Biomedical subjects

Emmanuel A M Mylanus

Publications and source records attributed to Emmanuel A M Mylanus.

At least 19 recordsLinked to original sources

PubMed2006

Evaluation of the Parental Perspective instrument for pediatric cochlear implantation to arrive at a short version.

OBJECTIVE: Evaluation of the well-known and widely used Parental Perspectives questionnaire (PP) by means of statistical analysis and exploring the possibility to develop a short version, as the instrument is often regarded as being rather lengthy with 74 questions. METHODS: One hundred and thirty parents of children participated in this study. To assess internal consistency among the PP items of the domains, Cronbach's alpha coefficients were calculated. Corrected item-total correlations were carried out to investigate the strength of individual items' associations with the construct. Factor analysis was performed to identify the statistical factors of the original PP and in exploring a revised short form PP. RESULTS: Instead of the expected 8-factor structure (eight suggested domains), factor analyses found a 15-factor structure. Nevertheless, when the proposed eight domain structure is followed, some items can be disposed of, based upon the Cronbach's alpha analyses and consistent reasoning. After reducing the number of factors based on standard criteria, a three-domain structure was shown as main concept. The cumulative variance explained by this new model was 39.4% and the final number of items in this probable revised version is 23. Reliability analyses of the new domains of the proposed short version PP (sPP) showed good internal consistency (Cronbach's alpha 0.79). The corrected item-total correlations represent strong individual items' associations with the construct as R items-total varies between 0.34 and 0.64. CONCLUSIONS: The Parental Perspectives instrument (PP) is an important tool to assess the impact of cochlear implantation of a child for the quality of life for the family and the child itself. This statistical investigation showed a possible option for development of a short form usable in prospective follow-up studies.

Adolescent↗

PubMed2006

No hearing loss associated with the use of artemether-lumefantrine to treat experimental human malaria.

Artemisinin derivatives are becoming the first-line treatment for uncomplicated malaria in areas with widespread resistance to chloroquine. Although generally safe and well tolerated, it has been suggested from animal experiments, and more recently from one human study with artemether-lumefantrine, that these compounds are potentially neurotoxic, affecting particularly the brainstem auditory pathways. We report here the auditory analyses of 15 volunteers who underwent an experimental human malaria infection and were treated with artemether-lumefantrine. The subjects underwent audiological examination before the start of the study, during infection, and after treatment. Examination included standard tone audiometry, high frequency tone audiometry and auditory brainstem response (ABR). No effects on hearing loss that were deemed to be caused by drug treatment were found using tone audiometry. ABR analysis similarly failed to demonstrate any auditory pathway damage in the volunteers after treatment. We have thus not found any clear evidence of a detrimental effect on the auditory system by artemether-lumefantrine treatment in uncomplicated malaria. Our results support the continued implementation of artemisinin derivatives in the fight against drug-resistant malaria.

Adolescent↗

PubMed2006

Estimated cost-effectiveness of active middle-ear implantation in hearing-impaired patients with severe external otitis.

OBJECTIVE: To determine the cost-effectiveness of middle-ear implantations in hearing-impaired patients with severe external otitis in the Netherlands. DESIGN: Cost-effectiveness analysis, using single-subject repeated measures of quality of life and total cost determinations. SETTING: Hospital based. Patients Moderately to severely sensorineurally hearing-impaired patients (n = 21) with severe chronic external otitis, eligible to receive a middle-ear implant. Main Outcome Measure Cost per quality-adjusted life-year (QALY), based on scores of the Medical Outcomes Study Short-Form Health()Survey (SF-36) generic quality of life questionnaire. Only direct costs were included in cost calculation of middle-ear implantation. RESULTS: Mean health utility gain was 0.046 (0.012-0.079) (P = .01) measured at the mental component of the SF-36. With a mean profitable time of 19.4 years and an overall cost of euro 14,354, minimal cost-effectiveness of middle-ear implantation was euro 16,085/QALY. CONCLUSION: Based on the cost per QALY, middle-ear implantation proved to be a cost-effective and justified health care intervention in the Netherlands.

Adolescent↗

PubMed2006

Fitting range of the BAHA Cordelle.

