Biomedical subjects

Paul Van de Heyning

Publications and source records attributed to Paul Van de Heyning.

17 recordsLinked to original sources

PubMed2007

Vestibular function testing.

PURPOSE OF REVIEW: This review provides an overview of vestibular function testing and highlights the new techniques that have emerged during the past 5 years. RECENT FINDINGS: Since the introduction of video-oculography as an alternative to electro-oculography for the assessment of vestibular-induced eye movements, the investigation of the utricle has become a part of vestibular function testing, using unilateral centrifugation. Vestibular evoked myogenic potentials have become an important test for assessing saccular function, although further standardization and methodological issues remain to be clarified. Galvanic stimulation of the labyrinth also is an evolving test that may become useful diagnostically. SUMMARY: A basic vestibular function testing battery that includes ocular motor tests, caloric testing, positional testing, and earth-vertical axis rotational testing focuses on the horizontal semicircular canal. Newer methods to investigate the otolith organs are being developed. These new tests, when combined with standard testing, will provide a more comprehensive assessment of the complex vestibular organ.

Electrodiagnosis↗

PubMed2006

A new autosomal recessive form of Stickler syndrome is caused by a mutation in the COL9A1 gene.

Stickler syndrome is characterized by ophthalmic, articular, orofacial, and auditory manifestations. It has an autosomal dominant inheritance pattern and is caused by mutations in COL2A1, COL11A1, and COL11A2. We describe a family of Moroccan origin that consists of four children with Stickler syndrome, six unaffected children, and two unaffected parents who are distant relatives (fifth degree). All family members were clinically investigated for ear, nose, and throat; ophthalmologic; and radiological abnormalities. Four children showed symptoms characteristic of Stickler syndrome, including moderate-to-severe sensorineural hearing loss, moderate-to-high myopia with vitreoretinopathy, and epiphyseal dysplasia. We considered the COL9A1 gene, located on chromosome 6q13, to be a candidate gene on the basis of the structural association with collagen types II and XI and because of the high expression in the human inner ear indicated by cDNA microarray. Mutation analysis of the coding region of the COL9A1 gene showed a homozygous R295X mutation in the four affected children. The parents and four unaffected children were heterozygous carriers of the R295X mutation. Two unaffected children were homozygous for the wild-type allele. None of the family members except the homozygous R295X carriers had any signs of Stickler syndrome. Therefore, COL9A1 is the fourth identified gene that can cause Stickler syndrome. In contrast to the three previously reported Stickler syndrome-causing genes, this gene causes a form of Stickler syndrome with an autosomal recessive inheritance pattern. This finding will have a major impact on the genetic counseling of patients with Stickler syndrome and on the understanding of the pathophysiology of collagens. Mutation analysis of this gene is recommended in patients with Stickler syndrome with possible autosomal recessive inheritance.

Child↗

PubMed2006

Primary and secondary auditory cortex stimulation for intractable tinnitus.

INTRODUCTION: Recent research suggests tinnitus is a phantom phenomenon based on hyperactivity of the auditory system, which can be visualized by functional neuroimaging, and transiently modulated by transcranial magnetic stimulation (TMS). We present the results of the first implanted electrodes on the primary and secondary auditory cortex after a successful TMS suppression. METHODS AND MATERIALS: Twelve patients underwent an auditory cortex implantation, 10 for unilateral and 2 for bilateral tinnitus, based on >50% suppression applying TMS. Results were analyzed for pure tone tinnitus and white noise tinnitus. RESULTS: TMS results in 77% pure tone tinnitus and 67% white noise reduction. Electrical stimulation via an implanted electrode results in a mean of 97% pure tone tinnitus and 24% white noise suppression. Mean Visual Analogue Scale score decreases from 9.5 to 1.5 for pure tone and from 8.8 to 6.8 for white noise postoperatively. DISCUSSION: Pure tone tinnitus might be the conscious percept of focal neuronal hyperactivity of the auditory cortex. Once visualized, this hyperactivity can be modulated by neurostimulation. CONCLUSION: The preliminary results of the first implantations suggest that patients with unilateral pure tone tinnitus are good surgical candidates for electrode implantation and permanent electrical stimulation of the auditory cortex, provided that the tinnitus is of recent origin and can be suppressed by TMS.

