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The development of cochlear implantation has allowed the majority of patients deafened after the development of language to regain significant auditory benefit. In a subset of patients, however, loss of hearing results from destruction of the cochlear nerves, rendering cochlear implantation ineffective. The most common cause of bilateral destruction of the cochlear nerves is neurofibromatosis type 2 (NF2). The hallmark of this genetic disorder is the development of bilateral acoustic neuromas, the growth or removal of which causes deafness in most patients. Patients with NF2 may benefit from direct stimulation of the cochlear nucleus. We describe the development, use, and results of the auditory brainstem implant (ABI), which is typically implanted via craniotomy at the time of tumor removal. Most patients with the implant have good appreciation of environmental sounds, but obtain more modest benefit with regard to speech perception. The majority of patients make use of the implant to facilitate lip reading; some can, to varying degrees, comprehend speech directly. We discuss future directions in central implants for hearing, including the penetrating ABI, the use of ABI in nontumor patients, and the auditory midbrain implant.
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http://dx.doi.org/10.1016/j.nurt.2007.10.068 | DOI Listing |
Neuroscience
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.
View Article and Find Full Text PDFPLoS Biol
September 2025
Neuroscience Training Program, University of Colorado Anschutz Medical Campus, Aurora, Colorado, United States of America.
Early sensory experience can exert lasting perceptual consequences. For example, a brief period of auditory deprivation early in life can lead to persistent spatial hearing deficits. Some forms of hearing loss (i.
View Article and Find Full Text PDFJ Oral Maxillofac Surg
August 2025
Associate Professor, Department of Dentistry, Government Medical College, Nagpur, Maharashtra, India.
Background: Cleft palate repair aims to achieve tension-free closure without compromising Eustachian tube function and hearing outcomes. The effect of pterygoid hamulotomy on postoperative hearing remains inconclusive.
Purpose: This study evaluated the effect of pterygoid hamulotomy during palatoplasty on hearing thresholds and middle ear status in nonsyndromic cleft palate patients.
Objective: This study aims to evaluate long-term auditory outcomes in patients with inner ear malformations (IEMs) treated with cochlear or auditory brainstem implants (CI/ABI), and to 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 (SRS) at defined intervals.
Otol Neurotol
August 2025
Department of Otolaryngology, Institute of Science Tokyo, 1-5-45 Yushima, Bunkyo-ku, Tokyo 113-8519, Japan.
Objective: To characterize the transition of audiometric features in patients with inner ear schwannoma (IES) with a special focus on transient mixed hearing loss.
Patients: Twelve patients were clinically diagnosed with an IES.
Interventions: All patients underwent otoscopic and audiological examinations, including serial pure-tone audiometry and magnetic resonance imaging (MRI) scans.