The middle ear muscle reflex in the diagnosis of cochlear neuropathy.

Cochlear neuropathy, i.e. the loss of auditory nerve fibers (ANFs) without loss of hair cells, may cause hearing deficits without affecting threshold sensitivity, particularly if the subset of ANFs with high thresholds and low spontaneous rates (SRs) is preferentially lost, as appears to be the case in both aging and noise-damaged cochleas. Because low-SR fibers may also be important drivers of the medial olivocochlear reflex (MOCR) and middle-ear muscle reflex (MEMR), these reflexes might be sensitive metrics of cochlear neuropathy. To test this hypothesis, we measured reflex strength and reflex threshold in mice with noise-induced neuropathy, as documented by confocal analysis of immunostained cochlear whole-mounts. To assay the MOCR, we measured contra-noise modulation of ipsilateral distortion-product otoacoustic emissions (DPOAEs) before and after the administration of curare to block the MEMR or curare + strychnine to also block the MOCR. The modulation of DPOAEs was 1) dominated by the MEMR in anesthetized mice, with a smaller contribution from the MOCR, and 2) significantly attenuated in neuropathic mice, but only when the MEMR was intact. We then measured MEMR growth functions by monitoring contra-noise induced changes in the wideband reflectance of chirps presented to the ipsilateral ear. We found 1) that the changes in wideband reflectance were mediated by the MEMR alone, and 2) that MEMR threshold was elevated and its maximum amplitude was attenuated in neuropathic mice. These data suggest that the MEMR may be valuable in the early detection of cochlear neuropathy.

Perceptual consequences of "hidden" hearing loss.

Dramatic results from recent animal experiments show that noise exposure can cause a selective loss of high-threshold auditory nerve fibers without affecting absolute sensitivity permanently. This cochlear neuropathy has been described as hidden hearing loss, as it is not thought to be detectable using standard measures of audiometric threshold. It is possible that hidden hearing loss is a common condition in humans and may underlie some of the perceptual deficits experienced by people with clinically normal hearing. There is some evidence that a history of noise exposure is associated with difficulties in speech discrimination and temporal processing, even in the absence of any audiometric loss. There is also evidence that the tinnitus experienced by listeners with clinically normal hearing is associated with cochlear neuropathy, as measured using Wave I of the auditory brainstem response. To date, however, there has been no direct link made between noise exposure, cochlear neuropathy, and perceptual difficulties. Animal experiments also reveal that the aging process itself, in the absence of significant noise exposure, is associated with loss of auditory nerve fibers. Evidence from human temporal bone studies and auditory brainstem response measures suggests that this form of hidden loss is common in humans and may have perceptual consequences, in particular, regarding the coding of the temporal aspects of sounds. Hidden hearing loss is potentially a major health issue, and investigations are ongoing to identify the causes and consequences of this troubling condition.

Cervical vestibular evoked myogenic potentials and caloric test results in individuals with auditory neuropathy spectrum disorders.

Auditory neuropathy spectrum disorder is a type of hearing loss where outer hair cell function are normal (as evidenced by the preservation of OAEs and cochlear microphonics), whereas auditory nerve functions are abnormal (as evidenced by abnormal auditory brainstem evoked potentials beginning with wave I of the ABR) and acoustic reflexes to ipsilateral and contralateral tones are absent. It is likely that in cases with auditory neuropathy spectrum disorder not only the cochlear nerve, but also the vestibular nerves might get involved. The present study was conducted with an aim of finding out the inferior and superior vestibular nerve involvement through cervical vestibular evoked myogenic potentials and Caloric test results respectively in individuals with Auditory Neuropathy Spectrum Disorders. Total 26 participants who fulfilled the criteria of auditory neuropathy spectrum disorder participated for the study. Vestibular evoked myogenic potentials results showed absence of responses from most of the subjects also caloric responses showed bilateral hypofunctional responses in most of the participants, which is suggestive of involvement of both the inferior as well as superior vestibular nerve in individuals with auditory neuropathy spectrum disorders. Additionally there was no association between the pattern and degree of hearing loss to caloric test results and vestibular evoked myogenic potentials results findings.

Vestibular evoked myogenic potentials: review.

BACKGROUND: Disorders of balance often pose a diagnostic conundrum for clinicians, and a multitude of investigations have emerged over the years. Vestibular evoked myogenic potential testing is a diagnostic tool which can be used to assess vestibular function. Over recent years, extensive study has begun to establish a broader clinical role for vestibular evoked myogenic potential testing. OBJECTIVES: To provide an overview of vestibular evoked myogenic potential testing, and to present the evidence for its clinical application. REVIEW TYPE: Structured literature search according to evidence-based medicine guidelines, performed between November 2008 and April 2009. No restrictions were applied to the dates searched. CONCLUSION: The benefits of vestibular evoked myogenic potential testing have already been established as regards the diagnosis and monitoring of several clinical conditions. Researchers continue to delve deeper into potential new clinical applications, with early results suggesting promising future developments.

