Outcomes of cochlear implantation in Usher syndrome: a systematic review.

PURPOSE: This study is a systematic review of the literature which seeks to evaluate auditory and quality of life (QOL) outcomes of cochlear implantation in patients with Usher syndrome. METHODS: Systematic review of studies indexed in Medline via PubMed, Ovid EMBASE, Web of Science, CENTRAL and clinicaltrials.gov was performed up to March 9th 2022, conducted in accordance with the PRISMA statement. Patient demographics, comorbidity, details of cochlear implantation, auditory, and QOL outcomes were extracted and summarized. RESULTS: 33 studies reported over 217 cochlear implants in 187 patients with Usher syndrome, comprising subtypes 1 (56 patients), 2 (9 patients), 3 (23 patients), and not specified (99 patients). Auditory outcomes included improved sound detection, speech perception, and speech intelligibility. QOL outcomes were reported for 75 patients, with benefit reported in the majority. CONCLUSIONS: Many patients with Usher syndrome develop improved auditory outcomes after cochlear implantation with early implantation being an important factor.

Cochlear Implant Outcomes in Patients With Otosclerosis: A Systematic Review.

OBJECTIVE: This study aimed to establish hearing outcomes after cochlear implantation in patients with otosclerosis. MATERIALS AND METHODS: We conducted a systematic review and narrative synthesis. Databases searched were as follows: MEDLINE, PubMed, Embase, Web of Science, Cochrane Collection, and ClinicalTrials.gov . No limits were placed on language or year of publication. The review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. RESULTS: Searches identified 474 abstracts and 180 full texts, with 68 studies meeting the inclusion criteria and reporting outcomes in a minimum of 481 patients with at least 516 implants. Patient-reported outcome measures (PROMs) were reported in five studies involving 51 patients. Intraoperative adverse events/surgical approach details and preoperative radiological assessment were reported in 46 and 38 studies, respectively. The methodological quality of included studies was modest, predominantly consisting of case reports and noncontrolled case series with small numbers of patients. Most studies were Oxford Centre for Evidence Based Medicine grade IV. DISCUSSION: Access to good rehabilitation support is essential to achieving the good hearing outcomes and PROMs that can be expected by 12 months after implantation in most cases. There was a significant association between the radiological severity of otosclerosis and an increase in surgical and postoperative complications. Postoperative facial nerve stimulation can occur and may require deactivation of electrodes and subsequent hearing detriment. CONCLUSIONS: Hearing outcomes are typically good, but patients should be counseled on associated surgical complications that may compromise hearing. Modern diagnostic techniques may help to identify potentially difficult cases to aid operative planning and patient counseling. Further work is needed to characterize PROMs in this population.

A Micro-Computed Tomography Study of Round Window Anatomy and Implications for Atraumatic Cochlear Implant Insertion.

HYPOTHESIS: The goal of this study was to interrogate high-resolution three-dimensional reconstructions of round window anatomy to illustrate and characterize structural variability with implications for atraumatic cochlear implant insertion. BACKGROUND: Cochlear implants are increasingly used to improve sound detection in patients with substantial residual hearing. However, traumatic cochlear implant insertion through the round window involving upward deviation of the electrode into the spiral ligament, basilar membrane, and osseous spiral lamina, medial impaction on the modiolus, or interscalar excursion into the scala vestibuli are associated with lower rates of hearing preservation and poorer speech perception.Successful atraumatic insertion is dependent on an anatomical understanding of the middle and inner ear. The round window bony niche lacks distinct demonstrable anatomical landmarks for the position of the round window membrane, and there is limited guidance on the amount of bony overhang that can be safely drilled away. A greater understanding of the anatomical variation around the round window could enhance treatment efficacy. METHODS: Fourteen human cadaver temporal bones were imaged using microcomputed tomography. Resulting scans were digitally reconstructed, segmented, and measured. RESULTS: Round window niche walls vary substantially in size and projection. Round window average short diameter measured 1.30 mm (range 1.07-1.44), and is limited by the crista fenestrae at the inferoanterior margin of the round window. Crista fenestrae size and morphology varied considerably. Reconstructions with solid and translucent panels are presented. CONCLUSION: Anatomical heterogeneity should be considered in cochlear implant selection, drilling, and choice of insertion vector.