Adult↗

PubMed2006

Body surface area in normal-weight, overweight, and obese adults. A comparison study.

Values for body surface area (BSA) are commonly used in medicine, particularly to calculate doses of chemotherapeutic agents and index cardiac output. Various BSA formulas have been developed over the years. The DuBois and DuBois (Arch Intern Med 1916;17:863-71) BSA equation is the most widely used, although derived from only 9 subjects. More recently, Mosteller (N Engl J Med 1987;317:1098) produced a simple formula, [weight (kg) x height (cm)/3600](1/2), which could be easily remembered and evaluated on a pocket calculator, but validation data in adults are rare. The purpose of the present study was to examine the BSA based on Mosteller's formula in normal-weight (body mass index [BMI], 20-24.9 kg/m(2)), overweight (BMI, 25-29.9 kg/m(2)), and obese (BMI, >/=30 kg/m(2)) adults (>18 years old) in comparison with other empirically derived formulas (DuBois and DuBois, Boyd [The growth of the surface area of the human body. Minneapolis: University of Minnesota Press; 1935], Gehan and George [Cancer Chemother Rep 1970;54:225-35], US Environmental Protection Agency [Development of statistical distributions or ranges of standard factors used in exposure assessments Washington, EPA/600/8-85-010. Office of Health and Environmental Assessment; 1985), Haycock et al [J Pediatr 1978;93:62-6], Mattar [Crit Care Med 1989;17:846-7], Livingston and Scott [Am J Physiol Endocrinol Metab 2001;281:E586-91]) and with the new 3-dimensional-derived formula of Yu et al (Appl Ergon. 2003;34:273-8). One thousand eight hundred sixty-eight patients were evaluated (397 normal weight [BMI, 23 +/- 1 kg/m(2); age, 50 +/- 14 years; M/F, 289/108], 714 overweight [BMI, 27 +/- 1 kg/m(2); age, 52 +/- 11 years; M/F, 594/120], and 757 obese [BMI, 36 +/- 6 kg/m(2); age, 53 +/- 11 years; M/F, 543/215]). The overall BSA was 2.04 +/- 0.24 m(2): 1.81 +/- 0.19 m(2) in normal-weight, 1.99 +/- 0.16 m(2) in overweight, and 2.21 +/- 0.22 m(2) in obese subjects. These values were significantly higher in overweight and obese patients compared with the values using the DuBois-DuBois formula (overall, 2.00 +/- 0.22 m(2), P < .01; normal weight, 1.81 +/- 0.19 m(2), P = .93; overweight, 1.97 +/- 0.16 m(2), P < .01; obese, 2.14 +/- 0.21 m(2), P < .001). We could show an excellent correlation between the results obtained from each formula, with all correlations of 0.97 or higher (between 0.971 and 0.999). Body surface area prediction with the commonly used DuBois formula underestimated BSA in obese patients by as much as 3% (male) to 5% (female). Based on the formula of Yu et al, however, BSA is overestimated when these traditional formulas are used. Although Mosteller's formula is recommended based on its simplicity and suitability for laboratory and clinical work in adults, accuracy studies in whites with 3-dimensional one-pass whole-body scanning are needed.

Adult↗

PubMed2006

Behaviour of middle ear cleft mucosa during inflammation: histo- morphometric study.

CONCLUSION: This histo-morphometric study shows a significant reduction in the distance between the blood vessel's centre and the basal membrane of the middle ear cleft mucosa during inflammatory process. This could suggest that gaseous exchange is faster and more abundant in inflamed mucosa. The distance is significantly shorter in the postero-superior compartment of the middle ear cleft, possibly favouring a specific gas exchange function. OBJECTIVES: The aim was to try to understand the morphologic changes that occur when the middle ear cleft mucosa suffers from chronic inflammation. MATERIALS AND METHODS: Light microscopic computerized morphometry was used to measure the distance between the centre of the blood vessels and the basement membrane of the middle ear cleft mucosa. RESULTS: In inflammation, the distance from blood vessel to mucosa is reduced in all five regions of the middle ear cleft. This distance is statistically the shortest in the postero-superior compartment of the middle ear cleft.

Adolescent↗

PubMed2006

Audiometric analyses confirm a cochlear component, disproportional to age, in stapedial otosclerosis.