The performance of the most powerful Bone-Anchored Hearing Aid (BAHA) currently available, the BAHA Cordelle, was evaluated in 25 patients with severe to profound mixed hearing loss. Patients showed bone conduction thresholds at 500, 1000 and 2000 Hz, ranged between 30 and 70 dB HL, and an additional air-bone gap of about at least 30 dB. With the BAHA Cordelle, free-field thresholds improve relative to bone-conduction thresholds with 1.5, 5.0, 17.8, and 4.3 dB at 500, 1000, 2000, and 4000 Hz, respectively, with substantial inter-individual variability. The differences in unaided air conduction thresholds and aided free-field thresholds amount to 45.3, 45.8, 47.5, and 43.5 dB at 500, 1000, 2000, and 4000 Hz, respectively. Speech perception, measured both with monosyllables of the consonant-vowel-consonant type and with bisyllables, showed highly similar results. The fitting range of a (linear) hearing aid is determined by its gain characteristics. Requiring aided speech reception thresholds at or better than 65 dB SPL results in an upper limit of the fitting range of the BAHA Cordelle for bone-conduction thresholds of 51, 56, 67, and 58 dB HL at 500, 1000, 2000, and 4000 Hz, respectively. The dynamic range provided by the BAHA Cordelle was estimated from loudness growth functions at 500, 1500, and 3000 Hz employing 7-point categorical scaling. On average, aided loudness growth functions exhibit normal slopes but they level off at input levels of about 80, 70, 65 dB SPL for 500, 1500, and 3000 Hz stimuli, respectively. Measurements with a skull simulator demonstrated that the levelling-off reflects saturation of the output of the Cordelle. The relatively low saturation levels of the device suggest that increasing maximum output levels may be a worthwhile consideration for candidates with more profound sensorineural loss.

Acoustic Stimulation↗

PubMed2006

Rehabilitation of patients with conductive hearing loss and moderate mental retardation by means of a bone-anchored hearing aid.

OBJECTIVE: To evaluate whether the bone-anchored hearing aid (BAHA) can be applied successfully to patients with conductive hearing loss and moderate mental retardation. STUDY DESIGN: Retrospective clinical evaluation. SETTING: Tertiary referral center. PATIENTS: Twenty-two patients with congenital moderate mental retardation and conductive or mixed hearing loss were selected to receive a BAHA at the University Medical Centre Nijmegen, the Netherlands. Four of them were fitted despite a limited air-bone gap. INTERVENTION: Rehabilitative BAHA application. MAIN OUTCOME MEASURES: Implantation results, skin reactions, and audiological data were evaluated during a mean follow-up of 36 months. RESULTS: All the patients were still using the BAHA 7 days a week and for more than 8 hours a day after a follow-up period between 5 and 96 months. Two implants (9%) were lost due to insufficient integration but were reimplanted successfully. With the BAHA, mean free-field thresholds showed a clear mean improvement of 9 dB compared with the previous hearing aid. Considerable improvements in daily activities were seen in at least five patients. CONCLUSION: Moderate mental retardation should no longer be considered as a contraindication for BAHA application. Although implant loss was low, extra attention may be required from the personal care providers to maintain the percutaneous implant. The BAHA was well-accepted by the patients with moderate mental retardation and was being used for most of the day. Implementation of the BAHA as hearing aid treatment in patients with moderate mental retardation proved to be sufficiently effective and may have strongly positive effects on activities at school or at work.

Adolescent↗

PubMed2006

Quality of life and cochlear implantation in Usher syndrome type I.

OBJECTIVES: The objectives of this descriptive, retrospective study were to evaluate quality of life, hearing, and vision in patients with Usher syndrome type I with and without cochlear implant. METHODS: Quality of life (QoL) of 14 patients with Usher type I (USH1) with a cochlear implant (CI) (seven adults, seven children) was compared with those of 14 patients with USH1 without a CI (12 adults, two children) by means of three questionnaires: NCIQ, SF12, and the Usher Lifestyle Survey. Additional information on hearing level was obtained by the equivalent hearing loss (EHL) principle and on the visual deterioration by the functional vision score (FVS). RESULTS: A significant benefit of CI was seen in the hearing-specific questionnaire NCIQ. This difference could not be detected in the generic SF12 survey. The Usher Lifestyle Survey indicated that patients with USH1 with a CI tend to be able to live an independent life more easily than the profoundly deaf unimplanted patients with USH1. EHL and FVS scores varied in both groups. CONCLUSIONS: Overall QoL can be enhanced by CI in patients with USH1, although effects are mostly seen in hearing-related QoL items.

