{"title":"Cochlear implant in Wolfram syndrome: A case report.","authors":"Hetal Marfatia, Anav Rattan, Anushka Jain","doi":"10.1080/14670100.2024.2442826","DOIUrl":"10.1080/14670100.2024.2442826","url":null,"abstract":"<p><strong>Introduction: </strong>Wolfram syndrome, a rare autosomal recessive disorder, is characterised by diabetes insipidus, juvenile diabetes mellitus, optic nerve atrophy and deafness (DIDMOAD).</p><p><strong>Case report: </strong>We present a case of a 21-year-old male diagnosed with Wolfram syndrome who underwent cochlear implantation due to progressive hearing loss. The patient first complained of bilateral hearing loss at the age of 8 years. As the hearing loss progressed hearing aids provided minimal benefit. A multidisciplinary team evaluated his extensive medical history, which included juvenile-onset diabetes mellitus, seizures, vision abnormalities and hypergonadotrophic hypogonadism. Pure tone audiometry was done which showed sloping bilateral severe to profound hearing loss, more at higher frequencies. Hrct and Mri temporal bone showed normal cochlear architecture and cochlear nerve. The patient received a Nucleus Profile™ CI 632 cochlear implant. Postoperative evaluation revealed significant improvement, with a speech discrimination score of 90% at the most comfortable level three months post-implantation.</p><p><strong>Conclusion: </strong>Wolfram syndrome is a rare genetic disorder with multisystem involvement and debilitating symptoms. High-frequency sensorineural hearing loss is a common association and hearing rehabilitation using hearing aids and cochlear implants must be considered to improve the quality of life.</p>","PeriodicalId":53553,"journal":{"name":"COCHLEAR IMPLANTS INTERNATIONAL","volume":" ","pages":"487-491"},"PeriodicalIF":1.4,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142883624","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Beata Diosi, Zsofia Bere, Angela Csomor, Tamara Tapai, Zoltan Toth, Edina Kovacs, Balint Posta, Miklos Csanady, Antal Nogradi, Zsigmond Tamas Kincses, Laszlo Rovo, Adam Perenyi
{"title":"Artefacts on magnetic resonance imaging with Osia®2 bone conduction hearing aid: A cadaver study.","authors":"Beata Diosi, Zsofia Bere, Angela Csomor, Tamara Tapai, Zoltan Toth, Edina Kovacs, Balint Posta, Miklos Csanady, Antal Nogradi, Zsigmond Tamas Kincses, Laszlo Rovo, Adam Perenyi","doi":"10.1080/14670100.2024.2398833","DOIUrl":"10.1080/14670100.2024.2398833","url":null,"abstract":"<p><strong>Objectives: </strong>Hearing implants often limit the assessment of magnetic resonance examinations due to susceptibility artefacts. Our aim was to evaluate the impact of artefacts attributed to the Osia®2 implant system in terms of utility in visualizing selected cranial structures.</p><p><strong>Methods: </strong>A BI300 implant and an OSI200 actuator were implanted into a human cadaver head in the audiologically most favourable position according to the manufacturer's guidelines. Scanning was accomplished using the institutional <i>head and inner ear</i> protocol with a General Electric 1.5 Tesla scanner with retained and removed implant magnet, extended with T1 and T2 weighted sequences with metal-artefact reduction (MAVRIC SL). Image quality was evaluated by three radiologists.</p><p><strong>Results: </strong>The Osia®2 produced significant artefacts in most of the series of standard imaging sequences predominantly on the ipsilateral side of the head. The majority of the artefacts were caused by the implant magnet. Even without removing the magnet, MAVRIC SL improved image quality to such an extent that it became comparable with that after magnet removal.</p><p><strong>Conclusions: </strong>The standard sequences suffer considerable quality loss due to the artefacts, attributed predominantly to the magnetic component. Metal-artefact reduction sequences are effective in obtaining sufficient-to-good quality images without surgical magnet removal.</p>","PeriodicalId":53553,"journal":{"name":"COCHLEAR IMPLANTS INTERNATIONAL","volume":" ","pages":"434-444"},"PeriodicalIF":1.4,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142480595","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Guillaume Lloret, Christophe Vincent, Michaël Risoud, Cyril Beck, Pierre Emmanuel Lemesre, Christian Renard, Jérôme André, Philippine Toulemonde
{"title":"Evaluation of a personalized auditory-cognitive training on the improvement of speech understanding in noise in cochlear implanted patients.","authors":"Guillaume Lloret, Christophe Vincent, Michaël Risoud, Cyril Beck, Pierre Emmanuel Lemesre, Christian Renard, Jérôme André, Philippine Toulemonde","doi":"10.1080/14670100.2024.2441612","DOIUrl":"10.1080/14670100.2024.2441612","url":null,"abstract":"<p><strong>Objective: </strong>The cochlear implant is a commonly used implantable device for the auditory rehabilitation of severe bilateral sensorineural hearing loss. The effectiveness of the implant, depends on many factors, including intensive auditory training, which is crucial. Intelligibility in a noisy environment is a current issue and poses a major difficulty for implanted patients. The aim of this study is to evaluate the improvement in auditory performance in noise among cochlear implant patients who underwent personalized auditory-cognitive training for speech understanding tasks in noise.