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Hearing Disorders: HELP
Articles by Blake Croll Papsin
Based on 69 articles published since 2010
(Why 69 articles?)
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Between 2010 and 2020, B. Papsin wrote the following 69 articles about Hearing Disorders.
 
+ Citations + Abstracts
Pages: 1 · 2 · 3
1 Editorial Editorial. 2013

Szyfter, Witold / Papsin, Blake C. · ·Audiol Neurootol · Pubmed #24335001.

ABSTRACT: -- No abstract --

2 Review Binaural integration: a challenge to overcome for children with hearing loss. 2017

Gordon, Karen A / Cushing, Sharon L / Easwar, Vijayalakshmi / Polonenko, Melissa J / Papsin, Blake C. ·Archie's Cochlear Implant Laboratory, The Hospital for Sick Children, Toronto, Ontario, Canada. ·Curr Opin Otolaryngol Head Neck Surg · Pubmed #29049038.

ABSTRACT: PURPOSE OF REVIEW: Access to bilateral hearing can be provided to children with hearing loss by fitting appropriate hearing devices to each affected ear. It is not clear, however, that bilateral input is properly integrated through hearing devices to promote binaural hearing. In the present review, we examine evidence indicating that abnormal binaural hearing continues to be a challenge for children with hearing loss despite early access to bilateral input. RECENT FINDINGS: Behavioral responses and electrophysiological data in children, combined with data from developing animal models, reveal that deafness in early life disrupts binaural hearing and that present hearing devices are unable to reverse these changes and/or promote expected development. Possible limitations of hearing devices include mismatches in binaural place, level, and timing of stimulation. Such mismatches could be common in children with hearing loss. One potential solution is to modify present device fitting beyond providing audibility to each ear by implementing binaural fitting targets. SUMMARY: Efforts to better integrate bilateral input could improve spatial hearing in children with hearing loss.

3 Review Auditory neuropathy spectrum disorder (ANSD) and cochlear implantation. 2015

Harrison, Robert V / Gordon, Karen A / Papsin, Blake C / Negandhi, Jaina / James, Adrian L. ·Department of Otolaryngology - HNS, Program in Neuroscience and Mental Health, The Hospital for Sick Children, 555 University Ave, Toronto, Ontario, Canada M5G 1X8; Department of Otolaryngology - Head and Neck Surgery, University of Toronto, 190 Elizabeth Street, Toronto, Ontario, Canada MG5 2N2. Electronic address: rvh@sickkids.ca. · Department of Otolaryngology - HNS, Program in Neuroscience and Mental Health, The Hospital for Sick Children, 555 University Ave, Toronto, Ontario, Canada M5G 1X8; Department of Otolaryngology - Head and Neck Surgery, University of Toronto, 190 Elizabeth Street, Toronto, Ontario, Canada MG5 2N2. · Department of Otolaryngology - HNS, Program in Neuroscience and Mental Health, The Hospital for Sick Children, 555 University Ave, Toronto, Ontario, Canada M5G 1X8. ·Int J Pediatr Otorhinolaryngol · Pubmed #26545793.

ABSTRACT: We discuss issues related to cochlear implantation in children with auditory neuropathy spectrum disorder (ANSD). We describe the varied nature of this disease category including the numerous potential causes of auditory neuropathy. The most prevalent etiology for infants with ANSD is associated with prolonged neonatal intensive care unit (NICU) stay. We discuss the potential contribution of cochlear hypoxia to this etiology. The second part of this review describes in detail our own experience at the Hospital for Sick Children in Toronto, with cochlear implantation of children diagnosed with ANSD. We outline the detection, diagnosis, and referral routes for our patients. We provide an overview of our "standard operation procedures" regarding candidacy, and discuss some of the special considerations that need to be applied to children with ANSD. This includes decisions to implant children with better audiometric thresholds that are standard in non-ANSD patients, concerns about the possibility of spontaneous remission and the appropriate timing of implantation. Finally we review an extensive published literature in outcomes after cochlear implantation (CI) in ANSD. This is not a systematic review but rather an exercise to distill out some important reoccurring themes and the general consensus of opinion to date. Our conclusion is that the hearing loss category ANSD, together with its numerous co-morbidities, is far too heterogeneous to make definitive statements about prognosis with CI.

4 Review Taking the History and Performing the Physical Examination in a Child with Hearing Loss. 2015

Cushing, Sharon L / Papsin, Blake C. ·Archie's Cochlear Implant Laboratory, Department of Otolaryngology Head and Neck Surgery, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada. Electronic address: sharon.cushing@sickkids.ca. · Archie's Cochlear Implant Laboratory, Department of Otolaryngology Head and Neck Surgery, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada. ·Otolaryngol Clin North Am · Pubmed #26409821.

ABSTRACT: Hearing loss is one of the most common childhood disorders and has far reaching effects on communication and socialization in children. Language acquisition, the most commonly sought and measured outcome, is tightly linked to age at diagnosis of the hearing loss and the speed with which rehabilitation is instituted. Treatment is often not affected by the underlying cause of the hearing loss and should be initiated at the time of initial identification. History-taking and physical examination in the setting of pediatric hearing loss are straightforward and should include an assessment of motor milestones, balance, and vestibular function.

5 Review What is the optimal timing for bilateral cochlear implantation in children? 2011

Gordon, K A / Jiwani, S / Papsin, B C. ·Archie's Cochlear Implant Laboratory, The Hospital for Sick Children, Toronto, ON, Canada. karen.gordon@utoronto.ca ·Cochlear Implants Int · Pubmed #21917210.

ABSTRACT: Bilateral cochlear implants (CIs) have been provided to children who are deaf in both ears with intent to promote binaural hearing. If it is possible to establish binaural hearing with two CIs, these children would be able to make use of interaural level and timing differences to localize sound and to distinguish between sounds separated in space. These skills are central to the ability to attend to one particular sound amidst a number of sound sources. This may be particularly important for children because they are typically learning and interacting in groups. However, the development of binaural processing could be disrupted by effects of bilateral deafness, effects of unilateral CI use, or issues related to the child's age at onset of deafness and age at the time of the first and second cochlear implantation. This research aims to determine whether binaural auditory processing is affected by these variables in an effort to determine the optimal timing for bilateral cochlear implantation in children. It is now clear that the duration of bilateral deafness should be limited in children to restrict reorganization in the auditory thalamo-cortical pathways. It has also been shown that unilateral CI use can halt such reorganization to some extent and promote auditory development. At the same time, however, unilateral input might compromise the development of binaural processing if CIs are provided sequentially. Mismatches in responses from the auditory brainstem and cortex evoked by the first and second CI after a long period of unilateral CI use suggest asymmetry in the bilateral auditory pathways which is significantly more pronounced than in children receiving bilateral implants simultaneously. Moreover, behavioural responses to level and timing differences between implants suggest that these important binaural cues are not being processed normally by children who received a second CI after a long period of unilateral CI use and at older ages. In sum, there may be multiple sensitive periods in the developing auditory system, which must be considered when determining the optimal timing for bilateral cochlear implantation.