Adolescent↗

PubMed2006

Audiologic performance and benefit of cochlear implantation in Usher syndrome type I.

OBJECTIVE: The objective of this retrospective study was to evaluate the benefit and performance of cochlear implantation in patients with Usher syndrome type 1 (USH1). METHODS: Fourteen patients with a clinical diagnosis of USH1 were included. Mutation analysis of USH1 genes was performed in all of them. All patients filled in the G(C)BI questionnaire, which measures the benefit of implantation. In addition, equivalent hearing level scores (EHL) were calculated to measure performance. Correlations between the mentioned parameters were studied. RESULTS: One or two pathogenic mutations were identified in seven of the 14 examined patients. Similar to previous studies, it was demonstrated that implantation at an earlier age results in better performance than implantation at higher age. Cochlear implantation performed within the first 2 decades of life was beneficial to 13 of 14 (93%) of the patients with USH1. Finally, the EHL score and the G(C)BI score showed a significant correlation; the benefit of implantation increases with a decreasing EHL score. CONCLUSIONS: Cochlear implantation in patients with USH1 improves the audiologic performance when patients are implanted at an earlier age and is beneficial according to the G(C)BI when performed within the first 2 decades of life.

Adolescent↗

PubMed2006

Classroom performance of children with cochlear implants in mainstream education.

OBJECTIVES: We compared classroom performance of children with a cochlear implant (CI) with that of their normal-hearing peers in mainstream education. METHODS: Thirty-two CI children in mainstream education, congenitally or prelingually deaf, participated in this study, as did 37 hearing classmates. Their teachers filled out 2 questionnaires: the Assessment of Mainstream Performance (AMP) and the Screening Instrument for Targeting Educational Risk (SIFTER). A high Fletcher index and open-set speech perception scores were obtained. RESULTS: The children with CIs scored above average on the AMP and sufficiently well in all but one area (communication) of the SIFTER questionnaire. Class rankings did not differ significantly between the CI students and their normal-hearing peers. Overall, the normal-hearing group outperformed the CI group. The classroom performance of CI children correlated negatively with duration of deafness and age at implantation. All longitudinal audiological data of the CI children showed improvement in open-set speech recognition. CONCLUSIONS: Although the results are encouraging, the CI group scored significantly less well than their normal-hearing peers on most questionnaire domains of both the AMP and the SIFTER. The most important variables for the outcome in this study were age at implantation and duration of deafness.

Child↗

PubMed2005

Does the bone-anchored hearing aid have a complementary effect on audiological and subjective outcomes in patients with unilateral conductive hearing loss?

OBJECTIVES: To study the effect of a bone-anchored hearing aid (BAHA) in patients with unilateral conductive hearing loss. STUDY DESIGN: Prospective evaluation on 18 subjects. METHODS: Aided and unaided binaural hearing was assessed in the sound field using a sound localization test and a speech recognition in noise test with spatially separated sound and noise sources. The patients also filled out a disability-specific questionnaire. PATIENTS: 13 out of the 18 subjects had normal hearing on one side and acquired conductive hearing loss in the other ear. The remaining 5 patients had a unilateral air-bone gap and mild symmetrical sensorineural hearing loss. RESULTS: Sound localization with the BAHA improved significantly. Speech recognition in noise with spatially separated speech and noise sources also improved with the BAHA. Fitting a BAHA to patients with unilateral conductive hearing loss had a complementary effect on hearing. Questionnaire results showed that the BAHA was of obvious benefit in daily life. CONCLUSIONS: The BAHA proved to be a beneficial means to optimize binaural hearing in patients with severe (40-60 dB) unilateral conductive hearing loss according to audiometric data and patient outcome measures.