</p><p><strong>Design: </strong>This was a prospective study involving cochlear implanted patients divided into two groups. One group underwent auditory training in a noisy environment at home for 2 months (G1) while the other group served as a control (G0). A test of intelligibility performance in noise was conducted at inclusion and two months later.</p><p><strong>Results: </strong>52 patients were included in the study. The trained group, G1, showed a significant improvement with an increase of 4.8 dB in signal-to-noise ratio (SNR) between the two tests (P < 0.01). There was no significant improvement in the control group (G0) (P = 0.756).</p><p><strong>Conclusions: </strong>This study demonstrated a significantly positive impact of personalized auditory training in a noisy environment for cochlear implant patients.</p>","PeriodicalId":53553,"journal":{"name":"COCHLEAR IMPLANTS INTERNATIONAL","volume":" ","pages":"467-476"},"PeriodicalIF":1.4,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142900382","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Teresa Y C Ching, Vicky Zhang, Esti Nel, Sanna Hou, Paola Incerti, Anke Plasmans
{"title":"Effects of automatic auditory scene classification on speech perception in noise and real-world functional communication in children using cochlear implants.","authors":"Teresa Y C Ching, Vicky Zhang, Esti Nel, Sanna Hou, Paola Incerti, Anke Plasmans","doi":"10.1080/14670100.2024.2415193","DOIUrl":"10.1080/14670100.2024.2415193","url":null,"abstract":"<p><strong>Objectives: </strong>To investigate the effects of automatic scene classification (SCAN) on speech perception in noise and real-world functional performance in children using cochlear implants (CIs).</p><p><strong>Methods: </strong>We used a within-subjects repeated measures design in two studies. The first study assessed speech perception in noise with or without SCAN enabled in 25 school-aged children. The second study evaluated functional auditory performance in real life. Parents of 18 children provided ratings using the Parents' Evaluation of Aural/oral Performance of Children (PEACH) questionnaire; and children provided ratings using the Self Evaluation of Listening Function (SELF) questionnaire. Analyses of variance with repeated measures were used to examine the effect of SCAN.</p><p><strong>Results: </strong>On average, speech perception in noise was significantly better with SCAN enabled (mean SRT: -4.1 dB; SD: 4.0), compared to SCAN disabled (mean SRT: 0.5 dB; SD: 3.5). Children's functional performance in real life was similar between the two device settings.</p><p><strong>Conclusion: </strong>Automatic auditory scene classification provides significant benefits for speech perception in noise (4.6 dB improvement). On average, there were no perceived detrimental or beneficial effects in real life. These findings support the use of SCAN in CIs for young children.</p>","PeriodicalId":53553,"journal":{"name":"COCHLEAR IMPLANTS INTERNATIONAL","volume":" ","pages":"422-433"},"PeriodicalIF":1.4,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11952983/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142480597","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Christina Batthyany, Marc van der Schroeff, Jantien Vroegop
{"title":"Translation and validation of the Hearing Environments and Reflection on Quality of Life (HEAR-QL) questionnaire for preschool children in Dutch.","authors":"Christina Batthyany, Marc van der Schroeff, Jantien Vroegop","doi":"10.1080/14670100.2024.2440977","DOIUrl":"10.1080/14670100.2024.2440977","url":null,"abstract":"<p><strong>Objective: </strong>Early diagnosis, intervention and consistent follow-up of hearing loss is of great importance in children, given the broad impact of untreated childhood hearing loss. Currently, no hearing-specific QOL proxy questionnaire exists for preschool children with hearing loss in the Dutch language. Therefore, the aim of this study was to translate and validate the Preschool HEAR-QL questionnaire into Dutch.</p><p><strong>Methods: </strong>Translation was conducted according the forward-backward procedure. Parents or caregivers of 60 normal-hearing children and 50 children with bilateral hearing loss aged 2-6 years old were invited to digitally complete the questionnaire.</p><p><strong>Results: </strong>The Preschool HEAR-QL questionnaire showed good discriminant validity between the normal-hearing and the hearing loss group, for its total score and some subscales. We concluded satisfying internal consistency given Cronbach's alpha values being above 0.70, nearly all alpha if item deleted values approaching the overall values, and item-subscale correlations above 0.30. Test-retest reliability was satisfying with all intraclass correlation coefficients being greater than 0.70.</p><p><strong>Conclusion: </strong>This Dutch hearing-specific QOL proxy questionnaire can play an important role in the management and follow-up of early childhood hearing loss in Dutch health care and hearing centers. Future research on clinical data will further verify its utility in clinical practice.