Audiological results in a group of children with auditory neuropathy spectrum disorder.

OBJECTIVE: To enrich clinical information of auditory neuropathy spectrum disorder. METHODS: Forty-eight infants and young children with severely abnormal auditory brainstem responses (ABRs) along with preserved distortion product otoacoustic emissions (DPOAEs) and/or cochlear microphonics (CMs) were included in this retrospective study. Click ABRs, CMs, DPOAEs, behavioral thresholds and tympanograms were established. RESULTS: Audiological information of 88 ears (40 cases were bilateral and 8 unilateral) that met the inclusion criteria were analyzed. Most of the ears (68.2%) had absent click ABRs at the maximum presentation level of 100 dB nHL, while other ears had repeatable wave V with lowest thresholds of 80 dB nHL. Behavioral hearing of 23 cases ranged from mild (n = 1), moderate (n = 2) to severe and profound loss (n = 20). CMs were present in all ears in spite of those with middle ear pathology, while DPOAEs were present only in 40% of the affected ears. CONCLUSION: Diverse results of audiological assessments are shown in this group of children. Concurrent pathologies may make auditory neuropathy spectrum disorder undetected. Absent or severely abnormal ABR along with present CMs are the most reliable measures for detecting this disorder at this stage.

Auditory steady state response in auditory neuropathy.

REVIEW: Auditory neuropathy is a disorder characterised by preservation of outer hair cell function, with normal otoacoustic emissions and/or cochlear microphonics, but an absent or distorted auditory brainstem response. PURPOSE: This study aimed to objectively assess hearing thresholds in patients with auditory neuropathy, using the auditory steady state response. MATERIALS AND METHODS: Thirteen patients with auditory neuropathy and 15 normal hearing subjects were examined. Audiological evaluation included basic audiological tests, otoacoustic emissions, auditory brainstem response and auditory steady state response. RESULTS: In the auditory neuropathy patients, the auditory brainstem response was absent in 11 patients, while the auditory steady state response was absent in only three. CONCLUSION: The auditory steady state response may serve as a valuable objective measure for assessing the hearing threshold across different frequencies in patients with auditory neuropathy. We recommend that auditory steady state response be used to complete the evaluation of patients with auditory neuropathy.

N100 cortical potentials accompanying disrupted auditory nerve activity in auditory neuropathy (AN): effects of signal intensity and continuous noise.

OBJECTIVE: Auditory temporal processes in quiet are impaired in auditory neuropathy (AN) similar to normal hearing subjects tested in noise. N100 latencies were measured from AN subjects at several tone intensities in quiet and noise for comparison with a group of normal hearing individuals. METHODS: Subjects were tested with brief 100 ms tones (1.0 kHz, 100-40 dB SPL) in quiet and in continuous noise (90 dB SPL). N100 latency and amplitude were analyzed as a function of signal intensity and audibility. RESULTS: N100 latency in AN in quiet was delayed and amplitude was reduced compared to the normal group; the extent of latency delay was related to psychoacoustic measures of gap detection threshold and speech recognition scores, but not to audibility. Noise in normal hearing subjects was accompanied by N100 latency delays and amplitude reductions paralleling those found in AN tested in quiet. Additional N100 latency delays and amplitude reductions occurred in AN with noise. CONCLUSIONS: N100 latency to tones and performance on auditory temporal tasks were related in AN subjects. Noise masking in normal hearing subjects affected N100 latency to resemble AN in quiet. SIGNIFICANCE: N100 latency to tones may serve as an objective measure of the efficiency of auditory temporal processes.

Novel subtype of idiopathic bilateral vestibulopathy: bilateral absence of vestibular evoked myogenic potentials in the presence of normal caloric responses.

To characterize clinical features of those patients who showed an absence of vestibular evoked myogenic potential (VEMP) responses in the presence of normal caloric responses bilaterally, we reviewed clinical records of 1,887 consecutive outpatients who complained of balance problems, and identified three patients, who showed absent VEMPs in the presence of normal caloric responses bilaterally with unknown causes. All three patients had episodes of recurrent vertigo without spontaneous, gaze-evoked, or positional nystagmus at the time of examination. They complained of oscillopsia while moving their body or head and showed positive Romberg's signs. Drawing on these cases, we underscore the importance of examining the function of the inferior vestibular nerve system, even with no nystagmus and normal caloric findings, in patients complaining of dizziness or oscillopsia during locomotion.

