Search

Background: Cochlear implants (CIs) are viable treatment options in patients with severe to profound hearing loss. Speech recognition difficulties were reported in some CI recipients even with a good-aided hearing threshold. The aim of this study was to report a mapping strategy based on different target-aided hearing thresholds to achieve optimal speech recognition and maximize functional outcomes. The safety and efficacy of the mapping strategy were also inspected in the article., Methods: This prospective repeated measures study enrolled 20 adult CI recipients with postlingual deafness using the MED-EL CI system. Word and sentence discrimination assessment and administration of a questionnaire pertaining to comfort level were conducted at the end of each session. The electrophysiological features of the CI mapping were recorded., Results: The correlation between audiometry results and word and sentence recognition was not high. CIs performed best at an audiometry threshold between 25 and 35 dB., Conclusion: CI performance with the best perception relies on a balance between minimizing the hearing threshold and maximizing the dynamic range while maintaining an appropriate comfort level, which was achieved when the target hearing threshold was set at 25-35 dB in this study., Competing Interests: Conflicts of interest: The authors declare that they have no conflicts of interest related to the subject matter or materials discussed in this article., (Copyright © 2022, the Chinese Medical Association.)

Objectives: Recessive variants in the MYO15A gene constitute an important cause of sensorineural hearing impairment (SNHI). However, the clinical features of MYO15A-related SNHI have not been systemically investigated. This study aimed to delineate the hearing features and outcomes in patients with pathogenic MYO15A variants., Design: This study recruited 40 patients with biallelic MYO15A variants from 31 unrelated families. The patients were grouped based on the presence of N-terminal domain variants (N variants). The longitudinal audiological data and for those undergoing cochlear implantation, the auditory and speech performance with cochlear implants, were ascertained and compared between patients with different genotypes., Results: At the first audiometric examination, 32 patients (80.0%) presented with severe to profound SNHI. Patients with at least one allele of the N variant exhibited significantly better hearing levels than those with biallelic non-N variants (78.2 ± 23.9 dBHL and 94.7 ± 22.8 dBHL, respectively) (p = 0.033). Progressive SNHI was observed in 82.4% of patients with non-profound SNHI, in whom the average progression rate of hearing loss was 6.3 ± 4.8 dBHL/year irrespective of the genotypes. Most of the 25 patients who underwent cochlear implantation exhibited favorable auditory and speech performances post-implantation., Conclusions: The hearing features of patients with biallelic pathogenic MYO15A variants are characterized by severe to profound SNHI, rapid hearing progression, and favorable outcomes with cochlear implants. Periodic auditory monitoring is warranted for these patients to enable early intervention., Competing Interests: The authors have no conflicts of interest relevant to this article to disclose., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Objectives: The purpose of this study was to compare the hearing preservation outcomes of patients who received extended versus single-dose steroid therapy in cochlear implant surgery., Design: Case-control., Setting: Tertiary referral centers in Taiwan from April 2017 to 2019., Participants: A total of 70 patients aged 1 to 78 years old (mean = 18.04, standard deviation [SD] = 21.51) who received cochlear implantation via the round window approach were included in the study. Prospectively, 35 cases were enrolled for cochlear implantation with single-dose therapy. Thirty-five controls who underwent cochlear implantation with extended therapy were retrospectively enrolled after frequency matching., Outcome Measures: The main outcome measure was the rate of hearing preservation. This was calculated based on the HEARRING Network formula and results were categorized as complete, partial, and minimal. Impedances served as secondary outcomes., Results: There was no significant difference in the complete hearing preservation rates between the extended and single-dose groups at 6 months postoperatively. Impedances were significantly lower in the extended group after 1 month and 6 months of follow up. When the complete and partial hearing preservation groups were compared, the size of round window opening and speed of insertion were found to be statistically significant., Conclusions: Both extended and single-dose therapies result in good hearing preservation in patients who undergo cochlear implantation. However, better impedances can be expected from patients who received extended therapy. A slower speed of insertion and a widely opened round window play a role in hearing preservation.

Objectives: The purpose of the study is to investigate feasibility of early activation after cochlear implantation by evaluating long-term impedance change and speech perception., Design: Case-control study SETTING: Between July 2015 and December 2016, we prospectively enrolled 20 subjects for early activation (within 24 hours after cochlear implantation). On the other hand, from November 2013 to July 2015, 20 age- and sex-matched control subjects from the database of cochlear implantees treated with conventional activation schedule (4 weeks after surgery) were retrospectively enrolled., Participant: Forty patients who underwent cochlear implantation surgeries., Main Outcome Measures: The series impedance and speech perception score of both groups were compared., Results: No statistical difference in long-term follow-up between the two groups was found using GEEs and multivariate analysis. In the early activation group, impedance reached a steady level by the 2nd postoperative week, and the hearing perception ability significantly improved by the 4th postoperative week., Conclusion: This comparative study illustrated sequential impedance data during early activation (24 hours) and conventional activation (4 weeks) after CI surgery. There were no major complications in either group, and the safety of early activation with respect to impedance changes, postoperative residual hearing preservation and speech perception scores were non-inferior to that of the conventional group. Therefore, in this study, we established the feasibility of early activation 24 hours after cochlear implantation., (© 2019 John Wiley & Sons Ltd.)

