UNLABELLED OBJECTIVE. Auditory neuropathy spectrum disorder (ANSD) affects approximately 10% of patients with sensorineural hearing loss. While many studies report abnormalities at the level of the cochlea, auditory nerve, and brainstem in children with ANSD, much less is known about their cortical development. We examined central auditory maturation in 21 children with ANSD. DESIGN. Morphology...

Friedreich ataxia (FRDA) is a neurodegenerative disease affecting motor and sensory systems. This study aimed to investigate the presence and perceptual consequences of auditory neuropathy (AN) in affected individuals and examine the use of personal-FM systems to ameliorate the resulting communication difficulties. Ten individuals with FRDA underwent a battery of auditory function tests and the...

BACKGROUND Auditory neuropathy/dysynchrony (AN/AD) typically develops early in life and is bilateral in nature. PURPOSE Herein, we describe an unusual finding of late-onset unilateral AN/AD based on reported case history and audiometric findings. RESEARCH DESIGN A 64-year-old female presented with a complaint of a progressive unilateral hearing loss that had developed over the past two-thre...

PURPOSE To analyze speech perception in children with pre-lingual hearing loss with auditory neuropathy spectrum disorder users of bilateral hearing aid. METHODS This is a descriptive and exploratory study carried out at the Research Center Audiological (HRAC/USP). The study included four children aged between 8 years and 3 months and 12 years and 2 months. Lists of monosyllabic words, two sy...