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Speech perception and cortical event related potentials in children with auditory neuropathy.

OBJECTIVES: 1) To investigate the unaided and aided speech perception abilities of children with auditory neuropathy (AN) and to compare their performance to children with sensorineural hearing loss. 2) To establish whether cortical event related potentials (ERPs) could be recorded in children with AN, and to determine the relationship between the presence of these responses and speech perception. DESIGN: Unaided and aided speech perception assessments (PBK words), and cortical-ERP testing was carried out in a group of 18 children with AN. Data also were obtained from a cohort of age and hearing level matched children with sensorineural hearing loss. RESULTS: The speech perception performance of the 15 children with AN able to complete a PBK-word assessment, fell into two distinct categories. The children either showed no open-set speech perception ability (7/15 cases), or performance levels similar to their sensorineural counterparts (8/15 cases). Approximately 50% of children with AN showed ERPs of normal latency, amplitude and morphology. In all cases, response presence (at normal latencies) was consistent with reasonable speech perception ability, and response absence was consistent with negligible speech perception. CONCLUSIONS: In approximately 50% of children with auditory neuropathy, the provision of amplification results in significant open-set speech perception improvements. The results confirm the previously published reports that speech perception ability cannot be reliably estimated from the behavioral audiogram in children with AN. Obligatory ERP test results may offer a means of predicting perceptual skills in newly diagnosed youngsters as the presence of ERPs (with age-appropriate latency and morphology) was correlated with significant open set speech perception abilities and amplification benefit. The absence of the ERP in contrast, indicated profound hearing disability evidenced by profound hearing loss and/or extremely poor speech perception.

Adolescent↗

Speech recognition in fluctuating and continuous maskers: effects of hearing loss and presentation level.

Listeners with normal-hearing sensitivity recognize speech more accurately in the presence of fluctuating background sounds, such as a single competing voice, than in unmodulated noise at the same overall level. These performance differences are greatly reduced in listeners with hearing impairment, who generally receive little benefit from fluctuations in masker envelopes. If this lack of benefit is entirely due to elevated quiet thresholds and the resulting inaudibility of low-amplitude portions of signal + masker, then listeners with hearing impairment should derive increasing benefit from masker fluctuations as presentation levels increase. Listeners with normal-hearing (NH) sensitivity and listeners with hearing impairment (HI) were tested for sentence recognition at moderate and high presentation levels in competing speech-shaped noise, in competing speech by a single talker, and in competing time-reversed speech by the same talker. NH listeners showed more accurate recognition at moderate than at high presentation levels and better performance in fluctuating maskers than in unmodulated noise. For these listeners, modulated versus unmodulated performance differences tended to decrease at high presentation levels. Listeners with HI, as a group, showed performance that was more similar across maskers and presentation levels. Considered individually, only 2 out of 6 listeners with HI showed better overall performance and increasing benefit from masker fluctuations as presentation level increased. These results suggest that audibility alone does not completely account for the group differences in performance with fluctuating maskers; suprathreshold processing differences between groups also appear to play an important role. Competing speech frequently provided more effective masking than time-reversed speech containing temporal fluctuations of equal magnitude. This finding is consistent with "informational" masking resulting from competitive processing of words and phrases within the speech masker that would notoccur for time-reversed sentences.

Acoustic Stimulation↗

Quantifying the intelligibility of speech in noise for non-native talkers.

The intelligibility of speech pronounced by non-native talkers is generally lower than speech pronounced by native talkers, especially under adverse conditions, such as high levels of background noise. The effect of foreign accent on speech intelligibility was investigated quantitatively through a series of experiments involving voices of 15 talkers, differing in language background, age of second-language (L2) acquisition and experience with the target language (Dutch). Overall speech intelligibility of L2 talkers in noise is predicted with a reasonable accuracy from accent ratings by native listeners, as well as from the self-ratings for proficiency of L2 talkers. For non-native speech, unlike native speech, the intelligibility of short messages (sentences) cannot be fully predicted by phoneme-based intelligibility tests. Although incorrect recognition of specific phonemes certainly occurs as a result of foreign accent, the effect of reduced phoneme recognition on the intelligibility of sentences may range from severe to virtually absent, depending on (for instance) the speech-to-noise ratio. Objective acoustic-phonetic analyses of accented speech were also carried out, but satisfactory overall predictions of speech intelligibility could not be obtained with relatively simple acoustic-phonetic measures.

