Arnold Starr
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Pathophysiological mechanisms and functional hearing consequences of auditory neuropathy Open
The effects of inner ear abnormality on audibility have been explored since the early 20th century when sound detection measures were first used to define and quantify 'hearing loss'. The development in the 1970s of objective measures of c…
Audibility, speech perception and processing of temporal cues in ribbon synaptic disorders due to OTOF mutations Open
Mutations in the OTOF gene encoding otoferlin result in a disrupted function of the ribbon synapses with impairment of the multivesicular glutamate release. Most affected subjects present with congenital hearing loss and abnormal auditory …
Mutations of Human NARS2, Encoding the Mitochondrial Asparaginyl-tRNA Synthetase, Cause Nonsyndromic Deafness and Leigh Syndrome Open
Here we demonstrate association of variants in the mitochondrial asparaginyl-tRNA synthetase NARS2 with human hearing loss and Leigh syndrome. A homozygous missense mutation ([c.637G>T; p.Val213Phe]) is the underlying cause of nonsyndromic…
Cortical activity during perception of musical rhythm: Comparing musicians and nonmusicians. Open
This study investigates the effects of musical training on brain activity to violations of rhythmic expectancies. We recorded behavioral and event-related brain potential (ERP) responses of musicians and non-musicians to discrepancies of r…
Auditory neuropathies and electrocochleography Open
Auditory neuropathy (AN) is a hearing disorder characterized by disruption of temporal coding of acoustic signals in auditory nerve fibres resulting in impairment of auditory perceptions relying on temporal cues (1, 2). Mechanisms suggeste…
Review of Hair Cell Synapse Defects in Sensorineural Hearing Impairment Open
Hair cell ribbon synapses are highly specialized to enable indefatigable sound encoding with utmost temporal precision. Their dysfunctions, which we term auditory synaptopathies, impair audibility of sounds to varying degrees but commonly …
Loudness adaptation accompanying ribbon synapse and auditory nerve disorders Open
Abnormal auditory adaptation is a standard clinical tool for diagnosing auditory nerve disorders due to acoustic neuromas. In the present study we investigated auditory adaptation in auditory neuropathy owing to disordered function of inne…
Auditory cortical activity in normal hearing subjects to consonant vowels presented in quiet and in noise Open
VOTs of stop consonants affect auditory cortical activities differently as a function of the position of the consonant in the utterance.
Spatiotemporal distribution of cortical processing of first and second languages in bilinguals. II. Effects of phonologic and semantic priming Open
This study determined the effects of phonology and semantics on the distribution of cortical activity to the second of a pair of words in first and second language (mixed pairs). The effects of relative proficiency in the two languages and…
Spatiotemporal distribution of cortical processing of first and second languages in bilinguals. I. Effects of proficiency and linguistic setting Open
The study determined how spatiotemporal distribution of cortical activity to words in first and second language is affected by language, proficiency, and linguistic setting. Ten early bilinguals and 14 late adult bilinguals listened to pai…
Auditory processing deficits in individuals with primary open-angle glaucoma Open
The finding that a significant proportion of subjects with open-angle glaucoma presented with auditory dysfunction provides evidence of systemic neuronal susceptibility. Affected individuals may suffer significant communication difficultie…
Auditory cortical N100 in pre- and post-synaptic auditory neuropathy to frequency or intensity changes of continuous tones Open
Abnormalities of auditory cortical N100 in AN reflect disorders of both temporal processing (low frequency) and neural adaptation (high frequency). Auditory N100 latency to the low frequency provides an objective measure of the degree of i…
A comparison of auditory evoked potentials to acoustic beats and to binaural beats Open
The purpose of this study was to compare cortical brain responses evoked by amplitude modulated acoustic beats of 3 and 6 Hz in tones of 250 and 1000 Hz with those evoked by their binaural beats counterparts in unmodulated tones to indicat…
Cortical evoked potentials to an auditory illusion: Binaural beats Open
Brain activity corresponding to an auditory illusion of low frequency beats can be recorded from the scalp.
N100 cortical potentials accompanying disrupted auditory nerve activity in auditory neuropathy (AN): Effects of signal intensity and continuous noise Open
N100 latency to tones may serve as an objective measure of the efficiency of auditory temporal processes.
Intensity changes in a continuous tone: Auditory cortical potentials comparison with frequency changes Open
These results suggest that intensity and frequency discrimination employ distinct central processes.
Auditory-evoked potentials to frequency increase and decrease of high- and low-frequency tones Open
The effects of base frequency and direction of change may reflect brain networks involved in more complex processing such as speech that are differentially sensitive to frequency modulations of high (consonant discrimination) and low (vowe…
ApoE genotype and abnormal auditory cortical potentials in healthy older females Open
Apolipoprotein E (ApoE) status and gender are risk factors for the development of Alzheimer's disease. Alzheimer's disease is more prevalent in female relative to male carriers of the ApoE epsilon 4 gene. We examined cortical sensory (P50,…
Frequency changes in a continuous tone: Auditory cortical potentials Open
These data provide electrophysiological evidence that central processing of spectral changes in the cortex differs for low and high frequencies. Some of these differences may be related to both temporal- and spectral-based coding at the au…
Auditory nerve is affected in one of two different point mutations of the neurofilament light gene Open
The absence of "deafness" may reflect the ability of central mechanisms to compensate for the slowly developing auditory nerve abnormalities.
The N1 complex to gaps in noise: Effects of preceding noise duration and intensity Open
Duration and intensity interact to define the dual peaked N-Complex, signaling the cessation of an ongoing sound.
Cholinesterase inhibitors affect brain potentials in amnestic mild cognitive impairment Open
Amnestic mild cognitive impairment (MCI) is an isolated episodic memory disorder that has a high likelihood of progressing to Alzheimer's disease. Auditory sensory cortical responses (P50, N100) have been shown to be increased in amplitude…
Memory Evaluation in Mild Cognitive Impairment using Recall and Recognition Tests Open
Amnestic mild cognitive impairment (MCI) is a selective episodic memory deficit that often indicates early Alzheimer's disease. Episodic memory function in MCI is typically defined by deficits in free recall, but can also be tested using r…
Relapsing polychondritis with features of dementia with Lewy bodies Open
We describe a 72-year old man with clinical features suggestive of dementia with Lewy bodies (DLB) who proved neuropathologically to have degeneration induced by relapsing polychondritis (RP), an autoimmune inflammatory disorder of cartila…
OTOF mutations revealed by genetic analysis of hearing loss families including a potential temperature sensitive auditory neuropathy allele Open
Introduction: The majority of hearing loss in children can be accounted for by genetic causes. Non-syndromic hearing loss accounts for 80% of genetic hearing loss in children, with mutations in DFNB1/GJB2 being by far the most common cause…