Pudendal nerve somatosensory evoked potentials in probable multiple sclerosis

One of the most frequent causes of sensorineural hearing loss in childhood is damage to outer hair cells of the cochlea. The presence of otoacoustic emissions, generated by outer hair cells, provides evidence for normal hearing. This finding, however, may give rise to false reassurance, because even severe hearing loss, localized behind the cochlea, can be associated with normal otoacoustic emissions. The coexistence of otoacoustic emissions and hearing loss calls for the prompt exclusion of neurological disease.     

Changes in cochlear function after double-membrane rupture in the guinea pig

We measured the transiently evoked otoacoustic emissions (TEOAEs), compound action potentials (CAPs) and cochlear microphonics (CMs) in guinea pigs after rupture of the round window membrane alone (n = 5) or of the round window membrane with localized cochlear damage (n = 10). The localized cochlear damage entailed rupture of Reissner's membrane with damage to the stria vascularis. We determined the time course of changes in the total echo power (TEP) in TEOAEs and the minimal detectable levels of CAPs and CMs. The endocochlear potential (EP) was measured in the cochlea with localized damage. There were no changes in TEOAEs, CAPs or CMs in the guinea pigs subjected to round window membrane rupture alone, but the minimal detectable levels of CAPs and CMs were increased in all the guinea pigs in which TEOAEs were absent after rupture of the round window membrane with localized cochlear damage. Our results suggest that double-membrane rupture (rupture of the round window membrane with localized cochlear damage) produces acute sensorineural hearing loss. The hearing loss appeared to be related to damage to the cochlea, which may be induced by influx of potassium-rich endolymph into the perilymph, and by morphological damage to the scala media.     

Basal cochlear lesions result in increased amplitude of otoacoustic emissions

We have measured the changes in transient otoacoustic emissions (TEOAEs) and distortion product otoacoustic emissions (DPOAEs) during and after ototoxic amikacin treatment in an animal (chinchilla) model. TEOAE and DPOAE were recorded from 6 adult chinchillas over a 6-week time course starting just before a 5-day or 7-day treatment period with amikacin sulphate (400 mg/kg/day, i.m.). After final recordings, cochlear morphology was assessed by scanning electron microscopy. Generally, both DPOAE and TEOAE amplitudes change during and after treatment in a systematic fashion. High-frequency components change first, followed by lower-frequency components. We note that there is often a long latency to the onset of changes in otoacoustic emissions (OAE), and that these changes can continue for weeks after treatment. Most importantly we report that when the basal region of the cochlea is damaged in the frequency region above the OAE recording bandwidth (0.6-6 kHz for TEOAE; 1-6.7 kHz for DPOAE), we often find an increase in OAE amplitudes. More specifically, we note that as a cochlear lesion progresses apically, there is often a transient increase in a frequency-specific OAE before it reduces or is lost. Our results suggest that the increase in OAE amplitudes precedes the expression of detectable cochlear pathology.     

Time-frequency distributions of click-evoked otoacoustic emissions

Emissions evoked by broad-band stimuli, such as clicks, show a 'frequency dispersion' reminiscent of the place-frequency distribution along the cochlea. Analysis of the time-frequency properties of transiently evoked otoacoustic emissions (TEOAEs) is therefore of considerable interest due to their close relation with cochlear mechanisms. In particular, since OAEs in response to click stimuli are expected to evoke a cumulative response from the whole cochlea, the analysis of click-evoked OAEs can yield a global view of cochlear function. Wavelet analysis is performed to obtain time-frequency distributions of click-evoked OAEs at various intensity levels from normal ears. By means of the inverse wavelet transform, the recorded responses are decomposed into elementary components representing the contribution within a narrow frequency band to the cumulative OAE. The relationship between the frequency of the elementary components, latency and level of stimulation is described.     

Transitory evoked and distortion products of otoacoustic emissions in absent auditory evoked potentials

Transient click-evoked otoacoustic emissions (TEOAE) and distortion-product otoacoustic emissions (DPOAE) are produced by an active biomechanical process in the cochlea, presumably related to outer hair-cell activity. Although it is generally accepted that in most cases of hearing loss with absent auditory evoked potentials neither TEOAE nor DPOAE can be found, some cases with such a constellation have been described. Here we report another four cases of children with severe to profound hearing loss where we discovered reproducible TEOAE and DPAOE, whereas auditory evoked potentials were missing. TEOAE and DPOAE recordings in these cases indicate substantially preserved outer hair-cell function independent of profound pre-sensineural hearing loss. Since the incidence of children with preserved otoacoustic emissions together with impairment of synaptic or postsynaptic function of the first neuron is not known, the unconditioned use of TEOAE nor DPOAE as a screening instrument must be seriously questioned. Secondly, in conjunction with subjective audiometry and brain-stem-evoked potentials, emission recordings is an indispensable measurement prior to cochlear implantation and use of high-power hearing aids.     

