Absence of Both Auditory Evoked Potentials and Auditory Percepts Dependent on Timing Cues

Starr, Arnold; McPherson, David L.; Patterson, Julie V.; Don, Manuel; Luxford, William M.; Shannon, Robert V.; Sininger, Yvonne S.; Tonakawa, L.; Waring, Michael D.

doi:10.1093/brain/114.3.1157

Cited by 173 publications

(175 citation statements)

References 1 publication

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“…A similar pattern of auditory nerve atrophy with normal hair cells had been noted previously in temporal bone studies of individual patients with deafness and hereditary neuropathy (Spoendlin 1974;Hallpike et al 1980) before the availability of clinical test procedures [otoacoustic emissions (OAEs), auditory brainstem responses (ABRs)] now used to distinguish between disorders of hair cells and auditory nerve (Starr et al 1991).…”

Section: Introductionsupporting

confidence: 65%

“…Separate tone burst averages were made to condensation and rarefaction signals to define cochlear microphonics by the appearance of phase-reversed potentials in the superimposed averages. Cochlear microphonics can be distinguished from recordings of electrical artifacts of the voltage applied to the earphone if clamping of the tube conducting the sound to the ear is accompanied by a marked attenuation of the potentials (see Starr et al 1991 for details). Amplifier bandpass for clicks and tone bursts was set between Direct Current (DC) and 3 kHz.…”

Section: Neurophysiologymentioning

confidence: 99%

“…The hearing loss is characterized by disproportionate effects on auditory temporal processes relative to pure tone thresholds with speech perception and binaural hearing being profoundly impaired (Starr et al 1991;Zeng et al 1999). The use of hearing aids to amplify acoustic signals is usually without benefit.…”

Section: Introductionmentioning

confidence: 99%

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A Dominantly Inherited Progressive Deafness Affecting Distal Auditory Nerve and Hair Cells

Starr

Isaacson

Michalewski

et al. 2004

JARO

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We have studied 72 members belonging to a large kindred with a hearing disorder inherited in an autosomal dominant pattern. We used audiological, physiological, and psychoacoustic measures to characterize the hearing disorders. The initial phenotypic features of the hearing loss are of an auditory neuropathy (AN) with abnormal auditory nerve and brainstem responses (ABRs) and normal outer hair cell functions [otoacoustic emissions (OAEs) and cochlear microphonics (CMs)]. Psychoacoustic studies revealed profound abnormalities of auditory temporal processes (gap detection, amplitude modulation detection, speech discrimination) and frequency processes (difference limens) beyond that seen in hearing impairment accompanying cochlear sensory disorders. The hearing loss progresses over 10-20 years to also involve outer hair cells, producing a profound sensorineural hearing loss with absent ABRs and OAEs. Affected family members do not have evidence of other cranial or peripheral neuropathies. There was a marked improvement of auditory functions in three affected family members studied after cochlear implantation with return of electrically evoked auditory brainstem responses (EABRs), auditory temporal processes, and speech recognition. These findings are compatible with a distal auditory nerve disorder affecting one or all of the components in the auditory periphery including terminal auditory nerve dendrites, inner hair cells, and the synapses between inner hair cells and auditory nerve. There is relative sparing of auditory ganglion cells and their axons.

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Section: Introductionsupporting

confidence: 65%

Section: Neurophysiologymentioning

confidence: 99%

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A Dominantly Inherited Progressive Deafness Affecting Distal Auditory Nerve and Hair Cells

Starr

Isaacson

Michalewski

et al. 2004

JARO

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“…We recently reported a patient with bilateral auditory nerve timing dysfunction (Starr et al, 1991) who was unable to fuse binaural signals. Van der Poel et al (1989) reported two patients with multiple sclerosis who were similarly unable to perform an interaural timing difference task because of a failure of binaural fusion.…”

Section: Discussionmentioning

confidence: 99%

Auditory time-intensity cues in the binaural interaction component of the auditory evoked potentials

