Time-intensity trade of bilaterally bone-conducted sounds in normal hearing subjects.

OBJECTIVE AND METHODS: In an effort to examine the rules by which information of bilaterally applied bone-conducted signals arising from interaural time differences (ITD) and interaural intensity differences (IID) is combined, data were measured for continuous 500 Hz narrow-band noise at 60 dBHL in 30 normal-hearing subjects using a centering method. Time-intensity trading functions were obtained by means of a sound image shifted towards one side by presenting an ITD, and shifted back to a centered sound image by varying the IID in the same ear. ITD values were varied from -600 to +600 microseconds at 200 microseconds steps, where negative values indicate delays to the right ear. RESULTS: Time-intensity trading functions in response to bone-conducted signals showed significantly lower discrimination thresholds across IIDs, when compared to a control group with applied air-conducted signals. These findings can be interpreted as a constructive interference effect related to the intimate mechanism of bilateral bone conduction, where interaural time differences play a major role. CONCLUSION: Time-intensity trade of bilaterally bone-conducted sounds in normal-hearing subjects is the highly sensitive. The high speed of sound through the skull may be the main reason for the high sensitivity of time-intensity trading.

Frequency sensitivity range of the saccule to bone-conducted stimuli measured by vestibular evoked myogenic potentials.

Vestibular evoked myogenic potentials (VEMPs) occurring in cervical muscles after intense sound stimulation conducted by air or bone are thought to be a polysynaptic response of otolith-vestibular nerve origin. We report the results of an experiment to investigate whether acoustic stimulation of the saccule by bone conduction produces VEMPs in which response amplitudes are somewhat sensitive to stimulus frequency, as appears to be the case with air-conducted stimuli. Prior to this we investigated the effect of stimulation repetition rate on bone-conducted VEMPs (B-VEMPs) at stimulus frequencies of 200 and 400 Hz with five different repetition rates (5, 10, 20, 40, and 80 Hz). B-VEMPs were recorded from 12 normal hearing subjects in response to bone-conducted 70 dB (normal hearing level), 10-ms tone bursts (rise/fall time=1 ms and plateau time=8 ms) at frequencies of 100, 200, 400, 800, 1600 and 3200 Hz. Our study showed that B-VEMP amplitudes were highest at 10 Hz but decreased as the repetition rate increased. B-VEMP response amplitudes were found to be maximal for stimulus frequencies from 200 to 400 Hz. This response may contribute to the perception of loud sounds.

Auditory nerve fiber differences in the normal and neurofilament deficient Japanese quail.

A primary axonal disease affecting the central and peripheral nervous system was discovered in a mutant strain of the Japanese quail, named quiver (Quv). We have previously demonstrated altered auditory evoked potentials in the neurofilament (NF) deficient quail. In this current study we attempt to find relationships between the auditory evoked potential results and the histo-pathological abnormalities of the auditory neurons. No abnormalities in the external auditory meatus and tympanic cavity were observed in either Quv or control quails and the ganglion cell bodies and their nuclei appeared normal by light microscopy. The myelin staining pattern was found to be similar in both strains with hematoxylin and eosin and Klüver-Barrera staining. The frequency histograms of fiber and axonal diameters of myelinated fibers showed an unimodal pattern in both strains. In Quv quails myelinated fibers and their axoplasm were smaller in diameter than in controls resulting in smaller neural tissue mass. In electron microscopic observation the axons of the Quv quail were composed of mitochondria and microtubules and smooth endoplasmic reticuli. In Quv quail electron micrographs of cochlear nerve myelinated fibers NFs were not seen in the axons and the neuronal cell bodies. Our current findings indicate that the previously reported reduction of conduction velocity of auditory evoked potentials may be due to smaller fiber and/or axonal diameter. The g-ratio, myelin thickness and fiber circularity were found to be the same for both strains. In conclusion, loss of axonal cytoskeletal elements (NFs) correlates well with our electrophysiological findings. Reduced conduction velocity and severely distorted auditory evoked potentials in NF deficient quails seem to be primarily due to axonal hypotrophy.

An isolated and sporadic auditory neuropathy (auditory nerve disease): report of five patients.

