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Purpose The aim of this study was to compare the outcomes of different mapping procedures based on anatomic or default frequency distribution in postlingual deafness adults who underwent cochlear implantation (CI). Methods Forty-eight adults with postlingual deafness who underwent CI (MED-EL) from January 2021 to May 2022 in our hospital were prospectively recruited. The participants were randomly assigned to two groups (the anatomic group and the default group). Postoperative computerized tomography (CT) scans were evaluated with Otoplan ® to determine the angular insertion depth (AID) and the specific locations of the intracochlear electrodes. Anatomic maps were imported into MAESTRO 9.0 software (MED-EL) for anatomy-based fitting for anatomic group, while default mapping program was set up for the default group. Hearing thresholds, Speech Recognition Scores (SRS), and subjects’ auditory and musical abilities were evaluated 1 year after using the CI. Differences were determined in two groups using Stata statistical software, with significance defined as p  < 0.05. Results SRS under noisy conditions was significantly greater for anatomic group than the default group ( p  = 0.02). Under quiet conditions, however, mean hearing thresholds (0.5, 1, 2, and 4 kHz) and SRS did not differ significantly between the two groups ( p  = 0.07). Modified questionnaires showed that auditory ( p  = 0.02) and musical ( p  = 0.01) quality were significantly better following the anatomic mapping than the default procedure. Conclusion CI program based on the anatomic distribution may bring better SRS under noise conditions as well as better auditory and musical qualities than based on the default frequency distribution.

Purpose: Both theoretical models of infant language acquisition and empirical studies posit important roles for attention to speech in early language development. However, deaf infants with cochlear implants (CIs) show reduced attention to speech as compared with their peers with normal hearing (NH; Horn, Davis, Pisoni, & Miyamoto, 2005; Houston, Pisoni, Kirk, Ying, & Miyamoto, 2003), which may affect their acquisition of spoken language. The main purpose of this study was to determine (a) whether infant-directed speech (IDS) enhances attention to speech in infants with CIs, as compared with adult-directed speech (ADS), and (b) whether the degree to which infants with CIs pay attention to IDS is associated with later language outcomes. Method: We tested 46 infants--12 prelingually deaf infants who received CIs before 24 months of age and had 12 months of hearing experience (CI group), 22 hearing experience-matched infants with NH (NH-HEM group), and 12 chronological age-matched infants with NH (NH-CAM group)--on their listening preference in 3 randomized blocks: IDS versus silence, ADS versus silence, and IDS versus ADS. We administered the Preschool Language Scale--Fourth Edition (PLS-4; Zimmerman, Steiner, & Pond, 2002) approximately 18 months after implantation to assess receptive and expressive language skills of infants with CIs. Results: In the IDS versus silence block, all 3 groups looked significantly longer to IDS than to silence. In the ADS versus silence block, both the NH-HEM and NH-CAM groups looked significantly longer to ADS relative to silence; however, the CI group did not show any preference. In the IDS versus ADS block, whereas both the CI and NH-HEM groups preferred IDS over ADS, the NH-CAM group looked equally long to IDS and ADS. IDS preference quotient among infants with CIs in the IDS versus ADS block was associated with PLS-4 Auditory Comprehension and PLS-4 Expressive Communication measures. Conclusions: Two major findings emerge: (a) IDS enhances attention to speech in deaf infants with CIs; (b) the degree of IDS preference over ADS relates to language development in infants with CIs. These results support a focus on input in developing intervention strategies to mitigate the effects of hearing loss on language development in infants with hearing loss.

Objectives To determine the Incremental Cost-Utility Ratio (ICUR) of cochlear implantation in the treatment of adult patients with single-sided deafness (SSD) and asymmetric hearing loss (AHL). Methods This prospective multicenter pragmatic study including a randomized controlled trial (RCT) enrolled 155 subjects with SSD or AHL. Subjects chose a treatment option between: abstention, Contralateral Routing Of the Signal hearing aids, Bone Conduction Device or Cochlear Implant (CI). Participants who opted for CI were then randomized between two arms: “immediate CI” where the cochlear implantation was performed within one month and “initial observation” where subjects were first observed. The ICUR of CI was determined at 6 months follow-up by comparing the two arms. Utility was measured using EuroQoL- 5 dimensions (EQ-5D), to calculate the gain in Quality-Adjusted Life Years (QALY). Individual costs were extracted from the French National Health Insurance database. A Markovian MultiState (MMS) model assessed the ICUR evolution over the lifetime horizon. Results Among the 155 included participants, 51 opted for a CI and were randomized. For a 6 months follow-up period, the ICUR was €422,279/QALY gained after CI. Using the MMS model, the ICUR of CI decreased to €57,561/QALY at 10 years follow-up, €38,006/QALY at 20 years, and dropped to €26,715 at 50 years. In the participants with severe tinnitus, mean ICUR was €31,105/QALY at 10 years. Conclusions CI can be considered as an efficient treatment in SSD and AHL from 20 years follow-up in the global population, and before 10 years follow-up in patients with severe associated tinnitus.

