OBJECTIVE: To assess the influence of three factors using retrospective chart review: age at which 2nd cochlear implant (CI) is implanted, prior hearing aid (HA) experience in the 2nd CI ear, and long-term experience with bilateral cochlear implants (BICIs) on sound localization in children with sequential BICIs.
METHODS: Mean absolute error (MAE) in localizing speech noise of 60 children with sequential BICIs was compared across four age groups of the 2nd CI (1-5.0; 5.1-10.0; 10.1-14.0; & 14.1-19.0 years) and two extents of prior HA experience (more than and less than one year). MAE was also longitudinally analyzed after 4-6 years of experience with BICI involving 18 participants out of 60.
RESULTS: Children who received 2nd CI before five years of age demonstrated significantly better localization than those who received it after ten years of age. More than one year of prior HA experience in the 2nd CI ear and extensive experience with sequential BICIs significantly enhanced localization performance. Inter-implant intervals and age at the 2nd CI showed a significant positive correlation with the MAE (poorer localization).
CONCLUSIONS: The results indicate that age at 2nd CI is important in developing sound localization skills. Based on the results, obtaining 2nd CI within the first five years of life and no later than ten years old is recommended. The results also suggest that longer use of amplification before 2nd CI and prolonged BICI experience significantly fosters localization development.

This study investigated sound localization abilities in patients with bilateral conductive and/or mixed hearing loss (BCHL) when listening with either one or two middle ear implants (MEIs). Sound localization was measured by asking patients to point as quickly and accurately as possible with a head-mounted LED in the perceived sound direction. Loudspeakers, positioned around the listener within a range of +73°/-73° in the horizontal plane, were not visible to the patients. Broadband (500 Hz-20 kHz) noise bursts (150 ms), roved over a 20-dB range in 10 dB steps was presented. MEIs stimulate the ipsilateral cochlea only and therefore the localization response was not affected by crosstalk. Sound localization was better with bilateral MEIs compared with the unilateral left and unilateral right conditions. Good sound localization performance was found in the bilaterally aided hearing condition in four patients. In two patients, localization abilities equaled normal hearing performance. Interestingly, in the unaided condition, when both devices were turned off, subjects could still localize the stimuli presented at the highest sound level. Comparison with data of patients implanted bilaterally with bone-conduction devices, demonstrated that localization abilities with MEIs were superior. The measurements demonstrate that patients with BCHL, using remnant binaural cues in the unaided condition, are able to process binaural cues when listening with bilateral MEIs. We conclude that implantation with two MEIs, each stimulating only the ipsilateral cochlea, without crosstalk to the contralateral cochlea, can result in good sound localization abilities, and that this topic needs further investigation.

OBJECTIVE: In prior work using non-speech stimuli, children with hearing loss show impaired perception of binaural cues and no significant change in cortical responses to bilateral versus unilateral stimulation. Aims of the present study were to: 1) identify bilateral responses to envelope and spectral components of a speech syllable using the frequency-following response (FFR), 2) determine if abnormalities in the bilateral FFR occur in children with hearing loss, and 3) assess functional consequences of abnormal bilateral FFR responses on perception of binaural timing cues.
METHODS: A single-syllable speech stimulus (/dα/) was presented to each ear individually and bilaterally. Participants were 9 children with normal hearing (MAge = 12.1 ± 2.5 years) and 6 children with bilateral hearing loss who were experienced bilateral hearing aid users (MAge = 14.0 ± 2.6 years). FFR temporal and spectral peak amplitudes were compared between listening conditions and groups using linear mixed model regression analyses. Behavioral sensitivity to binaural cues were measured by lateralization responses as coming from the right or left side of the head.
RESULTS: Both temporal and spectral peaks in FFR responses increased in amplitude in the bilateral compared to unilateral listening conditions in children with normal hearing. These measures of \"bilateral advantage\" were reduced in the group of children with bilateral hearing loss and associated with decreased sensitivity to interaural timing differences.
CONCLUSIONS: This study is the first to show that bilateral responses in both temporal and spectral domains can be measured in children using the FFR and is altered in children with hearing loss with consequences to binaural hearing.