Adult↗

PubMed2005

Long-term results of bone-anchored hearing aid recipients who had previously used air-conduction hearing aids.

OBJECTIVE: To study the long-term results (use, care, satisfaction, ear infections, and audiometry) of the application of a bone-anchored hearing aid (BAHA) to patients with conventional indications who had previously used air-conduction hearing aids. DESIGN: Follow-up study (mean duration, 9 years). SETTING: Tertiary referral center. PATIENTS: The study population comprised 27 patients with conductive or mixed hearing loss and who had participated in a previous study (N = 34). Seven could not be included anymore as a result of death, Alzheimer disease, or problems related to the implant. Everyone filled out the questionnaire, and 23 patients underwent audiometric evaluation. MAIN OUTCOME MEASURES: The patients filled out the adapted Nijmegen questionnaire. Aided free-field thresholds were measured as well as scores for speech in noise and in quiet. Results were compared with those obtained in the initial study. RESULTS: All 27 patients were still using their BAHA and appreciated it with regard to speech recognition in quiet, sound comfort, and improvements in ear infections. The audiometric results showed that most patients tested had stable bone-conduction thresholds over the years (after correction for age). Despite the treatment with BAHA, a significant deterioration in the cochlear hearing was observed in the other patients in the ear under study (their best hearing ear). CONCLUSIONS: Positive patient outcome measures emphasized the importance of BAHA application to patients with conventional indications. The audiometric data showed fairly stable cochlear function but not for all patients. This underlines that conservative treatment should be chosen (fitting of bone-conduction devices).

Audiometry↗

PubMed2005

The Usher lifestyle survey: maintaining independence: a multi-centre study.

Patients with Usher syndrome face a special set of challenges in order to maintain their independence when their sight and hearing worsen. Three different types of Usher (I, II and III) are distinguished by differences in onset, progression and severity of hearing loss, and by the presence or absence of balance problems. In this study 93 Usher patients from seven European countries filled out a questionnaire on maintaining independence (60 patients type I, 25 patients type II, four patients type III and four patients type unknown). Results of Usher type I and II patients are presented. Following the Nordic definition of maintaining independence in deaf-blindness, three domains are investigated: access to information, communication and mobility. Research variables in this study are: age and type of Usher, considered hearing loss- and the number of retinitis pigmentosa-related sight problems. Usher type I patients tend to need more help than Usher type II patients and the amount of help that they need grows when patients get older or when considered hearing loss worsens. No patterns in results were seen for the number of retinitis pigmentosa related sight problems.

Activities of Daily Living↗

PubMed2005

Bone-anchored hearing aids in unilateral inner ear deafness: an evaluation of audiometric and patient outcome measurements.

OBJECTIVE: To evaluate the benefit of a bone-anchored hearing aid contralateral routing of sound hearing aid (BAHA CROS hearing aid) in 29 patients with unilateral inner ear deafness. STUDY DESIGN: Prospective clinical follow-up study. SETTING: Tertiary referral center. PATIENTS: Thirty patients were recruited. There were 19 patients with a history of acoustic neuroma surgery and 11 patients with unilateral inner ear deafness due to other causes; 1 patient was excluded. The first 21 patients had also participated in a previous evaluation. INTERVENTION: Audiometric measurements were taken before intervention, when fitted with a conventional CROS, and after BAHA implementation. Patients' subjective benefit was quantified with four different hearing aid-specific instruments: the Abbreviated Profile of Hearing Aid Benefit, the Glasgow Hearing Aid Benefit Profile, the International Outcome Inventory for Hearing Aids, and the Single-Sided Deafness questionnaire. MAIN OUTCOME MEASURES: The same instruments were used at a mean long-term follow-up of 1 year after BAHA implantation. RESULTS: Sound localization in an audiologic test setting was no different from chance level. The main effect of the BAHA CROS that was found was the "lift the head shadow" effect in the speech-in-noise measurements. All instruments also showed positive results in favor of the BAHA CROS at long-term follow-up. CONCLUSIONS: The poor sound-localization results in an audiologic test setting illustrated the inability of patients with unilateral inner ear deafness to localize sounds. The speech-in-noise measurements demonstrated the efficacy of the BAHA CROS to lift the head shadow. Patients were still satisfied at 1-year follow-up, according to the four instruments.