Five patients of various ages with difficulty in speech discrimination were evaluated. All showed evidence of abnormal auditory brainstem responses (ABRs) beginning with the VIIIth cranial nerve. Broad summating potentials were evoked on their electrocochleograms (EcochGs) and they all exhibited almost normal cochlear outer hair cell function by otoacoustic emissions (OAEs) recordings. Behavioural audiometric testing revealed a mild to moderate elevation of pure-tone threshold in all patients. The shape of their pure-tone losses varied, being predominantly low-frequency in four patients (rising slope pattern) and flat across all frequencies in one patient. Speech intelligibility scores of all patients were poor and out of proportion to what would have been expected if threshold elevation of pure-tone was of cochlear origin (i.e. markedly poor scores on the speech audiogram with good scores on the auditory comprehension test). Patients were neurologically normal when the hearing impairment was first manifested. We suggest that this type of hearing impairment is due to an isolated and sporadic disorder of auditory nerve function. It occurs in isolation and does not seem to be part of a generalized neuropathological process.

Electrophysiological measures of auditory function in the neurofilament-deficient mutant quail (Quv).

Auditory pathway electrophysiological studies were performed on the mutant quail 'Quv'. This mutation is known to result in neurofilament deficiencies of both the peripheral and central nervous systems. Auditory evoked brainstem responses (ABRs), electrocochleograms (EcochGs) and middle latency responses (MLRs) were evaluated. ABRs in Quv quails demonstrated markedly altered waveforms exhibiting longer latencies, absence of the later peaks and lower amplitudes. The EcochG showed normal cochlear microphonics with no obvious abnormalities in amplitude or latency and normal latencies for peak N1. Quv quails had a mild threshold elevation with a normal latency for the first peak of the ABR (P1). The Quv MLRs showed no significant differences in amplitude but they revealed a latency prolongation for peaks N0, Pa and Na relative to the controls. We have discovered abnormal findings of auditory evoked potentials in the neurofilament-deficient quail (Quv). We suggest that the smaller axonal size and axonal hypotrophy due to altered neurofilament expression underlies these abnormal auditory evoked potential responses.

Bone-conducted vestibular evoked myogenic potentials in patients with congenital atresia of the external auditory canal.

OBJECTIVE: The purpose of this study was to determine whether vestibular evoked myogenic potentials from the sternocleidomastoid muscle in response to bone-conducted clicks and short tone-bursts can be used to assess vestibular apparatus function in patients with conductive hearing problems, particularly bilateral external auditory canal atresia. DESIGN: Evoked-potential responses to bone-conducted auditory stimuli were recorded from the sternocleidomastoid muscle of 15 patients (11 male and four female, aged 4--20 years) with congenital bilateral atresia of the external auditory canal, with or without the middle ear anomalies. SETTING: This study was conducted in the outpatient clinic of the Tokyo University Hospital, Department of Otolaryngology, University of Tokyo. INTERVENTION: Diagnostic. OUTCOME MEASURES: Bone-conducted vestibular evoked myogenic potentials in response to clicks and short tone-bursts were recorded with surface electrodes over both sternocleidomastoids in each patient. RESULTS: In all patients, bone-conducted clicks and short tone-bursts evoked larger biphasic responses from the sternocleidomastoid ipsilateral to the stimulated ear. Short tone-bursts evoked vestibular evoked myogenic potentials with higher amplitude and better waveform morphology than clicks at the same subjective intensity. CONCLUSION: Loud auditory stimuli delivered by bone conduction can evoke myogenic potentials from the sternocleidomastoid. This method is a noninvasive, rapid, and convenient test for investigating the vestibular system function in patients with bilateral external auditory canal atresia, with or without the middle ear anomalies.

The effect of sternocleidomastoid electrode location on vestibular evoked myogenic potential.

OBJECTIVE: The purpose of this study was to investigate the effect of a sternocleidomastoid (SCM) electrode array on the vestibular evoked myogenic potential (VEMP) and the most optimal recording site for clinical use. METHODS: Fifteen normal adults (10 men and 5 women, aged 18 to 38 years) were tested. We placed electrodes at four different locations over the SCM muscle: the upper part of the SCM muscle at the level of mandibular angle, the middle part of the muscle, and immediately above sternal and clavicular origins of the SCM muscle. Sound evoked myogenic potentials in response to monoaurally delivered short tone-bursts (500 Hz at 95 dBnHL, rise/fall time=1 ms and plateau=2 ms) were recorded with surface electrodes over the isometrically contracting SCM muscle. RESULTS: On the clavicle, the upper and middle parts of SCM from all subjects, air-conducted short tone burst evoked biphasic responses (p13-n23). VEMPs recorded at the upper part of the muscle showed the largest amplitude, followed by that at the middle part. However, the latency of the first peaks (p13-n23) was not constant in the upper part. Recording from the middle part of SCM muscle were more consistent. CONCLUSION: Our findings suggest that the middle part of the SCM muscle is the optimal location for recording vestibular evoked myogenic potential.

