Audiology & neuro-otology · 2025
Tailored Auditory Implantation in Inner Ear Malformations: A Long-Term Prospective Study Integrating Imaging and Functional Outcomes.
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Lorente-Piera Joan, Imizcoz Fabra Teresa, Manrique-Huarte Raquel, Calvo Imirizaldu Marta, Domínguez Pablo, Lima Janaina P, Picciafuoco Sebastián, Alkorta-Aramburu Gorka, Manrique Manuel
Аннотация
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 ± 14.52 dB, and mean SRS improved by 56.88 ± 25.32%. On the other hand, patients treated with ABI presented a gain of 45.00 ± 18.22 and 32.62 ± 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 ± 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.
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 ± 14.52 dB, and mean SRS improved by 56.88 ± 25.32%. On the other hand, patients treated with ABI presented a gain of 45.00 ± 18.22 and 32.62 ± 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 ± 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.