6 Review Use it or lose it? Lessons learned from the developing brains of children who are deaf and use cochlear implants to hear. 2011

Gordon, K A / Wong, D D E / Valero, J / Jewell, S F / Yoo, P / Papsin, B C. ·Archie's Cochlear Implant Laboratory, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, Canada. karen.gordon@utoronto.ca ·Brain Topogr · Pubmed #21479928.

ABSTRACT: In the present paper, we review what is currently known about the effects of deafness on the developing human auditory system and ask: Without use, does the immature auditory system lose the ability to normally function and mature? Any change to the structure or function of the auditory pathways resulting from a lack of activity will have important implications for future use through an auditory prosthesis such as a cochlear implant. Data to date show that deafness in children arrests and disrupts normal auditory development. Multiple changes to the auditory pathways occur during the period of deafness with the extent and type of change being dependent upon the age and stage of auditory development at onset of deafness, the cause or type of deafness, and the length of time the immature auditory pathways are left without significant input. Structural changes to the auditory nerve, brainstem, and cortex have been described in animal models of deafness as well in humans who are deaf. Functional changes in deaf auditory pathways have been evaluated by using a cochlear implant to stimulate the auditory nerve with electrical pulses. Studies of electrically evoked activity in the immature deaf auditory system have demonstrated that auditory brainstem development is arrested and that thalamo-cortical areas are vulnerable to being taken over by other competitive inputs (cross-modal plasticity). Indeed, enhanced peripheral sight and detection of visual movement in congenitally deaf cats and adults have been linked to activity in specific areas of what would normally be auditory cortex. Cochlear implants can stimulate developmental plasticity in the auditory brainstem even after many years of deafness in childhood but changes in the auditory cortex are limited, at least in part, by the degree of reorganization which occurred during the period of deafness. Consequently, we must identify hearing loss rapidly (i.e., at birth for congenital deficits) and provide cochlear implants to appropriate candidates as soon as possible. Doing so has facilitated auditory development in the thalamo-cortex and allowed children who are deaf to perceive and use spoken language.

7 Clinical Trial Preliminary experience using a cochlear implant with a novel linear pedestal design. 2017

Parkes, William J / Gnanasegaram, Joshua J / Cushing, Sharon L / James, Adrian L / Gordon, Karen A / Papsin, Blake C. ·Archie's Cochlear Implant Laboratory, Department of Otolaryngology, The Hospital for Sick Children, Toronto, Ontario, Canada; Department of Otolaryngology - Head and Neck Surgery, University of Toronto, Toronto, Ontario, Canada. Electronic address: wjparkes@gmail.com. · Archie's Cochlear Implant Laboratory, Department of Otolaryngology, The Hospital for Sick Children, Toronto, Ontario, Canada; The Institute of Medical Science, University of Toronto, Toronto, Ontario, Canada. · Archie's Cochlear Implant Laboratory, Department of Otolaryngology, The Hospital for Sick Children, Toronto, Ontario, Canada; Department of Otolaryngology - Head and Neck Surgery, University of Toronto, Toronto, Ontario, Canada. · Archie's Cochlear Implant Laboratory, Department of Otolaryngology, The Hospital for Sick Children, Toronto, Ontario, Canada; The Institute of Medical Science, University of Toronto, Toronto, Ontario, Canada; Department of Otolaryngology - Head and Neck Surgery, University of Toronto, Toronto, Ontario, Canada. ·Int J Pediatr Otorhinolaryngol · Pubmed #28109496.

ABSTRACT: OBJECTIVE: To assess the safety and efficiency of cochlear implantation using a novel device with a linear silastic pedestal (2 mm wide, 2 mm deep, 10 mm long) on the flat undersurface. METHODS: Operative times required to drill a linear groove (LG) for the new linear pedestal design were prospectively accrued for 46 implantations in 30 children (median age 3). Intra-operative safety was assessed during each case. Instances of dural exposure in the base of the LG were noted. Length of stay was also recorded as a secondary measure of efficiency. RESULTS: Across all surgeons, the mean time needed to create the LG was 1.9 ± 1.5 min (±SD) with a median time of 1.5 min (95% Cl: 1-2 min). The range in time was 1-10 min. No intraoperative complications occurred. Intended device positioning was confirmed with on-table post-operative x-rays in all cases. 43% of patients were discharged on the day of surgery. CONCLUSIONS: The novel linear pedestal design allows for deliberate device placement while adding little additional operative time and complexity, an improvement on our current standard of care.

8 Article Etiology and therapy indication for cochlear implantation in children with single-sided deafness : Retrospective analysis. 2019

Cushing, S L / Gordon, K A / Sokolov, M / Papaioannou, V / Polonenko, M / Papsin, B C. ·Department of Otolaryngology, Head and Neck Surgery, Hospital for Sick Children, Room 6103C Burton Wing, 555 University Avenue, M5G 1X8, Toronto, ON, Canada. sharon.cushing@sickkids.ca. · Department of Otolaryngology, Head and Neck Surgery, University of Toronto, Toronto, ON, Canada. sharon.cushing@sickkids.ca. · Archie's Cochlear Implant Laboratory, Hospital for Sick Children, Toronto, ON, Canada. sharon.cushing@sickkids.ca. · Department of Otolaryngology, Head and Neck Surgery, University of Toronto, Toronto, ON, Canada. · Archie's Cochlear Implant Laboratory, Hospital for Sick Children, Toronto, ON, Canada. · Department of Communication Disorders, Hospital for Sick Children, Toronto, ON, Canada. · Department of Otolaryngology, Head and Neck Surgery, Hospital for Sick Children, Room 6103C Burton Wing, 555 University Avenue, M5G 1X8, Toronto, ON, Canada. · Department of Neurosciences & Mental Health, Hospital for Sick Children, Toronto, ON, Canada. · Institute of Medical Science, University of Toronto, Toronto, ON, Canada. ·HNO · Pubmed #31478064.

ABSTRACT: OBJECTIVE: The characteristics of children with single-sided deafness (SSD) who become candidates for unilateral cochlear implantation (uCI) were identified. STUDY DESIGN: In all, 118 children with SSD presenting from 2013-2019 to a tertiary pediatric children's hospital were retrospectively assessed regarding candidacy for uCI. RESULTS: Of the 118 children, 103 had completed uCI candidacy assessment, while 15 were undergoing this assessment at the time of review. More than half of children did not go on to implantation (63/103, 61%), with the 2 main reasons being (1) half (31/63) did not meet candidacy criteria for implantation, most commonly due to cochlear nerve aplasia/hypoplasia (31/82 who were assessed with MRI, 38%) and (2) families (30/103; 29%) declined participation in the surgical arm of the trial. The most common etiologies of SSD in the 37/103 (36%) children who both met candidacy and consented to implantation were congenital cytomegalovirus (cCMV; 16/37, 43%), unknown (6/37, 16%), cochleovestibular anomaly and trauma (each 5/37, 14%). CONCLUSIONS: Many children with SSD who present for implant candidacy assessment do not ultimately receive uCI. Major factors contributing to noncandidacy are cochlear nerve aplasia and parental acceptance of the intervention. While approximately half of children with SSD in our cohort were candidates for implantation, only 1/3 of the total cohort proceeded with implantation with the main predictors of acceptability of this intervention being an etiology (i.e., cCMV) that carries risk of progressive deterioration in the better hearing ear or SSD that was sudden in onset. These findings provide important insight into this new population of cochlear implant users and the emerging acceptance of intervention in children with SSD.

