Emmanuel Mylanus - Academia.edu (original) (raw)

Papers by Emmanuel Mylanus

Otology & Neurotology, Oct 1, 2016

Objective: Identifying aspects for establishing cochlear implantation guidelines for patients wit... more Objective: Identifying aspects for establishing cochlear implantation guidelines for patients with ocular coloboma, heart defects, atresia of the choanae, retardation (of growth and/or of development), genital anomalies, and ear anomalies (CHARGE) syndrome (CS). Study Design: Explorative retrospective study. Setting: Cochlear implant (CI)-centers of tertiary referral centers in The Netherlands. Patients: Ten patients with CS who received a CI between 2002 and 2012. Interventions: Describing the challenges and benefits of cochlear implantation in CS. Main Outcome Measures: Imaging and surgical findings, language development, and Quality-of-life (QoL), compared with two control groups: 1) 34 non-syndromic CI-users and 2) 13 patients with CS without CI because of sufficient hearing. Results: Subjective and objective audiometry and magnetic resonance imaging were necessary to confirm the presence of the cochlear nerve. Surgery in CS was challenging because of enlarged emissary veins, semi-circular-canal aplasia, aberrant facial nerve, and dysplastic cochlear

Clinical Otolaryngology, Nov 6, 2017

Funding information Cochlear Objectives: Performance of a superpower bone-anchored hearing aid (B... more Funding information Cochlear Objectives: Performance of a superpower bone-anchored hearing aid (Baha), the Baha Cordelle from Cochlear Bone-Anchored Solutions (BCD1), was compared to its successor, the Baha 5 SuperPower (BCD2). Design: A comparative study in which each patient served as its own control. Setting: Tertiary clinic. Participants: Ten experienced BCD1 users with profound mixed hearing loss. For comparison, data from another study with 10 experienced users with a severe mixed hearing loss using a Cochlear Baha 5 power sound processor (BCD-P) were included. Main outcome measures: Speech reception thresholds in noise and APHAB and SSQ questionnaires. Results: Speech reception thresholds for the digits-in-noise (DIN) test were significantly lower (P < 5%), that is more favourable, for BCD2 in the speech and noise frontal condition and in the speech frontal and noise contralateral condition than for BCD1. For the group with severe mixed loss fitted with BCD-P, the SRTs were not significantly different (P > 5%) from the BCD2 values. With the APHAB questionnaire scores were significantly lower, that is more favourable, for the ease of communication (P < 5%) and the background noise (P < 1%) domains for BCD2 than for BCD1. APHAB scores for the aversiveness of loud sounds domain were not significantly different for both devices (P > 5%). Scores for the speech and quality domains of the SSQ questionnaire were significantly higher, that is more favourable, for BCD2 than for BCD1. APHAB and SSQ scores for BCD-P were not significantly different from those for BCD2 (P > 5%). Conclusions: Data for BCD2 in profound mixed loss are similar to those for BCD-P and a severe mixed loss. Of 10 patients, 2 expressed a strong preference for BCD2 over BCD1, and 7 patients had a preference for BCD2 over BCD1. One patient preferred BCD1 because of its built-in telecoil facility. 1 | INTRODUCTION Patients with profound hearing loss may experience problems with air-conduction hearing aids due to tightly fitted ear moulds and/or maximum gain restrictions by acoustic feedback. In profound mixed hearing loss consisting of a moderate sensorineural loss and a large air-bone gap, a powerful direct-drive bone-conduction device (BCD) is a viable alternative for a conventional hearing aid, owing to the relatively favourable bone-conduction thresholds. In essence, a percutaneous BCD system consists of a titanium implant anchored in the temporal bone with a skin-penetrating titanium abutment, and a sound processor. The sound processor converts

Otology & Neurotology, Aug 1, 2006

Objective: To evaluate whether the bone-anchored hearing aid (BAHA) can be applied successfully t... more 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.

International Journal of Audiology, 2009

Audiometric characteristics of a recently introduced more powerful, behind-the-ear, BAHA sound pr... more Audiometric characteristics of a recently introduced more powerful, behind-the-ear, BAHA sound processor, the BAHA Intenso, were evaluated in 23 patients with contra-indications for using conventional hearing aids. All patients had mixed hearing loss with boneconduction thresholds ranging between 30 and 50 dB HL. Boneconduction gain, defined as the difference in aided free-field detection thresholds and unaided boneconduction thresholds, was calculated at the octave frequencies between 500 Hz and 4 kHz. Median boneconduction gain of the Intenso ranges from 0 dB at 500 ;Hz to 12 ;dB at 2 kHz with substantial interindividual variability. The upper limit of the BAHA Intenso&amp;amp;amp;amp;amp;amp;amp;#39;s fitting range was established by requiring aided speech reception thresholds with CVC-monosyllables of at most 60 dB SPL. The fitting range of the BAHA Intenso appeared to be limited to 42, 44, 58, and 48 dB HL for boneconduction thresholds at 0.5, 1, 2, and 4 ;kHz, respectively. Loudness growth functions at 0.5 and 3 kHz as obtained with 7-point categorical scaling showed an adequate aided dynamic range.

