Objective: In auditory neuropathy (AN) a dyssynchrony in the nerve conduction of the auditory nerve fibers is observed. Typically, patients with AN exhibit moderate to profound sensorineural hearing loss, and treatment using cochlear implants (CIs) or hearing aids should be performed as early as possible for a better hearing rehabilitation. The aim of this study is to evaluate the satisfaction level of patients with AN spectrum disorder treated using CIs. The Satisfaction with Amplification in Daily Life questionnaire was selected to evaluate 10 patients with AN treated using CIs.
Materials And Methods: Clinical study of patients with AN spectrum disorder submitted to CI. A retrospective data analysis, genetic and clinical evaluation in a tertiary referral center was done.
Results: The means of the subscales for positive effects, services and costs, negative factors, and personal image were 6.15, 4.6, 3.26, and 3.33, respectively.
Conclusions: Patients with AN treated using CIs consider themselves satisfied.
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http://dx.doi.org/10.5152/iao.2015.1695 | DOI Listing |
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.
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