{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T03:01:10Z","timestamp":1790391670207,"version":"4.1.0"},"reference-count":45,"publisher":"S. Karger AG","issue":"1","license":[{"start":{"date-parts":[[2025,9,4]],"date-time":"2025-09-04T00:00:00Z","timestamp":1756944000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/karger.com\/pages\/terms-and-conditions"},{"start":{"date-parts":[[2025,9,4]],"date-time":"2025-09-04T00:00:00Z","timestamp":1756944000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/karger.com\/pages\/terms-and-conditions"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Audiol Neurotol"],"abstract":"<jats:p>Introduction: This study aimed to evaluate long-term auditory outcomes in patients with inner ear malformations (IEMs) treated with cochlear or auditory brainstem implants (CI\/ABI) and assess the influence of anatomical subtype, electrode design, insertion depth, and genetic\/syndromic background on hearing performance over a 10-year follow-up. Methods: We conducted a prospective cohort study including patients with radiologically confirmed IEMs and bilateral severe-to-profound hearing loss, all of whom underwent implantation and completed at least 10 years of follow-up. Outcomes were assessed using pure-tone average (PTA) and speech recognition scores (SRSs) at defined intervals. Additional analyses explored the influence of electrode array design, insertion depth, genetic and syndromic diagnosis, and intraoperative complications. Results: Eighty-two patients were included in the study, of whom 71 (86.58%) received a CI, while the remaining 11 patients (13.41%) were treated with an ABI. After a 10-year follow-up, the mean PTA gain with CI was 64.56 \u00b1 14.52 dB, and mean SRS improved by 56.88 \u00b1 25.32%. On the other hand, patients treated with ABI presented a gain of 45.00 \u00b1 18.22 and 32.62 \u00b1 10.67% dB, respectively. Enlarged vestibular aqueduct had the highest 10-year SRS (80.04%), whereas cochlear nerve agenesis, Michel deformity, and cochlear hypoplasia type I had the lowest (23.72%, 24.00%, and 26.50%, respectively). The mean insertion depth was 19.66 \u00b1 3.74 mm and showed a non-significant correlation with final SRS (r = 0.243, p = 0.084). The presence of residual auditory brainstem responses was significantly associated with better pre-implant PTA (p = 0.041) and higher SRS at 10 years (p = 0.034). Finally, having a syndromic condition was not significantly associated with the risk of a worse SRS at 10 years post-implantation (p = 0.091), nor with poorer auditory outcomes measured in PTA prior to surgery (p = 0.315). Conclusion: Auditory outcomes in IEMs are modulated by malformation subtype, neural status, and electrode design. A stratified, anatomy-guided approach is essential, especially when considering perimodiolar arrays. Delayed gains in some subtypes underscore the value of long-term follow-up, and integrating imaging and genetic data may enhance personalized implant strategies.<\/jats:p>","DOI":"10.1159\/000548098","type":"journal-article","created":{"date-parts":[[2025,9,4]],"date-time":"2025-09-04T16:00:56Z","timestamp":1757001656000},"page":"56-70","source":"Crossref","is-referenced-by-count":1,"title":["Tailored Auditory Implantation in Inner Ear Malformations: A Long-Term Prospective Study Integrating Imaging and Functional Outcomes"],"prefix":"10.1159","volume":"31","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9676-7879","authenticated-orcid":false,"given":"Joan","family":"Lorente-Piera","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Teresa","family":"Imizcoz 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