OBJECTIVE: To determine the cost-effectiveness or cost-utility of cochlear implants (CI) in adults with severe to profound bilateral hearing loss.
UNASSIGNED: PubMed (Medline), The Cochrane Library, Embase via Elsevier, EBSCOhost CINAHL, and Scopus.
METHODS: The study included adult participants with severe to profound bilateral neurosensory hearing loss. The analysis encompassed quality of life improvements, costs, cost-effectiveness, cost-utility, cost-benefit, quality-adjusted life year (QALY), incremental cost-effectiveness ratio (ICER), and incremental cost-utility ratio (ICUR). Systematic reviews, meta-analyses, case series, and prospective or retrospective cohort studies published in English between 2010 and 2023 were retrieved. Exclusion criteria included incomplete studies, abstracts, clinical cases, editorials, letters, studies involving pediatric populations, single-side deafness, methodology research, noneconomic aspects of CI, mixed child and adult data, and studies published before 2010. The risk of bias was assessed following the criteria outlined in Appendix I of the economic evaluation\'s quality assessment as per the NICE Guideline Development Method.
RESULTS: Ten articles met the criteria and were included in the qualitative synthesis. One study conducted a prospective cost-utility analyses, one carried out a cost-benefit analyses, one was a randomized controlled clinical trial focusing on cost-utility, and another was a clinical trial addressing cost-effectiveness. Six studies employed Markov models, and one study utilized uniquely the Monte Carlo method. None quantified the economic impact of improved hearing on cognitive function.
CONCLUSIONS: The heterogeneity of sources impacted data quality. Unilateral and sequential bilateral CI appeared to be cost-effective when compared with bilateral hearing aids or nontechnological support. When properly indicated, simultaneous bilateral CIs are cost-effective compared to no interventions and to unilateral cochlear implantation through differential discounting or variations from the base cases, especially with a life expectancy of 5-10 years or longer.

UNASSIGNED: Children with profound hearing loss (HL) and vestibular impairment have worse cochlear implant outcomes compared with those without vestibular impairment. However, the decision for cochlear implantation is rarely based on vestibular function assessment as a complement to audiologic testing.
UNASSIGNED: To identify the prevalence of vestibular impairment according to HL origin and to assess the association between vestibular impairment and delayed posturomotor development in children with profound HL.
UNASSIGNED: This cohort study was conducted in a pediatric referral center for cochlear implantation in Paris, France, using medical records data on HL origin, vestibular assessment, and ages of developmental milestone achievement. The cohort included children with profound HL (loss >90 dB HL) who completed vestibular assessment prior to cochlear implantation between January 1, 2009, and December 31, 2019. Data analyses were conducted between January and June 2023.
UNASSIGNED: The primary outcome was prevalence of vestibular impairment according to HL origin. Children were classified into 3 groups according to their responses to vestibular testing: normal vestibular function (NVF), partially impaired vestibular function (PVF), and complete bilateral vestibular loss (CBVL). Generalized logit models were performed to evaluate the association between vestibular impairment and causes of HL as well as posturomotor development delay.
UNASSIGNED: A total of 592 children were included (308 males [52.0%]; mean [SD] age, 38 [34] months). In children with documented HL origin (n = 266), 45.1% (120) had HL with genetic origin, 50.0% of which were syndromic (mainly Usher and Waardenburg syndromes) and 50.0% were nonsyndromic (mainly associated with connexin 26). Among patients with infectious HL origin (n = 74), 70.3% (52) had cytomegalovirus (CMV) infection. Vestibular impairment was found in 44.4% (263 of 592) of the children; it was mostly symmetrical in 88.9% (526) and was CBVL in 5.7% (34) of the cases. Vestibular impairment was present in 78.3% (47) of children with genetic syndromic HL (56.7% [34] with PVF; 21.7% [13] with CBVL) and in 69.2% (36) of children with CMV infection (57.7% [30] with PVF; 11.5% [6] with CBVL). Genetic syndromic HL origin was found to be more often associated with both PVF and CBVL than other HL causes. The odds of having delays in 4 developmental milestones (head holding, sitting, standing with support, and independent walking) were higher in both PVF and CBVL (eg, head-holding odds ratios: 2.55 and 4.79) compared with NVF, and the age of achieving these milestones was higher in CBVL than PVF (eg, head holding: 7.33 vs 4.03 years; P < .001). All 4 developmental milestones were associated with the degree of vestibular impairment.
UNASSIGNED: This cohort study found that among children with profound HL, vestibular impairment was prevalent, varied according to HL origin, and associated with posturomotor development; while all developmental milestones were associated with vestibular impairment severity, not all HL causes were associated with vestibular impairment severity. Children with profound HL may benefit from complete vestibular assessment before cochlear implantation, which would support early and adapted management, such as physical therapy for CBVL and cochlear implantation strategy.

This study aimed to investigate the role of hearing aid (HA) usage in language outcomes among preschool children aged 3-5 years with mild bilateral hearing loss (MBHL). The data were retrieved from a total of 52 children with MBHL and 30 children with normal hearing (NH). The association between demographical, audiological factors and language outcomes was examined. Analyses of variance were conducted to compare the language abilities of HA users, non-HA users, and their NH peers. Furthermore, regression analyses were performed to identify significant predictors of language outcomes. Aided better ear pure-tone average (BEPTA) was significantly correlated with language comprehension scores. Among children with MBHL, those who used HA outperformed the ones who did not use HA across all linguistic domains. The language skills of children with MBHL were comparable to those of their peers with NH. The degree of improvement in audibility in terms of aided BEPTA was a significant predictor of language comprehension. It is noteworthy that 50% of the parents expressed reluctance regarding HA use for their children with MBHL. The findings highlight the positive impact of HA usage on language development in this population. Professionals may therefore consider HAs as a viable treatment option for children with MBHL, especially when there is a potential risk of language delay due to hearing loss. It was observed that 25% of the children with MBHL had late-onset hearing loss. Consequently, the implementation of preschool screening or a listening performance checklist is recommended to facilitate early detection.