OBJECTIVE: To report the preoperative audiometric profile of surgically confirmed otosclerosis. STUDY DESIGN: Retrospective, multicenter study. SETTING: Four tertiary referral centers. PATIENTS: One thousand sixty-four surgically confirmed patients with otosclerosis. INTERVENTIONS: Therapeutic ear surgery for hearing improvement. MAIN OUTCOME MEASURES: Preoperative audiometric air conduction (AC) and bone conduction (BC) hearing thresholds were obtained retrospectively for 1064 patients with otosclerosis. A cross-sectional multiple linear regression analysis was performed on audiometric data of affected ears. Influences of age and sex were analyzed and age-related typical audiograms were created. Bone conduction thresholds were corrected for Carhart effect and presbyacusis; in addition, we tested to see if separate cochlear otosclerosis component existed. Corrected thresholds were than analyzed separately for progression of cochlear otosclerosis. RESULTS: The study population consisted of 35% men and 65% women (mean age, 44 yr). The mean pure-tone average at 0.5, 1, and 2 kHz was 57 dB hearing level. Multiple linear regression analysis showed significant progression for all measured AC and BC thresholds. The average annual threshold deterioration for AC was 0.45 dB/yr and the annual threshold deterioration for BC was 0.37 dB/yr. The average annual gap expansion was 0.08 dB/year. The corrected BC thresholds for Carhart effect and presbyacusis remained significantly different from zero, but only showed progression at 2 kHz. CONCLUSION: The preoperative audiological profile of otosclerosis is described. There is a significant sensorineural component in patients with otosclerosis planned for stapedotomy, which is worse than age-related hearing loss by itself. Deterioration rates of AC and BC thresholds have been reported, which can be helpful in clinical practice and might also guide the characterization of allegedly different phenotypes for familial and sporadic otosclerosis.

Adult↗

PubMed2005

GJB2 mutations and degree of hearing loss: a multicenter study.

Hearing impairment (HI) affects 1 in 650 newborns, which makes it the most common congenital sensory impairment. Despite extraordinary genetic heterogeneity, mutations in one gene, GJB2, which encodes the connexin 26 protein and is involved in inner ear homeostasis, are found in up to 50% of patients with autosomal recessive nonsyndromic hearing loss. Because of the high frequency of GJB2 mutations, mutation analysis of this gene is widely available as a diagnostic test. In this study, we assessed the association between genotype and degree of hearing loss in persons with HI and biallelic GJB2 mutations. We performed cross-sectional analyses of GJB2 genotype and audiometric data from 1,531 persons, from 16 different countries, with autosomal recessive, mild-to-profound nonsyndromic HI. The median age of all participants was 8 years; 90% of persons were within the age range of 0-26 years. Of the 83 different mutations identified, 47 were classified as nontruncating, and 36 as truncating. A total of 153 different genotypes were found, of which 56 were homozygous truncating (T/T), 30 were homozygous nontruncating (NT/NT), and 67 were compound heterozygous truncating/nontruncating (T/NT). The degree of HI associated with biallelic truncating mutations was significantly more severe than the HI associated with biallelic nontruncating mutations (P<.0001). The HI of 48 different genotypes was less severe than that of 35delG homozygotes. Several common mutations (M34T, V37I, and L90P) were associated with mild-to-moderate HI (median 25-40 dB). Two genotypes--35delG/R143W (median 105 dB) and 35delG/dela(GJB6-D13S1830) (median 108 dB)--had significantly more-severe HI than that of 35delG homozygotes.

Adolescent↗

PubMed2005

GJB2 (connexin 26) mutations are not a major cause of hearing loss in the Indonesian population.

Although hereditary hearing loss is a very heterogeneous disorder, variants in one gene, GJB2 (connexin 26), account for up to 50% of autosomal recessive nonsyndromal sensorineural hearing loss in most populations. This study investigates the contribution of GJB2 to autosomal recessive nonsyndromal hearing loss in the Indonesian population. We performed DNA sequence analysis in 120 patients with profound early childhood nonsyndromal hearing loss and in 100 control individuals and identified three novel variations resulting in amino acid substitutions (p.Gly4Asp, p.Thr5Ala, and p.Gly160Arg). Although we proved that p.Gly4Asp was not disease-causing, the pathological nature of p.Thr5Ala and p.Gly160Arg could not be determined. No recurrent disease-causing mutation could be detected in this Indonesian population. These findings are in contrast with the results obtained in other populations where GJB2 is a major cause of congenital recessive hearing loss.

