The majority of included studies showed CWU to result in more disease recidivism compared to the CWD technique in adult patients with a primary acquired cholesteatoma. If recidivism risk is the most important factor to consider a certain surgical technique, we recommend application of the CWD procedure. However, many additional factors in patient care will define the best treatment decision, such as residual hearing and access to health care. Our recommendations are based on Level II evidence, which underlines the need for future high-level evidence studies.

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Objective: This review aimed to evaluate the additional benefit of pediatric cochlear implantation before 12 months of age considering improved speech and language development and auditory performance. Materials and Methods: We conducted a search in PubMed, EMBASE and CINAHL databases and included studies comparing groups with different ages at implantation and assessing speech perception and speech production, receptive language and/or auditory performance. We included studies with a high directness of evidence (DoE). Results: We retrieved 3,360 articles. Ten studies with a high DoE were included. Four articles with medium DoE were discussed in addition. Six studies compared infants implanted before 12 months with children implanted between 12 and 24 months. Follow-up ranged from 6 months to 9 years. Cochlear implantation before the age of 2 years is beneficial according to one speech perception score (phonetically balanced kindergarten combined with consonant-nucleus-consonant) but not on Glendonald auditory screening procedure scores. Implantation before 12 months resulted in better speech production (diagnostic evaluation of articulation and phonology and infant-toddler meaningful auditory integration scale), auditory performance (Categories of Auditory Performance-II score) and receptive language scores (2 out of 5; Preschool Language Scale combined with oral and written language skills and Peabody Picture Vocabulary Test). Conclusions: The current best evidence lacks level 1 evidence studies and consists mainly of cohort studies with a moderate to high risk of bias. Included studies showed consistent evidence that cochlear implantation should be performed early in life, but evidence is inconsistent on all speech and language outcome measures regarding the additional benefit of implantation before the age of 12 months. Long-term follow-up studies are necessary to provide insight on additional benefits of early pediatric cochlear implantation.

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Pooled results of Austin-Kartush studies showed that in patients with COM, with or without cholesteatoma, the malleus status is a significant predictor of postoperative hearing outcome, independent of the stapes condition. Studies reporting on individual ossicle status supported this finding by showing that only malleus condition influenced postoperative hearing outcome. These findings are based on level IV evidence, which indicates the need for future high-level evidence studies.

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IMPORTANCEAlthough current guidelines recommend cochlear implantation only for children with profound hearing impairment (HI) (>90 decibel [dB] hearing level [HL]), studies show that children with severe hearing impairment (>70-90 dB HL) could also benefit from cochlear implantation. OBJECTIVE To perform a systematic review to identify audiologic thresholds (in dB HL) that could serve as an audiologic candidacy criterion for pediatric cochlear implantation using 4 domains of speech and language development as independent outcome measures (speech production, speech perception, receptive language, and auditory performance).EVIDENCE REVIEW PubMed and Embase databases were searched up to June 28, 2017, to identify studies comparing speech and language development between children who were profoundly deaf using cochlear implants and children with severe hearing loss using hearing aids, because no studies are available directly comparing children with severe HI in both groups. If cochlear implant users with profound HI score better on speech and language tests than those with severe HI who use hearing aids, this outcome could support adjusting cochlear implantation candidacy criteria to lower audiologic thresholds. Literature search, screening, and article selection were performed using a predefined strategy. Article screening was executed independently by 4 authors in 2 pairs; consensus on article inclusion was reached by discussion between these 4 authors. This study is reported according to the Preferred Reporting Items for Systematic Review and Meta-analysis (PRISMA) statement.FINDINGS Title and abstract screening of 2822 articles resulted in selection of 130 articles for full-text review. Twenty-one studies were selected for critical appraisal, resulting in selection of 10 articles for data extraction. Two studies formulated audiologic thresholds (in dB HLs) at which children could qualify for cochlear implantation: (1) at 4-frequency pure-tone average (PTA) thresholds of 80 dB HL or greater based on speech perception and auditory performance subtests and (2) at PTA thresholds of 88 and 96 dB HL based on a speech perception subtest. In 8 of the 18 outcome measures, children with profound HI using cochlear implants performed similarly to children with severe HI using hearing aids. Better performance of cochlear implant users was shown with a picture-naming test and a speech perception in noise test. Owing to large heterogeneity in study population and selected tests, it was not possible to conduct a meta-analysis. CONCLUSIONS AND RELEVANCEStudies indicate that lower audiologic thresholds (Ն80 dB HL) than are advised in current national and manufacturer guidelines would be appropriate as audiologic candidacy criteria for pediatric cochlear implantation.

No evidence was noted for lower complication rates or improved hearing preservation between the MFRA and SMA for cochlear implantation in children and adults. Pediatric data were available for children implanted above the age of 24 months only. Level I evidence is needed to resolve the uncertainty regarding differences in postoperative outcomes of pediatric and adult MFRA and SMA.