9 Article Impact of Consistency in Daily Device Use on Speech Perception Abilities in Children with Cochlear Implants: Datalogging Evidence. 2018

Easwar, Vijayalakshmi / Sanfilippo, Joseph / Papsin, Blake / Gordon, Karen. ·Archie's Cochlear Implant Laboratory, The Hospital for Sick Children, Toronto, ON, Canada. · Collaborative Program in Neuroscience, The University of Toronto, Toronto, ON, Canada. · Otolaryngology, The Hospital for Sick Children, Toronto, Toronto, ON, Canada. · Otolaryngology, The University of Toronto, Toronto, Toronto, ON, Canada. ·J Am Acad Audiol · Pubmed #30278868.

ABSTRACT: BACKGROUND: Cochlear implants (CIs) give children with severe to profound hearing loss access to sound. There appears to be a dose effect of sound exposure on speech perception abilities as shown by the positive influence of early implantation and CI experience. The consistency in device use per day could also affect sound dose, potentially affecting perceptual abilities in children with CIs. PURPOSE: The objectives of the present study were to identify the impact of consistency in device use on: (1) speech perception abilities and (2) asymmetry in speech perception abilities between bilateral CIs. RESEARCH DESIGN: Retrospective analysis. STUDY SAMPLE: To achieve the first objective, data from 65 children (age range at speech test: 1.91-18.05 yrs) with one (unilaterally implanted or bimodal) or two CIs (sequentially or simultaneously implanted) were included. A subset of data from 40 children with bilateral CIs was included to achieve the second objective. Of the 40 children with two CIs, 15 received their CIs sequentially. DATA COLLECTION AND ANALYSIS: Device use information was extracted from datalogs stored in personal speech processors using custom software. Speech perception scores per CI collected in quiet were also evaluated. Multiple regression was used to assess the impact of daily CI use, while controlling for factors previously identified to affect speech perception: age at speech test, length of pre-CI (acoustic) hearing experience, length of CI hearing experience, and order of CI for the first objective, and CI category (simultaneous/sequential implantation), interimplant delay, and length of CI experience for the second objective. RESULTS: On average, children wore their CIs for 11.59 ± 2.86 hours/day and, with one CI, exhibited 65.07 ± 22.64% accuracy on speech perception tests. Higher monaural speech perception scores were associated with longer everyday CI use and CI experience (p < 0.05). Among children with bilateral CIs, those with simultaneously implanted CIs and similar bilateral hearing experience demonstrated a small but significant right ear advantage with higher speech perception scores when using the right rather than left CI (mean difference = 4.55 ± 9.83%). The asymmetry in speech perception between CIs was larger and more variable in children who received their CIs sequentially (mean difference CI1-CI2 = 27.48 ± 24.87%). These asymmetries decreased with longer/consistent everyday use of the newer CI (p < 0.05). Yet, despite consistent everyday device use of the second CI (>12 hours/day), only a small proportion of children implanted sequentially (one out of seven children) achieved symmetrical function similar to children with simultaneously received bilateral CIs. CONCLUSIONS: Consistent everyday CI use contributes to higher speech perception scores. Although consistent CI use can help reduce the asymmetry in speech perception abilities of children with sequentially implanted CIs subsequent to interimplant delay, residual asymmetry often persists.

10 Article Transmastoid access in branchio-oto-renal syndrome: A reappraisal of computed tomography imaging. 2018

Parkes, William J / Cushing, Sharon L / Blaser, Susan I / Papsin, Blake C. ·Division of Otolaryngology, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA; Department of Otolaryngology-Head and Neck Surgery, Thomas Jefferson University, Philadelphia, PA, USA. Electronic address: william.parkes@nemours.org. · Department of Otolaryngology-Head and Neck Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. · Department of Otolaryngology-Head and Neck Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada; Department of Diagnostic Imaging, Division of Neuroradiology, The Hospital for Sick Children, Toronto, Ontario, Canada. ·Int J Pediatr Otorhinolaryngol · Pubmed #30262375.

ABSTRACT: OBJECTIVE: To evaluate for temporal bone abnormalities that might affect transmastoid surgery such as cochlear implantation in cases of branchio-oto-renal syndrome (BOR). STUDY DESIGN: Retrospective review. METHODS: Qualitative assessment of temporal bone computed tomography imaging was performed by a neuroradiologist for 30 individuals with BOR (60 ears) and 20 controls with normal hearing (20 ears). Transmastoid access was assessed categorically across 4 features: tip development, cortex pneumatization, tegmen height, and facial recess pneumatization. The appearance of 4 standard landmarks (Koerner's septum, antrum, prominence of the horizontal semicircular canal, incudal short process) was also dichotomized as normal or abnormal. Data were compared using Fisher's exact testing. RESULTS: Mastoid height differed between the groups with tip underdevelopment noted in 72% of BOR ears vs. 40% of controls (p = 0.02), and a low tegmen was seen in 68% of BOR ears and 25% of controls (p < 0.01). Significant differences in pneumatization were also found for the mastoid cortex (28% non-pneumatized in BOR vs. 5% in controls; p = 0.03) and the facial recess (27% non-pneumatized in BOR vs. 0% in controls; p = 0.01). Standard landmarks were easily identified in all of the control mastoids. In the BOR group, Koerner's septum was abnormally located or absent in 45%, and the antrum was severely hypoplastic or absent in 50%. Similarly, the prominence of the horizontal semicircular canal and the short process of the incus were dysplastic in 73% (44/60) and 62% (37/60), respectively. CONCLUSIONS: Mastoid abnormalities are common in BOR syndrome. Restricted transmastoid access and abnormal or absent mastoid landmarks should be anticipated in those patients with BOR who become cochlear implant candidates. LEVEL OF EVIDENCE: 4.

