Theoretical note on some types of psychogenic deafness, i.e., central, conductive, perceptive.
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Usher syndrome is a congenital autosomal dominant inherited disease characterised by hearing, balance and vision problems. The aim of the work was an evaluation of hearing and vestibular organ in the chosen group of patients with Usher syndrome diagnosed. Material consisted of 10 persons (5 males and 5 females) aged from 16 to 46 years with Usher syndrome diagnosed. All the patients have been in constant ENT and Ophthalmology Clinics' care. Complex hearing and balance organ, including posturography, as well as complex ophthalmologic examination were performed in each case. Medium or severe perceptive cochlear hearing loss, proper function of vestibular organ and slight elevation of stabilograms parameters were found in 6 cases. In 4 cases there were severe bilateral hearing loss, lack of vestibular function and considerable worsening of stabilograms parameters. In ophthalmologic examination in all the cases there was bilateral symmetrical dystrophy of the retina of various intensification. We concluded that hearing and balance organ examination in Usher syndrome can state accessory diagnostic aspect that might help to distinguish subtypes of the syndrome. In this way audiologic and otoneurologic tests could help to define prognosis of the disease in the individual cases. A necessity of close co-operation of genetics, ophthalmologists and ENT doctors as well as psychologists was stressed.
OBJECTIVE: To assess the relationship of loudness growth and the click-evoked auditory brain stem response (ABR) wave V latency-intensity function (LIF) in listeners with normal hearing or cochlear hearing loss. The effect of hearing loss configuration on the intensity functions was also examined. DESIGN: Behavioral and electrophysiological intensity functions were obtained using click stimuli of comparable intensities in listeners with normal hearing (Group I; n = 10), and cochlear hearing loss of flat (Group II; n = 10) or sloping (Group III; n = 10) configurations. Individual intensity functions were obtained from measures of loudness growth using the psychophysical methods of absolute magnitude estimation and production of loudness (geometrically averaged to provide the measured loudness function), and from the wave V latency measures of the ABR. RESULTS: Slope analyses for the behavioral and electrophysiological intensity functions were separately performed by group. The loudness growth functions for the groups with cochlear hearing loss approximated the normal function at high intensities, with overall slope values consistent with those reported from previous psychophysical research. The ABR wave V LIF for the group with a flat configuration of cochlear hearing loss approximated the normal function at high intensities, and was displaced parallel to the normal function for the group with sloping configuration. The relationship between the behavioral and electrophysiological intensity functions was examined at individual intensities across the range of the functions for each subject. A significant relationship was obtained between loudness and the ABR wave V LIFs for the groups with normal hearing and flat configuration of cochlear hearing loss; the association was not significant (p = 0.10) for the group with a sloping configuration of cochlear hearing loss. CONCLUSION: The results of this study established a relationship between loudness and the ABR wave V latency for listeners with normal hearing, and flat cochlear hearing loss. In listeners with a sloping configuration of cochlear hearing loss, the relationship was not significant. This suggests that the click-evoked ABR may be used to estimate loudness growth at least for individuals with normal hearing and those with a flat configuration of cochlear hearing loss. Predictive equations were derived to estimate loudness growth for these groups. The use of frequency-specific stimuli may provide more precise information on the nature of the relationship between loudness growth and the ABR wave V latency, particularly for listeners with sloping configurations of cochlear hearing loss.