McPherson

Starr

1995

Hearing Research

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Binaural interaction in the brainstem and middle latency auditory evoked potentials to intensity (dl) and timing differences (dT) between the two ears was studied in 10 normal hearing young adults. A component reflecting binaural interaction in the brainstem potentials occurred at approximately 7 ms and was of largest amplitude when d I and dT were 0. The latency of the binaural interaction component gradually shifted and its amplitude decreased as dl or dT increased and binaural interaction became undetectable when dI = 16 dB or when dT_> 1.6 ms. In the middle latency potentials binaural interaction components peaking at 20, 32, and 45 ms were defined that were also largest when dl and dT = 0. The latency of the interaction did not shift with changes in dT and dl whereas the amplitude gradually decreased but binaural interaction components were still evident even at the largest values of dl (30 dB) and dT (3 ms). Psychophysical judgments of binaural perceptions showed binaural fusion of the stimuli to persist with dT values up to 1.6 ms and that lateralization of the intracranial image was complete when either dT = 1.6 ms or when dl = 16 dB. The results suggest that the presence of a binaural interaction component of auditory brainstem potentials correlates with the fusion of binaural click stimuli and the amplitude of the binaural interaction component correlates inversely with the degree of lateralization of the intracranial image. Binaural interaction components of middle latency potentials persist and continue to change even after the binaural stimuli cannot be fused.

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“…However, as the IHC loss exceeds 80-90%, as is seen in at least four of the premature infant ears in the present study (Figure 4), the remaining ABR waves will disappear (Liberman et al 1997), especially if the only remaining fibers are in the apical turn, where response latencies are not well synchronized (Antoli-Candela and Kiang 1978) due to the progressive slowing of the mechanical traveling wave. It is also commonly suggested that AN could arise from dys-synchrony in the sound-evoked responses of the cochlear nerve (Starr et al 1991). Although, theoretically, a demyelinating disorder could produce such a phenotype, there are no animal models of either genetic or acquired cochlear disorders in which true cochlear nerve dys-synchrony has been demonstrated, i.e., in which the response latencies of cochlear nerve populations are so jumbled that no coherent peak emerges in the gross potential.…”

Section: Selective Ihc Loss Prematurity and Auditory Neuropathymentioning

confidence: 99%

Selective Inner Hair Cell Loss in Prematurity: A Temporal Bone Study of Infants from a Neonatal Intensive Care Unit

Amatuzzi

Liberman

Northrop

2011

JARO

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Premature birth is a well-known risk factor for sensorineural hearing loss in general and auditory neuropathy in particular. However, relatively little is known about the underlying causes, in part because there are so few relevant histopathological studies. Here, we report on the analysis of hair cell loss patterns in 54 temporal bones from premature infants and a control group of 46 bones from full-term infants, all of whom spent time in the neonatal intensive care unit at the Hospital de Niños in San Jose, Costa Rica, between 1977 and 1993. The prevalence of significant hair cell loss was higher in the preterm group than the full-term group (41% vs. 28%, respectively). The most striking finding was the frequency of selective inner hair cell loss, an extremely rare histopathological pattern, in the preterm vs. the full-term babies (27% vs. 3%, respectively). The findings suggest that a common cause of non-genetic auditory neuropathy is selective loss of inner hair cells rather than primary damage to the cochlear nerve.

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Absence of Both Auditory Evoked Potentials and Auditory Percepts Dependent on Timing Cues

Cited by 173 publications

References 1 publication

A Dominantly Inherited Progressive Deafness Affecting Distal Auditory Nerve and Hair Cells

A Dominantly Inherited Progressive Deafness Affecting Distal Auditory Nerve and Hair Cells

Auditory time-intensity cues in the binaural interaction component of the auditory evoked potentials

Selective Inner Hair Cell Loss in Prematurity: A Temporal Bone Study of Infants from a Neonatal Intensive Care Unit

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