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Hearing disability and communication handicap for compensation purposes based on self-assessment and audiometric testing.

It is reasoned that for compensation or epidemiological studies an evaluation of hearing disability and the concomitant handicap must include the ability to perceive visual cues. A scaling procedure for hearing- and audiovisual communication handicap is presented. The procedure deviates in two ways from previous handicap assessments: (1) It is based on individual self-assessment of semantic speech perception but can be implemented by means of professional audiological test procedures. (2) The system does not make use of pure-tone auditory thresholds as a predominant audiological principle, but is based on speech perception. The interrelationship between auditory and audiovisual handicap is evaluated. A total score including audio- and audiovisual perception handicap is proposed and a suggestion for disability percentages is presented.

Audiometry↗

Cochlear mechanisms of frequency and intensity coding. II. Dynamic range and the code for loudness.

Our preceding paper described SPL-dependent changes in the shape of transfer functions recorded from inner and outer hair cells as well as supporting cells, in the 500-2500 Hz regions of the Mongolian gerbil cochlea. As SPL was increased, large shifts were observed in the peak of the transfer function. A strongly compressive nonlinearity was also observed at CF. This paper examines the data from the perspective of intensity coding in the auditory periphery. Based on the data, we offer a new explanation for the mechanisms underlying the different dynamic ranges of low and high threshold auditory neurons. We also find that, for pure tone stimuli, the growth of excitation at the characteristic place saturates rapidly, and cannot encode the wide dynamic range of loudness. The data are analyzed to explore other excitation pattern candidates for loudness coding. The growth of the peak of the IHC transfer function, as well as the growth of the response area, have been found to be linearly related to loudness growth over most of its dynamic range. Implications of the data for auditory intensity coding are discussed.

Animals↗

An experimental study of auditory dysfunction associated with hyperlipoproteinemia.

We sought to clarify whether or not hyperlipoproteinemia induces auditory dysfunction. In so doing, we studied the general states and cochlear pathologies of guinea pigs after the administration of a hyperlipid diet for 3 months. Serum biochemistries indicated marked elevations of cholesterol, high density lipoprotein (HDL)-cholesterol and low density lipoprotein (LDL) levels. An increased auditory threshold varying from 10 to 20 dB was observed in 40% of the guinea pigs using auditory brainstem responses. Histochemical study of the inner ear revealed variations in lipid metabolism and partial disorders of the outer hair cells. Electron microscopic observations showed vacuolar and parenchymal protrusions on the surfaces of the stria vascularis and Corti's organ, and vacuolar degeneration was seen around the capillary vessels of the vascular stria. Our data has shown that the auditory dysfunction present in the inner ear was less marked than were the morphological changes seen. Our findings suggest that other factors besides hyperlipoproteinemia are involved in the development of severe auditory damage.

Animals↗

Trichloroethylene ototoxicity: evidence for a cochlear origin.

Trichloroethylene (TCE) is known to produce an unusual pattern of hearing impairment in laboratory animals marked by a preferential loss of threshold sensitivity at midfrequencies. The purpose of this research was to determine whether the TCE-induced auditory deficit results from cochlear dysfunction. Adult Long Evans hooded rats were exposed via inhalation to either 0 (clean air) or 4000 ppm TCE (6 h/day for 5 days). Auditory thresholds for 1-40 kHz tones were determined 3 weeks after exposure using reflex modification audiometry (RMA; n = 12/group). Cochlear electropotentials were measured during subsequent testing (n = 3-10/group) 5 to 7 weeks after exposure, including thresholds for cochlear action potentials (CAP) and the 1-microV cochlear microphonic for 2-40 kHz tones, and the N1 amplitude intensity function (40-90 dB SPL). Cochlear histopathology was assessed in midmodiolar preparations of a separate set of animals, exposed as before (n = 4/group). RMA testing confirmed a TCE-induced loss in midfrequency threshold sensitivity (8 and 16 kHz). CAP thresholds were elevated at midfrequencies (8 and 16 kHz) among TCE-treated subjects, along with a suppression of the N1 amplitude from 50 to 90 dB SPL. The cochlear microphonic, a nonpropagated ac potential generated largely by the outer hair cells, was not affected by the TCE treatment. Cochlear histopathology revealed a loss of spiral ganglion cells that was significant in the middle turn, but not in the basal turn. There was an inconsistent loss of hair cells among treated subjects. The data suggest strongly that the behaviorally determined loss in auditory function can be accounted for by a cochlear impairment and that the spiral ganglion cell may be a prominent target of TCE.