11 Article Cranial orthosis after cochlear implantation in an infant: Helmet modifications. 2018

Faucett, Erynne A / Lam-Bellissimo, Samantha / Zawawi, Faisal / Cushing, Sharon L / Papsin, Blake C. ·Department of Otolaryngology, Head and Neck Surgery, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. · The Hospital for Sick Children, The Children's Orthotics Clinic, Toronto, Ontario, Canada. · Department of Otolaryngology, Head and Neck Surgery, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada; Archie's Cochlear Implant Laboratory, Hospital for Sick Children, Toronto, Ontario, Canada; Institute of Medical Science, University of Toronto, Toronto, Ontario, Canada. Electronic address: Sharon.cushing@sickkids.ca. · Department of Otolaryngology, Head and Neck Surgery, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada; Archie's Cochlear Implant Laboratory, Hospital for Sick Children, Toronto, Ontario, Canada; Institute of Medical Science, University of Toronto, Toronto, Ontario, Canada. ·Int J Pediatr Otorhinolaryngol · Pubmed #30262345.

ABSTRACT: We present an infant with bilateral sensorineural hearing loss caused by bacterial meningitis, and moderate/severe plagiocephaly requiring simultaneous treatment of cochlear implantation for hearing loss and cranial orthosis for plagiocephaly. A helmet modification was created, so that the infant was able to be treated for his plagiocephaly while bilateral cochlear implants were in place, bringing attention to serve needs of those patients requiring cochlear implant and cranial orthosis concurrently. While this case was the first time such a modification was required, which was due to the young age at implantation, the occurrence of the concurrent need may increase as we continue to push the boundaries of early implantation.

12 Article Limiting asymmetric hearing improves benefits of bilateral hearing in children using cochlear implants. 2018

Polonenko, Melissa Jane / Papsin, Blake Croll / Gordon, Karen Ann. ·Institute of Medical Science, The University of Toronto, Toronto, ON, M5S 1A8, Canada. melissa.polonenko@mail.utoronto.ca. · Neurosciences and Mental Health, The Hospital for Sick Children, Toronto, ON, M5G 1X8, Canada. melissa.polonenko@mail.utoronto.ca. · Institute of Medical Science, The University of Toronto, Toronto, ON, M5S 1A8, Canada. · Department of Otolaryngology - Head & Neck Surgery, The University of Toronto, Toronto, ON, M5G 2N2, Canada. · Otolaryngology - Head & Neck Surgery, The Hospital for Sick Children, Toronto, ON, M5G 1X8, Canada. · Neurosciences and Mental Health, The Hospital for Sick Children, Toronto, ON, M5G 1X8, Canada. ·Sci Rep · Pubmed #30181590.

ABSTRACT: Neurodevelopmental changes occur with asymmetric hearing loss, limiting binaural/spatial hearing and putting children at risk for social and educational challenges. These deficits may be mitigated by providing bilateral hearing in children through auditory prostheses. Effects on speech perception and spatial hearing were measured in a large cohort of >450 children who were deaf and used bilateral cochlear implants or bimodal devices (one cochlear implant and a contralateral hearing aid). Results revealed an advantage of bilateral over unilateral device use but this advantage decreased as hearing in the two ears became increasingly asymmetric. Delayed implantation of an ear with severe to profound deafness allowed asymmetric hearing, creating aural preference for the better hearing ear. These findings indicate that bilateral input with the most appropriate device for each ear should be provided early and without delay during development.

13 Article Natural History of Tympanic Membrane Retraction in Children with Cleft Palate. 2018

Parkes, William / Vilchez-Madrigal, Luis / Cushing, Sharon / Papsin, Blake / James, Adrian. ·Clinic of Otolaryngology, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA. · Clinic of Otolaryngology, National Children's Hospital, San Jose, Costa Rica. · Clinic of Otolaryngology, Hospital for Sick Children, Toronto, Ontario, Canada. ·J Int Adv Otol · Pubmed #30100539.

ABSTRACT: OBJECTIVES: The natural history of tympanic membrane retraction is unpredictable. To obtain prognostic information for guiding surveillance and treatment, a cohort of children with retraction from cleft palate were prospectively followed for over 5 years. MATERIALS AND METHODS: This was a prospective observational study at a tertiary academic institution. Children with pars tensa retraction were selected from a cohort of 143 children with cleft palate. Thirty-seven ears were assessed with otoendoscopic image capture and audiometry at a median age of 9 years and reassessed at a median follow-up interval of 6.4 years. The severity of tympanic membrane retraction in the serial images of each ear was compared by four pediatric otolaryngologists blinded to the dates of the images. RESULTS: Initially, 19/37 retractions (51%) demonstrated contact with the incus and/or promontory. Follow-up images were rated as stable (n=16) or better (n=12) for 28/37 retractions (76%). Of the nine retractions that became more extensive, two developed cholesteatoma (5% of the total). No ossicular erosion developed in ears without cholesteatoma. Conductive hearing loss (4-tone average air-bone gap >25 decibels hearing level) was initially present in five ears, worsened in one, and normalized without intervention in others. No ears with initial normal hearing developed hearing loss. CONCLUSION: Most tympanic membrane retractions remained stable or improved over time in this cohort of children who were at a risk of persistent eustachian tube dysfunction. Clinically significant progression occurred infrequently, justifying the conservative approach taken to manage these retractions. Such data are necessary to weigh the potential benefit of preventive intervention over observation.

14 Article Emberger syndrome: A rare association with hearing loss. 2018

Zawawi, Faisal / Sokolov, Meirav / Mawby, Thomas / Gordon, Karen A / Papsin, Blake C / Cushing, Sharon L. ·Department of Otolaryngology, Head and Neck Surgery, Hospital for Sick Children, Toronto, ON Canada; Department of Otolaryngology, Head and Neck Surgery, University of Toronto, Toronto, ON Canada. · Department of Otolaryngology, Head and Neck Surgery, Hospital for Sick Children, Toronto, ON Canada; Department of Otolaryngology, Head and Neck Surgery, University of Toronto, Toronto, ON Canada; Archie's Cochlear Implant Laboratory, Hospital for Sick Children, Toronto, ON Canada. · Department of Otolaryngology, Head and Neck Surgery, University of Toronto, Toronto, ON Canada; Archie's Cochlear Implant Laboratory, Hospital for Sick Children, Toronto, ON Canada; Department of Communication Disorders, Hospital for Sick Children, Toronto, ON Canada. · Department of Otolaryngology, Head and Neck Surgery, Hospital for Sick Children, Toronto, ON Canada; Department of Otolaryngology, Head and Neck Surgery, University of Toronto, Toronto, ON Canada; Archie's Cochlear Implant Laboratory, Hospital for Sick Children, Toronto, ON Canada. Electronic address: Sharon.cushing@sickkids.ca. ·Int J Pediatr Otorhinolaryngol · Pubmed #29605372.

ABSTRACT: Emberger Syndrome (ES) is a rare genetic disorder characterized by lymphedema and myelodysplasia. It is also associated with hearing loss. The genetic mutations associated with ES are not part of the comprehensive 80 gene next generation sequencing (NGS) panel. As a result, the otolaryngologist should maintain an index of suspicion for ES in any child with SNHL who presents repeatedly with recurrent infections, lymphedema and/or cutaneous warts. This paper describes the clinical evolution and management of two children who were followed up for hearing loss and eventually were diagnosed with ES.