</p>","PeriodicalId":53553,"journal":{"name":"COCHLEAR IMPLANTS INTERNATIONAL","volume":" ","pages":"459-466"},"PeriodicalIF":1.4,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143069485","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Silicone allergy can lead to cochlear implant complication and explantation: a case report.","authors":"Syed Ahsan","doi":"10.1080/14670100.2024.2413265","DOIUrl":"10.1080/14670100.2024.2413265","url":null,"abstract":"<p><strong>Introduction: </strong>We report an unusual case of cochlear implant complication and eventual explantation secondary to an allergic reaction to silicone.</p><p><strong>Case report: </strong>A 62-year-old man who underwent cochlear implantation for asymmetric hearing loss developed an allergic response to the implant within a few weeks of the surgery. This led to subsequent explantation. Patch testing using the various parts of the implant revealed a hypersensitive response to the silicone component of the receiver stimulator. There have only been 6 reported cases of cochlear implant explantation secondary to allergic reactions to cochlear implants. The inflammatory reaction has been to the silicone component of the cochlear implant found in the silicone used in Cochlear America's implants.</p><p><strong>Conclusion: </strong>Although rare, it's important to be aware of delayed hypersensitivity reactions to the silicone component of a cochlear implant. Attention should be made to associate symptoms such as pruritis, urticaria and loss of hair as symptoms and signs of possible allergic reaction to the implanted component. Steroids may help to alleviate symptoms; however, symptoms have been shown to recur after cessation of steroids. Treatment requires the removal of the device.</p>","PeriodicalId":53553,"journal":{"name":"COCHLEAR IMPLANTS INTERNATIONAL","volume":" ","pages":"492-495"},"PeriodicalIF":1.4,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142480598","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Vanessa Y J Tan, Rodney Hollow, Sylvia Tari, Alex Rousset, Raoul Wills, Robert J S Briggs, Richard Charles Dowell
{"title":"Cochlear implant usage in single sided deafness and factors affecting usage.","authors":"Vanessa Y J Tan, Rodney Hollow, Sylvia Tari, Alex Rousset, Raoul Wills, Robert J S Briggs, Richard Charles Dowell","doi":"10.1080/14670100.2024.2403224","DOIUrl":"10.1080/14670100.2024.2403224","url":null,"abstract":"<p><strong>Objectives: </strong>The primary objective was to examine duration of daily cochlear implant (CI) usage at 12 and 24 months post-operatively in single sided deafness (SSD). The secondary objective was to examine factors that could affect CI usage.</p><p><strong>Methods: </strong>Retrospective cohort of patients with SSD who received CI from January 2015 to March 2020. CI usage was evaluated at 12 and 24 months. Hearing loss duration, tinnitus scores and signal-to-noise ratio (SNR) were correlated with CI usage at 12 months.</p><p><strong>Results: </strong>Usage data was available for 54 patients at 12 months and 38 patients at 24 months. The mean usage was 8.2 h/day (SD 4.2) at 12 months, 7.0 h/day (SD 5.1) at 24 months. 5 out of 54 (9.3%) and 7 out of 38 patients (18.4%) were non-users at 12 and 24 months post-operatively. An improved mean SNR score from pre-operative 4.4 dB (SD 2.8) to - 0.70 dB (SD 4.2) at 12 months post-operative was positively correlated with CI usage at 12 and 24 months. Hearing loss duration and tinnitus scores were not associated with CI usage.</p><p><strong>Conclusions: </strong>18.4% of our patients were non-users at 24 months. Mean usage at 24 months was 7.0 h/day. Improved hearing in noise at 12 months was correlated with better usage.</p>","PeriodicalId":53553,"journal":{"name":"COCHLEAR IMPLANTS INTERNATIONAL","volume":" ","pages":"387-393"},"PeriodicalIF":1.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142395234","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Amy H C Ng, Jessica Banh, Laura Shaw, Yasmeen Aboulhawa, Maria Simone, Trung Le, Vincent Lin, Joseph Chen
{"title":"Comparing remote programming of cochlear implants using two methods: portable laptop and remote hosted site.","authors":"Amy H C Ng, Jessica Banh, Laura Shaw, Yasmeen Aboulhawa, Maria Simone, Trung Le, Vincent Lin, Joseph Chen","doi":"10.1080/14670100.2024.2427507","DOIUrl":"10.1080/14670100.2024.2427507","url":null,"abstract":"<p><strong>Objectives: </strong>Compare two remote programming methods as a clinical service for user satisfaction, ease of use, preparation time and accessibility.</p><p><strong>Method: </strong>Method 1 (Portable Laptop): A 'Programming Kit' including laptop was shipped to cochlear implant users' homes (<i>N</i> = 20). The audiologist at the implant center used remote desktop control of this laptop to adjust subjects' speech processors. Method 2 (Remote Hosted Site): Eight distant clinics were recruited as host sites to house cochlear implant programming hardware and software so that CI users (<i>N</i> = 19) could attend their facility. The audiologist at the implant center used remote desktop control of the host sites' computers to adjust the subjects' CI speech processors. All parties were asked to fill out a questionnaire following their remote session.