Adult↗

PubMed2005

Consensus statements on the BAHA system: where do we stand at present?

After more than 25 years of clinical experience, the BAHA (bone-anchored hearing aid) system is a well-established treatment for hearing-impaired patients with conductive or mixed hearing loss. Owing to its success, the use of the BAHA system has spread and the indications for application have gradually become broader. New indications, as well as clinical applications, were discussed during scientific roundtable meetings in 2004 by experts in the field, and the outcomes of these discussions are presented in the form of statements. The issues that were discussed concerned BAHA surgery, the fitting range of the BAHA system, the BAHA system compared to conventional devices, bilateral application, the BAHA system in children, the BAHA system in patients with single-sided deafness, and, finally, the BAHA system in patients with unilateral conductive hearing loss.

Adult↗

PubMed2004

Bone-anchored hearing aid in unilateral inner ear deafness: a study of 20 patients.

OBJECTIVE: To evaluate the benefit of a bone-anchored hearing aid (BAHA) contralateral routing of sound (CROS) in 20 patients with unilateral inner ear deafness. SUBJECTS: 21 patients were recruited; 15 had undergone acoustic neuroma surgery and 6 patients had unilateral profound hearing loss due to other causes; 1 patient was excluded. Only patients with thresholds of better than 25 dB HL (500-2000 Hz) and an air-bone gap of less than 10 dB in the best ear were included. METHODS: Evaluation involved audiometric measurements before intervention, when fitted with a conventional CROS and after implementation and quantification of the patients' subjective benefit with a hearing aid-specific instrument: the Abbreviated Profile of Hearing Aid Benefit (APHAB). RESULTS: Lateralization scores were not significantly different from chance (50%) in any of the three conditions. Measurements of speech perception in noise showed an increase in the signal to noise ratio (S/N ratio) with the conventional CROS (p = 0.001) and with the BAHA CROS compared to the unaided condition when speech was presented at the front with noise on the poor hearing side. On the other hand, a lower S/N ratio was seen with the BAHA CROS (p = 0.003) compared to the unaided situation when noise was presented at the front with speech on the poor hearing side. The patient outcome measure (APHAB) showed improvement, particularly with the BAHA CROS. CONCLUSIONS: The poor sound localization results illustrate the inability of patients with unilateral inner ear deafness to localize sounds. The speech-in-noise measurements reflect the benefit of a BAHA CROS in lifting the head shadow while avoiding some of the disadvantages of a conventional CROS. The benefit of the BAHA CROS was most clearly reflected in the patients' opinion measured with the APHAB.

Adult↗

PubMed2004

The bone-anchored hearing aid: quality-of-life assessment.

OBJECTIVES: To assess the impact of a bone-anchored hearing aid (BAHA) on the quality of life (QOL) of adults and to test the hypothesis that a BAHA improves QOL because otorrhea and/or skin irritations decrease. DESIGN: Prospective postal-based questionnaire study using validated health-related QOL instruments, combined with hearing-aid-related questions. PATIENTS AND METHODS: The study included 56 consecutive adult patients with acquired conductive or mixed hearing loss who were scheduled for BAHA implantation at the University Medical Centre Nijmegen, Nijmegen, the Netherlands. All 56 patients completed the 36-Item Short-Form Health Survey (SF-36), the EuroQol-5D (EQ-5D), and the Hearing Handicap and Disability Inventory (HHDI); 36 patients had been using an air-conduction hearing aid (ACHA) and 20 patients a conventional bone-conduction hearing aid (CBHA). Questionnaires were filled out before surgery and after 6 months of experience with the BAHA. RESULTS: In the SF-36 group, there was significant improvement in the scores of the mental health domain (P =.02). When the SF-36 patients were classified according to previous hearing aid, there was no statistically significant change in the scores in any of the domains. In the EQ-5D group and in its ACHA and CBHA subgroups, there were no important differences in the results before and after the patients received their BAHAs. In the HHDI group, the handicap and disability scales showed significant improvement (P<.01) irrespective of the type of previously worn hearing aid. CONCLUSIONS: Overall, generic health-related QOL was not influenced significantly by the use of a BAHA according to the SF-36 and the EQ-5D. The more disease-specific scales (HHDI) did show improved QOL with a BAHA.

