Objective: To report and evaluate the results of auditory brainstem implantation in a case of postmeningitis hearing loss with totally ossified cochleae on both sides.
Study Design: Case report.
Setting: Tertiary referral center.
Patient: A 49-year-old man was referred to the authors' department for bilateral total hearing loss subsequent to bacterial meningitis 2 years earlier. Pure tone audiometry, auditory brainstem response tests, and promontory tests did not reveal any cochlear activity. Computed tomography showed bilateral and totally ossified cochleae. Magnetic resonance imaging confirmed the diagnosis by showing no cochlear signal on T2-weighed images and ruled out brainstem and cerebellopontine angle abnormalities.
Intervention: A left auditory brainstem implantation was performed through a translabyrinthine route, using a Nucleus 22-channel device.
Main Outcome Measures: Word and sentence recognition tests in sound-only and sound plus vision modes.
Results: No postoperative complication was observed. Twelve electrodes could be activated, and their tonotopy was defined. Nine other electrodes were inactivated because of an absence of auditory response (4 electrodes) or paresthesia (5 electrodes). At the last follow-up visit, 26 months after the implantation, 50% of disyllabic word scores and 60% of sentence scores were achieved using auditory brainstem implant sound only. These scores reached 80% and 93%, respectively, with lip-reading.
Conclusion: Auditory brainstem implantation is an efficient means of auditory rehabilitation in cases of bilateral total hearing loss with totally ossified cochleae. It should be considered in cases of predictable failure in cochlear implantation.
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http://dx.doi.org/10.1097/00129492-200301000-00016 | DOI Listing |
Exp Eye Res
January 2025
Shenzhen Eye Hospital, Jinan University, Shenzhen Eye Institute, Shenzhen, 518040 Guangdong, China. Electronic address:
Usher syndrome is a rare autosomal recessive genetic disorder that primarily affects both vision and hearing, manifesting as sensorineural hearing loss and progressive vision loss caused by retinitis pigmentosa. The pathogenesis of retinal degeneration in Usher syndrome is still largely unknown. In this study, a novel Ush2a knockout mouse model was successfully constructed using CRISPR/Cas9 technology.
View Article and Find Full Text PDFPLoS One
January 2025
Institute of Endocrinology, Prague, Czech Republic.
Objectives: Hearing impairment can have major impacts on behavior, educational attainment, social status, and quality of life. In congenital hypothyroidism, the incidence of hearing impairment reaches 35-50%, while in acquired hypothyroidism there is a reported incidence of 25%. Despite this, knowledge of the pathogenesis, incidence and severity of hearing impairment remains greatly lacking.
View Article and Find Full Text PDFJ Neurol Surg B Skull Base
February 2025
Department of Neurosurgery, UC San Diego Medical Center, San Diego, California, United States.
Many patients with neurofibromatosis type 2 (NF2) suffer from sensorineural hearing loss, and associated cochlear nerve compromise in NF2 patients makes auditory brainstem implant (ABI) an attractive treatment option. The long-term outcomes and benefits of the device are still being explored. A retrospective review was conducted for 11 ABI recipients at a single-institution tertiary center between November 2017 and August 2022.
View Article and Find Full Text PDFInt J Audiol
January 2025
Department of Audiology, School of Rehabilitation Sciences, Iran University of Medical Sciences, Tehran, Iran.
Objective: Tinnitus and its pathophysiological mechanisms need more investigation because tinnitus may change the typical processing of sounds in the auditory system. Poor temporal resolution, which is not assessed with conventional subjective tinnitus evaluations, has been reported in some tinnitus sufferers.
Design: This study used a gap-in-noise paradigm to assess temporal resolution in tinnitus sufferers using both behavioural and electrophysiologic methods.
Elife
January 2025
Department of Anesthesia, Women's Hospital, Zhejiang University School of Medicine, Hangzhou, China.
The inferior colliculus (IC) has traditionally been regarded as an important relay in the auditory pathway, primarily involved in relaying auditory information from the brainstem to the thalamus. However, this study uncovers the multifaceted role of the IC in bridging auditory processing, sensory prediction, and reward prediction. Through extracellular recordings in monkeys engaged in a sound duration-based deviation detection task, we observed a 'climbing effect' in neuronal firing rates, indicative of an enhanced response over sound sequences linked to sensory prediction rather than reward anticipation.
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