Frequency-specific electrocochleography indicates that presynaptic and postsynaptic mechanisms of auditory neuropathy exist.

OBJECTIVES: The physiological mechanisms underlying auditory neuropathy (AN) remain unclear and it is likely that the multiple disruptions are classified under the broadly defined term. Cochlear implantation is being more widely used in this population to bypass the suspected site-of-lesion although a number of cases have been identified within the Sydney Cochlear Implant Centre where this management strategy has been unsuccessful. It is likely that this relates to the different physiological mechanisms underlying AN. DESIGN: To investigate the site-of-lesion in AN, frequency-specific round window electrocochleography (ECochG) was used to assess local hair-cell, dendritic, and axonal currents generated within the cochlea in 14 subjects with AN and compared with responses from two normally hearing subjects. ECochG results were then compared with electrically evoked auditory brain stem response (EABR) measured after cochlear implantation. RESULTS: The results of this study demonstrate that two dominant patterns of ECochG waveforms (produced by a high-frequency alternating tone burst) can be identified in this population of AN subjects: (a) gross waveform showing a prolonged summating potential (SP) latency that, in most cases, is followed by a small compound action potential; and (b) gross waveform showing a normal latency SP waveform followed by a broad negative potential [assumed to reflect the dendritic potential (DP) identified in anaesthetized guinea-pigs]. This study demonstrates that in most subjects (n = 7) with a prolonged latency SP but no DP, normal morphology EABR waveforms were elicited for all electrode channels. On the other hand, all subjects (n = 7) who showed a normal latency SP followed by a broad negative DP, showed EABR waveforms that were absent or having poor wave V morphology. The authors' interpretation of this is that ECochG results may provide a classification of AN into pre- and postsynaptic lesions. CONCLUSIONS: We suggest that a presynaptic and postsynaptic type of AN exist that may have implications for the fitting of cochlear implants.

An animal experimental model of auditory neuropathy induced in rats by auditory nerve compression.

Several animal models of auditory neuropathy (AN) have been produced by employing pharmacological agents to damage auditory neurons or hair cells selectively. The specificity of pharmacological lesions is generally assessed by observation of visible structural damage but it is difficult to localize the delivery, which could lead to functional side effects in other anatomical structures. Although genetic analyses of human AN patients have provided important information on the pathophysiology of AN, specific genetic defects have not been fully correlated with functional deficits in the auditory nervous system. To address this problem, we compressed rat auditory nerves to assess neural degeneration for up to 35 weeks. The method produced a good model of auditory neuropathy, including profound deterioration of the auditory brainstem response and preservation of both cochlear microphonics and distortion product otoacoustic emissions. Histological examination revealed that in spite of profound degeneration of the auditory nerve, the hair cells remained intact. The model provides a complementary alternative to those based on pharmacological lesions and genetic analyses of AN patients and should allow analysis of the pathophysiology of auditory neuropathy with less risk of the results being confounded by unknown deficits in other cell types.

Speech perception in individuals with auditory neuropathy.

PURPOSE: Speech perception in participants with auditory neuropathy (AN) was systematically studied to answer the following 2 questions: Does noise present a particular problem for people with AN? Can clear speech and cochlear implants alleviate this problem? METHOD: The researchers evaluated the advantage in intelligibility of clear speech over conversational speech in 13 participants with AN. Of these participants, 7 had received a cochlear implant. Eight sentence-recognition experiments were conducted to examine the clear speech advantage in 2 listening conditions (quiet and noise) using 4 stimulation modes (monaural acoustic, diotic acoustic, monaural electric, and binaurally combined acoustic and electric stimulation). RESULTS: Participants with AN performed more poorly in speech recognition in noise than did the normal-hearing, cochlear-impaired, and cochlear implant controls. A significant clear speech advantage was observed, ranging from 9 to 23 percentage points in intelligibility for all listening conditions and stimulation modes. Electric stimulation via a cochlear implant produced significantly higher intelligibility than acoustic stimulation in both quiet and in noise. Binaural hearing with either diotic acoustic stimulation or combined acoustic and electric stimulation produced significantly higher intelligibility than monaural stimulation in quiet but not in noise. CONCLUSIONS: Participants with AN most likely derive the clear speech advantage from enhanced temporal properties in clear speech and improved neural synchrony with electric stimulation. Although the present result supports cochlear implantation as one treatment choice for people with AN, it suggests that the use of innovative hearing aids may be another viable option to improve speech perception in noise.

