OBJECTIVE: To identify and evaluate the evidence for the benefits of cochlear implants for people with cognitive impairment or dementia in terms of speech recognition, quality of life, behavioural and psychological symptoms of dementia, cognition, function in daily life, mental well-being, and caregiver burden.
METHODS: Ten electronic databases were searched systematically from inception to December 2023 for studies reporting on outcomes for cochlear implants that included adults identified with cognitive impairment, mild cognitive impairment, or dementia.
RESULTS: Thirteen studies were included in this review with a combined total of 222 cochlear implant patients with cognitive impairment, mild cognitive impairment. Two studies were non-randomised controlled design, the remainder were single group studies, case series or single case studies. Evidence suggested that people with cognitive impairment benefit in terms of improved speech recognition from cochlear implants, although they may benefit less than those with healthy cognition and the degree of benefit depends on the level of cognitive impairment. There was no evidence for increased adverse events among those with cognitive impairment. There was limited or no evidence for any other outcome.
CONCLUSIONS: People with cognitive impairment or dementia do benefit from cochlear implants. To inform policy and clinical practice, further data are needed about the broader benefits of cochlear implants for people with cognitive impairment or dementia, and referral, eligibility, and cochlear implant support needs for people with cognitive impairment and their caregivers.

Cochlear implantation has emerged as a transformative intervention in addressing profound hearing loss, offering a paradigm shift in auditory rehabilitation for individuals with restricted auditory function. Throughout its history, the understanding of contraindications for cochlear implant (CI) surgery has evolved significantly. This review comprehensively analyzes the chronological advancements in the understanding of CI contraindications, examining studies conducted from historical timelines to the present. Recent research has revealed significant developments in the field, prompting a reevaluation of established criteria and resulting in expanded indications for CI. The chronological evolution of contraindications underscores the transformative nature of the field, offering potential improvements in outcomes and enhancing the quality of life for individuals with profound hearing loss. In conclusion, this narrative review emphasizes the dynamic nature of the field, where the reevaluation of contraindications has created new opportunities and broader indications for CI. The emerging prospects, including improved outcomes and enhanced quality of life, hold promise for individuals with profound hearing loss.

OBJECTIVE: To report two cases of bilateral cochlear implantation (CI) in Charcot-Marie-Tooth disease (CMT) patients with novel mutations. Furthermore, we conducted a detailed literature review on the profile and outcomes of CI in this uncommon clinical circumstance.
METHODS: Case 1 involved a 25-year-old woman who was referred for sudden hearing loss (HL) in her left ear and had a 7-year history of HL in her right ear. She was diagnosed with CMT type 1 with a thymidine phosphorylase gene mutation. CI was performed on her left side because her hearing gradually worsened to deafness in both ears. At 3 months post-operation, her speech discrimination score without lip-reading improved from 0 to 100%. She underwent a second CI on her right ear 6 months after her first CI. Two years from her first operation, the speech discrimination score was 100%. Case 2 received her first CI on her right ear at the age of nine for her bilateral HL. She was diagnosed with CMT type 2 with a Twinkle mitochondrial DNA helicase gene mutation. Preoperatively, the speech discrimination score in both ear-aided conditions was 70%. At the 7-year post-operation follow-up, the speech discrimination score was 76%. A second CI was performed due to decreasing hearing ability in her left ear. The speech discrimination score showed 100% at 7 months after the second CI.
CONCLUSIONS: CI is an effective hearing rehabilitation option for CMT patients with severe-to-profound SNHL. Neuro-otologists should consider CI as a treatment option, even though hearing loss in CMT is associated with auditory neuropathy spectrum disease (ANSD).

