Neurofibromatosis Type 2 and Dual Sensory Loss: A Recipient's Perspective

Hannah McPierzie is an international disability advocate, speaker, and consultant who joins us in the studio to discuss Neurofibromatosis Type 2 (NF2), its far-reaching consequences, and the complex decisions individuals and families may face following diagnosis. Drawing on both her professional and lived experience, Hannah shares her journey with NF2, including the progression of hearing loss, her experience with auditory brainstem implants (ABIs), and the process of navigating a dual sensory loss. She offers valuable insights into self-advocacy, communication accessibility, and what it means to identify as deaf-blind. Weblinks Watch the fifth episode of MED-EL's Beyond Basics Rehabilitation Vodcast on the MED-EL Academy.

In this episode, we take an in-depth look at Augmentative and Alternative Communication (AAC) for children with cochlear implants who have complex needs. Joined by AAC expert and researcher Gunilla Thunberg, the discussion covers everything from defining AAC, best-practice for assessments, and use of predictive text. This conversation gives all professionals working with children with hearing loss valuable insights to help them get started with AAC. Weblinks Watch the fourth episode of MED-EL's Beyond Basics Rehabilitation Vodcast on the MED-EL Academy. AKKtiv Website 

Join Maximilian Schwaninger and Mario Schinnerl as they take a closer look at the test results and user experiences behind the new Standard Pro and Micro Pro rechargeable batteries for SONNET 3. Discover how extended runtimes, compact design, and simplified handling make these new options perfect for everyday use. Weblinks: MED-EL Professionals Blog: SONNET 3 Reloaded: Smaller size. Added power.

In this episode, Maximilian Schwaninger is joined by Scott Murray, a long-time cochlear implant user and outdoor enthusiast, to explore how the waterproof technology of SONNET 3 is transforming everyday life. From rainy concerts to water sports, discover how this next-level technology brings freedom, flexibility, and peace of mind to CI users—no matter the weather. Weblinks: MED-EL Website: SONNET 3 Audio Processor  MED-EL Professionals Blog: 11 Facts Clinicians Should Know About SONNET 3 & MAESTRO 11

In this episode, Patrick Spörr, Product Manager for External Devices at MED-EL, talks with Andreas Neurauter from MED-EL's R&D department about the new AudioLink XT and its standout features. They explore how this next-generation connectivity device builds on user feedback to offer ASHA-based TV streaming, FM receiver support, improved battery efficiency, broader compatibility, and more. Weblinks: MED-EL Website: AudioLink XT MED-EL Blog: Get the Most Out of SONNET 3 With AudioLink XT and AudioKey 3

In this final episode of Closest to Natural Hearing, Marcus Schmidt speaks with Dr. Hinrich Staecker and Dr. Peter Nopp about the future of cochlear implantation. They explore how advances in AI, anatomy‑based fitting, hearing preservation, and fully implantable systems are reshaping outcomes for current and future CI users. The conversation also highlights emerging developments in gene therapy, drug‑emitting electrodes, robotics, and individualized surgical planning—all pointing toward a future in which hearing with a CI is more natural, seamless, and accessible than ever. Literature References: Helbig S, Rajan GP, Stöver T, et al. Hearing preservation after cochlear reimplantation. Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology. 2013 Jan;34(1):61-65. DOI: 10.1097/mao.0b013e318277a44e. PMID: 23202149. Lee S, Lee S, Park S: Comprehensive genetic profiling of sensorineural hearing loss using an integrative diagnostic approach. Cell Reports Medicine, 2025; 6. Manrique-Huarte, Raquel; Zulueta-Santos, Cristina; Calavia, Diego; Linera-Alperi, Marta Álvarez de; Gallego, Maria Antonia; Jolly, Claude; Manrique, Manuel. Cochlear Implantation With a Dexamethasone Eluting Electrode Array: Functional and Anatomical Changes in Non-Human Primates. Otology & Neurotology 41(7):p e812-e822, August 2020. | DOI: 10.1097/MAO.0000000000002686. Michael Nieratschker, Pavel Mistrik, Zdenek Petrasek, Erdem Yildiz, Anselm J. Gadenstaetter, Matthias Gerlitz, Anne-Margarethe Kramer, Monika Kwiatkowska, Susanne Braun, Reimar Schlingensiepen, Clemens Honeder, Christoph Arnoldner. Silicone-based AC102-loaded cochlear implant coatings protect residual hearing in an animal model of cochlear implantation, Hearing Research, Volume 454, 2024, doi.org/10.1016/j.heares.2024.109150. Prenzler N, Salcher R, Büchner A, Warnecke A, Kley D, Batsoulis C, Vormelcher S, Mitterberger-Vogt M, Morettini S, Schilp S, Hochmair I, Lenarz T. Cochlear implantation with a dexamethasone-eluting electrode array: First-in-human safety and performance results. Hear Res. 2025 Jun;461:109255. doi: 10.1016/j.heares.2025.109255. Epub 2025 Mar 23. PMID: 40158223.  Shearer AE, Hildebrand MS, Odell AM, et al. Genetic Hearing Loss Overview. 1999 Feb 14 [Updated 2025 Apr 3]. In: Adam MP, Bick S, Mirzaa GM, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2026. Available from: https://www.ncbi.nlm.nih.gov/sites/books/NBK1434/.