Recording otoacoustic emissions in expert evaluation of chronic noise-induced hearing loss

In order to assess occupational hearing loss, damage to outer hair cell function must be demonstrated. Measurements of transiently evoked otoacoustic emissions (TEOAE) and distortion products (DPOAE) allow clinicians to investigate objectively the cochlear amplification process. In this prospective study, 50 persons with suspected occupational hearing losses were tested with pure-tone and speech audiometry. Additionally, TEOAE and DPAOE were determined. Significant linear correlations were observed when comparing hearing thresholds and speech discriminations with evoked emissions. Three groups were formed based on the OAE results: group A, TEOAE- and DPOAE-positive; group B, TEOAE-negative, DPOAE positive; group C, TEOAE- and DPOAE-negative. The groups were compared with regard to the weighted and simple discrimination scores and percentage of hearing loss computed according to the Boenninghaus-R?ser tables. Analysis of variance showed significant differences in hearing losses among the three groups, allowing an objective statement to be made about the degree of work compromise: group A, < 10%; group B, 10-15% group C, > or = 20%.     

Multi-variant analysis of otoacoustic emissions and estimation of hearing thresholds: transient evoked otoacoustic emissions

Evaluation of cochlear hearing loss by means of transiently evoked otoacoustic emissions is already established in clinical practice. However, accurate prediction of pure-tone thresholds is still questioned and is still regarded as troublesome. Both click- and tone-burst-evoked otoacoustic emissions at several intensity levels were measured and analysed in 157 ears from normally hearing and 432 ears from patients with different degrees of pure sensory hearing loss using the ILO88/92 equipment. Results of otoacoustic emissions (OAE), elicited by clicks and tone-bursts at centre frequencies from 1 to 5 kHz, were analysed using two different statistical methods. Both multivariate discriminant analysis and forward multiple regression analysis were used to determine which OAE variables were most discriminating and best at predicting hearing thresholds. We found that a limited set of variables obtained from both tone-burst and click measurements can accurately predict and categorize hearing loss levels up to a limit of 60 dB HL. We found correct classification scores of pure-tone thresholds between 500 and 4000 Hz up to 100 per cent when using combined click and tone-burst otoacoustic measurements. Prediction of pure-tone thresholds was correct with a maximum estimation error of 10 dB for audiometric octave frequencies between 500 and 4000 Hz. Measurements of multiple tone-bursts OAEs have a significant clinical advantage over the use of clicks alone for clinical applications, and a good classification and prediction of pure-tone thresholds with otoacoustic emissions is possible.     

Distortion products in normal hearing patients with tinnitus

Otoacoustic emissions are the result of cochlear active non-linear micromechanical mechanisms which probably originate within the OHC. OAE findings in patients with tinnitus are not univoque and there is no clear correlation between OAE modifications and tinnitus. We investigated distortion products in 20 normal hearing patients with tinnitus; all patients were selected with restrictive criteria (audiogram within 20 dB for all the frequencies, ABR and other tests normal, no history of ototoxic, nootropic drug intake, normal psychological evaluation, etc.). 12 patients out of 20 (60%) showed DP alterations. This finding is interpreted as an abnormality or a dysregulation of the efferent system (olivo-cochlear pathways) or of the other structures of the control loop which could modify outer hair cell activity in an otherwise normal cochlea with the development of tinnitus.     

Otoacoustic emissions in full-term newborns at risk for hearing loss

Distortion product otoacoustic emissions (DPOEs) and click-evoked otoacoustic emissions (CEOEs) characteristics of the normal newborn population have been previously reported in the literature. There is little information about DPOE evaluations in the newborn population at risk for hearing loss. The authors now report the DPOE and/or CEOE data from six full-term subjects at risk for hearing loss or with highly suspected hearing loss. These subjects were less than 1 year of age and at risk for hearing loss secondary to a history of hereditary hearing loss, meningitis, hyperbilirubinemia, and ototoxic drug exposure. Audiometric evaluation included auditory brainstem responses (ABR), behavioral observation audiometry, and tympanometry. The CEOEs and DPOEs were found to be decreased or absent in the subjects with suspected hearing loss secondary to cochlear pathology; they were found to be normal in a subject with a suspected central hearing loss. This study's data suggest that otoacoustic emissions when combined with ABR can provide a frequency-specific evaluation of cochlear function and help determine the anatomic site of a pathologic lesion.     