International Journal of Audiology, 2008

In 1984 the Bone-Anchored Hearing Aid, or BAHA, system was introduced. Its transducer is coupled ... more In 1984 the Bone-Anchored Hearing Aid, or BAHA, system was introduced. Its transducer is coupled directly to the skull percutaneously to form a highly effective bone-conduction hearing device. Clinical studies on adults with conductive hearing loss have shown that the BAHA system outperforms conventional bone-conduction hearing aids. Therefore, the next step was to apply the BAHA system in children with congenital or acquired conductive hearing loss. Reviewed data showed that, on average, such children benefited significantly more from the BAHA than from reconstructive surgery. Thus, BAHA application appears to be the best option to achieve normal communication and speech and language development in children with bilateral conductive hearing loss. However, in children under the age of three to four years, a conventional solution must be applied, e.g. a bone conductor with a transcutaneous coupling, because they are too young to undergo BAHA implant surgery. In the case of unilateral congenital conductive hearing loss, there is no convincing evidence in the clinical literature for early intervention. In summary, the BAHA system can be considered a new, indispensable tool for children with bilateral conductive hearing loss.

Otology & Neurotology, Apr 1, 2008

Objective: To study whether unilateral Bone-anchored Hearing Aid (BAHA) fitting led to subjective... more Objective: To study whether unilateral Bone-anchored Hearing Aid (BAHA) fitting led to subjective hearing benefit in patients with congenital unilateral conductive hearing impairment. Study Design: Prospective evaluation on 20 patients. Setting: Tertiary referral center. Patients: Ten adults and 10 children with congenital unilateral conductive hearing impairment, with a mean air-bone gap of 50 dB, were included. Methods: Subjective bilateral hearing benefit after BAHA fitting was measured using 2 disability-specific questionnaires: Chung and Stephens and the Speech, Spatial and Qualities of hearing profile (children_s version in the patients aged G18 yr). The Glasgow children_s benefit inventory was also used to measure patient_s health benefit after BAHA fitting. Results: Chung and Stephens_ questionnaire showed an overall preference for the BAHA in several specific hearing situations.

Laryngoscope, May 1, 2006

Objective: The objective of this retrospective study was to evaluate the benefit and performance ... more 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.

Otolaryngologic Clinics of North America, Feb 1, 1995

Laryngoscope, Sep 1, 2008

Objectives/Hypothesis: To define audiological application criteria for different implantable hear... more Objectives/Hypothesis: To define audiological application criteria for different implantable hearing aid devices. Study Design: Retrospective study. Methods: Comparisons were made between aided speech recognition scores obtained at conversational level (65 dB) in patients with the Vibrant Soundbridge (VSB) (n ϭ 22), the Otologics middle ear transducer (MET) (n ϭ 10), conventional hearing aids (behind-the-ears) (n ϭ 47), and cochlear implants (CIs) (n ϭ 123). Results: In relation to hearing loss, only for mild hearing loss, speech recognition scores with VSB were comparable to that with conventional hearing aids. In the Otologics MET users, speech recognition scores were comparable with those of the conventional hearing aid users until a mean hearing loss of about 75 dB HL. At a sensorineural hearing loss of about 65 dB HL or more, the Otologics MET users have better speech recognition scores than the VSB users. For comparison with CI users, we followed a more conservative approach. In 90% of the users of a CI, speech recognition scores were better than those in: 1) patients with a conventional hearing aid and a mean hearing loss of about 95 dB HL or worse; 2) patients with an Otologics MET and a mean hearing loss of 85 dB HL or worse. Conclusions: Patients fitted with a VSB or an Otologics MET middle ear implant do not demonstrate better speech recognition scores than patients fitted with today's conventional hearing aids. Results might even been worse. However, the VSB and Otologics MET are a good option in patients with moderate (VSB) to severe (Otologics MET) sensorineural hearing loss and external otitis.

Clinical Otolaryngology, 1997

Clinical Otolaryngology, Feb 1, 2013

Sir, Colquitt et al.'s 1 comprehensive review of bone-anchored hearing aids (BAHAs) emphasised th... more Sir, Colquitt et al.'s 1 comprehensive review of bone-anchored hearing aids (BAHAs) emphasised the overall lack of evidence on their effect on the quality of life experienced by patients (QoL). They reported just one relevant study 2 that assessed QoL using validated generic instruments, the SF-36 and the EQ-5D. The SF-36 has 8 scales (physical functioning, physical limitation, bodily pain, general health, vitality, social functioning, emotional limitation, and mental health), each of which are measured on a scale from 0 to 100. The EQ-5D has one overall scale, measured from 0 to 1, which is derived from five ques

Nederlands Tijdschrift voor Geneeskunde, 2000

Audiology and Neuro-otology, Aug 19, 2009

sentence scores between the poor and good performers. The two groups did not differ in age at ons... more sentence scores between the poor and good performers. The two groups did not differ in age at onset of hearing loss, duration of hearing loss, progression, age at onset of deafness, or duration of deafness. Conclusions: The clinical presentation of the otosclerosis (rapid or slow progression) did not influence speech perception. Better performance was related to less severe signs of otosclerosis on CT scan, full insertion of the electrode array, little or no facial nerve stimulation and little or no need to switch off electrodes.