15 Article Delayed access to bilateral input alters cortical organization in children with asymmetric hearing. 2018

Polonenko, Melissa Jane / Papsin, Blake Croll / Gordon, Karen Ann. ·Institute of Medical Sciences, University of Toronto, Toronto, ON M5S 1A8, Canada. · Neurosciences & Mental Health, Hospital for Sick Children, Toronto, ON M5G 1X8, Canada. · Department of Otolaryngology - Head & Neck Surgery, University of Toronto, Toronto, ON M5G 2N2, Canada. · Otolaryngology - Head & Neck Surgery, Hospital for Sick Children, Toronto, ON M5G 1X8, Canada. ·Neuroimage Clin · Pubmed #29159054.

ABSTRACT: Bilateral hearing in early development protects auditory cortices from reorganizing to prefer the better ear. Yet, such protection could be disrupted by mismatched bilateral input in children with asymmetric hearing who require electric stimulation of the auditory nerve from a cochlear implant in their deaf ear and amplified acoustic sound from a hearing aid in their better ear (bimodal hearing). Cortical responses to bimodal stimulation were measured by electroencephalography in 34 bimodal users and 16 age-matched peers with normal hearing, and compared with the same measures previously reported for 28 age-matched bilateral implant users. Both auditory cortices increasingly favoured the better ear with delay to implanting the deaf ear; the time course mirrored that occurring with delay to bilateral implantation in unilateral implant users. Preference for the implanted ear tended to occur with ongoing implant use when hearing was poor in the non-implanted ear. Speech perception deteriorated with longer deprivation and poorer access to high-frequencies. Thus, cortical preference develops in children with asymmetric hearing but can be avoided by early provision of balanced bimodal stimulation. Although electric and acoustic stimulation differ, these inputs can work sympathetically when used bilaterally given sufficient hearing in the non-implanted ear.

16 Article Cortical Processing of Level Cues for Spatial Hearing is Impaired in Children with Prelingual Deafness Despite Early Bilateral Access to Sound. 2018

Easwar, Vijayalakshmi / Yamazaki, Hiroshi / Deighton, Michael / Papsin, Blake / Gordon, Karen. ·Archie's Cochlear Implant Laboratory, The Hospital for Sick Children, Room 6D08, 555 University Avenue, Toronto, ON, M5G 1X8, Canada. veaswar@nca.uwo.ca. · Collaborative Program in Neuroscience, University of Toronto, Toronto, Canada. veaswar@nca.uwo.ca. · Archie's Cochlear Implant Laboratory, The Hospital for Sick Children, Room 6D08, 555 University Avenue, Toronto, ON, M5G 1X8, Canada. · Department of Otolaryngology, University of Toronto, Toronto, Canada. · Department of Otolaryngology, The Hospital for Sick Children, Toronto, Canada. ·Brain Topogr · Pubmed #29119311.

ABSTRACT: Bilateral cochlear implantation aims to restore binaural hearing, important for spatial hearing, to children who are deaf. Improvements over unilateral implant use are attributed largely to the detection of interaural level differences (ILDs) but emerging evidence of impaired sound localization and binaural fusion suggest that these binaural cues are abnormally coded by the auditory system. We used multichannel electroencephalography (EEG) to assess cortical responses to ILDs in two groups: 13 children who received early bilateral cochlear implants (CIs) simultaneously, known to protect the developing auditory cortices from unilaterally driven reorganization, and 15 age matched peers with normal hearing. EEG source analyses indicated a dominance of right auditory cortex in both groups. Expected reductions in activity to ipsilaterally weighted ILDs were evident in the right hemisphere of children with normal hearing. By contrast, cortical activity in children with CIs showed: (1) limited ILD sensitivity in either cortical hemisphere, (2) limited correlation with reliable behavioral right-left lateralization of ILDs (in 10/12 CI users), and (3) deficits in parieto-occipital areas and the cerebellum. Thus, expected cortical ILD coding develops with normal hearing but is affected by developmental deafness despite early and simultaneous bilateral implantation. Findings suggest that impoverished fidelity of ILDs in independently functioning CIs may be impeding development of cortical ILD sensitivity in children who are deaf but do not altogether limit benefits of listening with bilateral CIs. Future efforts to provide consistent/accurate ILDs through auditory prostheses including CIs could improve binaural hearing for children with hearing loss.

17 Article Cortical organization restored by cochlear implantation in young children with single sided deafness. 2017

Polonenko, Melissa Jane / Gordon, Karen Ann / Cushing, Sharon Lynn / Papsin, Blake Croll. ·Institute of Medical Sciences, The University of Toronto, Toronto, ON, M5S 1A8, Canada. melissa.polonenko@mail.utoronto.ca. · Neurosciences and Mental Health, The Hospital for Sick Children, Toronto, ON, M5G 1X8, Canada. melissa.polonenko@mail.utoronto.ca. · Institute of Medical Sciences, The University of Toronto, Toronto, ON, M5S 1A8, Canada. · Neurosciences and Mental Health, The Hospital for Sick Children, Toronto, ON, M5G 1X8, Canada. · Department of Otolaryngology - Head & Neck Surgery, The University of Toronto, Toronto, ON, M5G 2N2, Canada. · Otolaryngology - Head & Neck Surgery, The Hospital for Sick Children, Toronto, ON, M5G 1X8, Canada. ·Sci Rep · Pubmed #29203800.

ABSTRACT: Early treatment of single sided deafness in children has been recommended to protect from neurodevelopmental preference for the better hearing ear and from social and educational deficits. A fairly homogeneous group of five young children (≤3.6 years of age) with normal right sided hearing who received a cochlear implant to treat deafness in their left ears were studied. Etiology of deafness was largely cytomegalovirus (n = 4); one child had an enlarged vestibular aqueduct. Multi-channel electroencephalography of cortical evoked activity was measured repeatedly over time at: 1) acute (0.5 ± 0.7 weeks); 2) early chronic (1.1 ± 0.2 months); and 3) chronic (5.8 ± 3.4 months) cochlear implant stimulation. Results indicated consistent responses from the normal right ear with marked changes in activity from the implanted left ear. Atypical distribution of peak amplitude activity from the implanted ear at acute stimulation marked abnormal lateralization of activity to the ipsilateral left auditory cortex and recruitment of extra-temporal areas including left frontal cortex. These abnormalities resolved with chronic implant use and contralateral aural preference emerged in both auditory cortices. These findings indicate that early implantation in young children with single sided deafness can rapidly restore bilateral auditory input to the cortex needed to improve binaural hearing.