Adolescent↗

PubMed2005

Transcranial magnetic stimulation for tinnitus: influence of tinnitus duration on stimulation parameter choice and maximal tinnitus suppression.

OBJECTIVE: Tinnitus is a distressing symptom for which few treatments exist. It leads to an important decrease in quality of life in 2 to 3% of the population. Tinnitus is considered a phantom sound, the result of cortical reorganization. Transcranial magnetic stimulation (TMS) is a noninvasive method to modulate cortical reorganization and has been shown to be able to influence tinnitus perception. STUDY DESIGN: Retrospective analysis. SETTING: Tertiary referral center. PATIENTS: The effect of TMS of the contralateral auditory cortex in 114 patients with unilateral tinnitus is investigated as one of the selection criteria used for surgical implantation of electrodes on the auditory cortex. INTERVENTION: TMS is performed at 90% of motor threshold at 1, 3, 5, 10, and 20 Hz, with each stimulation session consisting of 200 pulses. Results were classified as no effect (0-19% improvement), partial effect (20-79% improvement), and good effect (80-100 suppression). MAIN OUTCOME MEASURES: TMS had a good effect in 25% of the patients studied, partial effect in 28% patients, and no effect in 47%. RESULTS: TMS at 200 pulses is capable of tinnitus suppression for seconds only. The results were influenced by tinnitus duration: the longer the tinnitus exists, the lower the stimulation frequency that yields maximal tinnitus suppression (p < 0.001). The maximal amount of tinnitus suppression decreases in time (p < 0.01), resulting in a 2% decrease of potential tinnitus suppression per year. CONCLUSION: TMS of the auditory cortex is capable of modifying tinnitus perception for a very short time. The maximal amount of suppression and best stimulation frequency depends on the tinnitus duration.

Auditory Cortex↗

PubMed2005

Pulsatile tinnitus and the intrameatal vascular loop: why do we not hear our carotids?

OBJECTIVE: Pulsatile tinnitus is characterized by hearing the heart beat or respiration in one or both ears. In 15% of patients with pulsatile tinnitus, no cause can be found. Other investigators have suggested that a vascular loop entering the internal auditory meatus can be another cause of arterial, pulse synchronous tinnitus. If so, we should constantly hear the arterial pulsations of the carotid arteries passing through the petrous bone. METHODS: Using magnetic resonance imaging, 17 patients with unilateral pulsatile tinnitus and 46 with non-pulsatile tinnitus were analyzed for the presence of a vascular loop entering into the internal acoustic meatus. Four temporal bones were sectioned to find structural differences between the internal acoustic meatus and the pericarotid area. Four patients with intrameatal vascular loops and ipsilateral pulsatile tinnitus underwent surgery by Teflon interpositioning between the loop and the cochlea. RESULTS: In unilateral pulsatile tinnitus, a statistically highly significant amount of intrameatal vascular loops was noted in comparison to non-pulsatile tinnitus. A well-developed pericarotid venous plexus was found histologically. Three of the four patients who underwent surgery were initially tinnitus free, but pulsations recurred after 3 months in one patient. CONCLUSION: Vascular loops in the internal auditory canal may generate pulsatile tinnitus. It may be treated by placing Teflon between the cochlea and the intrameatal vascular loop. One then does not hear the pulsation of the carotids due to a dampening effect of a pericarotid venous plexus.

Adolescent↗

PubMed2004

A novel Z-score-based method to analyze candidate genes for age-related hearing impairment.

