Patients with auditory neuropathy can hear but they cannot understand. Their difficulties have been traced to timingrelated deficits, revealing the importance of the neural encoding of timing cues for understanding speech. The absence of brain stem evoked responses indicates that firing of neurons in the early stages of the auditory pathway is abnormally asynchronous in these patients. Understa...

Is there a relationship? I t became clear in the last decades that neuropathy is not a separate clinical entity, but a component of several related complications (1). Although the functional consequences of neuropathy are well defined in various organ systems, the relationship of the alterations in the networks of the neuronal system is still poorly documented. Assessment of the potential commo...

Abstract Background Auditory neuropathy spectrum disorder (ANSD) is characterized by normal OHCs function as shown intact cochlear microphonics (CMs) and/or otoacoustic emissions (OAEs); absent or grossly abnormal auditory brainstem responses (ABRs) and middle ear muscle reflexes. This study designed to address whether the input/output of distortion product OAEs (DPOAEs I/O) in ANSD patients si...

PURPOSE To define the phenotype and elucidate the molecular basis for an autosomal recessively inherited optic atrophy and auditory neuropathy in a consanguineous family with two affected children. METHODS Family members underwent detailed ophthalmologic, electrophysiological, and audiological assessments. An autozygosity mapping strategy using high-density single nucleotide polymorphism micr...

Background. The aim of this study was to investigate the individual effects of envelope enhancement and high-pass filtering (500 Hz) on word identification scores in quiet for individuals with Auditory Neuropathy. Method. Twelve individuals with Auditory Neuropathy (six males and six females) with ages ranging from 12 to 40 years participated in the study. Word identification was assessed using...

We performed brainstem auditory evoked potential (BAEP) examinations in 15 patients with long-standing type 1 diabetes mellitus. We applied cardiovascular reflex tests for assessment of autonomic neuropathy. The aim of our investigation was to compare the BAEP results of this patient group with those of controls and to look for the possible correlation between alteration of the auditory brainst...

The auditory nerve is the primary conveyor of hearing information from sensory hair cells to the brain. It has been believed that loss of the auditory nerve is irreversible in the adult mammalian ear, resulting in sensorineural hearing loss. We examined the regenerative potential of the auditory nerve in a mouse model of auditory neuropathy. Following neuronal degeneration, quiescent glial cell...