Animals↗

Effects of click rate and electrode orientation on threshold of the auditory brainstem response.

This study evaluated the effects of stimulus presentation rate and electrode orientation on ABR threshold. Six normal-hearing adults served as subjects. ABRs were recorded from three orthogonal electrode pairs in response to click stimuli at rates of 48 and 21/s. Psychophysical thresholds were determined for each of these stimuli, and ABRs were recorded with stimuli between 0 and 10 dB SL in 2-dB steps. A ratio of ABR signal strength to noise variance (Fsp) was the criterion used to define threshold. Specifically, an Fsp value of 2.25, equivalent to a 95% response confidence using 5 and 250 degrees of freedom, was the criterion used to determine response presence within a maximum of 10,000 sweeps per average. Results revealed that, for normal-hearing adult subjects, click rate up to 48/s does not influence the sensation level at which a detectable response can be found, and a nearly vertical electrode orientation is optimal for recording the response at low levels. The predicted difference between psychophysical and ABR thresholds with a vertical electrode orientation, using 10,000 sweeps per average, up to 48 clicks per second, and an Fsp criterion of 2.25 is 4.4 dB.

Auditory Threshold↗

Normal auditory brainstem and cochlear function in extreme pediatric plumbism.

Lead (Pb) intoxication in children has been associated with encephalopathy, sensory and cognitive impairments. We investigated the prevalence and neuro-sensory effects of Pb exposure in children living in Andean villages of Ecuador with high Pb contamination from discarded automobile batteries used in the local ceramics glazing industry. Venous blood samples were collected from 107 children in the Pb glazing area and from 39 children living in a geographically distant area with no known Pb contamination and measured for blood lead (PbB) levels. Auditory brainstem responses (ABR) and audiological/otological tests were conducted on children in the Pb-Glazing Group. The median PbB level for children in the Pb-Glazing Group was 40.0 microg per dl (range: 6.2-128.2 microg per dl) and for the non Pb-Glazing Group 6.0 microg per dl (1.9-18.0 microg per dl). The differences in PbB levels for children in the study and control areas were statistically significant (t-test, P<0.0001). ABR tests on the Pb-Glazing Group indicated normal wave latencies and neural transmission times, and no statistical correlation between PbB level and interpeak latencies. Audiological tests showed normal cochlear function and no statistical relation between auditory thresholds and PbB level. Contrary to prevailing assumptions, elevated PbB levels in children do not invariably impair auditory brainstem neural transmission or sensory-neural cochlear function, both of which have been implicated as significant contributors to the neurodevelopmental disabilities associated with childhood plumbism.

Adolescent↗

Auditory steady-state response audiometry in profound SNHL: the impact of abnormal middle ear function.

Auditory steady-state response (ASSR) audiometry is a commercially available tool that is used to predict behavioral auditory threshold levels. Its particular value stems from the technology's ability to measure frequency-specific responses in the background electroencephalogram to auditory stimuli presented across a broad range of frequencies and sound pressure levels. It is clearly of benefit when used to assess threshold levels in infants and children with severe-to-profound hearing impairment (i.e., cochlear implant candidates). Although numerous authors have provided evidence of the usefulness of ASSR testing, their reports have concerned patients whose middle ear impedance measures were normal. We report the cases of 2 patients who, following improvement of abnormal middle ear impedance values, experienced a marked improvement in measurable thresholds by ASSR testing.

Acoustic Impedance Tests↗

Auditory steady-state responses in infants with perinatal brain injury.

Infants with perinatal brain injury present impairments in motor, visual, auditory, and cognitive functions. The most useful methods for detecting auditory alterations are auditory brainstem responses and otoacoustic emissions. Auditory steady-state responses have been reported as a reliable and objective technique for evaluating the hearing threshold. Auditory brainstem responses and auditory steady-state responses were carried out in 53 infants with perinatal brain injury and abnormal neurologic findings. With auditory brainstem responses, 33 (62.26%) infants presented normal and 20 abnormal results; 8 (15.09%) exhibited mild alterations, 8 (15.09%) moderate, and 4 (7.54%) severe alterations. With auditory steady-state responses, 17 (32.0%) infants were normal and 36 (67.9%) had abnormal results. When auditory steady-state responses were compared with auditory brainstem responses gold standard, the assessment gave 100% sensitivity, 51.51% specificity, 55.55% positive predictive value, and 100% negative predictive value. Abnormalities were mild in 21 (39.6%) infants, moderate in 10 (18.9%), and 5 (9.4%) exhibited severe hearing loss. We conclude that hearing loss is a frequent abnormality in infants with perinatal brain injury, and auditory steady-state responses have a high sensitivity for detecting hearing impairment, which is more evident in mild hearing loss for specific frequencies.