In this episode, Marcus Schmidt discusses with Prof. Stefan Weder and Dr. Ilona Anderson how a flexible electrode array design and advanced surgical tools, such as OTOPLAN and OTODRIVE, are helping surgeons achieve more precise outcomes while supporting residual hearing preservation by protecting the delicate structures in the cochlea. They also look at the latest clinical data on hearing preservation, including FDA-approval for hearing preservation, and they discuss how these innovations are improving quality of life for CI users.  Weblinks: MED-EL Professional Blog: 5 Ways to Maximize Residual Hearing Preservation With Precision Surgery How MED-EL Cochlear Implants Provide the Closest to Natural Hearing Literature References: Aebischer, P., Anschuetz, L., Caversaccio, M., Mantokoudis, G., & Weder, S. (2024). Quantitative in-vitro assessment of a novel robot-assisted system for cochlear implant electrode insertion. International Journal of Computer Assisted Radiology and Surgery, 1-10.  Heyning, P. H. V. de, Dazert, S., Gavilan, J., Lassaletta, L., Lorens, A., Rajan, G. P., Skarzynski, H., Skarzynski, P. H., Tavora-Vieira, D., Topsakal, V., Usami, S., Rompaey, V. V., Weiss, N. M., & Polak, M. (2022). Systematic Literature Review of Hearing Preservation Rates in Cochlear Implantation Associated With Medium- and Longer-Length Flexible Lateral Wall Electrode Arrays. Frontiers in Surgery, 9, 893839. https://doi.org/10.3389/fsurg.2022.893839. Oetiker Y, Aebischer P, Caversaccio M, Mantokoudis G, Weder S. Hearing preservation outcomes with motorized cochlear implant electrode insertion: matched-cohort observations. Front Surg. 2025 Nov 7;12:1700744. doi: 10.3389/fsurg.2025.1700744. PMID: 41282565; PMCID: PMC12637026. US Food & Drug Administration (FDA) approval confirming that there is sufficient evidence that MED-EL's FLEX electrodes preserve residual functional hearing in the majority of recipients: Document "Summary of safe-ty and effectiveness Data" of Premarket Approval Application (PMA) Number: P000025/S129, MED-EL Cochlear Implant System, https://www.accessdata.fda.gov/cdrh_docs/pdf/P000025S129B.pdf. von Ilberg C, Kiefer J, Tillein J, Pfenningdorff T, Hartmann R, Stürzebecher E, Klinke R. Electric-acoustic stimulation of the auditory system. New technology for severe hearing loss. ORL J Otorhinolaryngol Relat Spec. 1999 Nov-Dec;61(6):334-40. doi: 10.1159/000027695. PMID: 10545807.  