OBJECTIVE: Approximately half of the variance of Age-Related Hearing Impairment (ARHI) is attributable to environmental risk factors, and the other half to genetic factors. None of these genes has ever been identified, but the genes involved in monogenic nonsyndromic hearing impairment are good candidates. Here we define and validate a quantitative trait value for ARHI, correcting for age and gender, to allow the genetic study of ARHI as a quantitative trait. DESIGN: Based on the ISO 7029 standard, we convert audiometric data into a Z-score, an age- and gender-independent value expressing to what extent a person is affected by ARHI. The validity of this approach is checked using a test population of randomly collected subjects. The power to evaluate the contribution of a candidate gene to ARHI is assessed using simulated populations. As an example, one ARHI candidate gene is analyzed. RESULTS: In our test population, Z-scores were normally distributed although the mean did not equal zero. Z-scores were independent of age, and there was no difference between men and women. Power studies using simulated populations indicated that to detect moderate genetic effects, sample sizes of at least 500 random subjects are necessary. CONCLUSION: The Z-score conversion appears to be a valid method to describe to what extent a subject is affected by ARHI, allowing to compare persons from different age and gender. This method can be the basis of future, powerful studies to identify ARHI genes.

Adult↗

PubMed2004

Functional anatomy of the human cochlear nerve and its role in microvascular decompressions for tinnitus.

OBJECTIVE: The functional anatomy (i.e., tonotopy) of the human cochlear nerve is unknown. A better understanding of the tonotopy of the central nervous system segment of the cochlear nerve and of the pathophysiology of tinnitus might help to ameliorate the disappointing results obtained with microvascular decompressions in patients with tinnitus. METHODS: We assume that vascular compression of the cochlear nerve can induce a frequency-specific form of hearing loss and that when the nerve is successfully decompressed, this hearing loss can recuperate. Thirty-one patients underwent a microvascular decompression of the vestibulocochlear nerve for vertigo or tinnitus. Preoperative audiograms were subtracted from postoperative audiograms, regardless of the surgical result with regard to the tinnitus and vertigo, because the hearing improvement could be the only sign of the vascular compression. The frequency of maximal improvement was then correlated to the site of vascular compression. A tonotopy of the cochlear nerve was thus obtained. RESULTS: A total of 18 correlations can be made between the site of compression and postoperative maximal hearing improvement frequency when 5-dB hearing improvement is used as threshold, 13 when 10-dB improvement is used as threshold. A clear distribution can be seen, with clustering of low frequencies at the posterior and inferior side of the cochlear nerve, close to the brainstem, and close to the root exit zone of the facial nerve. High frequencies are distributed closer to the internal acoustic meatus and more superiorly along the posterior aspect of the cochlear nerve. CONCLUSION: The tonotopic organization of the cisternal segment of the cochlear nerve has an oblique rotatory structure as a result of the rotatory course of the cochlear nerve in the posterior fossa. Knowledge of this tonotopic organization of the auditory nerve in its cisternal course might benefit surgeons who perform microvascular decompression operations for the vestibulocochlear compression syndrome, especially in the treatment of unilateral severe tinnitus.

Adult↗

PubMed2003

Voice quality change in future professional voice users after 9 months of voice training.

Sixty-eight students of a school for audiovisual communication participated in this study. A part of them, 49 students, received voice training for 9 months (the trained group); 19 subjects received no specific voice training (the untrained group). A multidimensional test battery containing the GRBAS scale, videolaryngostroboscopy, Maximum Phonation Time (MPT), jitter, lowest intensity (IL), highest frequency (FoH), Dysphonia Severity Index (DSI) and Voice Handicap Index (VHI) was applied before and after training to evaluate training outcome. The voice training is made up of technical workshops in small groups (five to eight subjects) and vocal coaching in the ateliers. In the technical workshops, basic skills are trained (posture, breathing technique, articulation and diction), and in the ateliers, the speech and language pathologist assists the subjects in the practice of their voice work. This study revealed a significant amelioration over time for the objective measurements [Dysphonia Severity Index: from 2.3 to 4.5 ( P<0.001)] and the self-evaluation [Voice Handicap Index, from 23 to 18.4 ( P=0.016)] for the trained group only. This outcome favors the systematic introduction of voice training during the schooling of professional voice users.

Adult↗

PubMed2003

Chronic subglottic and tracheal stenosis: endoscopic management vs. surgical reconstruction.