Brain Diseases↗

Trophic effects of insulin-like growth factor-I (IGF-I) in the inner ear.

Insulin-like growth factors (IGFs) have a pivotal role during nervous system development and in its functional maintenance. IGF-I and its high affinity receptor (IGF1R) are expressed in the developing inner ear and in the postnatal cochlear and vestibular ganglia. We recently showed that trophic support by IGF-I is essential for the early neurogenesis of the chick cochleovestibular ganglion (CVG). In the chicken embryo otic vesicle, IGF-I regulates developmental death dynamics by regulating the activity and/or levels of key intracellular molecules, including lipid and protein kinases such as ceramide kinase, Akt and Jun N-terminal kinase (JNK). Mice lacking IGF-I lose many auditory neurons and present increased auditory thresholds at early postnatal ages. Neuronal loss associated to IGF-I deficiency is caused by apoptosis of the auditory neurons, which presented abnormally increased levels of activated caspase-3. It is worth noting that in man, homozygous deletion of the IGF-1 gene causes sensory-neural deafness. IGF-I is thus necessary for normal development and maintenance of the inner ear. The trophic actions of IGF-I in the inner ear suggest that this factor may have therapeutic potential for the treatment of hearing loss.

Animals↗

Hyperthermia increases aminoglycoside ototoxicity.

Similarities were noted between the nature of inner ear damage produced by loud sounds and by aminoglycoside antibiotics. Since body temperature affects cochlear function and influences the effects of noise on the ear, a similar effect was predicted for the aminoglycoside, kanamycin. By environmentally elevating the body temperatures of preweanling mice to approximately 1 degree C above that of the normal adult, kanamycin ototoxicity was increased (an average 20.4 dB threshold elevation, vs. 9.3 dB for kanamycin injected mice reared at room temperature). Hyperthermia per se had no influence on auditory thresholds. This may be of relevance to humans with fever who are treated with aminoglycoside antibiotics.

Animals↗

Quantitative analysis of nerve fibre densities in the cochlea of the house mouse (Mus musculus).

Transverse sections of the cochlear nerve, silver-stained surface preparations of the cochlea, and silver- and osmium-stained tangential sections of the cochlea of the house mouse were made to determine the number and density of nerve fibres entering the organ of Corti and their distribution to inner and outer hair cells along the cochlear spiral. A number of 12,578 +/- 819 fibres was counted within the cochlear nerve, which is almost equal to the total number of 12,350 +/- 810 fibres entering the organ of Corti. The 12,350 fibres divide into 9,780 (= 79%) fibres running to the inner hair cells, 703 (= 6%) basilar (afferent) fibres and 1,867 (= 15%) upper tunnel radial (efferent) fibres innervating the outer hair cells. About 93% of all afferent fibres are connected to the inner hair cells, and only 7% innervate outer hair cells. The density of fibres running to the inner hair cells varies considerably and has a significant (p less than 0.01) absolute and relative maximum 3.7 mm and 2.9 mm from the apex respectively (total length: 6.84 mm) and decreases toward apex and base. The density of afferent fibres running to the outer hair cells shows a slow increase up to 2 mm from the apex, and remains on a constant low level (5.5 fibres per 40 micrometer) down to the base. The density of efferent fibres running to the outer hair cells increases linearly up to about 2 mm from the apex, remains rather constant (10 fibres per 40 micrometer) from 2 mm to 4 mm, and then decreases toward the base. Generally, the outer hair cells show a convergent innervation pattern, the inner hair cells a divergent one. The significance of the present measurements is discussed in relation to respecitve results from other mammals and in relation to auditory thresholds.

Animals↗

Influence of electrode position on near-threshold recording of auditory evoked brainstem potentials.

Use of brainstem potentials in audiology is based on identifying a response in the near-threshold range. Wave V has turned out to be the component of the brainstem potentials that can be detected with a great deal of reliability when assessment of the threshold for the particular stimulus is desirable. As the detection of Wave I can be dispensed within audiologic diagnosis, the question arises as to whether or not a non-cephalic reference electrode compared to the common lateral position of the reference electrode on the ipsilateral mastoid produces a more stable Wave V of increased amplitude. In a group of 20 normal-hearing adults, near-threshold stimulation with clicks was conducted and the brainstem potentials recorded simultaneously with the reference electrode placed in four different locations (ipsilateral mastoid, contralateral mastoid, non-cephalic electrode, and ipsilateral earlobe). At all of the three intensities studied (10 dB nHL, 20 dB nHL and 30 dB nHL), recording with the non-cephalic reference yielded the highest amplitudes, the finding being statistically significant.