Adult↗

Manufacturer predicted and measured REAR values in adult hearing aid fitting: accuracy and clinical usefulness.

Audiologists may fit hearing aids using real-ear aided response (REAR) values predicted by manufacturer's fitting software, rather than actual measured REARs. This study examined how well one manufacturer's software was able to predict REARs for a DSP behind-the-ear product in 41 adults (N = 79 ears) under four conditions (two hearing loss configurations and two inputs). Results showed that for all ears tested, measured REAR values were significantly different from predicted for most of the audiometric frequencies for all four test conditions. Discrepancies between measured and predicted REAR values were greater in males' ears than females'. Few ears' REAR values (< or = 12%) were judged to be clinically similar to predicted. Results suggest audiologists should consider using individual real-ear measures in adult hearing aid fittings until manufacturer software is shown to accurately predict real-ear hearing aid performance.

Adult↗

Digital hearing aids for high-frequency sensorineural hearing loss: preliminary experience with the RetroX device.

CONCLUSIONS: The RetroX outer ear hearing aid seems to represent a means of overcoming problems with understanding speech in noise in patients with high-frequency sensorineural hearing loss (SNHL) without the need to wear conventional completely-in-the-canal (CIC) hearing aids, which are usually reported to annoy patients as a result of the occlusion effect. OBJECTIVE: To present preliminary data from a study carried out to compare the efficacy, in the same individual, of a standard digital CIC hearing aid and a new implantable outer ear canal device, the RetroX. MATERIAL AND METHODS: Three out of 15 adults affected by high-frequency SNHL who were candidates for auditory rehabilitation were evaluated by using speech audiometric tests in quiet and noise as well as a questionnaire shortly after use of a CIC hearing aid and the RetroX device, i.e. at 7 and 14 days. The efficacy of the RetroX was anticipated by testing all the subjects using a RetroX simulating system before starting the study protocol. RESULTS: In all three implanted patients, the RetroX provided better audiological benefit for speech understanding in noise. These findings were corroborated by the results of the questionnaire, which showed greater satisfaction with the RetroX, especially regarding the absence of the occlusion effect.

Adult↗

Comparison of speech perception in background noise with acceptance of background noise in aided and unaided conditions.

Background noise is a significant factor influencing hearing-aid satisfaction and is a major reason for rejection of hearing aids. Attempts have been made by previous researchers to relate the use of hearing aids to speech perception in noise (SPIN), with an expectation of improved speech perception followed by an increased acceptance of hearing aids. Unfortunately, SPIN was not related to hearing-aid use or satisfaction. A new measure of listener reaction to background noise has been proposed. The acceptable noise level (ANL), expressed in decibels, is defined as a difference between the most comfortable listening level for speech and the highest background noise level that is acceptable when listening to and following a story. The ANL measure assumes that speech understanding in noise may not be as important as is the willingness to listen in the presence of noise. It has been established that people who accept background noise have smaller ANLs and tend to be "good" users of hearing aids. Conversely, people who cannot accept background noise have larger ANLs and may only use hearing aids occasionally or reject them altogether. Because this is a new measure, it was important to determine the reliability of the ANL over time with and without hearing aids, to determine the effect of acclimatization to hearing aids, and to compare the ANL to well-established measures such as speech perception scores collected with the SPIN test. Results from 50 listeners indicate that for both good and occasional hearing aid users, the ANL is comparable in reliability to the SPIN test and that both measures do not change with acclimatization. The ANLs and SPIN scores are unrelated. Although the SPIN scores improve with amplification, the ANLs are unaffected by amplification, suggesting that the ANL is inherent to an individual and can be established prior to hearing aid fitting as a possible predictor of hearing-aid use.

Adaptation, Physiological↗

Connexin-associated deafness and speech perception outcome of cochlear implantation.

