Impact of cochlear abnormalities on hearing outcomes for children with cochlear implants.

OBJECTIVE: Evaluate the impact of cochlear anomalies on hearing outcomes for pediatric patients with cochlear implants. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary care center. SUBJECTS AND METHODS: Charts were retrospectively reviewed for cases where pediatric cochlear implant surgery was performed between 2002 and 2018 at a single, tertiary care institution. Patients were divided into groups based on the presence or absence of radiological cochlear abnormalities, which were further classified as low or high risk anomalies. Hearing outcomes were evaluated by measuring pure tone averages and word recognition scores preoperatively, 3 and 12 months postoperatively, in addition to the most recent test results. RESULTS: There were 154 ears implanted in our cohort of 100 patients. 107 ears had normal cochlear anatomy, 31 had low risk, and 16 had high risk abnormalities. The most common modality of preoperative imaging was CT scan. Postoperative mean pure tone average (PTA) was significantly higher in patients with inner ear anomalies compared to those with normal anatomy. No significant difference in PTA was noted between low versus high risk patients. <50% of patients had word recognition scores available within the first year following surgery. CONCLUSION: Abnormalities of the inner ear significantly influenced hearing outcomes over time following cochlear implant surgery when compared to pediatric patients with normal anatomy. Obtaining hearing testing can be difficult in very young children and therefore future studies are warranted to further investigate the impact that cochlear abnormalities may have on hearing outcomes following cochlear implant surgery.

Cochlear implantation in children with anomalous cochleovestibular anatomy: a systematic review.

OBJECTIVE: To determine the influence of inner ear dysplasia on both surgical and audiologic outcomes following pediatric cochlear implant (CI) surgery. DATA SOURCES: MEDLINE (1982-2009) and data from Massachusetts Eye and Ear Infirmary. REVIEW METHODS: A systematic review of the literature was performed. Variables assessed included age at implantation, duration of CI use, radiologic and operative findings, and speech perception outcome data. RESULTS: The initial search yielded 1326 articles. Including data from our own study, twenty-two fulfilled criteria for inclusion, representing 311 patients. Data for bilateral implants were recorded only for the first implant. Data on simultaneous bilateral implants were not recorded. The most common anomaly seen was large vestibular aqueduct (89/311 or 29%). When comparing patients with mild-moderate or severe dysplasia, rates of cerebrospinal fluid gusher were 31% versus 35% (odds ratio [OR] = 0.50), anomalous facial nerve anatomy was seen in 11% versus 51% (OR = 0.15), and postoperative speech perception abilities were found in 84% versus 54% (OR = 1.93), respectively. A large heterogeneity was found among studies regarding all outcome measures. CONCLUSION: Although we found that severe inner ear dysplasia was associated with increased surgical difficulty and lower speech perception, the lack of uniformity in published clinical data limited the strength of these results. Standardization of surgical and radiologic reporting as well as more consistent speech perception testing is needed to better determine the association between anomalous cochleovestibular anatomy and clinical outcomes.