Modern magnetic resonance imaging has significantly improved the diagnosis of cochlear nerve deficiencies. A careful assessment of all clinical, imaging, and auditory data is of utmost importance in such cases in order to properly establish the site of the abnormality. We report the case of a 3-year-old girl with unilateral cochlear nerve aplasia, normal middle and inner ear anatomy, and an absence of otoacoustic emissions, all of which erroneously suggested cochlear damage. We also briefly review the embryogenesis of the inner ear and auditory pathway. A patient with similar findings in the setting of a bilateral hearing loss and insufficient imaging would be at risk of inappropriate cochlear implantation.
Download full-text PDF |
Source |
|---|
Publication Analysis
Top Keywords
Similar Publications
Anatomical investigation of safety determining factors for keyhole drilling trajectories for robotic cochlear implant surgery.
Eur Arch Otorhinolaryngol
January 2025
Vrije Universiteit Brussel, Brussels Health Centre, Brussels, Belgium.
Purpose: Cochlear implants (CI) are the most successful bioprosthesis in medicine probably due to the tonotopic anatomy of the auditory pathway and of course the brain plasticity. Correct placement of the CI arrays, respecting the inner ear anatomy are therefore important. The ideal trajectory to insert a cochlear implant array is defined by an entrance through the round window membrane and continues as long as possible parallel to the basal turn of the cochlea.
View Article and Find Full Text PDFObjective: The aim of this study is to test the feasibility of a custom 3D-printed guide for performing a minimally invasive cochleostomy for cochlear implantation.
Study Design: Prospective performance study.
Setting: Secondary care.
Improving Real-Time Feedback During Cochlear Implantation: The Auditory Nerve Neurophonic/Cochlear Microphonic Ratio.
Ear Hear
January 2025
Department of Otorhinolaryngology and Head and Neck Surgery, Radboud University Medical Centre, Donders Institute for Brain, Cognition and Behaviour, Nijmegen, The Netherlands.
Objectives: Real-time monitoring of cochlear function to predict the loss of residual hearing after cochlear implantation is now possible. Current approaches monitor the cochlear microphonic (CM) during implantation from the electrode at the tip of the implant. A drop in CM response of >30% is associated with poorer hearing outcomes.
View Article and Find Full Text PDFDeciphering compromised speech-in-noise intelligibility in older listeners: the role of cochlear synaptopathy.
eNeuro
January 2025
Hearing Technology @ WAVES, Department of Information Technology, Ghent University, Technologiepark 216, 9052 Zwijnaarde, Belgium
Speech intelligibility declines with age and sensorineural hearing damage (SNHL). However, it remains unclear whether cochlear synaptopathy (CS), a recently discovered form of SNHL, significantly contributes to this issue. CS refers to damaged auditory-nerve synapses that innervate the inner hair cells and there is currently no go-to diagnostic test available.
View Article and Find Full Text PDFCircadian rhythm disruptions exacerbate inner ear damage in a murine endolymphatic hydrops model.
FASEB J
January 2025
Department of Otolaryngology-Head and Neck Surgery, Xiangya Hospital, Central South University, Changsha, Hunan, China.
Meniere's disease (MD) is an inner ear disease characterized by endolymphatic hydrops (EH). Maintaining a regular daily routine is crucial for MD patients. However, the relationship between circadian rhythms and MD remains unclear.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!