OBJECTIVE: To compare performance after cochlear implantation in children with mutations in connexin (Cx) 26 (GJB2) or Cx30 (GJB6) and children with deafness of unknown etiology. DESIGN: Genetic analysis and speech perception evaluation was performed in the children with and without Cx mutations who had undergone cochlear implantation. Speech perception performance was retrospectively analyzed 6, 12, 24, 36, and 48 months after implantation. Test material was selected according to the child's age and cognitive and language abilities. SETTING: The study took place at speech and hearing and genetic centers of a hospital in the central part of Israel and the genetics departments of 3 additional centrally located hospitals. PATIENTS: A total of 30 children who had undergone cochlear implantation were selected for the study, with control patients matched according to age at implantation, duration of implant use, and mode of communication. There was no evidence for additional disabilities or handicaps in either group. MAIN OUTCOME MEASURES: Speech perception measurements included a questionnaire, as well as closed and open-set tests. RESULTS: Overall, the 2 groups showed significant improvement in speech perception results after implantation. Four years after implantation, both groups achieved mean open-set speech perception scores of approximately 60%, 75%, and 90% for monosyllabic, 2 syllables, and words in sentences tests, respectively. CONCLUSIONS: There were no apparent differences in speech perception performance after implantation between the children with Cx mutations and children with deafness of unknown etiology. These data have important implications as a prognostic indicator when counseling candidates for cochlear implantation.

Child↗

Effect of release time in compression hearing aids: paired-comparison judgments of quality.

Paired-comparison judgments of quality were obtained from 20 hearing-impaired listeners for speech processed through simulated compression hearing aids varying in release time (60, 200, 1000 ms) at three different compression ratios (1.5, 2, 3:1) and for three different background noises (ventilation, apartment, cafeteria). Analysis revealed that the main effect of release time did not have a significant effect on perceived quality. The interaction between release time and noise type was found to be significant. While no significant difference in preference for release times was evident for the ventilation noise, the longer release times (200 and 1000 ms) were preferred for the higher level noises (apartment noise, cafeteria noise). Post hoc testing revealed that the mean preference scores for the 200- and 1000-ms release time were significantly greater than that of the 60-ms release time with the competing cafeteria noise (p < 0.05). Analysis of individual subject data revealed statistically significant preferences that differed from the group mean, suggesting that individualized fitting of this parameter of a compression hearing aid might be warranted.

Adolescent↗

Effects of three amplification strategies on speech perception by children with severe and profound hearing loss.

OBJECTIVE: Traditionally in the United Kingdom, children with severe and profound hearing loss have been fitted with linear, analog hearing aids. Fast-acting, wide-dynamic-range compression (WDRC) has been shown to give better discrimination of speech than linear amplification for moderately hearing-impaired young adults. For severe and profound hearing losses, higher compression ratios are needed. The resultant distortion of the temporal envelope and reduced modulation depth may offset improvements in audibility offered by WDRC. In this study, speech recognition and discrimination were assessed for severely and profoundly hearing-impaired children, using three different amplification strategies, including WDRC. DESIGN: Fifteen children (ages 7 to 15 yr) with severe and profound hearing loss were fitted bilaterally with high-power, multichannel compression hearing aids, incorporating one of three different amplification strategies: linear with peak clipping, linear with compression limiting, or WDRC. Output responses were matched to Desired Sensation Level (DSL i/o) targets. The children wore hearing aids programmed with each of the amplification strategies in turn, for at least 1 wk, in a counterbalanced order across children. After using a particular amplification strategy for at least 1 wk, speech perception tests were carried out. RESULTS: Speech scores on closed-set testing for the profound group showed significant benefit for WDRC over the other two algorithms. None of the other results showed a statistically significant effect of algorithm on speech performance. CONCLUSIONS: WDRC amplification sometimes led to better performance than linear amplification with peak clipping or output limiting, and it never led to poorer performance. Therefore, it appears to be safe to use well-designed WDRC for hearing-impaired children with severe or profound hearing loss.

Adolescent↗

Evaluation of the list of numerals in the Danish speech audiometry material. Dantale.