Bone-conducted evoked myogenic potentials from the sternocleidomastoid muscle.

The aim of this study was to show that bone-conducted clicks and short tone bursts (STBs) can evoke myogenic potentials from the sternocleidomastoid muscle (SCM) and that these responses are of vestibular origin. Evoked potential responses to bone-conducted auditory stimuli were recorded from the SCMs of 20 normal volunteers and from 12 patients with well-defined lesions of the middle or inner ear or the VIIIth cranial nerve. The subjects, who had various labyrinthine and retro-labyrinthine pathologies, included five patients with bilateral profound conductive hearing loss, two with bilateral acoustic neuroma post-total neurectomy and five with bilateral sensorineural hearing loss. Air- and bone-conducted evoked myogenic potentials in response to clicks and STBs were recorded with surface electrodes over each SCM of each subject. In normal subjects, bone- and air-conducted clicks and STBs evoked biphasic responses from the SCM ipsilateral to the stimulated ear. The bone-conducted clicks evoked short-latency vestibular-evoked myogenic potential (VEMP) responses only in young subjects or in subjects with conductive hearing loss. STBs evoked VEMPs with higher amplitude and better waveform morphology than clicks with the same acoustic intensity. Patients with total VIIIth cranial nerve neurectomy showed no responses to air- or bone-conducted click or STB stimuli. Clear VEMP responses were evoked from patients with conductive or sensorineural hearing loss. It is concluded that loud auditory stimuli delivered by bone- as well as air conduction can evoke myogenic potentials from the SCM. These responses seem to be of vestibular origin.

Otoacoustic emissions and auditory brainstem responses after neonatal hyperbilirubinemia.

Severe hyperbilirubinemia often results in hearing loss. Behavioral audiometry, auditory-evoked brainstem responses (ABRs) and otoacoustic emissions (OAEs) were performed in three such patients in an attempt to localize the pathophysiology of this hearing loss. Behavioral audiometric findings in these patients (all male, 4, 15 and 25 years old) ranged from severe in the 4-year-old, moderate in the 15-year-old and slight in the 25-year-old. Where obtained, ABR wave V thresholds were elevated or ABR were absent. However, absolute and inter-wave latency measurements were not indicative of brainstem pathology. OAEs (transient and distortion product) could only be obtained in the high- or low-frequency ranges in these patients. Our findings suggest that at least some lesions producing hearing loss in severe hyperbilirubinemia are in the cochlea, especially at the outer hair cells. Finally, we found that only moderately elevated serum bilirubin levels (<20 mg/dl) may contribute to the development of sensorineural hearing loss.

Vestibular function in auditory neuropathy.

Auditory neuropathy is characterized by mild-to-moderate pure-tone hearing loss, poor speech discrimination out of proportion with this loss, absent or abnormal auditory brainstem responses and normal outer hair cell function as measured by otoacoustic emissions and cochlear microphonics. We followed three patients in our clinic whom we classified as auditory neuropathy patients. These patients also complained of balance disorders and we report our auditory and vestibular system analyses of these patients. The data presented herein include results of audiometric tests (serial pure-tone audiometry and speech discrimination tests), otoacoustic emissions, auditory-evoked brainstem responses and vestibular function tests (clinical tests of balance, electronystagmography, damped rotation tests and vestibular-evoked myogenic potentials). In all patients, pure-tone audiometry revealed mild-to-moderate sensorineural hearing loss, markedly poor speech discrimination scores and absent auditory-evoked brainstem responses, all in the presence of normal otoacoustic emissions. Balance tests (caloric tests and damped rotation test) were abnormal. Saccades, smooth pursuit eye movements and optokinetic nystagmus were normal in all patients. Neurological and motor system evaluations were normal in all patients. These three auditory neuropathy patients manifest a disorder of cochlear nerve function in the presence of normal outer hair cell activity. They additionally manifest a disorder of the vestibular nerve and its end organs. We conclude that, in patients with isolated auditory neuropathy, the vestibular branch of the VIIIth cranial nerve and its innervated structures may also be affected. We suggest the use of the term "cochlear neuropathy" to characterize those patients with involvement of only the auditory branch of the VIIIth cranial nerve and its innervation.