Objective: To evaluate the pathological position of auditory neuropathy and investigate the hearing and speech rehabilitation results of cochlear implantation in patients with auditory neuropathy.
Method: In our hospital, among the patients received cochlear implantation, 8 cases with auditory neuropathy were selected, and 8 cases of non-auditory neuropathy patients with profound deafness were selected as matched control group with the background close to the study group. The preoperative hearing data of these two groups were retrospectively analyzed. During operation, the homemade stimulation electrodes were inserted to test the electric evoked auditory brainstem response(EABR) for assessing the auditory pathway; EABR and neural response telemetry(NRT) were tested after implantation, and T, C value were acquired 1 month later. CAP, SIR and speech recognition rate were used to assess hearing and speech rehabilitation effect 12 months after booting.
Result: Intra-operative EABR wave can be derived in 8 cases of auditory neuropathy, but the wave pattern exhibited variations compared with normal wave. It needed increased stimulation or adjusted parameters, with variable V latency. After cochlear implantation, the waveforms of NRT and EABR were similar between the two groups, and the post-operative V waveform was close to the intra-operative EABR. 8 pairs of patients can present listening response after booting. There was no statistically significant difference in T, C value, CAP (6.50 +/- 0.94 and 6.90 +/- 0.77) and speech recognition rate (85.00% +/- 11.66% and 89.50% +/- 9.02%) between the auditory neuropathy group and the control group 1 year after booting.
Conclusion: Pre-operative EABR can be used as an effective tool to assess the auditory pathway of auditory neuropathy patients, improving the pre-operative examination and helping with selecting the suitable cochlear implant patient. Cochlear implantation can help patients with auditory neuropathy to improve hearing and speech.
Download full-text PDF |
Source |
---|
Ann Otol Rhinol Laryngol
January 2025
Department of Otolaryngology-HNS, MedStar Georgetown University Hospital, Washington, DC, USA.
Objective: To assess outcomes of CI in adolescent patients with ANSD, a population which has not yet been comprehensively reviewed through a scoping review.
Methods: A scoping review in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. A comprehensive search of MEDLINE, EMBASE, Cochrane DSR, Cochrane CENTRAL, CINAHL, and Web of Science was performed.
Front Mol Neurosci
January 2025
Department of Otorhinolaryngology, The Affiliated Changsha Central Hospital, Hengyang Medical School, University of South China, Changsha, China.
Auditory neuropathy spectrum disorder (ANSD) is an auditory dysfunction disorder characterized by impaired speech comprehension. Its etiology is complex and can be broadly categorized into genetic and non-genetic factors. mutation is identified as a causative factor in ANSD.
View Article and Find Full Text PDFJ Commun Disord
December 2024
MRC Cognition and Brain Sciences Unit, University of Cambridge, Cambridge, UK.
Objectives: The objectives of this study were to evaluate the relationships between auditory processing, speech discrimination, and listening and communication abilities in children diagnosed with auditory neuropathy spectrum disorder (ANSD) compared to those with normal hearing.
Methods: This was a case-control study involving seventeen participants with a diagnosis of ANSD who used spoken language as their primary mode of communication and wore hearing aids or were unaided and 21 normally hearing controls. All underwent a battery of behavioural measures of hearing, speech perception and auditory processing skills.
Orphanet J Rare Dis
January 2025
Senior Department of Otolaryngology Head and Neck Surgery, The 6th Medical Center of Chinese PLA General Hospital, Chinese PLA Medical School, Beijing, 100048, China.
Background: Non-isolated auditory neuropathy (AN), or syndromic AN, is marked by AN along with additional systemic manifestations. The diagnostic process is challenging due to its varied symptoms and overlap with other syndromes. This study focuses on two mitochondrial function-related genes which result in non-isolated AN, FDXR and TWNK, providing a summary and enrichment analysis of genes associated with non-isolated AN to elucidate the genotype-phenotype correlation and underlying mechanisms.
View Article and Find Full Text PDFJ Otol
July 2024
Department of Audiology, All India Institute of Speech and Hearing, Mysuru, Karnataka, India.
Purpose: The present systematic review examined imaging findings in the Auditory Neuropathy Spectrum Disorder (ANSD) population.
Methods: Electronic databases such as Pub Med, Google Scholar, J Gate, and Science Direct were used to conduct a literature search. The articles retrieved through the literature search were assessed in two stages.
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!