Purpose of Review
This review will highlight recent outcome-based evidence guiding decision making for cochlear implantation in advanced otosclerosis, related complications, and technical surgical considerations in otosclerosis and the obstructed cochlea.
Cochlear implantation in advanced otosclerosis results in consistent, excellent auditory outcomes with improvement in both objective speech recognition scores and subjective quality of life measures. Facial nerve stimulation may occur at higher rates in otosclerosis cochlear implant recipients. Cochlear implantation in the setting of luminal obstruction in osteosclerotic patients may be managed with altered surgical technique to achieve successful auditory improvements. Pre-operative imaging with high resolution CT or MRI may help anticipate intraoperative challenges and post-operative complications in cochlear implantation.
Cochlear implantation is an established, successful treatment for profound hearing loss in advanced otosclerosis. Surgeon knowledge of outcomes, complications, and potential surgical challenges is important to appropriately counsel patients regarding auditory rehabilitation options in advanced otosclerosis.
Objective To systematically review the literature to determine safety of cochlear implantation in pediatric patients 12 months and younger. Data Source Ovid MEDLINE, EMBASE, CINAHL, and Cochrane Central Register of Controlled Trials (CENTRAL) databases were searched from inception to March 20, 2021. Review Methods Studies that involved patients 12 months and younger with report of intraoperative or postoperative complication outcomes were included. Studies selected were reviewed for complications, explants, readmissions, and prolonged hospitalizations. Two independent reviewers screened all studies that were selected for the systematic review and meta-analysis. All studies included were assessed for quality and risk of bias. Results The literature search yielded 269 studies, of which 53 studies underwent full-text screening, and 18 studies were selected for the systematic review and meta-analysis. A total of 449 patients and 625 cochlear implants were assessed. Across all included studies, major complications were noted in 3.1% of patients (95% CI, 0.8-7.1) and 2.3% of cochlear implantations (95% CI, 0.6-5.2), whereas minor complications were noted in 2.4% of patients (95% CI, 0.4-6.0) and 1.8% of cochlear implantations (95% CI, 0.4-4.3). There were no anesthetic complications reported across all included studies. Conclusion The results of this systematic review and meta-analysis suggest that cochlear implantation in patients 12 months and younger is safe with similar rates of complications to older cohorts.
Variations in the POU Class 3 Homeobox 4 ( POU3F4) gene are associated with X-linked mixed deafness. Here, the identification of a novel variant of POU3F4 in a male paediatric patient (the proband) with incomplete partition type III (IP-III) hearing impairment, is described. Clinical data were collected from the proband and his biological parents. Whole exome sequencing of the proband revealed a novel frameshift insertion mutation in POU3F4 (c.717_718ins GTGCCTTGCAG : p.Leu240Valfs*5) in a hemizygous state. This variant likely truncates the protein within the POU-specific domain, and the proband’s biological mother was found to be a carrier of this variant. After excluding all contraindications, the proband underwent cochlear implantation in the right ear in June 2020. Cerebrospinal fluid (CSF) gushing was observed during surgery, but there were no postoperative complications, such as CSF leak, meningitis, or facial nerve stimulation. A novel pathogenic frameshift variant of POU3F4 was identified, enriching the known mutation spectrum of POU3F4. Effective perioperative prevention and response measures should be taken to reduce the incidence of CSF gushing and meningitis in patients receiving IP-III cochlear implantation.