OBJECTIVE: To compare changes in music appreciation after cochlear implant (CI) surgery for patients with bilateral and single-sided deafness (SSD).
METHODS: A retrospective cohort study was performed on all adult CI unilateral or bilateral recipients from November 2019 to March 2023. Musical questionnaire subset data from the Cochlear Implant Quality of Life (CIQOL) - 35 Profile Instrument Score (maximum raw score of 15) was collected. Functional CI assessment was measured with CI-alone speech-in-quiet (SIQ) scores (AzBio and CNC).
RESULTS: 22 adults underwent CI surgery for SSD and 21 adults for bilateral deafness (8 sequentially implanted). Every patient group had clinically significant improvements (p < 0.001) in mean SIQ scores in the most recently implanted ear (Azbio (% correct) SSD: 14.23 to 68.48, bilateral: 24.54 to 82.23, sequential: 6.25 to 82.57). SSD adults on average had higher music QOL scores at baseline (SSD: 11.05; bilateral: 7.86, p < 0.001). No group had significant increases in raw score at the first post-operative visit (SSD: 11.45, p = 0.86; bilateral: 8.15, p = 0.15). By the most recent post-implantation evaluation (median 12.8 months for SSD, 12.3 months for bilateral), SSD adults had a significant increase in raw score from baseline (11.05 to 12.45, p = 0.03), whereas bilaterally deafened (7.86 to 9.38, p = 0.12) adults had nonsignificant increases.
CONCLUSIONS: SSD patients demonstrate higher baseline music appreciation than bilaterally deafened individuals regardless of unilateral or bilateral implantation and are more likely to demonstrate continued improvement in subjective music appreciation at last follow-up even when speech perception outcomes are similar.

BACKGROUND: Wernicke\'s encephalopathy (WE) is an acute neurological syndrome resulting from thiamine (vitamin B1) deficiency. It has been recognized increasingly in non-alcoholic patients, such as in the condition of malnutrition. Recent literature has shed light on uncommon symptoms and neuroimaging findings.
METHODS: We reported a case of a 44-year-old male who initially presented with bilateral hearing loss, and exhibited abnormality in the splenium of the corpus callosum on magnetic resonance imaging (MRI) diffusion-weighted imaging sequence. On the following day the patient developed new symptoms, including unstable walking, double vision and hallucination. The subsequent brain MRI demonstrated lesions involving periaqueductal grey matter and bilateral medial thalamus, indicating the diagnosis of WE. Empirical treatment with intravenous thiamine resulted in complete clinical and radiological resolution.
CONCLUSIONS: To the best of our knowledge, the current case is the first report of WE in literature with uncommon but reversible manifestations. This case warns us to maintain a heightened level of suspicion for WE in malnourished patients with neurological deficits, despite the possibility of atypical presentations encompassing bilateral hearing disturbances and unusual neuroradiological results. Early diagnosis and timely administration of thiamine in WE are likely to lead to a favorable outcome and full recovery.

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OBJECTIVE: To comprehensively examine the characteristics and prognosis of bilateral sudden sensorineural hearing loss (BSSHL) and its subtypes compared to unilateral sudden sensorineural hearing loss (USSHL).
METHODS: PubMed, Scopus, and CINAHL.
METHODS: Databases were searched from inception to December 5, 2023, for studies reporting patient characteristics and audiometric outcomes for BSSHL and its simultaneous (Si-BSSHL) and sequential (Se-BSSHL) subtypes. Meta-analysis of continuous measures, proportions (%), mean differences (Δ), and odds ratio (OR) were performed.
RESULTS: Eleven studies were included, consisting of 368 patients with BSSHL and 2,705 patients with USSHL. The pooled prevalence among all SSHL cases was 88.1% (95% CI: 81.2%-93.6%) for USSHL and 11.9% (95% CI: 6.4% to 18.8%) for BSSHL. PTA improvement following treatment with steroids was significantly worse in patients with BSSHL (Δ15.3 dB; 95% CI: 14.6 to 15.9; p < 0.0001) compared to patients with USSHL. There was no significant difference in post-treatment PTA improvement between the BSSHL subtypes. Patients with Si-BSSHL were significantly less likely to have an idiopathic etiology (OR: 0.4; 95% CI: 0.2 to 0.8; p = 0.01) and significantly more likely to have an autoimmune disease etiology (OR: 27.4; 95% CI: 2.2 to 336.1; p = 0.01), comorbid cardiovascular disease (OR: 2.3; 95% CI: 1.1 to 5.1; p = 0.03), and comorbid hypertension (OR: 2.5; 95% CI: 1.6 to 3.8; p < 0.0001) compared to patients with USSHL.
CONCLUSIONS: BSSHL is a considerably rarer form of SSHL with worse prognosis compared to USSHL. BSSHL, and Si-BSSHL in particular, has significantly greater associations with systemic pathologies compared to USSHL. Laryngoscope, 134:3883-3891, 2024.