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  • 1
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    Online Resource
    Rate of Neural Recovery in Implanted Children with Auditory Neuropathy Spectrum Disorder
    Wiley ; 2011
    In:  Otolaryngology–Head and Neck Surgery Vol. 144, No. 2 ( 2011-02), p. 274-279
    Details
    In: Otolaryngology–Head and Neck Surgery, Wiley, Vol. 144, No. 2 ( 2011-02), p. 274-279
    Abstract: The study objectives were to compare the rate of neural recovery and speech perception performance in children with auditory neuropathy spectrum disorder (ANSD) and children with sensorineural hearing loss (SNHL) from other etiologies. Study Design Cohort study. Setting Academic hospital and cochlear implant center. Subjects and Methods Ten children with ANSD were matched based on type of implant and age at implantation with peers diagnosed with SNHL. Electrically evoked compound action potential (ECAP) recovery functions were obtained to measure neural refractory behaviors in response to stimulation from the cochlear implant. Speech perception performance was measured using speech recognition thresholds (SRTs) for monosyllable and spondee word stimuli. These outcome measures were compared between groups. Results There was no difference in average recovery function exponent in children with ANSD compared to children with SNHL. Similarly, there were no differences in average SRTs in quiet and in noise in children with ANSD compared to children with SNHL. Relationships between SRT and recovery rate were not present within groups or for all subjects for SRT in quiet, but a significant relationship was found for all subjects for SRT in noise ( P =. 04). Conclusions Dyssynchronous neural activity in ANSD may affect temporal encoding of electrical stimulation from a cochlear implant. As a group, children with ANSD did not demonstrate slower neural recovery compared to those with SNHL, but there was slower neural recovery observed for some subjects. The utility of ECAP recovery functions on optimizing the stimulation rate for individual patients with ANSD requires further investigation.
    Type of Medium: Online Resource
    ISSN: 0194-5998 , 1097-6817
    URL: Article
    DOI: 10.1177/0194599810391603
    Language: English
    Publisher: Wiley
    Publication Date: 2011
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  • 2
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    Effect of Magnetic Resonance Imaging on Internal Magnet Strength in Med‐El Combi 40+ Cochlear Implants
    Wiley ; 2004
    In:  The Laryngoscope Vol. 114, No. 8 ( 2004-08), p. 1355-1361
    Details
    In: The Laryngoscope, Wiley, Vol. 114, No. 8 ( 2004-08), p. 1355-1361
    Abstract: Objective: Magnetic resonance imaging (MRI) has been contraindicated when cochlear implants containing an internal magnet are in place because of concerns regarding torque, force, demagnetization, artifacts, induced voltages, and heating. The objective was to determine the magnetic field strength of Med‐El Combi 40+ cochlear implant internal magnets after MRI studies. Study Design/Methods: Two fresh cadavers were used to study demagnetization using a repeated measures design and a magnetometer. Pre‐ and postMRI measurement of magnetic field strength was completed. Five sets of sagittal T1‐weighted, axial T1‐weighted, and axial T2‐weighted sequences were performed on a cadaver at 0.2 Tesla in the device‐up and device‐down positions. In the other cadaver, 15 sets of sagittal T1‐weighted, axial T1‐weighted, and axial T2‐weighted sequences were performed on a cadaver at 1.5 Tesla were conducted, 5 each with the head oriented at 80, 90, and 100 degrees rotated around the yaw plane (rotated around the z‐axis). Subsequently, three cochlear implant patients completed 0.2 Tesla MRIs. For these patients, subjective and objective assessment of cochlear implant performance was performed. Setting: Academic medical center. Results: In the cadaver studies, analysis of variance showed no significant difference in the magnetic field strength after the 0.2 or 1.5 Tesla scans. There was no significant difference in the magnetic field strength for the three patients undergoing 0.2 Tesla MRIs and no adverse consequences, including no changes in telemetry, auditory sensations, nonauditory sensations, and sound quality. Conclusions: No significant demagnetization of the internal magnet occurred during repeated 1.5 Tesla MRI scans with the head orientations used in this study. In the cochlear implant patients, no significant demagnetization of the internal magnet occurred after a 0.2 Tesla MRI.