European Archives of Oto-rhino-laryngology, Apr 4, 2009

In Fig. 2 as it is printed in the article, an indication arrow is pointing to a location that doe... more In Fig. 2 as it is printed in the article, an indication arrow is pointing to a location that does not correspond with the location of the cochlea. The correct Fig. 2 is given here.

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Otology & Neurotology, Sep 1, 2009

To study age-related patient satisfaction with the bone-anchored hearing aid (BAHA) compact. A re... more To study age-related patient satisfaction with the bone-anchored hearing aid (BAHA) compact. A retrospective postal questionnaire, the International Outcome Inventory for Hearing Aids (IOI-HA), was sent to 211 BAHA Compact users. Questionnaire responses from 135 BAHA users were analyzed related to age, sex, years of BAHA experience, and the hearing thresholds (pure-tone average) at the aided side. Age ranged from 18 to 77 years. The IOI-HA showed that the BAHA Compact was greatly appreciated by almost all of the users: most patients stated that they were using the device for most of the day; it helped them to hear better and it reduced the number of situations in which hearing impairment was problematical. The cumulative score on the questionnaire was negatively influenced by age (rho = -0.191, p = 0.05). Furthermore, increase in sensorineural hearing loss (SNHL) component was associated with decrease in total IOI-HA scores (Spearman rho = -0.193, p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.05). A significant correlation was found between age and the SNHL component (Spearman rho = 0.525, p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.001).There were no significant differences in the levels of difficulty with placing the BAHA on the implant or with handling the BAHA between the age groups. Cleaning the skin around the implant causes the most difficulties in the youngest age group (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.02). The BAHA Compact enhances participation in various domains of communication. Differences in patients&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39; satisfaction seemed to be correlated with the SNHL component rather than age.

International Journal of Pediatric Otorhinolaryngology, Jun 1, 2017

Existing literature only reports a few patients with Noonan syndrome (NS) and Noonan syndrome wit... more Existing literature only reports a few patients with Noonan syndrome (NS) and Noonan syndrome with multiple lentigines (NSML) who underwent cochlear implantation (CI). The present study describes four NS patients and one NSML patient with a PTPN11 mutation. They all had severe to profound hearing loss, and they received a CI. The age at which the CI surgery occurred ranged from 1 to 13 years old, and the audiological results in all five patients improved after the CI. Otological and audiological examinations in NS and NSML are important, and for those with severe hearing loss, the CI surgery improved the audiological outcome regardless of age.

International Journal of Rehabilitation Research, Dec 1, 2005

Patients with Usher syndrome face a special set of challenges in order to maintain their independ... more 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.

Annals of Otology, Rhinology, and Laryngology, Jul 1, 2010

We performed an evaluation of the audiological and subjective benefits of the bone-anchored heari... more We performed an evaluation of the audiological and subjective benefits of the bone-anchored hearing aid (Baha) as a device for transcranial routing of sound (Baha CROS) in 56 patients with unilateral inner ear deafness. We performed a prospective clinical follow-up study in a tertiary referral center. Previously reported results of 29 patients were supplemented with a second series of 30 patients with unilateral inner ear deafness; 3 patients dropped out during the evaluation. Audiometric measurements were taken before and after Baha CROS fitting. Subjective benefits were quantified with 4 different patient questionnaires. The sound localization results in a well-structured test setting were not differentiable from chance. The 5 patients with congenital hearing loss showed better scores in the unaided sound localization measurements. Overall, most patients reported some subjective improvement in their capacity to localize sounds with the Baha CROS in daily life. The main effect of the Baha CROS was to alleviate the head shadow effect during the speech-in-noise test. Poor sound localization in this larger series of patients confirms the findings of previous studies. Improvements in the speech-in-noise scores corroborated the efficacy of the Baha CROS in alleviating the head shadow effect. The 4 different patient questionnaires revealed subjective benefit and satisfaction in various domains.

Audiology Research, 2021

Electrically evoked auditory potentials have been used to predict auditory thresholds in patients... more Electrically evoked auditory potentials have been used to predict auditory thresholds in patients with a cochlear implant (CI). However, with exception of electrically evoked compound action potentials (eCAP), conventional extracorporeal EEG recording devices are still needed. Until now, built-in (intracorporeal) back-telemetry options are limited to eCAPs. Intracorporeal recording of auditory responses beyond the cochlea is still lacking. This study describes the feasibility of obtaining longer latency cortical responses by concatenating interleaved short recording time windows used for eCAP recordings. Extracochlear reference electrodes were dedicated to record cortical responses, while intracochlear electrodes were used for stimulation, enabling intracorporeal telemetry (i.e., without an EEG device) to assess higher cortical processing in CI recipients. Simultaneous extra- and intra-corporeal recordings showed that it is feasible to obtain intracorporeal slow vertex potentials wi...