OBJECTIVE: To evaluate the literature and summarize cochlear implantation (CI) outcomes after intralabyrinthine schwannoma (ILS) excision and tumor observation with CI.
METHODS: OVID Medline, Embase, Web of Science; conception to 2024.
METHODS: A literature review was performed using subject headings, MeSH terms, and keywords. Abstracts and full texts were reviewed by two independent reviewers and adjudicated by a third. Inclusion criteria included studies with ILS and CI with reported audiologic outcomes. Subjects were analyzed into two groups, ILS resection with CI and in situ ILS with CI. Patients with NF2 were included. The main outcome of interest was CI audiometric performance level, with secondary outcomes of CI user status and open-set speech attainment.
RESULTS: There were 29 articles with a total of 93 patients who met inclusion criteria. The resection group had 17% low performers, 44% intermediate performers, and 38% high performers. The in situ group had 40% low performers, 32% intermediate performers, 27% high performers. The resection group had 69 patients with 97% maintaining user status and 92% with open-set speech recognition. The observation group had 24 patients, with 87% user rate and 86% achieving open-set speech recognition. There was a greater percentage of NF2 diagnosis seen in the in situ group.
CONCLUSIONS: There is a paucity of literature on CI and ILS. Patients are managed with both resection of tumor and implantation in situ. Early data are limited, with improvement in hearing outcomes and high user rates in both populations.
METHODS: N/A Laryngoscope, 2024.

UNASSIGNED: To assess outcomes in autoimmune inner ear disease (AIED) after cochlear implantation (CI) through systematic review and meta-analysis.
UNASSIGNED: PubMed, MedLine, Embase, and CINAHL.
UNASSIGNED: Databases were queried for inclusion of AIED patients who underwent CI with outcomes recorded ≥3 months postoperatively. We examined demographics, pure-tone average (PTA), speech perception, preoperative imaging, intraoperative management, and postoperative complications.
UNASSIGNED: Twenty-six articles encompassing 124 patients met inclusion criteria. Mean implantation age was 26.2 years (range 4-65 years) with average length of follow-up at 28.2 months (range 3-120 months). Meta-analysis demonstrated significant improvement in speech perception following CI. There was a statistically significant improvement in speech recognition score (SRS) (standard mean difference [SMD] = 6.5, 95% confidence interval [CI], 4.8-8.0, P < 0.0001) as well as word recognition score (WRS) (SMD = 5.5, 95% CI, 4.2-6.8, P < 0.0001) after CI. Anomalous preoperative radiologic manifestations were reported by 57.7% (15/26) studies. Disease activity-related intraoperative adjustment was noted in 57.7% (15/26) studies; common consequences were cochlear drill-out (53.3%), difficult round window insertion (26.7%), and scala vestibuli insertion (26.7%). Frequent postoperative complications noted in 26.9% (7/26) studies included systemic AIED flares (71.4%) and wound healing delay (42.9%).
UNASSIGNED: Findings of this systematic review of AIED cochlear implant literature demonstrate a lack of consistent reporting standards for PTA and speech perception as well as a lack of long-term follow-up. Despite these findings, meta-analysis suggests that CI is a viable treatment for improving speech perception in AIED patients.

OBJECTIVE: The aim of this study was to provide an updated European narrative review spanning the last decade, focusing on the cost-effectiveness of cochlear implants (CIs) for adults with severe to profound post-lingual hearing loss.
METHODS: This review encompasses both prospective and retrospective approaches, as well as cross-sectional and longitudinal trials conducted on CIs in adults. All studies related to European countries (Austria, Germany, Switzerland, the Netherlands, Sweden, the UK and Poland) were conducted in English and were published between 2012 and June 2023.
RESULTS: Nine studies were included in the analysis. The patients\' ages ranged from 18 years to over 67 years, with sample sizes ranging from 20 to 100 patients; two of these studies were focused on single-sided deafness in adults. The Markov model was identified as the most commonly utilized analysis method.
CONCLUSIONS: This review identified a general consensus on CI cost-effectiveness, despite substantial variability among countries in factors such as observation time horizons, cost-effectiveness thresholds, methods of cost collection, discount rates, CI eligibility criteria and country-specific health systems. Generally, CIs yield positive societal benefits for working-age individuals, potentially less for seniors. Early unilateral CI enhances cost-effectiveness, highlighting the importance of prompt candidate identification. A consistent undersupply of CIs relative to the percentage of potential recipients emerged across countries. Therefore, further investigation into subcategories such as single-sided deafness is warranted, along with country-specific cost analyses. Emphasizing the significance of detailed information on health systems and associated costs and benefits is crucial for facilitating comparisons across different settings.