18 Article Music perception improves in children with bilateral cochlear implants or bimodal devices. 2017

Polonenko, Melissa J / Giannantonio, Sara / Papsin, Blake C / Marsella, Pasquale / Gordon, Karen A. ·Archie's Cochlear Implant Laboratory, Department of Otolaryngology, The Hospital for Sick Children, Room 6D08, Toronto M5G 1X8, Canada. · Audiology and Otosurgery Unit, Bambino Gesù Pediatric Hospital, Piazza di Sant'Onofrio 4, 00165, Rome, Italy. ·J Acoust Soc Am · Pubmed #28679263.

ABSTRACT: The objectives of this study were to determine if music perception by pediatric cochlear implant users can be improved by (1) providing access to bilateral hearing through two cochlear implants or a cochlear implant and a contralateral hearing aid (bimodal users) and (2) any history of music training. The Montreal Battery of Evaluation of Musical Ability test was presented via soundfield to 26 bilateral cochlear implant users, 8 bimodal users and 16 children with normal hearing. Response accuracy and reaction time were recorded via an iPad application. Bilateral cochlear implant and bimodal users perceived musical characteristics less accurately and more slowly than children with normal hearing. Children who had music training were faster and more accurate, regardless of their hearing status. Reaction time on specific subtests decreased with age, years of musical training and, for implant users, better residual hearing. Despite effects of these factors on reaction time, bimodal and bilateral cochlear implant users' responses were less accurate than those of their normal hearing peers. This means children using bilateral cochlear implants and bimodal devices continue to experience challenges perceiving music that are related to hearing impairment and/or device limitations during development.

19 Article Association of Gel-Forming Mucins and Aquaporin Gene Expression With Hearing Loss, Effusion Viscosity, and Inflammation in Otitis Media With Effusion. 2017

Samuels, Tina L / Yan, Justin C / Khampang, Pawjai / Dettmar, Peter W / MacKinnon, Alexander / Hong, Wenzhou / Johnston, Nikki / Papsin, Blake C / Chun, Robert H / McCormick, Michael E / Kerschner, Joseph E. ·Department of Otolaryngology and Communication Sciences, Medical College of Wisconsin, Milwaukee. · RDBiomed Limited, Hull, England. · Department of Pathology, Medical College of Wisconsin, Milwaukee. · Department of Microbiology and Molecular Genetics, Medical College of Wisconsin, Milwaukee. · Archie's Cochlear Implant Laboratory, Department of Otolaryngology, The Hospital for Sick Children, Toronto, Ontario, Canada. ·JAMA Otolaryngol Head Neck Surg · Pubmed #28594978.

ABSTRACT: Importance: Persistent, viscous middle ear effusion in pediatric otitis media (OM) contributes to increased likelihood of anesthesia and surgery, conductive hearing loss, and subsequent developmental delays. Biomarkers of effusion viscosity and hearing loss have not yet been identified despite the potential that such markers hold for targeted therapy and screening. Objective: To investigate the association of gel-forming mucins and aquaporin 5 (AQP5) gene expression with inflammation, effusion viscosity, and hearing loss in pediatric OM with effusion (OME). Design, Setting, and Participants: Case-control study of 31 pediatric patients (aged 6 months to 12 years) with OME undergoing tympanostomy tube placement and control individuals (aged 1 to 10 years) undergoing surgery for cochlear implantation from February 1, 2013, through November 30, 2014. Those with 1 or more episodes of OM in the previous 12 months, immunologic abnormality, anatomical or physiologic ear defect, OM-associated syndrome (ie, Down syndrome, cleft palate), chronic mastoiditis, or history of cholesteatoma were excluded from the study. All patients with OME and 1 control were recruited from Children's Hospital of Wisconsin, Milwaukee. The remainder of the controls were recruited from Sick Kids Hospital in Toronto, Ontario, Canada. Main Outcomes and Measures: Two to 3 middle ear biopsy specimens, effusions, and preoperative audiometric data (obtained <3 weeks before surgery) were collected from patients; only biopsy specimens were collected from controls. Expression of the mucin 2 (MUC2), mucin 5AC (MUC5AC), mucin 5B (MUC5B), and AQP5 genes were assayed in middle ear biopsy specimens by quantitative polymerase chain reaction. One middle ear biopsy specimen was sectioned for histopathologic analysis. Reduced specific viscosity of effusions was assayed using rheometry. Results: Of the 31 study participants, 24 patients had OME (mean [SD] age, 50.4 [31.9] months; 15 [62.5%] male; 16 [66.7%] white) and 7 acted as controls (mean [SD] age, 32.6 [24.4] months; 2 [26.6%] male; 6 [85.7%] white). Mucins and AQP5 gene expression were significantly higher in patients with OME relative to controls (MUC2: ratio, 127.6 [95% CI, 33.7-482.7]; MUC5AC: ratio, 3748.8 [95% CI, 558.1-25 178.4]; MUC5B: ratio, 471.1 [95% CI, 130.7-1697.4]; AQP5: ratio, 2.4 [95% CI, 1.1-5.6]). A 2-fold increase in MUC5B correlated with increased hearing loss (air-bone gap: 7.45 dB [95% CI, 2.65-12.24 dB]; sound field: 6.66 dB [95% CI, 6.63-6.69 dB]), effusion viscosity (2.75 mL/mg; 95% CI, 0.89-4.62 mL/mg), middle ear epithelial thickness (3.5 μm; 95% CI, 1.96-5.13 μm), and neutrophil infiltration (odds ratio, 1.7; 95% CI, 1.07-2.72). A 2-fold increase in AQP5 correlated with increased effusion viscosity (1.94 mL/mg; 95% CI, 0.08-3.80 mL/mg). Conclusions and Relevance: Further exploration of the role of MUC5B in the pathophysiology of OME holds promise for development of novel, targeted therapies to reduce effusion viscosity, facilitation of effusion clearance, and prevention of disease chronicity and hearing loss in patients with OME.

20 Article Children With Single-Sided Deafness Use Their Cochlear Implant. 2017

Polonenko, Melissa Jane / Papsin, Blake Croll / Gordon, Karen Ann. ·1Archie's Cochlear Implant Laboratory, The Hospital for Sick Children, Toronto, Ontario, Canada; 2Neurosciences & Mental Health, The Hospital for Sick Children, Toronto, Ontario, Canada; 3Institute of Medical Sciences, The University of Toronto, Toronto, Ontario, Canada; 4Collaborative Program in Neuroscience, The University of Toronto, Toronto, Ontario, Canada; 5Department of Otolaryngology - Head & Neck Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada; and 6Department of Otolaryngology - Head & Neck Surgery, The University of Toronto, Toronto, Ontario, Canada. ·Ear Hear · Pubmed #28542017.

