Explore the vast range of titles available.

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 BrownVialetto-Van-Laere syndrome (BWL), 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.

Objectives To establish audiological and other outcomes following cochlear implantation in humans and animals with eluting electrodes. Methods Systematic review and narrative synthesis. Databases searched (April 2023): MEDLINE, EMBASE, CENTRAL, ClinicalTrials.gov, and Web of Science. Studies reporting outcomes in either humans or animals following cochlear implantation with a drug‐eluting electrode were included. No limits were placed on language or year of publication. Risk of bias assessment was performed on all included studies using either the Brazzelli or Systematic Review Centre for Laboratory animal Experimentation (SYRCLE) assessment tools. The review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta‐Analyses (PRISMA) 2020 statement. Results Searches identified 146 s and 108 full texts. Of these, 18 studies met the inclusion criteria, reporting outcomes in 523 animals (17 studies) and 24 humans (1 study). Eluting electrodes included dexamethasone (16 studies), aracytine (1 study), nicotinamide adenine dinucleotide (1 study), the growth factors insulin‐like growth factor 1 (IGF1) and hepatocyte growth factor (HGF) (1 study), and neurotrophin‐3 (1 study). All included studies compare outcomes following implantation with an eluting electrode with a control non‐eluting electrode. In the majority of studies, audiological outcomes (e.g., auditory brainstem response threshold) were superior following implantation with an eluting electrode compared with a standard electrode. Most studies which investigated post‐implantation impedance reported lower impedance following implantation with an eluting electrode. The influence of eluting electrodes on other reported outcomes (including post‐implantation cochlear fibrosis and the survival of hair cells and spiral ganglion neurons) was more varied across the included studies. Conclusions Eluting electrodes have shown promise in animal studies in preserving residual hearing following cochlear implantation and in reducing impedance, though data from human studies remain lacking. Further in‐human studies will be required to determine the clinical usefulness of drug‐eluting cochlear implants as a future treatment for sensorineural hearing loss. This systematic review focusses on studies examining the use of drug‐eluting electrodes in cochlear implants. We include both human and animal studies in our review, summarize the range of outcomes reported across these studies, and discuss the significance of these findings for the future clinical use of eluting electrodes in cochlear implants.

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-Cl 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. 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.

Objective This systematic review aims to establish the expected hearing and speech outcomes following cochlear implantation (CI) in patients with profound congenital deafness secondary to Waardenburg syndrome (WS). Methods A systematic review of the literature and narrative synthesis was performed in accordance with the PRISMA statement. Databases searched: Medline, Pubmed, Embase, Web of Science, Cochrane Collection, and ClinicalTrials.gov. No limits were placed on language or year of publication. Results Searches identified 186 s and full texts. Of these, 16 studies met inclusion criteria reporting outcomes in 179 patients and at least 194 implants. Hearing outcomes of those receiving cochlear implantation were generally good. Five studies included genetic analysis of one or more of the participants. A total of 11 peri/post‐operative complications were reported. The methodological quality of included studies was modest, mainly comprising noncontrolled case series with small cohort size. All studies were OCEBM grade III–IV. Conclusion Cochlear implantation in congenitally deafened children with Waardenburg Syndrome is a well‐established intervention as a method of auditory rehabilitation. Due to the uncommon nature of the condition, there is a lack of large‐scale high‐quality studies examining the use of cochlear implantation in this patient group. However, overall outcomes following implantation are positive with the majority of patients demonstrating improved audiometry, speech perception and speech intelligibility supporting its use in appropriately selected cases. In this article, we present a systematic review which examines the the expected hearing outcomes following cochlear implantation in Waardenburg Syndrome, a rare but important cause of congenital hearing loss.

BACKGROUND: Congenital cholesteatomas account for just up to 5% of all cholesteatomas and most commonly arise in the petrous apex and middle ear. Congenital cholesteatomas arising in the mastoid are rare and typically present late. METHODS: In this study, we report a case series of 3 cases managed in our department between 2006 and 2021 and present a summary of the current literature. RESULTS: Congenital cholesteatomas arising in the mastoid is a rare finding and even among reported cases, not all are clearly mastoid in origin. Their location allows for considerable growth before symptoms develop. Pain and localized swelling in the temporal area are the most common presenting symptoms which can lead to diagnostic challenges. Our cases show that although surgery is often appropriate, conservative management may be suitable in certain situations. CONCLUSION: Congenital cholesteatoma of mastoid origin is rare and can present a diagnostic challenge. Greater awareness is important to facilitate early detection. A high index of suspicion is needed in those presenting with retro-auricular pain and swelling in the context of a normal ontological examination. KEYWORDS: Congenital cholesteatoma, ear surgery, otology, temporal bone

Objectives As the population ages and implantation criteria are relaxed, more patients with complex comorbidities are becoming eligible for cochlear implantation (CI). These patients have higher risks associated with general anesthesia. This systematic review assesses outcomes and complications following CI under local anesthetic to examine utility for patients deemed not suitable or at high risk for general anesthesia. Methods A systematic review and meta‐analysis performed according to the 2020 PRISMA guidelines. Databases searched were MEDLINE, PubMed, EMBASE, CINAHL, Web of Science Core Collection, Cochrane Database of Systematic Reviews, and Cochrane Central Register of Controlled Trials and ClinicalTrials.gov. There were no limitations on year of publication or language. Results Then, 132 unique were identified. After screening s and full texts for eligibility criteria a total of 18 articles were included. In the nine studies where audiological data were reported, all patients demonstrated improvement in audiological outcomes following implantation under local anesthetic (LA). Only minor complications of transient vertigo, wound infection, facial nerve paralysis, confusion, and tinnitus were reported but all were transient. Meta‐analysis showed surgical time was significantly shorter under LA. Conclusions CI under LA is safe for patients with comorbidities which preclude them from general anesthesia, with minimal complications and an improved cost‐effectiveness profile. However, larger scale, robust trials are required to assess this further. Cochlear implantation under local anesthetic is safe for patients with comorbidities which preclude them from general anesthesia, with minimal complications and an improved cost‐effectiveness profile. However, larger scale, robust trials are required to assess this further.