Using auditory-visual speech to probe the basis of noise-impaired consonant-vowel perception in dyslexia and auditory neuropathy.

Both dyslexics and auditory neuropathy (AN) subjects show inferior consonant-vowel (CV) perception in noise, relative to controls. To better understand these impairments, natural acoustic speech stimuli that were masked in speech-shaped noise at various intensities were presented to dyslexic, AN, and control subjects either in isolation or accompanied by visual articulatory cues. AN subjects were expected to benefit from the pairing of visual articulatory cues and auditory CV stimuli, provided that their speech perception impairment reflects a relatively peripheral auditory disorder. Assuming that dyslexia reflects a general impairment of speech processing rather than a disorder of audition, dyslexics were not expected to similarly benefit from an introduction of visual articulatory cues. The results revealed an increased effect of noise masking on the perception of isolated acoustic stimuli by both dyslexic and AN subjects. More importantly, dyslexics showed less effective use of visual articulatory cues in identifying masked speech stimuli and lower visual baseline performance relative to AN subjects and controls. Last, a significant positive correlation was found between reading ability and the ameliorating effect of visual articulatory cues on speech perception in noise. These results suggest that some reading impairments may stem from a central deficit of speech processing.

[Vestibular evoked muscle potentials dependency on neural origin and the location of an acoustic neuroma]. / Die vestibulär evozierten Muskelpotenziale in Abhängigkeit vom nervalen Ursprung und der Lage eines Akustikusneurinoms.

INTRODUCTION: Most acoustic neuromas (AN) originate from the inferior vestibular nerve (IVN). Vestibular evoked myogenic potentials (VEMP) are accepted as the only unilateral test for the function of the sacculus and the IVN. METHODS: The influence of the origin from the IVN and superior vestibular nerve (SVN), and the position of the AN in relation to the internal auditory canal on VEMPs was investigated. A total of 39 patients (aged: 30-67 years, mean: 53 years) were examined. The VEMPs were recorded on the activated sternocleidomastoid muscle and averaged over 200 stimuli. Tone bursts (95 dB nHL; 500 Hz; stimulation rate 5 Hz) were used to generate the VEMPs. RESULTS: The exact origin of the AN from the SVN or the IVN could be determined intraoperatively and correlated using VEMP in 28 patients. CONCLUSION: The origin of the AN has only a marginal influence on the results of VEMP measurements. The position of the AN in relation to the internal auditory canal seems to have more influence than the origin.

Vestibular-evoked myogenic potentials in two patients with Ramsay Hunt syndrome.

We report on the function of the inferior vestibular nerve, as monitored by the vestibular-evoked myogenic potentials (VEMP), in two patients suffering from Ramsay Hunt syndrome. Both the patients presented canal paresis (CP) and hearing loss, but in one patient normal VEMP was recorded while the other presented vagus nerve paralysis plus no VEMP response at the highest stimulus intensity used in our institute (i.e., 105 dB nHL).

Sound-evoked myogenic potentials and responses with 3-ms latency in auditory brainstem response.

OBJECTIVES: The vestibular-evoked myogenic potential (VEMP) has shed new light on vestibular testing. A large negative deflection with a 3-ms latency within the auditory brainstem response (ABR) has been reported in some patients with deafness. This negative deflection has been termed the N3 potential and it is assumed to be a vestibular-evoked potential. This study investigated the relationship between the VEMP and the N3 potential. STUDY DESIGN: Prospective evaluation of the VEMP and the N3 potential in 21 patients. METHODS: The oto-neurological tests, including caloric test, hearing sensitivity test, VEMP, and ABR, were performed and data were analyzed. RESULTS: The average hearing threshold ranged from 65 to above 110 dB, which includes 9 (37.5%) totally deaf ears. The N3 potentials were recorded in 10 (41.7%) ears. A normal VEMP was detected in 16 (66.7%) ears. Canal paresis was observed in 11 (45.8%) ears. CONCLUSIONS: Both the VEMP and the N3 potential appear to originate from the sacculus, but because the characteristics of these two responses are not identical, additional factors might be involved in the generation of the N3 potential.

Auditory neuropathy in children.

Auditory neuropathy is a sensorineural disorder characterized by absent or abnormal auditory brainstem evoked potentials and normal cochlear outer hair cell function. A variety of processes is thought to be involved in its pathophysiology and their influence on hearing may be different. We present here the diagnostic sequence and management of two new cases of auditory neuropathy in breastfeeding children.

Absent vestibular evoked myogenic potentials in vestibular neurolabyrinthitis. An indicator of inferior vestibular nerve involvement?