</p><p><strong>Results: </strong>Remote hosted site method was rated higher for ease of use by the Remote Experts (12/15, 80%), compared to portable laptop method (11/19, 57.9%) and is more accessible to CI users of all levels of computer abilities while requiring less preparation time per session.</p><p><strong>Conclusion: </strong>Remote hosted site method is an easier, more efficient method of remote programming as a clinical service delivery method compared to the Portable Laptop.</p>","PeriodicalId":53553,"journal":{"name":"COCHLEAR IMPLANTS INTERNATIONAL","volume":" ","pages":"360-372"},"PeriodicalIF":1.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142734264","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Nicole T Jiam, Archana Podury, Alicia M Quesnel, Ophir Handzel
{"title":"Worldwide differences in surgeon intraoperative practices for cochlear implantation.","authors":"Nicole T Jiam, Archana Podury, Alicia M Quesnel, Ophir Handzel","doi":"10.1080/14670100.2024.2367309","DOIUrl":"10.1080/14670100.2024.2367309","url":null,"abstract":"<p><strong>Objective: </strong>To characterize practice patterns of intraoperative imaging and/or functional confirmation of cochlear implant electrode location worldwide.</p><p><strong>Methods: </strong>A cross-sectional survey of otolaryngologists performing cochlear implantation was conducted between March 1 and May 6, 2023. Participants were recruited worldwide using an international otologic society membership email list and at professional meetings. Ninety-seven of the 125 invited participants (78%) completed the survey. Participants were categorized by continent.</p><p><strong>Results: </strong>North American surgeons use intraoperative X-rays more frequently than surgeons in Europe and Asia (<i>p</i> < 0.001). Otolaryngologists in Europe and Asia more frequently use no intraoperative imaging (<i>p</i> = 0.02). There is no regional difference between the intraoperative use of electrophysiologic instruments. European and Asian surgeons implant MED-EL devices (<i>p</i> = 0.012) more frequently than North American surgeons, who more frequently use Cochlear Corporation devices (<i>p</i> = 0.003). MED-EL use is related to less frequent intraoperative X-ray use (<i>p</i> = 0.02). Advanced Bionics use is related to more frequent intraoperative CT use (<i>p</i> = 0.03). No significant association existed between years of practice, number of cochlear implantation surgeries performed yearly, volume of pediatric CI practice, and use of intraoperative tools.</p><p><strong>Conclusion: </strong>Intraoperative practice for radiologic and functional verification of cochlear implant electrode positioning varies worldwide. Practice guidelines may help establish a standard of care for cochlear implantation.</p>","PeriodicalId":53553,"journal":{"name":"COCHLEAR IMPLANTS INTERNATIONAL","volume":" ","pages":"344-351"},"PeriodicalIF":1.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141472579","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Reading achievement and deaf students with cochlear implants.","authors":"Connie Mayer, Beverly J Trezek","doi":"10.1080/14670100.2024.2394313","DOIUrl":"10.1080/14670100.2024.2394313","url":null,"abstract":"<p><strong>Objectives: </strong>The purpose of this study was to investigate the reading outcomes of a Canadian cohort of school-aged deaf learners with cochlear implants (CIs). The goal was to investigate whether achievement approached that of hearing age peers and identify demographic factors influencing performance.</p><p><strong>Methods: </strong>Participants represent a subset of 13 students with CIs from a larger sample of 70 deaf students in grades four through 12 educated in inclusive settings within a large school board in central Canada. Data sources included demographic information, teachers' ratings on the Categories of Auditory Performance (CAP), and scores from the <i>Woodcock-Johnson III Diagnostic Reading Battery</i> [WJ III-DRB].Results/Discussion: Participants performed within the low average range in all areas except for Phonological Awareness, which was in the low range; however, there was wide variability in scores across participants. None of the demographic variables (e.g. home language, additional disabilities) had a statistically significant association with performance, although older students had higher mean scores on the Phonological Awareness cluster.</p><p><strong>Conclusion: </strong>These findings add to the body of research on literacy achievement and cochlear implantation, providing evidence that this technology has a significant positive effect on outcomes for a population that has heretofore underperformed in this area.</p>","PeriodicalId":53553,"journal":{"name":"COCHLEAR IMPLANTS INTERNATIONAL","volume":" ","pages":"394-402"},"PeriodicalIF":1.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142086399","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}