ABSTRACT: OBJECTIVES: To assess acceptance of a cochlear implant (CI) by children with single-sided deafness (SSD) as measured by duration of CI use across daily listening environments. DESIGN: Datalogs for 7 children aged 1.1 to 14.5 years (mean ± SD: 5.9 ± 5.9 years old), who had SSD and were implanted in their deaf ear, were anonymized and extracted from their CI processors. Data for all available follow-up clinical appointments were included, ranging from two to six visits. Measures calculated from each datalog included frequency and duration of time the coil disconnected from the internal device, average daily CI use, and both duration (hr/day) and percentage of CI use (% daily use) in different intensity ranges and environment types. Linear mixed effects regression analyses were used to evaluate the relationships between CI experience, daily CI use, frequency of coil-offs, and duration of coil-off time. Nonlinear regression analyses were used to evaluate CI use with age in different acoustic environments. RESULTS: Children with SSD used their CI on average 7.4 hr/day. Older children used their CI for longer periods of the day than younger children. Longitudinal data indicated consistent CI use from the date of CI activation. Frequency of coil-offs reduced with CI experience, but did not significantly contribute to hours of coil-off time. Children used their CI longest in environments that were moderately loud (50 to 70 dB A) and classified as containing speech-in-noise. Preschoolers tended to spend less time in quiet but more time in music than infants/toddlers and adolescents. CONCLUSIONS: Children with SSD consistently use their CI upon activation in a variety of environments commonly experienced by children. CI use in children with SSD resembles reported bilateral hearing aid use in children but is longer than reported hearing aid use in children with less severe unilateral hearing loss, suggesting that (1) the normal-hearing ear did not detract from consistent CI use; and (2) a greater asymmetry between ears presents a significant impairment that may facilitate device use to access bilateral sound.

21 Article Simultaneous bilateral cochlear implants: Developmental advances do not yet achieve normal cortical processing. 2017

Easwar, Vijayalakshmi / Yamazaki, Hiroshi / Deighton, Michael / Papsin, Blake / Gordon, Karen. ·Archie's Cochlear Implant Laboratory The Hospital for Sick Children Toronto ON Canada. · Collaborative Program in Neuroscience The University of Toronto Toronto ON Canada. · Otolaryngology The University of Toronto Toronto ON Canada. · Otolaryngology The Hospital for Sick Children Toronto ON Canada. ·Brain Behav · Pubmed #28413698.

ABSTRACT: BACKGROUND: Simultaneous bilateral cochlear implantation promotes symmetric development of bilateral auditory pathways but binaural hearing remains abnormal. To evaluate whether bilateral cortical processing remains impaired in such children, cortical activity to unilateral and bilateral stimuli was assessed in a unique cohort of 16 children who received bilateral cochlear implants (CIs) simultaneously at 1.97 ± 0.86 years of age and had ~4 years of CI experience, providing the first opportunity to assess electrically driven cortical development in the absence of reorganized asymmetries from sequential implantation. METHODS: Cortical activity to unilateral and bilateral stimuli was measured using multichannel electro-encephalography. Cortical processing in children with bilateral CIs was compared with click-elicited activity in 13 normal hearing children matched for time-in-sound. Source activity was localized using the Time Restricted, Artefact and Coherence source Suppression (TRACS) beamformer method. RESULTS: Consistent with dominant crossed auditory pathways, normal P1 activity (~100 ms) was weaker to ipsilateral stimuli relative to contralateral and bilateral stimuli and both auditory cortices preferentially responded to the contralateral ear. Right hemisphere dominance was evident overall. Children with bilateral CIs maintained the expected right dominance but differences from normal included: (i) minimal changes between ipsilateral, contralateral and bilateral stimuli, (ii) weaker than normal contralateral stimulus preference, (iii) symmetric activity to bilateral stimuli, and (iv) increased occipital lobe recruitment during bilateral relative to unilateral stimulation. Between-group contrasts demonstrated lower than normal activity in the inferior parieto-occipital lobe (suggesting deficits in sensory integration) and greater than normal left frontal lobe activity (suggesting increased attention), even during passive listening. CONCLUSIONS: Together, findings suggest that early simultaneous bilateral cochlear implantation promotes normal-like auditory symmetry but that abnormalities in cortical processing consequent to deafness and/or electrical stimulation through two independent speech processors persist.

22 Article Cortical Representation of Interaural Time Difference Is Impaired by Deafness in Development: Evidence from Children with Early Long-term Access to Sound through Bilateral Cochlear Implants Provided Simultaneously. 2017

Easwar, Vijayalakshmi / Yamazaki, Hiroshi / Deighton, Michael / Papsin, Blake / Gordon, Karen. ·Archie's Cochlear Implant Laboratory, Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canada, v.easwar@utoronto.ca. · Collaborative Program in Neuroscience, University of Toronto, Toronto, Ontario M5S 1A8, Canada. · Archie's Cochlear Implant Laboratory, Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canada. · Department of Otolaryngology, University of Toronto, Toronto, Ontario M5G 2N2, Canada, and. · Department of Otolaryngology, Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canada. ·J Neurosci · Pubmed #28123078.

ABSTRACT: Accurate use of interaural time differences (ITDs) for spatial hearing may require access to bilateral auditory input during sensitive periods in human development. Providing bilateral cochlear implants (CIs) simultaneously promotes symmetrical development of bilateral auditory pathways but does not support normal ITD sensitivity. Thus, although binaural interactions are established by bilateral CIs in the auditory brainstem, potential deficits in cortical processing of ITDs remain. Cortical ITD processing in children with simultaneous bilateral CIs and normal hearing with similar time-in-sound was explored in the present study. Cortical activity evoked by bilateral stimuli with varying ITDs (0, ±0.4, ±1 ms) was recorded using multichannel electroencephalography. Source analyses indicated dominant activity in the right auditory cortex in both groups but limited ITD processing in children with bilateral CIs. In normal-hearing children, adult-like processing patterns were found underlying the immature P1 (∼100 ms) response peak with reduced activity in the auditory cortex ipsilateral to the leading ITD. Further, the left cortex showed a stronger preference than the right cortex for stimuli leading from the contralateral hemifield. By contrast, children with CIs demonstrated reduced ITD-related changes in both auditory cortices. Decreased parieto-occipital activity, possibly involved in spatial processing, was also revealed in children with CIs. Thus, simultaneous bilateral implantation in young children maintains right cortical dominance during binaural processing but does not fully overcome effects of deafness using present CI devices. Protection of bilateral pathways through simultaneous implantation might be capitalized for ITD processing with signal processing advances, which more consistently represent binaural timing cues.

23 Article Benefits of Music Training for Perception of Emotional Speech Prosody in Deaf Children With Cochlear Implants. 2017

Good, Arla / Gordon, Karen A / Papsin, Blake C / Nespoli, Gabe / Hopyan, Talar / Peretz, Isabelle / Russo, Frank A. ·From the 1Department of Psychology, Ryerson University, Toronto, Ontario, Canada; 2Department of Otolaryngology, Cochlear Implant Program, The Hospital for Sick Children, Toronto, Ontario, Canada; 3International Laboratory for Brain, Music and Sound Research, University of Montreal, Montreal, Quebec, Canada; and 4Toronto Rehabilitation Institute, Toronto, Ontario, Canada. ·Ear Hear · Pubmed #28085739.