Adult↗

PubMed2004

Congenital malformation of the inner ear and pediatric cochlear implantation.

OBJECTIVES: To study the surgical aspects and performance outcome of cochlear implantation in children with malformed inner ears. STUDY DESIGN: Clinical and audiometric evaluation in 13 patients. METHODS: Patient data concerning surgery, postoperative follow-up, and pre- and postimplantation audiometry were obtained from the cochlear implant center's database and evaluated. A review of the literature has been included. SETTING: Tertiary referral center. PATIENTS: The patients had a variety of inner ear malformations and profound hearing loss. One patient with recurrent meningitis had a severe cochlear malformation (common cavity). RESULTS: Major complications did not occur. In one patient with an abnormal position of the cochlea and concurring middle ear disease, it was difficult to find the scala tympani during surgery. A cerebrospinal fluid gusher was encountered in two patients and an aberrant facial nerve in another, which did not lead to any complications. The patients with mild cochlear malformation such as an incomplete partition demonstrated a good performance in speech perception tests. Even the child with the common cavity deformity had some open-set speech perception 1 year after implantation. CONCLUSIONS: Viewing the patients from this study and patients from a review of the literature concerning cochlear implantation in children with malformed inner ears including severe cochlear malformations, the occurrence of an aberrant facial nerve was 17%, which increases to 27% if one reviews the surgical findings in children with severe malformed cochleae such as a common cavity or a severe cochlear hypoplasia. In the latter patients, results in speech perception vary. Although the result of cochlear implantation may be promising, as in our patient with a common cavity, during preoperative counseling the child's parents must be informed that the result is uncertain.

Audiometry↗

PubMed2004

Cochlear implantation in 53 patients with otosclerosis: demographics, computed tomographic scanning, surgery, and complications.

OBJECTIVES: To collect data from a large number of cochlear implant recipients with otosclerosis and to make an assessment of these patients' clinical characteristics, computed tomographic scans, surgical findings, and complications, and to quantify the occurrence of postoperative facial nerve stimulation. STUDY DESIGN: Retrospective multicenter study. PATIENTS: Fifty-three patients with otosclerosis from four cochlear implant centers in the United Kingdom and The Netherlands were reviewed. Sixty surgical procedures were performed in these patients: 57 devices were placed in 56 ears. RESULTS: The computed tomographic imaging demonstrated retrofenestral (cochlear) otosclerotic lesions in the majority of patients. Although not statistically significant, the extent of otosclerotic lesions on the computed tomographic scan as categorized in three types tends to be greater in patients with rapidly progressive hearing loss, in patients in whom there is surgically problematic insertion of the electrode array, and in patients with facial nerve stimulation. In four patients, revision surgery had to be performed. Twenty of 53 (38%) patients experienced facial nerve stimulation at various periods postoperatively. CONCLUSION: Cochlear implant surgery in patients with otosclerosis can be challenging, with a relatively high number of partial insertions and misplacements of the electrode array demanding revision surgery. A very high proportion of patients experienced facial nerve stimulation mainly caused by the distal electrodes. This must be discussed with patients preoperatively.

Adult↗

PubMed2003

Bone-anchored hearing aids in unilateral inner ear deafness.

In nine patients with unilateral deafness and normal hearing in the contralateral ear, measurements of sound localization and speech perception were obtained before intervention, with a conventional contralateral routing of sound (CROS) hearing aid and later with a bone-anchored hearing aid (BAHA) implanted in the deaf ear. Sound localization did not show any differences between the three conditions. Speech perception using short, everyday sentences showed a reduction in the head-shadow effect of 2 dB for both the conventional CROS hearing aid and the BAHA in comparison to the unaided condition. Patients' real-life experiences of the three conditions were evaluated using the Abbreviated Profile of Hearing Aid Benefit (APHAB) questionnaire. The results showed a significant benefit with the BAHA in situations involving background noise and reverberation and a reduced aversion to loud sounds in comparison to the unaided and conventional CROS conditions.

Audiometry, Speech↗