    Type of Medium: Online Resource
    ISSN: 0023-852X , 1531-4995
    URL: Article
    DOI: 10.1097/00005537-200408000-00007
    Language: English
    Publisher: Wiley
    Publication Date: 2004
    detail.hit.zdb_id: 2026089-1
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  • 3
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    Electrosurgery after Cochlear Implantation: Eighth Nerve Electrophysiology
    Wiley ; 2004
    In:  The Laryngoscope Vol. 114, No. 12 ( 2004-12), p. 2252-2254
    Details
    In: The Laryngoscope, Wiley, Vol. 114, No. 12 ( 2004-12), p. 2252-2254
    Abstract: Hypothesis: Monopolar electrosurgery below the neck in cochlear implant recipients can be performed without damage to the internal cochlear stimulator, electrode array, and the cochlear nerve. Study Design: Prospective pre‐ and postintervention electrically evoked compound action potential (ECAP) study of cochlear nerve function and behavioral sound perception assessment. Methods: Neural response telemetry (NRT) was used to measure ECAPs before and after the use of monopolar electrosurgery during coronary artery bypass surgery to assess prosthetic device function and electrophysiologic function of the cochlear nerve. In addition, electrode voltage impedances and behavioral sound perception was measured at the same time intervals. Results: ECAPs, behavioral sound perception, and electrode voltage impedances were within the normal range, within compliance, and similar preoperatively and on postoperative day 6. Conclusion: The studies reported herein were a series of measurements designed to test neural integrity and prosthetic device function before and after the use of monopolar electrosurgery. With appropriate precautions, use of monopolar electrosurgery below the neck in cochlear implant recipients can be performed safely.
    Type of Medium: Online Resource
    ISSN: 0023-852X , 1531-4995
    URL: Article
    DOI: 10.1097/01.mlg.0000149469.03575.91
    Language: English
    Publisher: Wiley
    Publication Date: 2004
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  • 4
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    Connexin 26 and Connexin 30 Mutations in Children with Nonsyndromic Hearing Loss
    Wiley ; 2004
    In:  The Laryngoscope Vol. 114, No. 4 ( 2004-04), p. 607-611
    Details
    In: The Laryngoscope, Wiley, Vol. 114, No. 4 ( 2004-04), p. 607-611
    Abstract: Objectives/Hypothesis Mutations in the connexin 26 (Cx26) or gap junction beta 2 gene are the leading cause of hereditary nonsyndromic sensorineural hearing loss in Caucasians. The Cx26 coding region of 68 children with nonsyndromic sensorineural hearing loss was sequenced to determine the frequency and type of Cx26 mutations in this population. Screening was also performed for a common connexin 30 (Cx30) or gap junction beta 6 mutation (del [GJB6‐D13S1830]). Children also underwent audiological testing to determine whether any correlation exists between Cx26 mutations and severity of hearing loss. Study Design In all, 68 children with nonsyndromic sensorineural hearing loss were screened for Cx26 and Cx30 mutations by polymerase chain reaction and direct sequencing. Methods Genomic DNA was amplified by polymerase chain reaction using primers that flank the entire Cx26 coding region. Screening for the 342‐kb Cx30 deletion was performed using primers that amplified the breakpoint junction of the deletion. The amplicons were then sequenced in both directions and analyzed for mutations. Audiometric testing, including pure‐tone audiometry and auditory evoked brainstem response, was also performed to determine the degree of hearing loss. Results Twenty‐seven of 68 children tested had mutations in Cx26 with 35delG being the most prevalent. Ten additional Cx26 mutations were detected including a novel compound heterozygote. Two children were heterozygous for the Cx30 del (GJB6‐D13S1830) mutation. Conclusion Cx26 and Cx30 mutations were present in 41.2% of children tested in the study population. Audiometric data supported previous studies demonstrating a greater degree of hearing loss in subjects who are homozygous for the 35delG mutation.
    Type of Medium: Online Resource
    ISSN: 0023-852X , 1531-4995
    URL: Article
    DOI: 10.1097/00005537-200404000-00003
    Language: English
    Publisher: Wiley
    Publication Date: 2004
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  • 5
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    Online Resource
    Case‐Control Analysis of Cochlear Implant Performance in the Elderly
    Wiley ; 2009
    In:  The Laryngoscope Vol. 119, No. S1 ( 2009-01)
    Details
    In: The Laryngoscope, Wiley, Vol. 119, No. S1 ( 2009-01)
    Type of Medium: Online Resource
    ISSN: 0023-852X , 1531-4995
    URL: Issue
    URL: Article
    DOI: 10.1002/lary.v119.1s
    DOI: 10.1002/lary.20427
    Language: English
    Publisher: Wiley
    Publication Date: 2009
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  • 6
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    Genetics in Otolaryngology: Translational Research
    Wiley ; 2011
    In:  Otolaryngology–Head and Neck Surgery Vol. 145, No. S2 ( 2011-08)
    Details
    In: Otolaryngology–Head and Neck Surgery, Wiley, Vol. 145, No. S2 ( 2011-08)
    Abstract: Recent years have seen great advances in our understanding of the molecular genetic basis of many clinical disorders. Building on the success of the Human Genome Project, new technologies are in development to identify disease‐causing mutations through genetic testing. However, there is a knowledge gap among otolaryngologists regarding which patients should undergo genetics evaluation and how genetic testing should be utilized in the care of patients. The three participants are clinician‐scientists who investigate the genetic factors underlying human disease, and also apply their understanding of genetics to the care of patients. This panel will discuss the state of the art in genetic testing in otolaryngology as well as translation of recent research which will soon be clinically available. Dr Gruber will address cancer genetics, applying his work in cancer in general to head and neck tumors; Dr Runge will discuss the evaluation of auditory neuropathy beyond routine clinical measures and also genetic testing as an example of genetic hearing loss. Dr Lesperance will discuss approaches to genetic evaluation of the pediatric patient, for children with rare genetic disorders, multiple congenital anomalies, and for Mendelian disorders. The emphasis will be on providing the practicing otolaryngologist with the tools to counsel and evaluate their patients with potential genetic disorders. Educational Objectives 1) Describe relationships between genotype and phenotype in auditory neuropathy spectrum disorder. 2) List the most common methods of genetic testing and the risks and benefits of each. 3) Understand the role of the geneticist in evaluating patients with otolaryngologic disorders.