Objectives/hypothesis: To explore the genetic characteristics of children with cochlear implants (CIs) and to correlate the auditory performance after implantation to the genetic diagnosis of children with CIs., Study Design: Prospective cohort study., Methods: Mutations of four common deafness-associated genes, GJB2, SLC26A4, the mitochondrial 12S rRNA gene, and OTOF, were screened in 743 unrelated children with idiopathic sensorineural hearing impairment, including 180 and 563 children with and without CIs, respectively. The allele frequencies and audiologic features were compared between both groups. The Categories of Auditory Performance (CAP) scores at 3 years after implantation were then analyzed according to the genotypes., Results: A definitive genetic diagnosis was made in 37 (20.6%) of the 180 CI children. A significant difference in allele frequencies between CI and non-CI children was found in GJB2 mutations (chi-square test, P < .01), but not in SLC26A4 mutations, mitochondrial 12S rRNA mutations, or OTOF mutations (all P > .05). Further analysis revealed that the difference might have resulted from distinct audiological features in each group. Among the 110 CI children who had received more than 3 years of rehabilitation after implantation, the 35 children with mutations had better CAP scores than the 75 children without mutations., Conclusions: A significant prevalence of genetic mutations was identified in children with CIs, suggesting the need for routine genetic assessments. The frequencies of common deafness-associated mutations were different between children with and without CIs. The presence of genetic mutations was associated with an excellent long-term auditory performance outcome after implantation., (Copyright © 2011 The American Laryngological, Rhinological, and Otological Society, Inc.)

In Mandarin Chinese, tonal patterns are lexically meaningful. In a multi-talker environment, competing tones may create interference in addition to competing vowels and consonants. The present study measured Mandarin-speaking cochlear implant (CI) users' ability to recognize concurrent vowels, tones, and syllables in a concurrent-syllable recognition test. Concurrent syllables were constructed by summing either one Chinese syllable each from one male and one female talker or two syllables from the same male talker. Each talker produced 16 different syllables (4 vowels combined with 4 tones); all syllables were normalized to have the same overall duration and amplitude. Both single- and concurrent-syllable recognition were measured in 4 adolescent and 4 adult CI subjects, using their clinically assigned speech processors. The results showed no significant difference in performance between the adolescent and adult CI subjects. With single syllables, mean vowel recognition was 90% correct, while tone and syllable recognition were only 63% and 57% correct, respectively. With concurrent syllables, vowel, tone, and syllable recognition scores dropped by 40-60 percentage points. Concurrent-syllable performance was significantly correlated with single-syllable performance. Concurrent-vowel and syllable recognition were not significantly different between the same- and different-talker conditions, while concurrent-tone recognition was significantly better with the same-talker condition. Vowel and tone recognition were better when concurrent syllables contained the same vowels or tones, respectively. Across the different vowel pairs, tone recognition was less variable than vowel recognition; across the different tone pairs, vowel recognition was less variable than tone recognition. The present results suggest that interference between concurrent tones may contribute to Mandarin-speaking CI users' susceptibility to competing-talker backgrounds., (2009 Elsevier B.V.)

Objective: To evaluate Mandarin-speaking cochlear implant patients' understanding of telephone speech., Design: Telephone speech was simulated by band-limiting broadband speech stimuli (300-3200 Hz) and adding Gaussian noise (35 dB signal-to-noise ratio). Recognition of multitalker vowels, consonants, voice gender, and Chinese tones was measured for both simulated telephone speech and broadband speech in fifteen Mandarin-speaking cochlear implant patients., Results: Results showed no significant difference in Chinese tone recognition scores between broadband and telephone speech. However, mean recognition scores for vowels, consonants and voice gender were significantly lower with telephone speech. The effect of the limited telephone bandwidth on speech recognition was highly variable among subjects. Some subjects were more sensitive to high-frequency speech cues, resulting in a significant drop in performance with band-limited telephone speech, while other subjects were less sensitive to high-frequency speech cues, resulting in similar performance between broadband and band-limited telephone speech., Conclusions: These results suggest that the limited bandwidth negatively affects cochlear implant patients' understanding of telephone speech. Because the effect of band-limited speech was highly variable among subjects, the results also suggest that the contribution of high frequency information to speech recognition may vary significantly among cochlear implant patients. For patients who receive little benefit from high-frequency speech cues, speech processor adjustments may be necessary to access the additional cues provided in broadband speech.

