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Management of Unilateral Hearing Loss in a 14-Year-Old with Internal Auditory Canal Duplication Using a Bonebridge Bone Conduction Implant.
Am J Case Rep
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Oto-Rhino-Laryngology Surgery Clinic, Institute of Physiology and Pathology of Hearing - World Hearing Center, Kajetany, Poland.
BACKGROUND Duplicated internal auditory canal (dIAC) is a rare congenital temporal bone anomaly associated with ipsilateral sensorineural hearing loss (SNHL). The Bonebridge bone conduction implant has a magnet, an internal transducer, and an external audio processor. This report is of a 14-year-old girl with unilateral SNHL and vestibulocochlear nerve (VIII cranial nerve) aplasia due to dIAC who was treated with a Bonebridge bone conduction implant.
View Article and Find Full Text PDFObjective: 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.
Gene therapy for inner ear disease: the next targets.
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Department of Otolaryngology Head and Neck Surgery, University of Vienna, Vienna, Austria.
Purpose Of Review: The recent successful otoferlin gene therapy trials have refocused the fields attention on the potential of gene therapy to cure hearing loss. With over 100 known monogenetic causes of hearing loss, the key question is which will be the next set of disorders that are treatable. The current review addresses potentially targetable hearing disorders that can be addressed with current gene therapy technologies.
View Article and Find Full Text PDFHearing Loss in Leucine-Rich Glioma-Inactivated 1 Encephalitis: Cochlear Implantation Considerations.
Cureus
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Otolaryngology - Head and Neck Surgery, Manatee Memorial Hospital, Bradenton, USA.
Leucine-rich glioma-inactivated 1 (LGI-1) antibody-associated autoimmune encephalitis is a rare neurologic disorder primarily presenting with memory impairment, behavioral changes, and seizures. Auditory symptoms, particularly sensorineural hearing loss (SNHL), are uncommon but may occur due to LGI-1 expression in auditory pathways. A 78-year-old male with a prior diagnosis of LGI-1 autoimmune encephalitis presented with progressive bilateral SNHL and frequent falls.
View Article and Find Full Text PDFLimited usable hearing unilaterally (LUHU) in infants and young children: a scoping review of technology considerations and developmental outcomes.
Int J Audiol
September 2025
School of Communication Sciences and Disorders and the National Centre for Audiology, Western University, London, Ontario, Canada.
Objectives: The objective of this scoping review was to examine the developmental impact of limited usable hearing unilaterally (LUHU) and surgical and non-surgical technology outcomes specific to infants and young children who have LUHU.
Design: The Joanna Briggs Institute (JBI) Model of Evidence-Based Healthcare provided a framework. Covidence software was used to manage the articles.