    Type of Medium: Online Resource
    ISSN: 0194-5998 , 1097-6817
    URL: Article
    DOI: 10.1177/0194599811415818a69
    Language: English
    Publisher: Wiley
    Publication Date: 2011
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  • 7
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    Online Resource
    TMTC2 variant associated with sensorineural hearing loss and auditory neuropathy spectrum disorder in a family dyad
    Wiley ; 2018
    In:  Molecular Genetics & Genomic Medicine Vol. 6, No. 4 ( 2018-07), p. 653-659
    Details
    In: Molecular Genetics & Genomic Medicine, Wiley, Vol. 6, No. 4 ( 2018-07), p. 653-659
    Abstract: Sensorineural hearing loss ( SNHL ) is a common form of hearing loss that can be inherited or triggered by environmental insults; auditory neuropathy spectrum disorder ( ANSD ) is a SNHL subtype with unique diagnostic criteria. The genetic factors associated with these impairments are vast and diverse, but causal genetic factors are rarely characterized. Methods A family dyad, both cochlear implant recipients, presented with a hearing history of bilateral, progressive SNHL , and ANSD . Whole‐exome sequencing was performed to identify coding sequence variants shared by both family members, and screened against genes relevant to hearing loss and variants known to be associated with SNHL and ANSD . Results Both family members are successful cochlear implant users, demonstrating effective auditory nerve stimulation with their devices. Genetic analyses revealed a mutation (rs35725509) in the TMTC 2 gene, which has been reported previously as a likely genetic cause of SNHL in another family of Northern European descent. Conclusion This study represents the first confirmation of the rs35725509 variant in an independent family as a likely cause for the complex hearing loss phenotype ( SNHL and ANSD ) observed in this family dyad.
    Type of Medium: Online Resource
    ISSN: 2324-9269 , 2324-9269
    URL: Issue
    URL: Article
    DOI: 10.1002/mgg3.2018.6.issue-4
    DOI: 10.1002/mgg3.397
    Language: English
    Publisher: Wiley
    Publication Date: 2018
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  • 8
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    Online Resource
    Electrophysiologic Effects of Placing Cochlear Implant Electrodes in a Perimodiolar Position in Young Children
    Wiley ; 2004
    In:  The Laryngoscope Vol. 114, No. 1 ( 2004-01), p. 71-76
    Details
    In: The Laryngoscope, Wiley, Vol. 114, No. 1 ( 2004-01), p. 71-76
    Abstract: Objective The purpose of this study was to intraoperatively record the electrically evoked auditory brainstem response (EABR) before and after placement of the electrode positioning system (EPS) (CII Bionic Ear with HiFocus I cochlear implant electrode array) as well as before and after stylet removal (Nucleus Contour cochlear implant electrode array). It was hypothesized that physiologic changes would occur after perimodiolar positioning of the electrode array and these changes would be evident from the EABR recordings. Study Design Consecutive young (11–36 month old) pediatric cochlear implant recipients (n = 17) had intraoperative EABRs recorded from three intracochlear electrodes that represented apical, medial, and basal locations. Wave V amplitudes and thresholds were studied relative to electrode location and pre‐ versus postperimodiolar positioning. These evoked potential measures were analyzed for statistical significance. Setting Tertiary referral children's hospital/medical college. Results Wave V thresholds of the EABR were lower, and amplitudes were larger after perimodiolar positioning, although the changes were dependent on electrode location and implant design. Statistically significant decreases in EABR wave V threshold and increases in suprathreshold wave V amplitude were found for the basal electrode for the CII Bionic Ear HiFocus I and for the apical electrode for the Nucleus Contour. Conclusions Placement of either the CII Bionic Ear HiFocus I or Nucleus Contour cochlear implant electrode array in the perimodiolar position in young children resulted in less electrical current necessary to stimulate the auditory system. Changes in electrophysiologic thresholds and amplitudes, measured with EABR, indicate that the electrode array is placed closer to the modiolus with both electrode designs.