Objective: To evaluate the effects of speech processing strategy on Chinese tone recognition by Nucleus-24 cochlear implant users., Methods: Recognition of Chinese tones was measured with the spectral peak (SPEAK), advanced combination encoders (ACE), and continuous interleaved sampling (CIS) strategies in nine Nucleus-24 cochlear implant users. Recognition of Chinese sentences was also measured with the ACE strategy (1200 pulse-per-sec on each electrode). As the stimulation rate in the SPEAK processor is fixed at 250 pulses per sec per electrode (ppspe), only one stimulation rate was tested with the SPEAK processor. Three stimulation rates (900, 1200, and 1800 ppspe) were tested in the ACE processing conditions. Four stimulation rates (1200, 1800, 2400, and 3600 ppspe) were tested in the CIS processing conditions. Because the number of stimulating electrodes determines the stimulation rate in CIS processors, the number of electrodes was reduced from 12 to 8 to 6 to 4 to achieve stimulation rates of 1200, 1800, 2400, to 3600 pps., Results: Results showed that mean Chinese tone recognition scores with the ACE and CIS strategies were significantly higher than those with the SPEAK strategy. For the ACE strategy, there was no significant difference in performance among the various stimulation rates (for the ACE processing conditions, the stimulation rate was varied while keeping the number of stimulating electrodes constant). For the CIS strategy, there was no significant difference among the various stimulation rates and electrode configurations (for the CIS processing conditions, the stimulation rate was varied by changing the number of stimulating electrodes). There was also a significant correlation between Chinese tone recognition and the recognition of everyday sentences., Conclusions: The latest-generation speech processing strategies were able to provide cochlear implant users with considerable amounts of tonal information. However, Chinese tone recognition was significantly affected by the choice in speech processing strategy. Relatively higher stimulation rates (>900 ppspe) may improve Chinese tone recognition. Furthermore, it may be necessary to develop speech processing strategies to specifically improve tone recognition, which is critical for understanding tonal languages.

Cochlear implantation is the treatment of choice for children with profound sensorineural hearing impairment (SNHI), yet the outcomes of cochlear implants (CI) vary significantly across individuals. To investigate the CI outcomes in pediatric patients with SNHI due to various etiologies, we prospectively recruited children who underwent CI surgery at two tertiary referral CI centers from 2010 to 2021. All patients underwent comprehensive history taking, next generation sequencing (NGS)-based genetic examinations, and imaging studies. The CI outcomes were evaluated using Categories of Auditory Performance (CAP) and Speech Intelligibility Rating (SIR) scores. Of the 160 pediatric cochlear implantees (76 females and 84 males) included in this study, comprehensive etiological work-up helped achieve clinical diagnoses in 83.1% (133/160) of the patients, with genetic factors being the leading cause (61.3%). Imaging studies identified certain findings in 31 additional patients (19.3%). Four patients (2.5%) were identified with congenital cytomegalovirus infection (cCMV), and 27 patients (16.9%) remained with unknown etiologies. Pathogenic variants in the four predominant non-syndromic SNHI genes (i.e., SLC26A4, GJB2, MYO15A, and OTOF) were associated with favorable CI outcomes (Chi-square test, p = 0.023), whereas cochlear nerve deficiency (CND) on imaging studies was associated with unfavorable CI outcomes (Chi-square test, p < 0.001). Our results demonstrated a clear correlation between the etiologies and CI outcomes, underscoring the importance of thorough etiological work-up preoperatively in pediatric CI candidates. [ABSTRACT FROM AUTHOR]