BACKGROUND: Benign paroxysmal positioning vertigo (BPPV) is generally thought to be caused by canalolithiasis in the posterior semicircular canal, an organ that is innervated by the inferior vestibular nerve. We hypothesized that absent vestibular evoked myogenic potentials (VEMPs) would indicate involvement of the inferior vestibular nerve and that posterior semicircular canal-type BPPV could not develop after vestibular neurolabyrinthitis (VNL) in patients with absent VEMPs. OBJECTIVE: To find out if VEMPs could be helpful in evaluating involvement of the inferior vestibular nerve in acute VNL. DESIGN: We reviewed the VEMP findings in 47 patients (34 men and 13 women) with acute VNL, 10 of whom had then developed posterior semicircular canal-type BPPV. RESULTS: While p13-n23, the first positive-negative peak of the VEMP, was ipsilaterally present on stimulation of the unaffected side in all patients, it was absent on the affected side in 16 patients (34%). The absence or presence of p13-n23 was independent of the results of caloric tests, pure tone audiometry, and auditory brain-stem responses. Typical posterior semicircular canal BPPV developed in 10 of the 47 patients after the acute attack of VNL, always on the same side as the neurolabyrinthitis. The p13-n23 potentials were preserved on stimulation of the affected ear in all 10 patients with BPPV. CONCLUSIONS: These results suggest that if VEMPs are absent from an ear that has suffered acute VNL, then posterior semicircular canal BPPV is unlikely to develop as a consequence of the VNL. The reason for this appears to be that the absence of VEMPs is due to involvement of the inferior vestibular nerve or involvement of the structures that it innervates.

Effects of olivocochlear bundle section on otoacoustic emissions in humans: efferent effects in comparison with control subjects.

The effects of contralateral acoustic stimulation on evoked otoacoustic emissions (OAE) were examined in three subject groups in order that the impact of efferent olivocochlear bundle section (as a consequence of vestibular neurectomy) could be compared with normal findings, and with a control surgical population. Results demonstrated that the inhibitory effect of contralateral noise on OAE amplitude was absent from the cochlea with severed efferent fibers. These findings appear to be independent of acoustic reflex activity, as suppression was absent despite normal reflexes. Inter-aural suppression of emissions recorded from the patients' intact cochleae act as a control and show a clear reduction in amplitude during contralateral stimulation in a frequency specific pattern consistent with normal findings. Patients who had undergone a similar surgical approach for vascular decompression of the VIIIth nerve without vestibular nerve section, were studied in order to assess the impact of retrolabyrinthine surgery on inter-aural suppression. Inhibition of OAE amplitude was maintained in all control cases in both the operated and intact sides, and was consistent with suppression observed in normal subjects, suggesting that the surgical procedures had not disturbed inter-aural suppression of otoacoustic emissions. It is concluded that the olivocochlear efferent system, when activated by low level contralateral acoustic stimulation, has an inhibitory role in controlling the cellular mechanisms responsible for the generation of otoacoustic emissions in humans. OAE techniques in conjunction with contralateral acoustic stimulation may thus prove to be of value in providing a rapid and non-invasive clinical test of efferent function and offer a means of investigating the functional significance of the efferent auditory system in humans.

Click-evoked responses from the exposed intracranial portion of the eighth nerve during vestibular nerve section: bipolar and monopolar recordings.

We compare the click-evoked compound action potentials from the exposed intracranial portion of the eighth nerve using bipolar and monopolar recording electrodes in patients undergoing vestibular nerve section. It is assumed that a bipolar recording electrode will only record propagated neural activity in the auditory nerve, whereas a monopolar recording electrode may in addition record electrical activity that is conducted passively to the recording site. The results of the present study confirm that the earliest detectable propagated neural activity in the intracranial portion of the auditory nerve occurs with a latency that is close to that of peak II of the brain-stem auditory evoked potentials, and the results also confirm that the late components in the click-evoked compound action potentials that have been demonstrated previously using the monopolar recording technique represent propagated neural activity in the auditory nerve. The results also indicate that the responses that are recorded by a bipolar recording electrode, when the small tips of which are placed on the eighth nerve when it is relatively dry, represent only small populations of nerve fibers. Even when an attempt is made to align the two tips of a bipolar electrode with the course of the auditory nerve, this type of electrode may record from different populations of nerve fibers.

Problems in the clinical measurement of acoustic reflex latency.

Two problems in the clinical measurement of acoustic reflex latency are discussed. First, the rise characteristic of the reflex is affected by the frequency of the eliciting signal. Due to the closed ipsilateral feedback loop system the reflex rises more rapidly at low frequencies than at high frequencies. Second, the temporal characteristic of the measurement apparatus may influence the apparent morphology of the reflex at low frequencies.