Active electronic cochlear implants for middle and inner ear hearing loss--a new era in ear surgery. I: Basic principles and recommendations on nomenclature

Hearing aids have fundamental disadvantages: (1) stigmatization of the patient; (2) the sound is often found to be unsatisfactory due to the limited frequency range and undesired distortion; (3) in many patients, the ear canal fitting device generally necessary leads to an occlusion effect; (4) acoustic feedback when amplification is high. Conventional hearing aids transmit sound into the ear canal via a small microphone. Sound has the disadvantage of requiring high output sound pressure levels for its transmission. This along with the necessary miniaturization of the loudspeaker as well as the resonances and reflections in the closed ear canal contribute to the disadvantages mentioned. In contrast, implantable hearing aids do not make sound signals but micromechanical vibrations. An implantable hearing aid has an electromechanical transducer instead of the loudspeaker of a conventional hearing aid. The hearing signal does not leave the transducer as sound but as a mechanical vibration which is directly coupled to the auditory system bypassing the air. This implantable hearing aid is either coupled to the tympanic membrane, the ossicular chain, the perilymph of the inner ear, or the skull. An implantable hearing aid is expected to have: 1 Better sound fidelity than a hearing aid 2 No ear canal fitting device, free ear canal 3 No feedback 4 Invisibility Requirements on electronic hearing implants designed for patients with conductive hearing loss differ from those on implants for sensorineural hearing loss. Conductive hearing loss requires the implant to replace the impedance transformation, thus being an impedance transformation implant (ITI). In various respects, the demands on an ITI are lower than the demands on an electronic hearing aid for patients with sensorineural hearing loss. The latter are mostly patients with a failure of the cochlea amplifier (CA). A damage to the CA is clinically discernible by a positive recruitment and loss of otoacoustic emissions (OAE). Since these patients form the majority of cases with sensorineural hearing loss, an active hearing implant for such patients should partially replace the function of the CA. Therefore, the suggestion is to refer to a CAI (cochlea amplifier implant). The implant expressions ITI (for patients with conductive hearing loss) and CAI (for patients with sensorineural hearing loss) used in this context allow nomenclatural association with the CI (cochlear implant) for complete inner ear failure as well as with the BSI (brainstem implant) in the case of hearing nerve failure.     

Neonatal hearing screening with the Echosensor automated device for otoacoustic emissions. A comparative study

Following the recommendations of the United States National Institutes of Health Consensus Conference in 1993, otoacoustic emissions (OAE) are now used internationally for hearing screening. The use of recording systems as well as the interpretation of results requires specially trained personnel although measurements are easier to perform than other recording methods available. To date, no objective method for general hearing screening, has been introduced in Germany, for neonates or children at risk, even though it would be desirable to detect and rehabilitate children with congenital hearing loss as early as possible (incidence, 1-6 per 1000). The Echosensor provides the means for carrying out OAE recordings within a short period of time and includes an automatic evaluation of results. Recordings can also be made by trained personnel. As a comparison test, audiologically trained staff recorded otoacoustic emissions in 111 new-born children in order to compare the results of the Echosensor with the results of a conventional OAE measurements device (the ILO88). The aim of this pilot study was to determine the reliability and validity of the recordings in comparison with available standards in brainstem audiometry. Our study showed that the results of the Echosensor corresponded well with the ILO88 results. Consequently, an OAE measuring method is now available is also provides high sensitivity and specificity and is easy to use. Our findings show that the Echosensor can meet the demands of systematic hearing screening in Germany.     

Cost of illness: an inextricable maze or an aid in decision making? The case of schizophrenia