ABSTRACT: OBJECTIVES: Children who use cochlear implants (CIs) have characteristic pitch processing deficits leading to impairments in music perception and in understanding emotional intention in spoken language. Music training for normal-hearing children has previously been shown to benefit perception of emotional prosody. The purpose of the present study was to assess whether deaf children who use CIs obtain similar benefits from music training. We hypothesized that music training would lead to gains in auditory processing and that these gains would transfer to emotional speech prosody perception. DESIGN: Study participants were 18 child CI users (ages 6 to 15). Participants received either 6 months of music training (i.e., individualized piano lessons) or 6 months of visual art training (i.e., individualized painting lessons). Measures of music perception and emotional speech prosody perception were obtained pre-, mid-, and post-training. The Montreal Battery for Evaluation of Musical Abilities was used to measure five different aspects of music perception (scale, contour, interval, rhythm, and incidental memory). The emotional speech prosody task required participants to identify the emotional intention of a semantically neutral sentence under audio-only and audiovisual conditions. RESULTS: Music training led to improved performance on tasks requiring the discrimination of melodic contour and rhythm, as well as incidental memory for melodies. These improvements were predominantly found from mid- to post-training. Critically, music training also improved emotional speech prosody perception. Music training was most advantageous in audio-only conditions. Art training did not lead to the same improvements. CONCLUSIONS: Music training can lead to improvements in perception of music and emotional speech prosody, and thus may be an effective supplementary technique for supporting auditory rehabilitation following cochlear implantation.

24 Article The combination of vestibular impairment and congenital sensorineural hearing loss predisposes patients to ocular anomalies, including Usher syndrome. 2017

Kletke, S / Batmanabane, V / Dai, T / Vincent, A / Li, S / Gordon, K A / Papsin, B C / Cushing, S L / Héon, E. ·Department of Ophthalmology and Vision Sciences, University of Toronto, Toronto, Ontario, Canada. · Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children, Toronto, Ontario, Canada. · Program of Genetics and Genomic Biology, The Hospital for Sick Children, Toronto, Ontario, Canada. · Department of Otolaryngology - Head & Neck Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada. · Archie's Cochlear Implant Laboratory, The Hospital for Sick Children, Toronto, Ontario, Canada. ·Clin Genet · Pubmed #27743452.

ABSTRACT: The co-occurrence of hearing impairment and visual dysfunction is devastating. Most deaf-blind etiologies are genetically determined, the commonest being Usher syndrome (USH). While studies of the congenitally deaf population reveal a variable degree of visual problems, there are no effective ophthalmic screening guidelines. We hypothesized that children with congenital sensorineural hearing loss (SNHL) and vestibular impairment were at an increased risk of having USH. A retrospective chart review of 33 cochlear implants recipients for severe to profound SNHL and measured vestibular dysfunction was performed to determine the ocular phenotype. All the cases had undergone ocular examination and electroretinogram (ERG). Patients with an abnormal ERG underwent genetic testing for USH. We found an underlying ocular abnormality in 81.81% (27/33) of cases; of which 75% had refractive errors, and 50% of those patients showed visual improvement with refractive correction. A total of 14 cases (42.42%; 14/33) had generalized rod-cone dysfunction on ERG suggestive of Usher syndrome type 1, confirmed by mutational analysis. This work shows that adding vestibular impairment as a criterion for requesting an eye exam and adding the ERG to detect USH increases the chances of detecting ocular anomalies, when compared with previous literature focusing only on congenital SNHL.

25 Article Factors Affecting Daily Cochlear Implant Use in Children: Datalogging Evidence. 2016

Easwar, Vijayalakshmi / Sanfilippo, Joseph / Papsin, Blake / Gordon, Karen. ·Archie's Cochlear Implant Laboratory, The Hospital for Sick Children, Toronto, ON, Canada. · Collaborative Program in Neuroscience, The University of Toronto, Toronto, ON, Canada. · Otolaryngology, The University of Toronto, Toronto, ON, Canada. · Otolaryngology, The Hospital for Sick Children, Toronto, ON, Canada. ·J Am Acad Audiol · Pubmed #27885978.

ABSTRACT: BACKGROUND: Children with profound hearing loss can gain access to sound through cochlear implants (CIs), but these devices must be worn consistently to promote auditory development. Although subjective parent reports have identified several factors limiting long-term CI use in children, it is also important to understand the day-to-day issues which may preclude consistent device use. In the present study, objective measures gathered through datalogging software were used to quantify the following in children: (1) number of hours of CI use per day, (2) practical concerns including repeated disconnections between the external transmission coil and the internal device (termed "coil-offs"), and (3) listening environments experienced during daily use. PURPOSE: This study aimed to (1) objectively measure daily CI use and factors influencing consistent device use in children using one or two CIs and (2) evaluate the intensity levels and types of listening environments children are exposed to during daily CI use. RESEARCH DESIGN: Retrospective analysis. STUDY SAMPLE: Measures of daily CI use were obtained from 146 pediatric users of Cochlear Nucleus 6 speech processors. The sample included 5 unilateral, 40 bimodal, and 101 bilateral CI users (77 simultaneously and 24 sequentially implanted). DATA COLLECTION AND ANALYSIS: Daily CI use, duration, and frequency of coil-offs per day, and the time spent in multiple intensity ranges and environment types were extracted from the datalog saved during clinic appointments. Multiple regression analyses were completed to predict daily CI use based on child-related demographic variables, and to evaluate the effects of age on coil-offs and environment acoustics. RESULTS: Children used their CIs for 9.86 ± 3.43 hr on average on a daily basis, with use exceeding 9 hr per day in ∼64% of the children. Daily CI use reduced significantly with increasing durations of coil-off (p = 0.027) and increased significantly with longer CI experience (p < 0.001) and pre-CI acoustic experience (p < 0.001), when controlled for the child's age. Total time in sound (sum of CI and pre-CI experience) was positively correlated with CI use (r = 0.72, p < 0.001). Longer durations of coil-off were associated with higher frequency of coil-offs (p < 0.001). The frequency of coil-offs ranged from 0.99 to 594.10 times per day and decreased significantly with age (p < 0.001). Daily CI use and frequency of coil-offs did not vary significantly across known etiologies. Listening environments of all children typically ranged between 50 and 70 dBA. Children of all ages were exposed to speech in noisy environments. Environment classified as "music" was identified more often in younger children. CONCLUSIONS: The majority of children use their CIs consistently, even during the first year of implantation. The frequency of coil-offs is a practical challenge in infants and young children, and demonstrates the need for improved coil retention methods for pediatric use. Longer hearing experience and shorter coil-off time facilitates consistent CI use. Children are listening to speech in noisy environments most often, thereby indicating a need for better access to binaural cues, signal processing, and stimulation strategies to aid listening. Study findings could be useful in parent counseling of young and/or new CI users.

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