UNASSIGNED: To describe outcomes after bilateral cochlear implantation (CI) in a patient with a pathologic PTPN11 variant associated with Noonan syndrome (NS) and Noonan syndrome with multiple lentigines (NSML). Additionally, to assess the utility of CI in this specific population based on our outcome and previous reports.
UNASSIGNED: Retrospective case report with literature review using Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines.
UNASSIGNED: A young boy with various multiorgan abnormalities, speech and language delay, and persistent hearing loss who was found to have a heterozygous PTPN11 gene mutation at age 2.
UNASSIGNED: Bilateral tympanostomy tube placement, diagnostic imaging, and eventual staged bilateral CI.
UNASSIGNED: Objective audiometric testing and developmental milestone attainment.
UNASSIGNED: Bilateral CI was successfully completed over a 2-month period. The patient illustrated significant improvement in objective audiologic measurement. However, he continues to sign as his main form of communication without significant speech progression.
UNASSIGNED: Early diagnostic and therapeutic intervention in patients with NS/NSML can help improve long-term audiologic and speech development. Given the heterogeneity of NS/NSML, a multidisciplinary approach is needed for optimal outcomes.

Subtotal petrosectomy (STP) is characterized by obliteration of the middle ear and occlusion of the external auditory canal. The advent of the endoscope has allowed a reduction in morbidity for some conditions such as cholesteatoma and other middle ear disorders, but STP still plays an important role. A retrospective review of medical records and videos of patients who had undergone STP was performed. Perioperative data and images were collected from various clinical cases who had undergone subtotal petrosectomy at our tertiary referral university hospital in Verona. We confronted our experience with a review of the literature to present the main indications for this type of procedure. STP allows a variety of diseases to be managed effectively as it offers the possibility of a definitive healing with radical clearance of temporal bone. Moreover, it can be safely combined with other procedures with a very low complication rate. Although the endoscope represents a revolution in ear surgery, STP, when indicated, is nowadays a surgical option that should be included in the otosurgeon\'s portfolio.

Cochlear implantation, a transformative intervention for individuals with profound hearing loss, has evolved significantly over the years. However, its impact on the vestibular system, responsible for balance and spatial orientation, remains a subject of ongoing research and clinical consideration. This narrative review highlights key aspects of vestibular evaluation in patients undergoing cochlear implantation. Preoperative vestibular assessment is crucial to establish baseline vestibular function and identify any pre-existing balance issues. Various tests, including caloric, rotational chair, vestibular-evoked myogenic potential, and video head impulse tests, play a vital role in evaluating vestibular function. The goal is to assess the risk of vestibular disturbances arising from the surgery, guide surgical planning, and detect pre-existing alterations that could be totally or partially compensated. While some patients experience minimal vestibular disruptions, others may encounter transient or persistent balance issues following cochlear implant surgery. Postoperative vestibular testing allows for the early detection of such disturbances, enabling timely interventions like vestibular rehabilitation and evaluating changes produced due to surgical complications or changes in the patient\'s prior conditions. Challenges in vestibular evaluation include individual variability in patient responses, the proximity of the cochlea to the vestibular system, and the need to tailor testing protocols to individual needs. Further research is essential to refine testing protocols, minimize vestibular disturbances, and improve outcomes for cochlear implant candidates. A multidisciplinary approach involving otolaryngologists, audiologists, and physical therapists is integral to comprehensive patient care in this context. In conclusion, vestibular evaluation in patients undergoing cochlear implantation is critical for optimizing surgical planning, managing postoperative issues, and enhancing the overall quality of life for individuals embarking on the journey of restored hearing.

[This corrects the article DOI: 10.3389/fnins.2022.850245.].