One study contributed 41/66 patients (62%). Hearing outcomes following CI in JLNS are generally good with the majority of patients experiencing useful hearing improvement. Significant peri-operative cardiac risks exist and should be discussed with the patient and family during pre-operative counselling and prompt thorough investigation, pre-operative optimisation and peri-operative monitoring.This study aimed to establish outcomes following cochlear implantation (CI) in patients with superficial siderosis (SS). MEDLINE, Embase, Web of Science, Cochrane, and ClinicalTrials.gov databases were searched for this systematic review. No limits were placed on the language or the year of publication. The review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. Of a total of 46 studies, 19 studies met the inclusion criteria reporting outcomes in 38 patients. Of the 44 implants, 23 implants (52.27%) had good hearing outcomes at the last follow-up, 9 (20.45%) were initially beneficial for the patient, but then the performance deteriorated (4 of which were re-implanted), and 12 (27.27%) were not beneficial for the patient. All studies were classified as grade 4 studies using the Oxford Centre for Evidence-Based Medicine (OCEBM) grading system, being retrospective in nature and consisting of case reports and noncontrolled case series with a small number of patients. Of the 44 implants, 32 (72.73%) showed improved hearing outcomes following CI for SS at some point in time, of which 23 (52.2%) implants showed sustained benefit at the last follow-up (average follow-up time was 21.97 months). It is difficult to predict the longevity of benefit owing to the progressive nature of the disease or the patients in whom it may be beneficial, as the preoperative investigations inadequately predict benefit. Preimplantation and postimplantation counseling with the patient and their family regarding the potential limited benefit and eventual parallel decline with the neurological disease are crucial, along with thorough clinical investigations.Establish outcomes following cochlear implantation (CI) in patients with Pendred syndrome. Systematic review and narrative synthesis. Databases searched Medline, Pubmed, Embase, Web of Science, Cochrane Collection and ClinicalTrials.gov. No limits placed on language or year of publication. Review conducted in accordance with the PRISMA statement. Searches identified 251 abstracts and 242 full texts. Of these, 22 studies met inclusion criteria reporting outcomes in 231 patients with at least 234 implants. Hearing outcomes were generally good with patients experiencing useful functional improvement. A total of 46 minor complications were reported in 78 cases. The methodological quality of included studies was modest, predominantly consisting of case reports and non-controlled case series with small numbers of patients. All studies were OCEBM grade III-IV. Hearing outcomes following CI in Pendred syndrome are generally good with useful functional improvement. However, outcomes reported in published studies lack long term follow up.To establish outcomes following cochlear implantation (CI) in patients with postsynaptic auditory neuropathy (AN). Systematic review and narrative synthesis. Databases searched MEDLINE, PubMed, EMBASE, Web of Science, Cochrane Collection and ClinicalTrials.gov. No limits placed on language or year of publication. Review conducted in accordance with the PRISMA statement. Searches identified 98 studies in total, of which 14 met the inclusion criteria reporting outcomes in 25 patients with at least 28 CIs. Of these, 4 studies focused on Charcot-Marie-Tooth disease (CMT), 3 on Brown-Vialetto-Van-Laere syndrome (BVVL), 2 on Friedreich Ataxia (FRDA), 2 on Syndromic dominant optic atrophy (DOA+), 2 on Cerebellar ataxia - areflexia - pes cavus - optic atrophy - sensorineural hearing loss (CAPOS) syndrome, and 1 on Deafness-dystonia-optic neuronopathy (DDON) syndrome. All studies were Oxford Centre for Evidence Based Medicine (OCEBM) grade IV. Overall trend was towards good post-CI outcomes with 22 of the total 25 patients displaying modest to significant benefit. Hearing outcomes following CI in postsynaptic ANs are variable but generally good with patients showing improvements in hearing thresholds and speech perception. In the future, development of a clearer stratification system into pre, post, and central AN would have clinical and academic benefits. Further research is required to understand AN pathophysiology and develop better diagnostic tools for more accurate identification of lesion sites. Multicenter longitudinal studies with standardized comprehensive outcome measures including health-related quality of life data will be key in establishing a better understanding of short and long-term post-CI outcomes.Summarise outcomes following cochlear implantation (CI) in patients with post-meningitis deafness. Systematic review and narrative synthesis. Databases searched Medline, Pubmed, Embase, Web of Science, Cochrane Collection and ClinicalTrials.gov. No limits placed on language or year of publication. Studies with a minimum of 20 individuals with post-meningitis deafness were included. Review conducted in accordance with the PRISMA statement. Searches identified 906 abstracts and 291 full texts. Of these, 19 studies met the inclusion criteria, reporting outcomes in 610 patients with 650 implants. Audiological outcomes improved across all studies following cochlear implantation. 7 studies demonstrated a statistically significant difference between pre and post-CI outcomes. Patients with no cochlear ossification, full electrode insertion, shorter duration of deafness and no neurological sequelae generally appeared to perform best. A total of 31 minor and 19 major complications were reported, with 15 cases of reimplantation. The methodological quality of the included studies was sufficient, predominantly consisting of cohort studies.  https://www.selleckchem.com/products/Mycophenolic-acid(Mycophenolate).html  15 studies were OCEBM grade III and 4 studies were OCEBM grade IV. All studies had a minimum of 20 individuals with post-meningitic deafness and used multi-channel cochlear implant devices. Audiological outcomes following cochlear implantation in meningitis are satisfactory, providing functional levels of speech perception and intelligibility. Improvement in hearing is dependent on the amount of cochlear ossification, duration of deafness prior to implantation, electrode insertion depth and presence of neurological sequalae. Cochlear implantation in meningitis patients can be challenging due to the presence of ossification and inaccuracies of pre-operative imaging. Therefore, early and bilateral implantation is recommended in all patients with post-meningitis hearing loss to improve the likelihood of full electrode insertion.