Distortion-product otoacoustic emissions (DPOAEs) are still undergoing evaluation for clinical use. Although the effects of ageing on otoacoustic emissions have been studied quite extensively in the past, DPOAE response-growth or input-output (I-O) measures, which are well suited as an objective method for monitoring cochlear function at specific frequencies, have been less thoroughly examined. The aim of the present study was to assess the 2f1-f2 DPOAEs in a clinical setting in order to examine the response of 20 normally hearing middle-aged adults and to compare the results with those of 20 people of the same age with ears of sensorineural high-frequency hearing loss (HL). The experiment consisted of two stages. First, the DPOAE-gram was recorded in 1-4-octave steps at a stimulus level of 70 dB SPL over a frequency range of the f2 primary tone which extended from 1.001 to 6.299 kHz. Secondly, in order to elicit DPOAE I-O functions, the two primary stimuli were presented at equilevel intensities ranging from 20 to 71 dB SPL. The stimulus-level step size was 3 dB. The I-O functions were recorded at five separate DPOAE frequencies, with the f2 frequency most closely related to the clinical audiogram (f2 = 1.0, 1.5, 2.0, 4.0 and 6.0 kHz). Two clearly separated portions in the form of the I-O function for normally hearing ears were found. The first portion, in response to primary levels of 60 dB SPL and below, showed a plateau (saturating) behaviour. If primary levels exceeded 60 dB SPL, I-O functions became more linear. The attenuation of the saturation portion of the I-O function in ears with high-frequency HL across the frequency-test range is difficult to explain because elevated behavioural thresholds were observed only for frequencies > 1.5 kHz. Thus, the more linear I-Os associated with the hearing-loss frequencies may indicate deficiencies in the active properties of outer hair cells (OHCs), whereas those for I-Os < 1.5 kHz, where hearing was normal, may indicate a beginning of damage to active OHC micromechanical processes prior to their clinical manifestation. DPOAE recordings from people with high-frequency HL, possibly age-related, supplement recordings of TEOAEs and give complementary information on degenerative changes in the outer hair-cells. DPOAE I-O functions may reveal discrete pathological alterations both in the active cochlear signal processing and in the passive mechanisms of the cochlea prior to their detection by clinical audiometric tests.     

Analysis of evoked otoacoustic emissions in patients with high frequency cochlear hearing loss

Evoked otoacoustic emissions (EOAE) and auditory brainstem evoked response (ABR) were tested in patients with high frequency cochlear hearing loss and subjects with normal hearing. Results showed that: 1. In the normal group, the main component of EOAE varied between 0.5-5kHz. 2. There was a close correlation between the pure-tone audiogram figure and EOAE spectrum. 3. The I/O function curves of EOAE displayed non-linear characteristics and a trend of saturation with higher stimulus levels in the normal group. In the patient group the growth rates of EOAE and ABR were remarkably higher than those in the normal group with high stimulus levels. It suggests that the abnormal EOAE are probably relevant to the recruitment phenomenon in cochlear hearing loss.     

Changes in otoacoustic emissions in patients with idiopathic sudden deafness

Transiently click-evoked (TEOAE), distortion product (DPOAE) and spontaneous otoacoustic emissions (SOAE) were recorded and changes in these tests were studied during the recovery process in 15 cases of idiopathic sudden deafness (ISD). In all these cases the amplitudes of TEOAEs and DPOAEs increased concurrently with the recovery of the hearing threshold. Ears with ISD were not different in their OAE characteristics from ears with other forms of sensorineural hearing loss (SNHL). In 4 of the 15 cases, SOAEs could be detected when hearing had recovered. These results suggest that the function of outer hair cells (OHCs) had deteriorated when the hearing threshold was elevated and that OHC activity recovered as hearing improved to nearly normal levels in ISD cases with good outcome.     

Otoacoustic emissions in the human

Otoacoustic emissions are sounds emitted by the cochlea, basically deriving from the active micromechanical properties of the outer hair cells of the organ of Corti. As they can be recorded painlessly and non-intrusively, they provide a good means of studying human cochlear functioning. In this report, the main types of otoacoustic emission are described, with their characteristics and relation to cochlear functioning. The contribution of otoacoustic emission studies to the physiology of the medial olivocochlear system is discussed, this being the only sensitive and non-intrusive way of studying this system, the function of which remains uncertain.     

An uncrossed tectopontine pathway mediates ipsiversive circling

Otoacoustic emissions have great promise for use in clinical tests of the functional status of outer hair cells, which represent cochlear structures that make a major contribution to the hearing process. A substantial literature is available concerning the evaluation of outer hair cell function by transiently evoked otoacoustic emissions. However, relatively little attention has been focused on the benefits of testing with distortion-product otoacoustic emissions. The purpose of this presentation is to provide knowledge of the principal advantages offered by distortion-product emissions testing.     