Hearing impairment was anecdotally reported in resistance to thyroid hormone (RTH), a condition caused by mutations in the beta-thyroid hormone receptor (beta TR) gene. Because of its ontogenic distribution in the cochlea, the beta TR may have a pivotal role in the development of auditory function. To assess the prevalence and mechanisms of hearing impairment in RTH, 82 RTH-positive (RTH+) patients and 55 unaffected relatives (RTH-) underwent systematic audiological examination, including puretone and speech reception thresholds, and tests studying middle ear (tympanometry and acoustic reflexes), cochlear (otoacoustic emissions), and retrocochlear integrity (brain stem auditory evoked potentials). Significant hearing loss was present in 21% of RTH+ patients vs. none in RTH- patients. More RTH+ patients had abnormal tympanometry (34% vs. 12%) and abnormal acoustic reflexes (39% vs. 19%). Isolated conductive deficit was found in 7 of 17 RTH+ patients with hearing loss, isolated sensorineural deficit in 7 cases, and mixed deficit in 3 cases. Cochlear dysfunction was found in 50% of all RTH+ patients, with or without hearing loss. Retrocochlear function was normal. No morphological cochlear abnormalities were detected on computed tomography of the temporal bone. In conclusion, hearing loss is a significant problem in RTH, with an equal frequency of conductive (probably related to the frequent ear infections) and sensorineural deficits. Abnormal otoacoustic emissions suggest that the mutant beta TR has a specific negative impact on cochlear function.
Sensorineural hearing loss (SNHL) has been documented in patients with otitis media. Despite a number of clinical and pathologic works dealing with this common problem, animal studies searching for possible relationships between the middle ear inflammation and cochlear function remain insufficient. Bacterial inoculation and ototoxins and inflammatory products in the middle ear cavity cause SNHL in rodents. Human serum albumin placed in the middle ear cavity in chinchillas also produces SNHL, owing to the effects of nonspecific inflammation in the middle ear cavity. Most of the middle ear inflammatory mediators enter the inner ear through the round window route, and alteration of the permeability of the round window membrane plays an important role in causing cochlear dysfunction. Although an immunologic response in the middle ear plays an important role in otitis media, the immunologic response in the inner ear as it relates to middle ear inflammatory mediators requires further study.
The effects of exposure to an augmented acoustic environment (AAE) on auditory function were evaluated in mouse strains that exhibit various degrees and time courses of progressive hearing loss (BXD-22, BXD-12, BXD-16, BXD-14, BALB/cJ), and in normal-hearing CBA/CaJ mice. Beginning at age 25 days, mice were exposed 12 h every night to a 70 dB SPL broadband noise AAE. The AAE was maintained for at least 30 days in each strain. Same-strain control mice were age-matched and maintained under normal vivarium acoustic conditions. The auditory brainstem response (ABR), acoustic startle response amplitude, and prepulse inhibition (PPI) were used to assess the auditory system. Exposure to the AAE resulted in improved auditory performance (better PPI, lower ABR thresholds) when hearing impairment was present, but not when hearing was normal. The ameliorative effects occurred irrespective of a mouse's age at the onset of hearing loss, as long as initiation of AAE treatment preceded the occurrence of severe hearing loss. If AAE treatment was delayed beyond such a point, loss of threshold sensitivity progressed as usual, although PPI could still benefit. Finally, AAE treatment can slow, but not prevent, the occurrence of severe genetically determined hearing loss.
Following Kemp's original studies, several others have confirmed the existence of otoacoustic emissions. Their clinical relevance remains, however, to be clarified. The various published studies have concerned small series. This study sought to specify otoacoustic emission characteristics in relation to sensorineural hearing loss (148 ears of 76 subjects). The results show that the presence of otoacoustic emissions drops as a function of hearing loss and that there is a highly statistically significant correlation between otoacoustic emission threshold and hearing loss at the 1000-Hz frequency. Otoacoustic emissions are never found when hearing loss at 1000 Hz exceeds 40 dB hearing level and when the mean audiometric hearing loss (at 500, 1000, 2000, and 4000 Hz) exceeds 45 dB hearing level. The main practical conclusion is that otoacoustic emission presence indicates middle frequency functional integrity of the outer hair cells of Corti's organ. Absence of otoacoustic emissions is harder to interpret.