With diverse etiologies and clinical features, the management of pediatric auditory neuropathy spectrum disorder (ANSD) is often challenging, and the outcomes of cochlear implants (CIs) are variable. This study aimed to investigate CI outcomes in pediatric patients with ANSD of different etiologies. Thirty-six children with ANSD who underwent cochlear implantation between 2001 and 2021 were included. Comprehensive etiological analyses were conducted, including a history review, next-generation sequencing-based genetic examinations, and imaging studies using high-resolution computed tomography and magnetic resonance imaging. Serial behavioral and speech audiometry were performed before and after surgery, and the outcomes with CI were evaluated using the Categories of Auditory Performance (CAP) and Speech Intelligibility Rating (SIR) scores. By etiology, 18, 1, 1, and 10 patients had OTOF-related, WFS1-related, OPA1-related, and cochlear nerve deficiency (CND)-related ANSD, respectively. Six patients had no definite etiology. The average CI-aided behavioral threshold was 28.3 ± 7.8 dBHL, and those with CND-related ANSD were significantly worse than OTOF-related ANSD. The patients' median CAP and SIR scores were 6 and 4, respectively. Favorable CI outcomes were observed in patients with certain etiologies of ANSD, particularly those with OTOF (CAP/SIR scores 5–7/2–5), WFS1 (CAP/SIR score 6/5), and OPA1 variants (CAP/SIR score 7/5). Patients with CND had suboptimal CI outcomes (CAP/SIR scores 2–6/1–3). Identifying the etiologies in ANSD patients is crucial before surgery and can aid in predicting prognoses. [ABSTRACT FROM AUTHOR]

Keywords: audit; cochlear implants; communication; diagnosis; genetics; hearing loss; inner ear; outcomes; sensorineural hearing loss EN audit cochlear implants communication diagnosis genetics hearing loss inner ear outcomes sensorineural hearing loss 341 346 6 02/17/22 20220301 NES 220301 Key Points LEOPARD syndrome is a rare hereditary disease characterised by several congenital defects in multiple organs, including sensorineural hearing impairment. The CI outcomes in LEOPARD syndrome patients are influenced by the I PTPN11 i genotypes because different I PTPN11 i mutations confer different pathogenicity on neurocognitive development. Audit, communication, diagnosis, genetics, inner ear, outcomes, sensorineural hearing loss, cochlear implants, hearing loss The authors attributed the unfavourable outcome to the patient's underlying cerebral palsy and late implantation.3 Nierop et al.4 reported another patient with LEOPARD syndrome caused by the I PTPN11 i p.Y279C mutation who received bilateral sequential CI at the ages of 1 years 4 months and 1 years 10 months. [Extracted from the article]

Cochlear implantation (CI) has developed for more than four decades. Initially, CI was used for profound bilateral hearing impairment. However, the indications for CI have expanded in recent years to include children with symptomatic partial deafness. Therefore, CI strategies to preserve residual hearing are important for both patients and otologists. The loss of residual low-frequency hearing is thought to be the result of many factors. All surgical methods have the same goal: protect the delicate intracochlear structures and preserve residual low-frequency hearing to improve speech perception abilities. Fully opening the round window membrane, a straight electrode array, slower insertion speed, and the use of corticosteroids result in a higher rate of hearing preservation. Several factors, like the way of surgical approaches, length of arrays and timing of activation, may not affect the residual hearing preservation. Therefore, the classic atraumatic technique, including the very slow and delicate insertion and administration of intraoperative corticosteroids, can improve hearing outcomes. [ABSTRACT FROM AUTHOR]

Abstract: Objective: This study aimed to assess the static balance function in deaf adolescents with cochlear implants. Methods: We included 24 adolescents who had received unilateral cochlear implantation for at least 5 years. Each subject underwent stabilometry testing under 4 different conditions: (A) firm surface with eyes open; (B) firm surface with eyes closed; (C) foam pad with eyes open; and (D) foam pad with eyes closed. All of them received tests with their cochlear implant turned on and off. Another 24 age- and sex-matched adolescents with normal hearing were tested in the same way for comparison. Sway velocity and circular area were measured and analyzed. Results: The mean sway velocity of the cochlear implant group under conditions A–D was 1.68, 1.98, 2.36, and 5.25cm/s, respectively, and the mean circular area of the cochlear implant group under conditions A–D was 7.39, 6.68, 12.21, and 34.27cm2, respectively. Both of the parameters showed statistical significance between the cochlear implant group and the normal hearing group for conditions A, C and D (p <0.05). Furthermore, there was no significant balance function change among cochlear implant group with their implant “on” and “off”. Conclusions: This study showed that the static balance function in adolescents with long-term use of cochlear implants was worse than those of normal hearing peers. The difference between the cochlear implant group and normal hearing group was the highest when both visual and somatosensory inputs were disrupted. The postural stability was similar whether or not the cochlear implant was activated. [Copyright &y& Elsevier]

This article focuses on the 4th congress of Asia Pacific Symposium on Cochlear Implant and Related Sciences which was held from December 4-6, 2003, in Taipei, Taiwan. This congress provided with a marvelous opportunity to share experiences and update knowledge not only in the technological and surgical aspects of cochlear implantation but also in basic sciences, images, audiology, rehabilitation and education. The academic program included nine keynote lectures, nine panel sessions, 221 free papers and five special workshops, presented to 622 delegates from 26 countries all over the world.