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Object: Neurofibromatosis Type 2 (NF2) has typically resulted in deafness after surgical removal of bilateral vestibular schwannomas (VSs). Cochlear implants are generally ineffective for this kind of deafness because of the loss of continuity in the auditory nerve after tumor removal. The first auditory brainstem implant (ABI) in such a patient was performed in 1979 at the House Ear Institute, and this individual continues to benefit from electrical stimulation of the cochlear nucleus complex. In 1992, an advanced multichannel ABI was developed and a series of patients with NF2 received this implant to study the safety and efficacy of the device.
Methods: At the time of first- or second-side VS removal, patients received an eight-electrode array applied to the surface of the cochlear nucleus within the confines of the lateral recess of the fourth ventricle. The device was activated approximately 6 weeks after implantation. and patients were tested every 3 months for the 1st year after the initial stimulation, and annually thereafter. The protocol included a comprehensive battery of psychophysical and speech perception tests.
Conclusions: The multichannel ABI proved to be effective and safe in providing useful auditory sensations in most patients with NF2. The ABI improved patients' ability to communicate compared with the lipreading-only condition, it allowed the detection and recognition of many environmental sounds, and in some cases it provided significant ability to understand speech by using just the sound from the ABI (with no lipreading cues). Its performance in most patients has continued to improve for up to 8 years after implantation.
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http://dx.doi.org/10.3171/jns.2002.96.6.1063 | DOI Listing |
Med Eng Phys
October 2025
Department of Bioengineering, Technological University of Havana "José Antonio Echeverría", Cuba.
Congenital hearing loss is a significant health problem, with a worldwide incidence of >6 per 1000 live births. Late diagnosis will delay appropriate treatment, leading to potential neurodevelopment problems. Early diagnosis requires neonatal hearing screening, where one of the most used techniques is automated Auditory Brainstem Responses (aABR).
View Article and Find Full Text PDFAm J Hum Genet
September 2025
Department of Clinical Genetics, Erasmus MC, University Medical Center Rotterdam, PO Box 2040, Rotterdam 3000 CA, the Netherlands.
Microtubule-actin cross-linking factor 1 (MACF1) is a large protein of the spectraplakin family, which is essential for brain development. MACF1 interacts with microtubules through the growth arrest-specific 2 (Gas2)-related (GAR) domain. Heterozygous MACF1 missense variants affecting the zinc-binding residues in this domain result in a distinctive cortical and brain stem malformation.
View Article and Find Full Text PDFIEEE Trans Biomed Eng
September 2025
Objective: Transcranial ultrasound (US) stimulation (TUS) has emerged as a promising technique for minimally invasive, localized, deep brain stimulation. However, indirect auditory effects during neuromodulation require careful consideration, particularly in experiments with rodents. One method to prevent auditory responses involves applying tapered envelopes to US bursts.
View Article and Find Full Text PDFEur J Pharm Sci
September 2025
Department of Otorhinolaryngology-Head and Neck Surgery, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea. Electronic address:
Intratympanic (IT) delivery of dexamethasone (DEX) is widely used for treating inner ear disorders; however, its therapeutic efficacy is limited by poor permeability of the round window membrane (RWM). This study aimed to evaluate and compare the efficacy and safety of three pharmacological agents-histamine (HIS), 3% hypertonic saline (3% HS), and sodium caprate (SC)-as adjuvants for enhancing RWM permeability and improving IT-DEX delivery in a murine model. Following IT administration of each permeability enhancer followed by DEX injection, perilymph DEX concentrations were measured using ultra-high-performance liquid chromatography, and DEX receptor expression in the organ of Corti was assessed by immunofluorescence.
View Article and Find Full Text PDFNeuroscience
September 2025
Department of Biomedical Engineering, Southern University of Science and Technology, Shenzhen, Guangdong 518055, China. Electronic address:
The auditory brainstem response (ABR) remains the gold standard for evaluating hearing function in both animal models and humans. Features of ABR, including threshold, wave I amplitude and latency are critical for diagnosing and investigating the mechanisms of hearing loss. Critically, the rapid proliferation of genetically engineered mouse models in hearing research has created an imperative demand for high-throughput ABR testing capabilities.
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