In the past decade, we have encountered two patients with unilateral long-term deaf ear having vestibular schwannoma. One was on the deaf ear in a 53 years' woman and the other was on the contralateral hearing ear in a 62 years' woman. These two patients had total deafness on the right ear since childhood, associated with absent auditory brainstem response (ABR) and caloric responses, whereas normal ABR and caloric responses were shown on the left ear. Although similar physiological findings were disclosed on the deaf ears, MRI scan demonstrated vestibular schwannoma on the different side, that is, one in deaf ear and the other in hearing ear. For the former, absent ABR and caloric results indicate that the tumor originates from the superior vestibular nerve. In contrast with the latter, normal ABR and caloric responses, but absent vestibular-evoked myogenic potentials on the hearing ear imply that the tumor originates from the inferior vestibular nerve. The ipsilateral tumor on the deaf ear underwent intracranial surgical excision, followed by radiosurgery due to tumor recurrence 10 years later, while the contralateral one on the only hearing ear received radiosurgery only. Both patients were rather well, 3 years after last treatment.
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http://dx.doi.org/10.1007/s00405-006-0236-2 | DOI Listing |
Trends Hear
January 2025
Key Laboratory of Noise and Vibration Research, Institute of Acoustics, Chinese Academy of Sciences, Beijing, China.
Wide dynamic range compression (WDRC) and noise reduction both play important roles in hearing aids. WDRC provides level-dependent amplification so that the level of sound produced by the hearing aid falls between the hearing threshold and the highest comfortable level of the listener, while noise reduction reduces ambient noise with the goal of improving intelligibility and listening comfort and reducing effort. In most current hearing aids, noise reduction and WDRC are implemented sequentially, but this may lead to distortion of the amplitude modulation patterns of both the speech and the noise.
View Article and Find Full Text PDFGenes (Basel)
January 2025
Department of Hearing Implant Sciences, Shinshu University School of Medicine, Matsumoto 390-8621, Japan.
Background/objectives: The gene is responsible for autosomal recessive non-syndromic sensorineural hearing loss and is assigned as DFNB18B. To date, 44 causative variants have been reported to cause non-syndromic hearing loss. However, the detailed clinical features for -associated hearing loss remain unclear.
View Article and Find Full Text PDFHear Res
December 2024
Bionics Institute, East Melbourne, Victoria 3002, Australia; Department of Medical Bionics, The University of Melbourne, Fitzroy, Victoria 3065, Australia; Department of Surgery (Otolaryngology), University of Melbourne, The Royal Victorian Eye and Ear Hospital, East Melbourne, Victoria 3002, Australia. Electronic address:
In the adult mammalian cochlea, hair cell loss is irreversible and causes deafness. The basic helix-loop transcription factor Atoh1 is essential for normal hair cell development in the embryonic ear. Over-expression of Atoh1 in the adult cochlea by gene therapy can convert supporting cells (cells that underlie hair cells) into a hair cell lineage.
View Article and Find Full Text PDFBrain Commun
January 2025
Centre for Cognitive Neuroscience, University of Salzburg, 5020 Salzburg, Austria.
Former studies have established that individuals with a cochlear implant (CI) for treating single-sided deafness experience improved speech processing after implantation. However, it is not clear how each ear contributes separately to improve speech perception over time at the behavioural and neural level. In this longitudinal EEG study with four different time points, we measured neural activity in response to various temporally and spectrally degraded spoken words presented monaurally to the CI and non-CI ears (5 left and 5 right ears) in 10 single-sided CI users and 10 age- and sex-matched individuals with normal hearing.
View Article and Find Full Text PDFEar Hear
January 2025
Dutch Foundation of the Deaf and Hard of Hearing Child (NSDSK), Amsterdam, The Netherlands.
Objectives: One important aspect in facilitating language access for children with hearing loss (HL) is the auditory environment. An optimal auditory environment is characterized by high signal to noise ratios (SNRs), low background noise levels, and low reverberation times. In this study, the authors describe the auditory environment of early intervention groups specifically equipped for young children with HL.
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