The effect of conductive hearing loss on the maturation of the auditory pathway was evaluated using the auditory brainstem response (ABR) in rhesus monkeys. Ten newborn rhesus monkeys were assigned to control (N = 4), unilateral hearing loss (N = 3), or bilateral hearing loss (N = 3) groups. Hearing loss was created by surgically excising a 3 mm section of the external auditory canal and suturing the canal. Auditory brainstem responses to click stimuli were recorded prior to and after the surgical procedure and bi-monthly or monthly for a 14 month follow-up period. Results showed that after surgery all ears developed an estimated 30-50 dB conductive hearing loss which was retained throughout the follow-up period. Contrary to expectations, the latencies of the ABR component waves decreased with age in all ears. When adjusted for hearing level, there were no differences between ears in maturation of the component waves of the ABR. These data suggest that, in primates, a conductive hearing loss does not affect the maturation of those aspects of the auditory pathway reflected in the ABR. Furthermore, the conductive losses were not accompanied by any discernible change in the neuronal sizes of brainstem auditory neurons or the volume of the cochlear nuclei.
The purpose of this chapter is to review the literature on hearing impairment, specifically the impact of hearing impairment on the functioning of elders, interventions that minimize the impact of hearing loss on functioning, and identification of issues raised by the review for nursing research. Computerized (MEDLINE, PsychINFO, and CINAHL) and manual searches were used to obtain research reports from a range of disciplines. Research articles including elders (> or = 60) and published between 1989 and 2001 were included. Twenty-five articles were selected for critical review, four written by nurses. The diversity of methodologies, the criteria used to define hearing impairment, the range of sample characteristics, and the assessment measures make comparisons across studies difficult. Most studies, however, support the negative impact of hearing impairment, especially on psychosocial functioning. Measures that are condition specific are generally more effective in capturing the impact of hearing loss than generic measures. Findings related to physical disability are less consistent. The results of intervention studies suggest that hearing devices can improve psychosocial and communication outcomes, but behavioral interventions have not shown long lasting benefit. For nurses to assist elders and their families manage the impact of hearing impairment, further research is needed in several areas that have been poorly explored. These include the dyadic experience of hearing impairment, the way in which culture influences the experience of hearing loss, the needs of hearing impaired individuals across settings, the long-term impact of ototoxic medications, and strategies to assist elders in coping with hearing impairment and utilizing available technologies.
Conductive hearing loss due to secretory otitis media is the commonest form of hearing loss in children. Eustachian tube dysfunction is often implicated as the most important factor in the origin and course of the disease. It is well known that myringotomy, aspiration of fluid from within the middle ear, and ventilation with any one of several types of tubes will restore hearing and mobility of the tympanic membrane in the vast majority of patients. The effects of these procedures on Eustacian tube function and on middle ear and mastoid volumes, particularly on a long-term basis, have not been clearly delineated. Thirty-six children (72 ears) with secretory otitis media were studied. During the course of the disease, fluid was aspirated from the middle ear and Silastic ventilation tubes were inserted. Hearing levels, tympanogram type, middle ear volume, and Eustachian tube function were determined before and after operation. After myringotomy, aspiration of fluid, and ventilation, we found that (1) middle ear volume progressively increased during a period of three to eight months after operation; (2) Eustachian tube function remained abnormal while ventilation tubes were in place, and (3) hearing was restored to normal levels.
The growth function of the acoustic stapedius reflex was measured in subjects with normal hearing and sensorineural hearing loss of cochlear origin. The effects of age and magnitude of hearing loss were controlled. Activating stimuli were 500, 1000, and 2000 Hz tones and broadband noise. Stapedius muscle activity was inferred from acoustic impedance measures in the contralateral ear. The mean growth functions for tones were essentially linear in log-log plots with the rate of growth equal for the two groups. The mean growth function for the noise signal was curvilinear for the normal hearing groud had linear for the hearing loss group. Comparison of slope functions derived from the fitted data indicated that the rate of reflex growth for the noise signal, over a limited range above reflex threshold, is greater in ears with cochlear lesions than normal ears. For higher level noise signals, however, the rate of reflex growth is similar for normal and pathological ears. The effect of a cochlear lesion on the input-output function of the cochlea for both tonal and noise stimuli is to maintain the rate of reflex growth but shift the function along the intensity axis of a tonal signal and the response axis for a noise signal.