IL-15 induces the release of soluble IL-2Ralpha from human peripheral blood mononuclear cells

The present study was designed to investigate the applicability of transient evoked otoacoustic emissions (TEOAEs) as a method of screening for hearing losses among recruits attending obligatory military service. TEOAEs, tympanometry and puretone audiometry were recorded in 95 male recruits. Sixty-one recruits were tested after a 2-month period of gunfire exposure in order to document any permanent change in cochlear function. Screening by pure-tone audiometry showed an unexpectedly high prevalence of hearing losses > 20 dBHL, probably due to technical reasons. Thresholds were corrected using lower thresholds obtained at the end of service or by ENT specialists. The accuracy with which normal and impaired ears could be identified with TEOAEs analysed in frequency bands was determined by decision theory. Impairment was defined as mean hearing thresholds > or = 30 dBHL averaged from three neighbouring frequencies. Adequate accuracy was obtained in the middle frequencies. Further improvement of the technique is needed before it can be deemed suitable for detecting hearing losses at low and high frequencies. TEOAEs are quicker to measure and offer greater objectivity than pure-tone audiometry. A small decrease in TEOAE level was found after the training period. The TEOAEs were highly repeatable and had a higher sensitivity than pure-tone audiometry to detection of small changes in cochlear function under conditions normally found when testing recruits.     

Distortion-product otoacoustic emissions in kanamycin-treated guinea pig cochlea

Measurement of distortion-product otoacoustic emissions (DPOAE) is widely accepted as one of the most valuable tools for evaluating the frequency of specific cochlear pathology. Previous studies have revealed that distortion-product levels in the ear canal are definitely correlated with degree of damage in the cochlea. However, there seem to be no clear data of help in predicting the distribution and grade of damage in the cochlea quantitatively on the basis of the results of this non-invasive test. The present study is designed to assess correlations between degree of outer hair cell (OHC) damage by a potent ototoxic antibiotic, kanamycin, and DPOAE levels at the characteristic frequency at the site. Guinea pigs were used after daily intramuscular administration of kanamycin for 7 or 10 days. DPOAE levels were measured using a system (CUBDIS: Etymotic Research) with 78 frequency combinations of iso-intensity primaries from 0.5kHz to 16kHz of f2. The frequency ratio (f2/f1) was set at 1.2. Distortion-product level plots versus f2 (DP-grams) were constructed. The integrity of the OHC system was evaluated histologically by the succinic dehydrogenase (SDH) method under a light microscope. Cochleograms were constructed by calculating percentages of intact OHCs along the basilar membrane in 1-mm blocks. The DP-grams and the histopathological cochleograms showed essentially identical patterns in the kanamycin-damaged guinea pig cochlea. The results suggest that: 1) The generation of DPOAE requires functioning OHCs. 2) DPOAE measurement provides information allowing prediction of OHC damage distribution in the cochlea without histological investigations. 3) Careful setting of primary levels and other parameters is necessary to reliably predict the pathology. 4) Attempts to detect of minimal OHC damage could fail. 5) DPOAE seem very useful for monitoring cochlear function in clinically.     

The effects of coenzyme Q10 treatment on maternally inherited diabetes mellitus and deafness, and mitochondrial DNA 3243 (A to G) mutation

Little is known about cisplatin ototoxicity in pediatric patients. Measurement of otoacoustic emissions is a rapid, reproducible, objective method of evaluating hearing. We examined whether transient-evoked otoacoustic emissions in pediatric patients exposed to cisplatin in the past correlated with audiographic findings. Twelve patients were entered into the study (mean age at treatment 7.8 years, mean cumulative dose 442.5 mg/mm2, mean 7.1 doses). Hearing at 3000 Hz was preserved in 82.6% of patients. In the higher frequencies significant sensorineural hearing loss was noted: 43.5% at 4 kHz; 81.0% at 6 kHz; and 90.5% at 8 kHz. Transient-evoked otoacoustic emissions were measurable in 11 of 12 patients. Middle ear disease accounted for abnormal otoacoustic emission seen in three patients (1 with effusion, 2 with significant negative middle ear pressure). When the middle ear was normal, a statistically significant correlation was seen between the transient-evoked otoacoustic emissions reproducibility and pure-tone threshold (correlation coefficient = -0.69, p = 0.008). Increased hearing loss was also associated with young age at first dose of cisplatin (p = 0.044), high number of chemotherapy cycles (p = 0.042), and high cumulative dose (p = 0.042).     

The application of otoacoustic emissions in paediatric hearing screening

We conducted auditory test of 132 high risk infants by using both otoacoustic emissions (OAE) and auditory brainstem response (ABR) screening. The result showed that the passing rate was 88.3% (233/264 ears) for OAE and 92% (243/264 ears) for ABR. The sensitivity and specificity of OAE in comparison to ABR were 90.5% (19/21 ears) and 95% (230/243 ears) respectively. The mean test time was 3 min for OAE and 30 min for ABR. We therefore conclude that OAE is a highly sensitive, reliable and convenient method to be used for paediatric hearing screening.