are not completely understood. It has been demonstrated that some ICSA patients have an increased CO2 drive, expressed by the hypercapnic ventilatory response (HCVR). The aim of our study was to evaluate whether CPAP can decrease the HCVR in successfully treated ICSA patients. Also the central apnea mean duration (CMD) was studied. Nine patients were evaluated. Their apnea-hypopnea index at baseline was high, but decreased from 50 +/- 5 to 8 +/- 3, during 5 +/- 1 cm H2O CPAP. After one month CPAP high values were still found for HCVR (2.69 +/- 0.23 L.min-1.mmHg-1 at baseline, 2.71 +/- 0.33 L.min-1.mmHg-1 after CPAP). Sleep quality was only improved by a decrease in the arousal index (from 29 +/- 12 to 6 +/- 1 a night) (p = 0.04). Daytime arterial O2 partial pressure (PaO2) increased from 73 +/- 4 mmHg to 85 +/- 3 mmHg (p = 0.02), whereas daytime arterial CO2 partial pressure (PaCO2) remained unchanged (from 41 +/- 1 mmHg to 40 +/- 1 mmHg). CMD was 21 +/- 6 s before and 13 +/- 1 s after CPAP (p = 0.05). The presence of hypersomnolence decreased from in 89% to in 33% of the patients. It is concluded that CPAP treatment can induce a subjective and objective improvement in ICSA patients, with improved sleep-related respiratory indices, daytime PaO2, but without a change in CO2 drive. High chemical drives seem to be the cause and not the consequence of sleep disordered breathing and persist after treatment.

Analysis of Variance↗

A second gene for otosclerosis, OTSC2, maps to chromosome 7q34-36.

Otosclerosis due to abnormal bone homeostasis of the otic capsule is a frequent cause of hearing loss in adults. Usually, the hearing loss is conductive, resulting from fixation of the stapedial footplate, which prevents normal ossicular vibration in response to sound. An additional type of sensorineural hearing loss may be caused by otosclerotic damage to the cochlea. The etiology of the disease is unknown, and both environmental and genetic factors have been implicated. Autosomal dominant inheritance with reduced penetrance has been proposed, but large families are extremely rare. To elucidate the pathogenesis of the disease, identification of the responsible genes is essential. In this study, we completed linkage analysis in a Belgian family in which otosclerosis segregates as an autosomal dominant disease. After excluding linkage to a known locus on chromosome 15 (OTSC1), we found linkage on chromosome 7q, with a multipoint LOD score of 3.54. Analysis of key recombinant individuals maps this otosclerosis locus (OTSC2) to a 16-cM interval on chromosome 7q34-36 between markers D7S495 and D7S2426.

Alleles↗

Therapeutic electrical stimulation of the hypoglossal nerve in obstructive sleep apnea.

BACKGROUND: Hypoglossal nerve stimulation has been demonstrated to relieve upper airway obstruction acutely, but its effect on obstructive sleep apnea is not known. OBJECTIVE: To determine the response in obstructive sleep apnea to electrical stimulation of the hypoglossal nerve. METHODS: Eight patients with obstructive sleep apnea were implanted with a device that stimulated the hypoglossal nerve unilaterally during inspiration. Sleep and breathing patterns were examined at baseline before implantation and after implantation at 1, 3, and 6 months and last follow-up. RESULTS: Unilateral hypoglossal nerve stimulation decreased the severity of obstructive sleep apnea throughout the entire study period. Specifically, stimulation significantly reduced the mean apnea-hypopnea indices in non-rapid eye movement (mean +/- SD episodes per hour, 52.0 +/- 20.4 for baseline nights and 22.6 +/- 12.1 for stimulation nights; P<.001) and rapid eye movement (48.2 +/- 30.5 and 16.6 +/- 17.1, respectively; P<.001) sleep and reduced the severity of oxyhemoglobin desaturations. With improvement in sleep apnea, a trend toward deeper stages of non-rapid eye movement sleep was observed. Moreover, all patients tolerated long-term stimulation at night and did not experience any adverse effects from stimulation. Even after completing the study protocol, the 3 patients who remained free from stimulator malfunction continued to use this device as primary treatment. CONCLUSION: The findings demonstrate the feasibility and therapeutic potential for hypoglossal nerve stimulation in obstructive sleep apnea.

Adult↗

Evaluation for sleep apnea in patients with Ehlers-Danlos syndrome and Marfan: a questionnaire study.

Sleep complaints are frequently reported by patients with Marfan and Ehlers-Danlos syndrome (EDS). We examined the exact nature of sleep complaints in these patients. A representative sample of Marfan and EDS patients responded to a general sleep questionnaire, including the Epworth Sleepiness Scale (ESS) and the Medical Outcomes Study Short-Form 36 (SF-36) health-related quality of life (QOL) questionnaire. Fifteen Marfan patients and 9 EDS patients were evaluated and compared to 24 healthy controls, matched for age, sex and body mass index. Maintaining sleep was frequently disturbed in Marfan (40%, p < 0.04) as well as in EDS patients (56%, p < 0.01). Sleep apnea was exclusively reported by Marfan patients (27%, p = 0.03). Periodic limb movements were much more reported in EDS (67%, p = 0.02) than in Marfan (27%, p = 0.25) compared to controls (8%). Pain and back complaints were highly presented in both groups, but most pronounced in EDS patients (47% in Marfan versus 77% in EDS). No differences for the scores in the ESS were found. For all SF-36 questionnaire items, scores were much lower in patient groups, except for emotional problems. We found that sleep complaints were not rare in Marfan and EDS patients and correlated well with different QOL items. Our study calls for greater attention to the presence of apnea, pain and periodic limb movements in these patients.

