Cochlear Implantation in Children With Single-Sided Deafness

Abstract

IMPORTANCEIn 2019, the US Food and Drug Administration approved cochlear implantation for children with single-sided deafness (SSD). The absence of robust clinical data specific to pediatric patients to guide shared decision-making and to identify potential advantages is a challenge in family counseling.OBJECTIVE To evaluate the audiological and patient-reported outcomes in children who underwent cochlear implantation for SSD and to assess the association between time of implantation, subjective outcomes, and cochlear implant device use rates.DATA SOURCE MEDLINE, Embase, Scopus, Cochrane, and PubMed were searched for English-language articles that were published in a peer-reviewed journal from database inception to February 18, 2020.STUDY SELECTION Inclusion criteria were designed to capture studies that evaluated pediatric patients (1) younger than 18 years, (2) with a diagnosis of SSD for which they underwent a cochlear implantation, and (3) with at least 1 outcome of interest measured numerically: speech perception, sound localization, device use, and patient-reported outcomes. Of the 526 articles reviewed, 12 (2.3%) met the selection criteria. DATA EXTRACTION AND SYNTHESISThe Meta-analyses Of Observational Studies in Epidemiology (MOOSE) reporting guidelines were followed. Data were pooled using fixed-effect and random-effect models. The following information was obtained from each article: study characteristics, patient characteristics, hearing loss and intervention characteristics, and outcomes. MAIN OUTCOMES AND MEASURESOutcomes were (1) postoperative changes in speech perception (in quiet was measured as a proportion of correct responses, and in noise was measured as decibel signal to noise ratio for speech reception threshold) and sound localization (measured in degree of localization error), (2) patient-reported audiological outcomes (measured by the speech, spatial, and qualities of hearing scale), and (3) device use rates among children who received cochlear implantation for SSD. RESULTSTwelve observational studies that evaluated 119 children (mean [SD] age, 6.6 [4.0] years) with SSD who received a cochlear implant were included. Most children showed clinically meaningful improvement in speech perception in noise (39 of 49 children [79.6%]) and in quiet (34 of 42 children [81.0%]). Long duration of deafness (>4 years in congenital SSD and >7 years in perilingual SSD) was the most commonly proposed reason for lack of improvement. Sound localization as measured by degrees of error from true location (mean difference [MD], -24.78°; 95% CI, -34.16°to -15.40°; I 2 = 10%) improved statistically significantly after cochlear implantation. Patients with acquired SSD and shorter duration of deafness compared with those with congenital SSD reported greater improvements in speech (MD, 2.27; 95% CI, 1.89-2.65 vs 1.58; 95% CI, 1.00-2.16) and spatial (MD, 2.95; 95% CI, 2.66-3.24 vs 1.68; 95% CI, 0.96-2.39) hearing qualities. The duration of deafness among device nonusers was statistically significantly longer than t...

Perception of Environmental Sounds in Cochlear Implant Users: A Systematic Review

“…These changes make extrapolation from earlier studies problematic. Unlike CI patients implanted in the 1980s and 1990s, the majority of whom were profoundly deaf in both ears, today's CI candidates often include adults and children who still retain usable, and in case of single-sided deafness, normal hearing in one ear (Benchetrit et al, 2021 ). Patients with greater overall hearing abilities prior to implantation may expect more from their hearing after implantation, and post-implantation PES scores that indicated an improvement in the past may no longer be sufficiently high.…”

“…Cochlear implants are the treatment of choice for a growing number of people afflicted with sensorineural hearing loss beyond the therapeutic capabilities of acoustic amplification with hearing aids (NIDCD, 2021 ). Although CIs were initially approved by the Food and Drug Administration (FDA) for adults with profound hearing loss in both ears (Sladen et al, 2017 ), today's CI candidates may include adults and children who still retain usable, and sometimes normal hearing in at least one ear (e.g., Benchetrit et al, 2021 ). Patients with greater overall hearing abilities prior to implantation may expect more from their implants afterwards.…”

American Cochlear Implant Alliance Task Force Guidelines for Clinical Assessment and Management of Cochlear Implantation in Children With Single-Sided Deafness

“…Studies have also provided evidence that some adult SSD + CI recipients have improved speech in noise performance secondary to binaural redundancy and binaural squelch (Vermeire & Van de Heyning 2009; Mertens et al 2015). Pediatric SSD + CI patients have shown improvement in SIN tasks as well (Sladen et al 2017; Thomas et al 2017; Zeitler et al 2019; Benchetrit et al 2021; Ehrmann-Mueller et al 2020; Park et al 2021a).…”

The Limitations of FDA Criteria: Inconsistencies with Clinical Practice, Findings, and Adult Criteria as a Barrier to Pediatric Implantation

“…The 2019 FDA labeling approval for implantation in cases of SSD in children aged 5 and older (MED-EL) was welcomed. Early studies and case reports have indicated benefits in chil-dren, 42 particularly when it comes to hearing in noise [43][44][45][46][47] and localization. 43,48,49 The age limit of 5 years was likely set due to safety, as the clinical trial used for approval did not include young children.…”