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Tailored Auditory Implantation in Inner Ear Malformations: A Long-Term Prospective Study Integrating Imaging and
Joan Lorente-Piera1, Teresa Imizcoz Fabra2, Raquel Manrique-Huarte3
1Department of Otorhinolaryngology, Clínica Universidad de Navarra, Pamplona, Spain, jlorentep@unav.es.
Audiology & Neuro-Otology
|September 4, 2025
Summary
Long-term cochlear implant (CI) and auditory brainstem implant (ABI) use in inner ear malformations (IEMs) shows significant hearing gains. Auditory outcomes depend on malformation type and neural status, highlighting the need for personalized strategies.
Area of Science:
- Otolaryngology and Neurotology
- Medical Imaging and Genetics in Hearing Loss
- Auditory Neurophysiology and Rehabilitation
Background:
- Inner ear malformations (IEMs) present unique challenges for hearing restoration.
- Cochlear implants (CI) and auditory brainstem implants (ABI) are options for severe-to-profound hearing loss in IEMs.
- Long-term outcomes and influencing factors in IEMs post-implantation require comprehensive evaluation.
Purpose of the Study:
- To evaluate the 10-year auditory outcomes of CI/ABI in patients with IEMs.
- To assess how anatomical subtype, electrode design, insertion depth, and genetic/syndromic factors impact hearing performance.
- To identify predictors of successful auditory rehabilitation in this patient cohort.
Main Methods:
- Prospective cohort study of 82 patients with radiologically confirmed IEMs and severe-to-profound hearing loss.
- 10-year follow-up assessing pure-tone average (PTA) and speech recognition scores (SRS) post-CI or ABI.
- Analysis of anatomical subtype, electrode characteristics, insertion depth, genetic/syndromic background, and residual auditory brainstem responses (ABRs).
Main Results:
- Mean PTA gain with CI was 64.56 dB and mean SRS improved by 56.88%; ABI yielded gains of 45.00 dB and 32.62% SRS.
- Enlarged vestibular aqueduct (EVA) showed the highest 10-year SRS (80.04%), while cochlear nerve agenesis had the lowest (23.72%).
- Residual ABRs correlated significantly with better pre-implant PTA and higher 10-year SRS; syndromic status did not significantly affect outcomes.
Conclusions:
- Auditory outcomes in IEMs are significantly influenced by malformation subtype, neural integrity, and implant type/design.
- A tailored, anatomy-guided implantation strategy is crucial, particularly concerning electrode choice and insertion.
- Long-term follow-up is essential, as delayed hearing gains are observed in certain IEM subtypes, supporting personalized implant approaches.