    Type of Medium: Online Resource
    ISSN: 0023-852X , 1531-4995
    URL: Article
    DOI: 10.1097/00005537-200401000-00012
    Language: English
    Publisher: Wiley
    Publication Date: 2004
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  • 9
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    Development of in‐house genetic screening for pediatric hearing loss
    Wiley ; 2020
    In:  Laryngoscope Investigative Otolaryngology Vol. 5, No. 3 ( 2020-06), p. 497-505
    Details
    In: Laryngoscope Investigative Otolaryngology, Wiley, Vol. 5, No. 3 ( 2020-06), p. 497-505
    Abstract: To evaluate the efficiency of in‐house genetic testing for mutations causing the most common types of inherited, nonsyndromic, sensorineural hearing loss (SNHL). Methods Retrospective cohort study of 200 patients at a single, pediatric medical center with suspected or confirmed hearing loss who underwent either send out vs in‐house genetic testing for mutations in GJB2/GJB6, SLC26A4, and MTRNR1. Primary outcome measure was the difference in mean turnaround time for send‐out vs in‐house genetic testing. Additional outcomes included associations between audiometric findings and genetic test results. Results One hundred four send‐out tests were performed between October 2010 and June 2014, and 100 in‐house tests were performed between November 2014 and November 2016. The mean turnaround time for send‐out testing was 53.7 days. The mean turnaround time for in‐house testing was 18.9 days. This difference was statistically significant ( P   〈  .001). The largest component of turnaround time was the amount of time elapsed between receipt of specimen in the lab and final test result. These intervals were 47.0 and 18.3 days for send‐out and in‐house tests, respectively. Notably, the longest turnaround time for in‐house testing (43 days) was less than the average turnaround time for send‐out testing. In addition, we identified two simple audiometric parameters (ie, bilateral newborn hearing screen referral and audiometry showing symmetric SNHL) that may increase diagnostic yield of genetic testing. Conclusions The development of in‐house genetic testing programs for inherited SNHL can significantly reduce testing turnaround times. Newborn hearing screening and audiometry results can help clinicians identify patients most likely to benefit from genetic testing. Level of Evidence IV.
    Type of Medium: Online Resource
    ISSN: 2378-8038 , 2378-8038
    URL: Issue
    URL: Article
    DOI: 10.1002/lio2.v5.3
    DOI: 10.1002/lio2.379
    Language: English
    Publisher: Wiley
    Publication Date: 2020
    detail.hit.zdb_id: 2851702-7
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  • 10
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    Temporal and spectral contributions to musical instrument identification and discrimination among cochlear implant users
    Wiley ; 2016
    In:  World Journal of Otorhinolaryngology - Head and Neck Surgery Vol. 2, No. 3 ( 2016-09), p. 148-156
    Details
    In: World Journal of Otorhinolaryngology - Head and Neck Surgery, Wiley, Vol. 2, No. 3 ( 2016-09), p. 148-156
    Abstract: To investigate the contributions of envelope and fine‐structure to the perception of timbre by cochlear implant (CI) users as compared to normal hearing (NH) listeners. Methods This was a prospective cohort comparison study. Normal hearing and cochlear implant patients were tested. Three experiments were performed in sound field using musical notes altered to affect the characteristic pitch of an instrument and the acoustic envelope. Experiment 1 assessed the ability to identify the instrument playing each note, while experiments 2 and 3 assessed the ability to discriminate the different stimuli. Results Normal hearing subjects performed better than CI subjects in all instrument identification tasks, reaching statistical significance for 4 of 5 stimulus conditions. Within the CI population, acoustic envelope modifications did not significantly affect instrument identification or discrimination. With envelope and pitch cues removed, fine structure discrimination performance was similar between normal hearing and CI users for the majority of conditions, but some specific instrument comparisons were significantly more challenging for CI users. Conclusions Cochlear implant users perform significantly worse than normal hearing listeners on tasks of instrument identification. However, cochlear implant listeners can discriminate differences in envelope and some fine structure components of musical instrument sounds as well as normal hearing listeners. The results indicated that certain fine structure cues are important for cochlear implant users to make discrimination judgments, and therefore may affect interpretation toward associating with a specific instrument for identification.
    Type of Medium: Online Resource
    ISSN: 2095-8811 , 2589-1081
    URL: Article
    DOI: 10.1016/j.wjorl.2016.09.001
    Language: English
    Publisher: Wiley
    Publication Date: 2016
    detail.hit.zdb_id: 2999715-X
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