Purpose: The purpose of this study was to evaluate the clinical and audiological outcomes in long-term cochlear implant (CI) users, focusing on hearing performance and device failures. Methods: A retrospective study was conducted on patients who underwent CI surgery, with a minimum follow-up of 10 years. Device survival was analyzed using Kaplan–Meier curves, and failure rates were classified as device failure explants or medical-related explants. The time to revision and causes of reimplantation were assessed. Statistical analyses included Pearson correlation for audiological outcomes, Student’s t-test for group comparisons, and the log-rank test for survival comparisons among implant brands. Results: In this study, data from a total of 211 CIs were collected. Fourteen major postoperative complications were reported in this series, resulting in a global major complication rate of 6.6%: 5.2% due to hard failure and 1.4% due to medical problems and soft failure. The revision rate was 4.1% for the children’s group and 10% for the adult group. The overall device survival rates were more than 96% at 10 years and 91% at 20 years. The median postoperative follow-up was 16.3 years. Moreover, a highly significant correlation was observed (r = 0.669, p < 0.0001) between pure tone average (PTA) thresholds at implant activation and those at the last follow-up, as analyzed using Pearson’s correlation coefficient. Conclusions: The CI is a lifelong device; however, the technology is constantly evolving. Therefore, careful patient counseling (or counseling of parents in the case of pediatric patients) is necessary. The device may wear out over time, requiring revision surgery. These data are essential for audiologists and ENT specialists when selecting patients and determining surgical indications.

Background: Beckwith–Wiedemann syndrome (BWS) is a rare congenital overgrowth disorder caused by genetic and epigenetic alterations on chromosome 11p15.5. While macroglossia, abdominal wall defects, and tumor predisposition are well recognized, hearing impairment has been sporadically reported. Objectives: The aim of this study is to review audiological features, surgical management, and rehabilitation in BWS, and we additionally present three cases with comprehensive longitudinal audiological follow-up. Methods: A systematic review of PubMed and Scopus was conducted according to PRISMA guidelines, including studies reporting audiological findings in patients with confirmed BWS. Studies without audiological data or reporting only normal-hearing patients were excluded. Data on hearing loss type, severity, genetics, clinical features, imaging, surgical interventions, and outcomes were extracted. A narrative synthesis was conducted; no meta-analysis was performed due to the heterogeneity and limited number of available studies. Data extraction was performed independently by two reviewers who independently screened titles, abstracts, and full texts, with disagreements resolved by discussion. In addition, three original case reports from our institution were included to further illustrate the clinical and rehabilitative variability of hearing impairment in BWS. Results: We identified 40 patients from the review, but only 12 of them reported audiological data (e.g., hearing thresholds, type of hearing loss, or diagnostic tests). Ossicular chain anomalies, particularly stapes fixation, were frequently observed. Surgical management improved hearing in selected cases, while bone conduction devices (BCD) or conventional amplification were effective alternatives when surgery was contraindicated. Genetic analyses revealed CDKN1C mutations or imprinting defects in nine patients. Conclusions: Hearing impairment in BWS is clinically relevant and often conductive, likely related to middle-ear anomalies. Early, multidisciplinary audiological evaluation—including imaging when indicated—and individualized rehabilitation can optimize auditory and communicative outcomes. The evidence is limited by the small number of studies and heterogeneous reporting of audiological outcomes.

Background/Objectives: Ichthyosis refers to a group of genetic disorders characterized by extensive scaling of the skin. Syndromic ichthyosis, such as KID syndrome, is associated with mutations in connexin 26, resulting in a triad of keratosis, ichthyosis, and deafness. Cochlear implant (CI) is considered an effective rehabilitation option for severe hearing loss in these patients, though challenges related to skin complications may arise. This study aims to systematically review the existing literature to evaluate the effectiveness of CI in patients with ichthyosis, focusing on auditory and communicative abilities. Methods: A comprehensive literature search was conducted across PubMed, Scopus, and Web of Science databases according to the PRISMA statement. Studies were included based on the presence of genetically confirmed ichthyosis patients who underwent CI. Results: A total of 29 studies were identified, of which 11 met the inclusion criteria, encompassing 47 patients. Genetic analysis revealed GJB2 mutations in 40 patients, with a prevalence of the c.148G>A (D50N) mutation. All patients experienced sensorineural hearing loss, predominantly severe to profound. CI was performed in all patients, with significant improvements in speech discrimination and auditory thresholds (89.4%). Complications post-implant were noted in 78.6% of cases, primarily involving wound infections and dehiscence. Conclusions: Despite the potential for significant complications, the overall outcomes suggest that CI can markedly enhance the quality of life of subjects. Multidisciplinary approaches and careful surgical planning are crucial to managing these patients effectively. Future research should aim for larger sample sizes and extended follow-up periods to further understand CI outcomes in this population.

Background: Narrow duplicated internal auditory canal (IAC) is a rare congenital malformation frequently associated with severe-to-profound sensorineural hearing loss. Case Presentation: We present a one-year-old girl with bilateral narrow duplicated IAC and profound hearing loss evaluated through CT/MRI and electrically evoked auditory brainstem response (EABR). Methods: We conducted a systematic review (1990–2023), identifying 59 published cases of which 24 were bilateral. The mean age at diagnosis was 10.34 years, and 25 cases presented additional inner ear malformations. Only seven patients underwent cochlear implantation, and EABR was performed in four cases. Outcomes of cochlear implantation were heterogeneous. Discussion: In our case, EABR showed a reproducible wave V on the right side, supporting candidacy for cochlear implantation which led to positive early auditory responses. Conclusions: This case and review highlight the role of EABR in identifying residual cochlear nerve functionality and guiding candidacy for cochlear implantation in narrow duplicated IAC.

Background/Objectives: Children with severe-to-profound hearing loss and additional disabilities, receiving cochlear implant (CI), require multidimensional outcome measures that extend beyond conventional assessments of auditory perception and spoken language. The Early Cognitive and Listening Links (EarlyCaLL) framework was originally developed by the Nottingham Auditory Implant Programme team, in the United Kingdom, led by Datta and colleagues, to support expectation counselling and to monitor post-cochlear implantation progress in children with CI and associated complex needs. The framework was designed to capture broader developmental outcomes, including cognition, social and emotional engagement, listening and understanding, vocalisations and early talk, and augmentative and alternative communication (AAC), alongside CI equipment use and signal tolerance. However, to date, no validated Italian version of the Framework has been available. This study aims to translate, culturally adapt, and perform a preliminary psychometric validation of the Italian version of the EarlyCaLL. Methods: A methodological study was conducted following international guidelines for translation and cross-cultural adaptation. The development phase included forward translation, expert committee review, back-translation, developer review, and pre-validation refinement. The validation phase involved 20 experienced professionals. Results: The expert panel demonstrated extensive clinical experience, with a 100% response rate. Face validity was excellent across all six domains (≥90% adequate ratings; Gwet’s AC1 = 0.82). Ninety percent of experts considered the questionnaire acceptable without substantial modifications, and 10% recommended only minor revisions. All 25 descriptors achieved perfect content validity, while the overall scale indices also indicated unanimous agreement regarding the relevance and adequacy of the instrument. Conclusions: Our results support the face and content validity of the Italian version of the EarlyCaLL, supporting further clinical validation as a culturally adapted multidimensional observational instrument for evaluating rehabilitation outcomes in children with CI and additional disabilities. The framework provides a comprehensive assessment of functional development and may facilitate individualized rehabilitation, multidisciplinary collaboration, and family-centred care in pediatric CI.