In this episode, Dr. Ilona Anderson welcomes Mariia Pazi and Krystian Koper, two cochlear implant users with unique hearing journeys. Together, they explore what "closest to natural hearing" means from a user's perspective.  Weblinks: How MED-EL Cochlear Implants Provide the Closest to Natural Hearing  

In this episode, Jennifer Robinson is joined by Dr. Anja Kurz and Dr. Margaret Dillon for an in‑depth look at anatomy‑based fitting (ABF) and its role in reducing frequency–place mismatch. Together, they explore what makes ABF so impactful—from the underlying science to everyday clinical challenges—and discuss recent findings that show how individualized frequency alignment can improve listening comfort, sound quality, and speech comprehension.   Literature References: Buchman CA, Dillon MT, King ER, Adunka MC, Adunka OF, Pillsbury HC. Influence of cochlear implant insertion depth on performance: a prospective randomized trial. Otol Neurotol. 2014 Dec;35(10):1773-9. doi: 10.1097/MAO.0000000000000541. PMID: 25122601.  Canfarotta MW, Dillon MT, Buchman CA, Buss E, O'Connell BP, Rooth MA, King ER, Pillsbury HC, Adunka OF, Brown KD. Long-Term Influence of Electrode Array Length on Speech Recognition in Cochlear Implant Users. Laryngoscope. 2021 Apr;131(4):892-897. doi: 10.1002/lary.28949. Epub 2020 Aug 1. PMID: 32738069; PMCID: PMC7855603.  Dillon MT, Canfarotta MW, Buss E, Rooth MA, Richter ME, Overton AB, Roth NE, Dillon SM, Raymond JH, Young A, Pearson AC, Davis AG, Dedmon MM, Brown KD, O'Connell BP. Influence of Electric Frequency-to-Place Mismatches on the Early Speech Recognition Outcomes for Electric-Acoustic Stimulation Users. Am J Audiol. 2023 Mar;32(1):251-260. doi: 10.1044/2022_AJA-21-00254. Epub 2023 Feb 17. PMID: 36800505; PMCID: PMC10166189.  Dillon MT, Buss E, Richter ME, Brown KD. Effects of Interaural Latency and Frequency Mismatch on Speech Recognition for Bimodal Cochlear Implant Users. Laryngoscope. 2025 Jun;135(6):2139-2145. doi: 10.1002/lary.32026. Epub 2025 Jan 27. PMID: 39871523.  Kurz A, Müller-Graff FT, Hagen R, Rak K. One Click Is Not Enough: Anatomy-Based Fitting in Experienced Cochlear Implant Users. Otol Neurotol. 2022 Dec 1;43(10):1176-1180. doi: 10.1097/MAO.0000000000003731. PMID: 36351223.   Kurz A, Herrmann D, Hagen R, Rak K. Using Anatomy-Based Fitting to Reduce Frequency-to-Place Mismatch in Experienced Bilateral Cochlear Implant Users: A Promising Concept. J Pers Med. 2023 Jul 8;13(7):1109. doi: 10.3390/jpm13071109. PMID: 37511722; PMCID: PMC10381201.  Kurz A, Herrmann D, Müller-Graff FT, Voelker J, Hackenberg S, Rak K. Anatomy-based fitting improves speech perception in noise for cochlear implant recipients with single-sided deafness. Eur Arch Otorhinolaryngol. 2025 Jan;282(1):467-479. doi: 10.1007/s00405-024-08984-4.  Landsberger DM, Svrakic M, Roland JT Jr, Svirsky M. The Relationship Between Insertion Angles, Default Frequency Allocations, and Spiral Ganglion Place Pitch in Cochlear Implants. Ear Hear. 2015 Sep-Oct;36(5):e207-13. doi: 10.1097/AUD.0000000000000163. PMID: 25860624; PMCID: PMC4549170.  Lee SE, Brown KD, Overton AB, Thompson NJ, Sloop AD, Richter ME, Canfarotta MW, Selleck AM, Dedmon MM, Dillon MT. Association of Tonotopic Mismatch With the Speech Recognition of Cochlear Implant Users With Unilateral Hearing Loss. Otol Neurotol. 2025 Sep 1;46(8):e302-e306. doi: 10.1097/MAO.0000000000004543. Epub 2025 May 15. PMID: 40423677.  Müller-Graff FT, Ilgen L, Schendzielorz P, Voelker J, Taeger J, Kurz A, Hagen R, Neun T, Rak K. Implementation of secondary reconstructions of flat-panel volume computed tomography (fpVCT) and otological planning software for anatomically based cochlear implantation. Eur Arch Otorhinolaryngol. 2022 May;279(5):2309-2319. doi: 10.1007/s00405-021-06924-0. Epub 2021 Jun 8. PMID: 34101009; PMCID: PMC8986679.  Müller-Graff FT, Voelker J, Kurz A, Hagen R, Neun T, Rak K. Accuracy of radiological prediction of electrode position with otological planning software and implications of high-resolution imaging. Cochlear Implants Int. 2023 May;24(3):144-154. doi: 10.1080/14670100.2022.2159128. Epub 2023 Jan 8. PMID: 36617441.  Müller-Graff FT, Spahn B, Herrmann DP, Kurz A, Völker J, Hagen R, Rak K. Comprehensive literature review on the application of the otological surgical planning software OTOPLAN® for cochlear implantation. HNO. 2024 Dec;72(Suppl 2):89-100. English. doi: 10.1007/s00106-023-01417-4. Epub 2024 Jun 11. PMID: 38861031.  Müller-Graff FT, Herrmann D, Spahn B, Voelker J, Kurz A, Neun T, Hackenberg S, Rak K. Position control of flexible electrodes with regard to intra-cochlear structure preservation and hearing outcomes: a retrospective study with implementation of the electrode contact view. Otol Neurotol. 2025 Jun 17. doi: 10.1097/MAO.0000000000004528. Online ahead of print.  Sloop AD, Richter ME, Scharf SP, Rooth MA, Brown KD, Dillon MT. Attrition in Cochlear Implant Research: Sociodemographic, Audiologic, and Performance Variables. Laryngoscope. 2026 Mar 6. doi: 10.1002/lary.70464. Epub ahead of print. PMID: 41792877.  