Children who are deaf or hard of hearing (DHH) and who use hearing aids or cochlear implants are more likely than their peers with typical hearing to exhibit behavior problems. Although multiple evidence-based interventions for child behavior problems exist, they are rarely delivered to children who are DHH, and no rigorous randomized controlled trials have been conducted to determine their effects with this population. This protocol describes a study aiming to test the effectiveness of an evidence-based behavioral parent training intervention adapted for parents of young children who are DHH and simultaneously to assess key implementation outcomes and multilevel contextual factors influencing implementation. The protocol for a Type 1 hybrid effectiveness-implementation trial of a behavioral parent training intervention for parents of young children who are DHH is presented, including details of the study design, participants, assessments, and analyses. Using a stakeholder-engaged, mixed-methods approach, we will test the effects of the intervention versus treatment as usual on parenting behaviors, child behaviors, and a range of secondary effectiveness outcomes, including adherence to using hearing aids and cochlear implants as well as measures of child speech and language. We will assess the acceptability, feasibility, fidelity, and costs of the intervention from the perspectives of peer coaches who deliver the intervention, hearing health care clinicians (including audiologists and speech-language pathologists), and administrators of programs serving young children who are DHH. Results of this trial will inform future efforts to close the gap between prevalence of behavioral problems in young children who are DHH and access to and use of evidence-based interventions to prevent and treat them. If effective, this intervention could be widely implemented using strategies informed by the findings of this study to benefit young children who are DHH and followed in hearing health care and their families.

The objective of this study was to compare the stability, survival, and tolerability of 2 percutaneous osseointegrated titanium implants for bone conduction hearing: a 4.5-mm diameter implant (test) and a 3.75-mm diameter implant (control). Fifty-seven adult patients were included in this randomized controlled clinical trial. Sixty implants were allocated in a 2:1 (test–control) ratio. Follow-up visits were scheduled at 7, 14, 21, and 28 days; 6 and 12 weeks; and 6 months. At every visit, implant stability quotient (ISQ) values were recorded by means of resonance frequency analysis (RFA) and skin reactions were evaluated according to the Holgers classification. Implants were loaded with the bone conduction device at 3 weeks. Hearing-related quality of life was evaluated using the Abbreviated Profile of Hearing Aid Benefit (APHAB), the Glasgow Benefit Inventory (GBI), and the Glasgow Health Status Inventory (GHSI). ISQ values were statistically significantly higher for the test implant compared to the control implant. No implants were lost and soft tissue reactions were comparable for both implants. Positive results were reported in the hearing-related quality of life questionnaires. These 6-month results indicate that both implants and their corresponding hearing devices are safe options for hearing rehabilitation in patients with the appropriate indications. Loading at 3 weeks did not affect the stability of either implant.

Objective We aimed to explore the educational outcome and influencing factors of ongoing verbal rehabilitation training together with inclusive education among prelingually deaf children with a cochlear implant. Methods Prelingually deaf children who underwent cochlear implantation, rehabilitation, and had inclusive education placement were randomly divided into two groups: one group received continuous verbal rehabilitation training under inclusive education status; the other group did not receive this training. Speech discrimination scores were determined. Results Among 60 included children, subjectively perceived academic adaptability, peer relations, initiative communication, and teacher’s involvement under inclusive education, as well as speech discrimination scores, were all significantly different between groups. Continuous verbal rehabilitation training influenced the subjective perception of children and resulted in higher speech discrimination scores and more positive subjective perception. Subjective perception was not significantly correlated with chronological age, sex, age at the time of cochlear implantation, or duration of inclusive education. Conclusion Ongoing verbal rehabilitation training within inclusive education can largely improve the education placement outcomes of prelingually deaf children with cochlear implants.

 The aim of this study was to compare the static and dynamic balance performance of deaf children with and without cochlear implants. This is a cross-sectional study of 145 school children, aged between 7 and 12 years comprising 85 children with congenital or early acquired bilateral profound sensorineural hearing loss (the hearing loss group) and 60 normal hearing aged-matched control counterparts were assessed using the balance subtest of Bruininks-Oseretsky test of Motor Proficiency (BOTMP). The hearing loss group, 50 without cochlear implants (the non-implant group) and 35 of them with unilateral cochlear implants (the implant group) were recruited from schools for the deaf and normal hearing children (the control group) randomly selected from two randomly selected elementary schools of Tehran city. The scores were analyzed using one-way ANOVA. The total score of deaf children especially the implant group were significantly lower than the control group )P<0.001). The balance performance of the control group was better than the implant group in all of the items as well as the non-implant group except the fourth tested item (walking forward on a line) (P<0.05). The balance score of the implant group was significantly lower than the non-implant group except for the third tested item (standing on the preferred leg on a balance beam with eyes closed). The findings suggested that deaf children, specifically those with cochlear implants are at risk for motor and balance deficits. Thus, vestibular and motor evaluations, as well as interventions to improve balance and motor skills, should be prioritized for this population.