Adult↗

Prevalence of otosclerosis in an unselected series of temporal bones.

BACKGROUND: Histologic otosclerosis is a disease process without clinical symptoms or manifestations that can be discovered only by sectioning of the temporal bone at autopsy. Clinical otosclerosis is otosclerosis at a site where it causes conductive hearing loss by interfering with the motion of the stapes or of the round window membrane. Various authors have studied the prevalence of histologic otosclerosis on laboratory collections of temporal bones. Some 12% to 15% of temporal bones with histologic otosclerosis have demonstrated stapedial fixation. Using these figures for calculating the prevalence of clinical otosclerosis gives an extrapolated clinical prevalence of 0.99% to 1.2%. This does not correlate well with the clinical data on otosclerotic families, from which a clinical prevalence of 0.3% has been estimated. OBJECTIVE: To study the prevalence of histologic otosclerosis in an unselected series of temporal bones. STUDY DESIGN: During a 1-year period, 118 consecutive pairs of temporal bones of deceased patients at a tertiary care center were collected to determine the prevalence of otosclerosis. Although histology remains the gold standard for evaluation of otosclerosis, the gross observation of temporal bone slices combined with microradiography was used to screen for otosclerotic lesions more rapidly and with a lower cost/benefit ratio. The temporal bones, which were suspected of having otosclerosis with these techniques, were further analyzed by conventional histology. RESULTS: 2.5% of the 236 temporal bones (or 3.4% of patients) studied demonstrated histologic otosclerosis. CONCLUSIONS: Although the prevalence of 2.5% is much lower than previously published figures on histologic otosclerosis, the extrapolated data (extrapolated clinical prevalence = 0.30% to 0.38%) correlate well with clinical studies of otosclerotic families. The previous studies based on laboratory collections were likely biased by hearing loss or other otologic diseases.

Adolescent↗

Continuous positive airway pressure and lung inflation in sleep apnea patients.

BACKGROUND: It was shown in normals that an important decrease in upper airway resistance can be obtained with continuous positive airway pressure (CPAP). It was suggested that lung inflation in patients with sleep apnea syndrome (SAS) could also be a mechanism of action of CPAP. OBJECTIVE: In the present study we wanted to evaluate the effects of nocturnal CPAP on the daytime lung function pattern in patients with SAS. METHODS: We measured arterial blood gases and possible changes in static lung volumes in 57 SAS patients (37 with normal lung function, 10 with COPD and 10 with restrictive lung disease) after at least one month of CPAP therapy. RESULTS: A significant increase in PaO(2) (from 79 to 84 mm Hg, p = 0.01) and a decrease in AaDO(2) (from 23 +/- 1 to 16 +/- 1, p < 0.01) was only observed in SAS patients with normal lung function. This improved gas exchange was parallelled by a small but non significant change in the FRC (from 96.5 +/- 3.2 to 105.4 +/- 3.7%pred, p = 0.07) and TLC (from 101.3 +/- 1.7 to 104.1 +/- 1.4%pred, p = 0.15). Similar changes in TLC and FRC were also observed in SAS patients with obstructive and restrictive lung disease. CONCLUSIONS: Chronic nocturnal CPAP therapy can improve daytime gas exchange and may influence lung inflation during the daytime. The small changes seem to be a functional effect but of no clinical relevance.

Female↗

Wegener's granulomatosis triggered by infection?

Wegener's granulomatosis is a systemic disease of unknown origin, although recent studies suggest that auto-immune mechanisms and infection play a role in the pathogenesis of this disease. Wegener is characterized by a necrotizing vasculitis involving the lungs (pulmonary infiltrates), the upper airways and the kidneys (rapidly progressive glomerulonephritis). We present a case of a male patient admitted because of progressive deterioration of the general condition with weight loss, a unilateral neck mass, unilateral purulent rhinorrea and fever. CT-scan evaluation demonstrated a unilateral expanding mass in the sing-nasal cavity, obliterating the ethmoid complex. MRI revealed signs of intracranial inflammatory reaction and onset of absedation. A malignancy was suspected but a diagnosis of Wegener's granulomatosis was established based on histologic criteria (nasal biopsy) and a positive titer for anti-cytoplasmic antibodies (cANCA). During follow-up, nasal carriage of Staphyloccocus Aureus could be documented. An overview of Wegener's granulomatosis will be provided with emphasis on the potential role of acute infections as a trigger for Wegener's granulomatosis and the head and neck manifestations.

Acute Disease↗

Refined localization and two additional linked families for the DFNA10 locus for nonsyndromic hearing impairment.

DFNA10 originally was mapped to the long arm of chromosome 6 in a large American family segregating for autosomal dominant progressive nonsyndromic hearing impairment. By extending this American family, we have reduced the original DFNA10 candidate region from 13 cM to 3.7 cM. We also report a Belgian family with autosomal dominant nonsyndromic hearing impairment linked to DFNA10 and a Norwegian family with the same condition in which linkage is suggestive, although maximum lod scores are only 2.5. The hearing phenotype in all three DFNA10 families is similar, with losses beginning in the middle frequencies and involving the low and high frequencies later in life.

Audiometry↗