In this episode, Dr. Peter Nopp is joined by Prof. Griet Mertens and Dr. Masoud Zoka Assadi to explore individualized cochlear implantation, from anatomy-based electrode selection to anatomy-based fitting. They also explain how OTOPLAN supports clinicians before and after surgery by visualizing the patient's unique cochlea, guiding electrode selection, and calculating frequencies tonotopically for a precise postoperative fitting. Literature References: Creff G, Lambert C, Coudert P, Pean V, Laurent S, Godey B. Comparison of Tonotopic and Default Frequency Fitting for Speech Understanding in Noise in New Cochlear Implantees: A Prospective, Randomized, Double-Blind, Cross-Over Study. Ear Hear. 2024 Jan-Feb 01;45(1):35-52. doi: 10.1097/AUD.0000000000001423. Epub 2023 Oct 12. PMID: 37823850.  Kurz A, Herrmann D, Müller-Graff FT, Voelker J, Hackenberg S, Rak K. Anatomy-based fitting improves speech perception in noise for cochlear implant recipients with single-sided deafness. Eur Arch Otorhinolaryngol. 2025 Jan;282(1):467-479. doi: 10.1007/s00405-024-08984-4. Epub 2024 Sep 19. PMID: 39299967; PMCID: PMC11735480. Mertens G, Van de Heyning P, Vanderveken O, Topsakal V, Van Rompaey V. The smaller the frequency-to-place mismatch the better the hearing outcomes in cochlear implant recipients? Eur Arch Otorhinolaryngol. 2022 Apr;279(4):1875-1883. doi: 10.1007/s00405-021-06899-y. Epub 2021 Jun 15. PMID: 34131770. Lassaletta L, Calvino M, Sánchez-Cuadrado I, Muñoz E, Gavilán J. Can anatomy-based fitting improve musical perception in adult cochlear implant users? Braz J Otorhinolaryngol. 2025 Mar-Apr;91(2):101533. doi: 10.1016/j.bjorl.2024.101533. Epub 2024 Nov 19. PMID: 39566293; PMCID: PMC11617376.