This study aimed to investigate whether adults with cochlear implants benefit from a change of fine structure (FS) coding strategies regarding the discrimination of prosodic speech cues, timbre cues, and the identification of natural instruments. The FS processing (FSP) coding strategy was compared to 2 settings of the FS4 strategy. A longitudinal crossover, double-blinded study was conducted. This study consisted of 2 parts, with 14 participants in the first part and 12 participants in the second part. Each part lasted 3 months, in which participants were alternately fitted with either the established FSP strategy or 1 of the 2 newly developed FS4 settings. Participants had to complete an intonation identification test; a timbre discrimination test in which 1 of 2 isolated cues changed, either the spectral centroid or the spectral irregularity; and an instrument identification test. A significant effect was seen in the discrimination of spectral irregularity with 1 of the 2 FS4 settings. The improvement was seen in the FS4 setting in which the upper envelope channels had a low stimulation rate. This improvement was not seen with the FS4 setting that had a higher stimulation rate on the envelope channels. In general, the FSP strategy and the 2 settings of the FS4 strategy provided similar levels in the perception of prosody and timbre cues, as well as in the identification of instruments.

Controversy regarding functional reorganization in the adult brain remains. To investigate whether neuroplasticity is present in adults with postlingual deafness, we examined the pattern of cerebral glucose metabolism on (18)F-FDG brain PET images of postlingually deaf patients by comparing the auditory cortical activation pattern with those of age- and sex-matched healthy control subjects. We also correlated the cerebral glucose metabolism in deaf patients with the duration of deafness using statistical parametric mapping. In the resting state (eye closed, ears unoccluded in a dark and quiet environment), (18)F-FDG brain PET scans were performed on 9 postlingually deaf patients and 9 age- and sex-matched healthy volunteers. Significant increases and decreases of regional cerebral metabolism in the patient group were estimated by comparing their PET images with those of the healthy volunteers using t statistics at every voxel. To reveal regions in which metabolism was significantly correlated with the duration of deafness, the general linear model with the duration of deafness as a covariate was tested at each voxel. When we compared (18)F-FDG brain PET images of postlingually deaf patients with those of age- and sex-matched healthy control subjects by performing a t test at every voxel, the glucose metabolism of deaf patients was significantly (P < 0.001) lower than that of the control subjects in both anterior cingulate gyri (Brodmann area 24 [BA24]) and superior temporal cortices (BA41, BA42) and in the right parahippocampal gyrus. No area showed a significant increase of metabolism in deaf patients with the same threshold. When we correlated glucose metabolism of deaf patients with the duration of deafness after total deprivation of hearing capability using a general linear model with the duration of deafness as a covariate at every voxel, metabolism in both anterior cingulate gyri (BA24) and superior temporal cortices (BA41, BA42) showed a significant (P < 0.005) positive correlation with the duration of deafness. This study suggests that plasticity is present in adult brains of postlingually deaf patients. In the mature brain, auditory deprivation decreased neuronal activity transiently in primary auditory and auditory-related cortices, and, over time, functional reorganization likely takes place in the auditory cortex. Plasticity was prominent in superior temporal and anterior cingulate gyri in the sensory-deprived mature brain and militated against postimplantation improvement in patients with cochlear implants.

GJB2 gene mutations are highly prevalent in pre-lingual hearing loss patients from China. Pre-lingual deafness is a sensorineural disorder that can only be treated with cochlear implantation. The prevalence of GJB2 gene mutations was examined in 330 randomly selected patients treated with cochlear implantation. Overall, 276 patients (83.64 per cent) carried variations in the GJB2 gene. Seventeen different genotypes were identified, including 10 confirmed pathogenic mutations (c.235delC, c.299delAT, c.176del16, p.E47X, p.T123N, p.V167M, p.C218Y, p.T86R, p.V63L and p.R184Q), 3 polymorphisms (p.V27I, p.E114 G and p.I203 T) and 2 unidentified mutations (p.V37I and c.571 T > C). A total of 103 patients (31.2 per cent) carried 2 confirmed pathogenic mutations. The frequency of c.235delC was higher than that reported previously in the Jiangsu province. The two novel mutations identified, 69C > G and 501G > A, are likely to be polymorphisms.