Objectives To assess outcomes following cochlear implantation (CI) in patients with hearing loss secondary to primary or secondary autoimmune inner ear disease (AIED). Methods A systematic review and narrative synthesis was completed according to PRISMA guidelines. Databases searched included MEDLINE, PubMed, EMBASE, Web of Science, Cochrane Collection, and ClinicalTrials.gov. No limits were placed on year of publication or language. Results A total of 551 studies were identified, of which 29 were included after removal of duplicates, and screening the title, , and full text. All except one study were OCEBM grade IV. 114 of 115 patients displayed improvement in hearing following cochlear implantation. With implant use, roughly a third of these patients had hearing that improved over time, a third improved and plateaued, and a third remained stable. There was no additional risk of perioperative complications found in AIED patients compared what is generally accepted in general cochlear implantation, although two episodes of device failure after 6 months were noted, and four patients with secondary AIED displayed poor initial audiological outcomes. Conclusion CI in both primary and secondary AIED provides marked improvement in hearing. Early CI may be a valid management option, provide long‐lasting hearing in patients and reduce the side effects of long‐term systemic immunosuppressants. However, patients should be counseled residual hearing may be lost if there is cochlear ossification or fibrosis which may make implant insertion more traumatic. Level of Evidence NA. Autoimmune inner ear disease is characterised by bilateral, assymetrical, progressive (or fluctuating) hearing loss that develops over weeks to months and is steroid responsive. Cochlear implantation has been suggested as a useful intervention in this group when medical management fails, however there is limited data in the literature. This review aims to systematically review all available evidence of post‐cochlear implantation hearing outcomes in patients with autoimmune inner ear disease to assess its benefit.

Establish outcomes following cochlear implantation (CI) in patients with Jervell and Lange-Nielsen Syndrome (JLNS). Methods Systematic review and narrative synthesis. Databases searched on 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 63 abstracts and 19 full texts. Of these, 9 studies met inclusion criteria reporting outcomes in 66 patients with at least 72 implants. Hearing outcomes were generally good. Mortality secondary to cardiac complications within the follow up period occurred in at least five cases (7.6%), though three of these were thought to be unrelated to surgery. Potentially dangerous arrhythmias without associated morbidity were also noted in at least five patients. 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 IV. 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's aim was to establish outcomes following cochlear implantation (CI) in patients with mitochondrial disorders associated with deafness. Systematic review and narrative synthesis. Databases searched: Medline, EMBASE, Web of Science, COCHRANE, and ClinicalTrials.gov. No limits on language or year of publication. Review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. Searches identified 437 abstracts and 37 full text articles, of which 11 studies met the inclusion criteria reporting outcomes in a total of 17 patients. All implants achieved good hearing outcomes, and follow-up ranged between 1 week and 12 months. The methodological quality of the included studies was sufficient, scoring grades 3 to 4 using the Oxford Centre for Evidence Based Medicine grading system. All studies were retrospective and consisted of case reviews and case reports. All cases of CI showed positive outcomes in speech perception and detection. There is some qualitative evidence to suggest improvement in quality of life and satisfaction postoperatively. There was very limited information available on secondary outcomes such as surgical complications, quality of life, and method of cochlear implant insertion. The small sample size of our patient cohort and quality of studies suggests a need for large-scale studies with more robust methodology to assess the effectiveness of CI. There is a need for studies that assess other factors to be considered when counseling patients about cochlear implants, such as adverse events, surgical complications, and long-term benefits.

Objective Establish anatomical considerations, audiological outcomes, and optimal management in patients with branchiootic/branchiootorenal syndrome (BO/BOR). Methods Databases reviewed: Medline, Pubmed, Embase, Web of Science, Cochrane Collection, and ClinicalTrials.gov. Clinical or radiological studies of patients with BOR syndrome describing either the audiological profile or anatomical changes were included. Articles in which BOR syndrome was associated with other syndromes, and those that were focused only on general and genetic aspects of BOR syndrome were excluded. Articles were assessed using Oxford Centre for Evidence‐Based Medicine (OCEBM) grading system and the Brazzelli risk of bias tool for nonrandomized studies. Results Searches identified 379 articles. Of these, 64 studies met the inclusion criteria, reporting outcomes in 482 patients from at least 95 families. In 308 patients, hearing loss was categorized as sensorineural (29%), conductive (20%), and mixed (51%). Hearing outcomes were variable in terms of onset, pattern, and severity; ranging from mild to profound deafness. One hundred sixty‐nine patients presented with inner ear anomalies, 145 had middle, and 151 had external ear abnormalities. In 44 studies, 58 ear operations were described. Mixed outcomes were reported in patients managed with hearing aids or middle ear surgery; however, successful cochlear implantation was described in all five cases. Conclusion The anatomical and audiological profiles of patients with BO/BOR are variable. A range of surgical procedures were described, however lacked objective outcome measures. Given the range of anatomical variants, management decisions should be made on an individual basis including full audiological and radiological assessment. Level of evidence NA.