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Theories concerning the influence of function disturbances of the cervical spine on the development of sudden hearing loss should be re-examined. Forty-eight patients with unilateral sudden hearing loss and 94 controls were examined. X-rays of the cervical spine were made in lateral projection in normal position, and in maximum flexion and extension. The posture and movement of the vertebrae were recorded using a computer digitalization program. Overall mobility of the cervical spine was reduced in the group with sudden hearing loss: reduced movement was found in segments C2/C3, C5/C6, and C6/C7. Segments P0/P1 and C1/C2 were conspicuous by very high standard deviations in the group with sudden hearing loss. The results indicate that sudden hearing loss and function disorders of the cervical spine are related.
Auditory and vestibular function was examined in 29 patients with unilateral sensorineural hearing loss to identify characteristic vestibulocochlear lesion patterns. In 45%, a vestibular lesion was found, of which 53% had a combined impairment of the cochlea and the ipsilateral posterior semicircular canal, possibly reflecting vascular disease in the common cochlear artery.
The World Health Organization's (WHO) Disability Assessment Scale II (WHO-DAS II) is a generic health-status instrument firmly grounded in the WHO's International Classification of Functioning, Disability and Health (WHO-ICF). As such, it assesses functioning for six domains: communication, mobility, self-care, interpersonal, life activities, and participation. Domain scores aggregate to a total score. Because the WHO-DAS II contains questions relevant to hearing and communication, it has good face validity for use as an outcome measure for audiologic intervention. The purpose of the present study was to determine the psychometric properties of the WHO-DAS II on a sample of individuals with adult-onset hearing loss, including convergent validity, internal consistency, and test-retest stability. Convergent validity was established by examining correlations between the WHO-DAS II (domain and total scores) and the Abbreviated Profile of Hearing Aid Benefit (APHAB) and the Hearing Aid Handicap for the Elderly (HHIE), two disease-specific measures, as well as with the Short Form-36 for veterans (SF-36V), a second generic measure. Data on all four measures were collected from 380 older individuals with adult-onset hearing loss who were not hearing aid users. The results of the convergent validity analysis revealed that the WHODAS II communication domain score was moderately and significantly correlated with scores on the APHAB and the HHIE. WHO-DAS II interpersonal and participation domain scores and the total scores were also moderately and significantly correlated with HHIE scores. These findings support the validity of using the WHO-DAS II for assessing activity limitations and participation restrictions of adult-onset hearing loss. Several WHO-DAS II domain scores and the total score were also significantly and moderately-markedly correlated with scores from the SF-36V. These findings support the validity of the WHO-DAS II as a generic health-status instrument. Internal consistency reliability for all the domain scores was adequate for all but the interpersonal domain. Test-retest stability for all the domain scores was adequate. Critical difference values were calculated for use in clinical application of the WHO-DAS II. From these findings, we concluded that the WHO-DAS II communication, participation, and total scores can be used to examine the effects of adult-onset hearing loss on functional health status. Further work examining the utility of the WHO-DAS II as an outcome measure for hearing aid intervention is warranted.
In normal-hearing subjects, the slope of the growth-of-masking (GOM) function obtained in simultaneous masking when the masker frequency (f(m)) is much less than the signal frequency (f(s)) often changes from a value near 2.0 to a value near 1.0 at high levels. The purpose of the present study was to evaluate whether this change in slope reflects a basal shift in the peak of the signal's basilar-membrane vibration pattern. To discourage the use of basally shifted peak excitation, GOM functions were obtained in seven subjects with a precipitously sloping high-frequency hearing loss. The signal was located at the normal-hearing edge of the loss, and the masker was located 3 equivalent rectangular bandwidths below f(s). In addition, GOM functions for an f(s) of 2000 Hz were obtained in four subjects with normal hearing, either "in quiet" or in the presence of a restrictor tone with a frequency of 2400 or 2600 Hz and a level of 90 dB SPL. Overall, the results generally are not consistent with the change in slope at high levels being due to a basal shift in the peak of the signal's basilar-membrane vibration pattern. Instead, the results are consistent with a decrease in compression at high input levels at the place corresponding to f(s).
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