Adolescent↗

Contralateral sound stimulation suppresses the compound action potential from the auditory nerve in humans.

BACKGROUND: In the past decade, contralateral sound suppression of otoacoustic emissions has been extensively used to study the role of the medial olivocochlear efferent in humans. In most studies, the suppressive effect of contralateral sound stimulation was not greater than 2 to 4 dB. However, the relation between the degree of otoacoustic emission reduction and the neural auditory threshold is unknown. METHODS: The current study investigates the effect of contralateral sound stimulation by measuring compound action potential response from the auditory nerve during retrosigmoid surgery in humans. RESULTS: Although only a small number of subjects responded to contralateral sound stimulation, we report that efferent activation by contralateral sound stimulation results in 10 dB effective neural attenuation. CONCLUSIONS: Together with previous otoacoustic emission measurements in humans, this result demonstrates that the suppressive effect of contralateral noise suppression is greater when measured with compound action potential than otoacoustic emissions, and that contralateral sound suppressive effect is at least as strong in humans as in animals.

Acoustic Stimulation↗

Auditory neuropathy: case study with hyperbilirubinemia.

Auditory neuropathy (AN) has been described in the literature as presenting with a combination of audiometric findings that include elevated behavioral audiometric thresholds, auditory brainstem response findings that are not consistent with audiometric findings, poor speech recognition, and present otoacoustic emissions (OAEs) and/or cochlear microphonics. Since the availability of clinical OAE testing, AN has come to be identified with increasing frequency; however, incidence and prevalence figures are unavailable. There is a great deal of discussion about the accurate diagnosis of AN, its characteristics, and its treatment. Some of this discussion is occurring on the Internet and over the telephones. The need to continue to provide information in accessible peer-reviewed journals is paramount. Following a review of the literature, a case study is presented of a boy who was diagnosed with AN as a newborn. He experienced hyperbilirubinemia and other neonatal health complications. His educational intervention was managed elsewhere until recently. Information is presented about the progression of the case over a 5-year period that includes audiologic data and communication development results.

Audiometry, Pure-Tone↗

D-Methionine and cisplatin ototoxicity in the guinea pig: D-methionine influences cisplatin pharmacokinetics.

D-Methionine has recently been advocated as a protectant against cisplatin toxicity. The use of systemic D-methionine as a protector was studied in 58 guinea pigs. Kinetics and distribution of [11CH(3)]D-methionine was analysed by positron emission tomography. Cisplatin and the monohydrated complex of cisplatin was quantified in blood ultrafiltrate using reversed-phase liquid chromatography with post-column derivatisation. Administration of 300 mg/kg of D-methionine caused a 30% decrease in the area under the concentration-time curve (AUC) of cisplatin. The toxic effect of cisplatin was studied after dose adjustment of cisplatin, i.e. with similar cisplatin AUC in the group receiving D-methionine and the saline control group. A significant ototoxic effect, measured as difference in pre- and 96 h post-treatment electrophysiological hearing threshold (auditory brainstem response), was observed at stimulus frequencies of 30 and 20 kHz. There was no difference between the groups in the extent of threshold shift. Quantitative outer hair cell counts showed a similar loss of cells in the two groups. All animals had a significant increase in plasma-creatinine but there was no difference between the groups. The results indicate that protection from cisplatin ototoxicity by systemic D-methionine can be explained by a lowered systemic exposure to the drug.

Animals↗

[Effect of myelography on the curve of the auditory evoked early brain stem potentials].

In 50 patients auditory threshold and brain stem evoked potential studies were carried out before and after myelography. Due to the analysis of amplitudes and latencies of auditory brain stem measurements, significant functional disorders of the hearing organ and the auditory pathway could be demonstrated. In most of the patients these functional disorders were found to be subclinical, whereas 12 patients showed alterations extending from a subjectively slight hearing loss to an audiometrically objectified acute hearing loss depending on its intensity in each case. The reasons of these functional disorders could not be clarified. An open cochlear aqueduct through which perilymph enters the subarachnoidal space leading to a secondary endolymphatic hydrops can be considered as the cause in cases where manifest symptoms develop. The changes in brain stem audiometry can be additionally explained by changes in osmolality of the inner ear fluids which may lead to the development of an endolymphatic hydrops.

Auditory Pathways↗