Laryngotracheal stenosis has been and remains one of the most vexing problems in the field of head and neck surgery. Two treatment modalities prevail, endoscopic and external. The indication for each modality is not yet clearly defined. This undefined situation motivated our current work, and we decided to assess laser-assisted endoscopy (with or without stenting) vs. open surgery for treating chronic laryngotracheal stenosis. Our study included 28 cases of chronic laryngotracheal stenosis that were classified according to treatment in two main groups: group I included 13 patients who were endoscopically treated and group II included 15 patients with surgical reconstruction. The mean follow-up period was 12.58 months for group I and 27.43 months for group II. Respiratory function tests (RFT) were carried out preoperatively, 1-month postoperatively and on completion of follow-up. Except for age, which was significantly higher in group I (P<0.001), there was no significant difference between both groups. Although the incidence of complications was higher in group I (69%) than in group II (47%), it was nonsignificant. The postoperative RFT improved significantly in both groups. Although the improvement was higher in group I than group II, the difference was nonsignificant. The correlation between preoperative stridor and all other variables demonstrated that preoperative stridor correlated with the diameter of the stenosed segment (rs=-0.631, P<0.001) and the peak expiratory flow rate (PEFR) (rs=-0.488, P=0.030). Our results indicate that open surgery is the treatment of choice. Compared with endoscopic treatment, it provides a higher success rate and better functional results, especially long term. However, if contraindications to open surgery exist, whether local or general, laser-assisted endoscopy with stenting can offer good palliative results.

Adult↗

PubMed2003

Acute respiratory distress after upper airway obstruction following palatoplasty.

We report a case of acute respiratory distress after upper airway obstruction following routine palatoplasty in an otherwise healthy 6-year-old boy. We believe that extreme variation of intrathoracal pressure was the basis of the development of the acute respiratory distress. We recommend after palatoplasty a more than careful postoperative clinical observation to detect and treat postoperative obstruction and hypoxemia.

Airway Obstruction↗

PubMed2003

Vocal hygiene in radio students and in radio professionals.

In this study 27 future professional voice users (i.e., radio students) and 53 professional voice users (i.e., radio professionals) were questioned on their vocal hygiene. Both groups differed clearly in age and in radio experience. The mean age of the radio students was 22 years, whereas the mean age of the radio professionals was 41 years; radio students had no radio experience and radio professionals' experience ranged from 2 to 32 years. This study shows that the vocal hygiene of radio professionals is not superior to the vocal hygiene of radio students. On the contrary, every group has a distinctive vocal hygiene profile. The intake of coffee was significantly higher in radio professionals (p < 0.001) and they indicated significantly more vocal fatigue (p = 0.028). The radio students, on the other hand, experienced more acid reflux problems (p < 0.001) and signalled significantly more hoarseness (p = 0.012). Next to this, both groups smoked more than one could expect from (future) professional voice users (radio students: 33%; radio professionals: 28%). This study demonstrates the necessity of an adapted vocal hygiene education programme for both future and professional voice users, as they seem to underestimate the negative implications of bad vocal hygiene.

Adult↗

PubMed2003

Reanimation of the paralyzed human larynx with an implantable electrical stimulation device.

OBJECTIVES/HYPOTHESIS: Electrical stimulation of the posterior cricoarytenoid muscle, when paced with inspiration, offers a physiological approach to restore ventilation in bilateral laryngeal paralysis without any of the disadvantages associated with conventional treatment. STUDY DESIGN: A prospective study of six patients. METHODS: The patients were successfully implanted with an Itrel II stimulator (Medtronic, Inc). In postoperative sessions, stimulated vocal fold abduction, patient ventilation, and voice were assessed and compared with preoperative values. RESULTS: The optimum stimulus paradigm was a 1- to 2-second train of 1-millisecond pulses delivered at a frequency of 30 to 40 Hz and amplitude of 2 to 7 V. Posterior cricoarytenoid stimulation produced a large dynamic abduction (3.5-7 mm) in three patients and moderate abduction (3 mm) in a fourth patient. The fifth patient showed a large but delayed response of 4 mm to stimulation with some lateralization of the vocal fold. In the sixth patient, stimulated abduction was noted on device implantation but was lost postoperatively. All five patients with stimulated abduction postoperatively met the ventilatory criteria for decannulation, and three patients subsequently had decannulation. Long-term stimulation of the posterior cricoarytenoid muscle had no appreciable effect on voice quality. CONCLUSIONS: Electrical stimulation of the posterior cricoarytenoid muscle shows potential as an improved therapy for bilateral vocal fold paralysis.

Adult↗