The psychometric function for the list of numerals in the Danish 'Dantale' speech audiometry material was obtained by presenting the lists to 30 normally hearing subjects at eight different sound pressure levels. Six of the subjects participated in a test of a possible learning effect. The psychometric function is presented, showing that 50% speech recognition is reached at a mean of 15 dB SPL, and the slope of the function around the 50% value is 8.3% per dB. It was found that in no more than 7% of the cases was each numeral on the list confused with another numeral. A strong correlation between the recorded RMS value and the SRT for the individual numeral was found. The short-term learning effect was found to be negligible.

Adolescent↗

Asymmetric suprathreshold speech recognition and the telephone ear phenomenon.

We compared interaural suprathreshold speech recognition scores in 62 adults with sensorineural hearing loss. Subjects were tested at two sites, 25 at the Baylor College of Medicine, Houston, TX and 37 at Brooklyn College, Brooklyn, NY. Ears were categorized according to telephone usage. Results showed, at both sites, a small but significant difference in speech understanding scores between the ear habitually used on the telephone and the opposite, nontelephone ear. The average speech recognition score was approximately 5 percent better on the telephone ear. Results are interpreted in relation to the theories of auditory deprivation and auditory acclimatization.

Adult↗

Investigation of binaural interference in normal-hearing and hearing-impaired adults.

When speech recognition testing is performed under diotic conditions, some elderly persons with asymmetric hearing loss exhibit a phenomenon in which the performance of the poorer ear interferes with that of the better ear. This binaural interference phenomenon has been estimated to occur in 8 to 10 percent of elderly hearing aid users. The purpose of the present study was to investigate the prevalence of this phenomenon in groups (n = 12) of young and elderly listeners with normal hearing, plus groups of elderly listeners with hearing loss who were aided or unaided. Of 48 subjects tested, only 2 exhibited significant evidence of binaural interference, a result that is close to that expected by chance. Although both of these subjects were elderly, one had normal hearing and the other was aided binaurally. A third elderly unaided subject exhibited a significant binaural advantage. Further studies are needed to determine the prevalence of binaural interference in normal-hearing or hearing-impaired listeners in any decade of life.

Adult↗

Evaluation of a portable two-microphone adaptive beamforming speech processor with cochlear implant patients.

A two-microphone noise reduction technique was tested with four cochlear implant patients. The noise reduction technique, known as adaptive beamforming (ABF), used signals from only two microphones--one behind each ear--to attenuate sounds not arriving from the direction directly in front of the patient. The algorithm was implemented in a portable digital signal processor, and was compared with a strategy in which the two microphone signals were simply added together (two-microphone broadside strategy). Tests with the four patients were conducted in a soundproof booth with target speech arriving from in front of the patient and multitalker babble noise arriving at 90 deg to the left. Results at 0-dB signal-to-noise level (S/N) showed large improvements in speech intelligibility for all patients, when compared to the two-microphone broadside strategy. Precautions were taken to avoid cancellation of the target speech, and, accordingly, subjective tests showed no deterioration in performance for the adaptive beamformer in quiet. Physical measurement of the directional characteristics of the ABF was made with the microphones placed behind the ears of a KEMAR manikin and in the same acoustic environment as used with the patients. Results showed directional gain of approximately 10 dB when the angle of incidence for interfering noise was shifted more than 20 to 30 deg from directly in front of or behind the manikin.(ABSTRACT TRUNCATED AT 250 WORDS)

Amplifiers, Electronic↗

Cochlear implantation in a patient with neurofibromatosis type 1 and profound hearing loss: evidence to support a cochlear site of lesion.

OBJECTIVE: To determine the efficacy of cochlear implantation in an individual with neurofibromatosis type 1 and profound hearing loss and to determine, to the extent possible in a living subject, the site of lesion of the hearing loss in an individual with neurofibromatosis type 1. STUDY DESIGN: Postoperative assessment of an adult male with neurofibromatosis type 1. SETTING: The study was completed in the Departments of Otolaryngology and Audiology, University of Massachusetts Medical Center, Massachusetts. PATIENTS: One adult male with neurofibromatosis type 1 and bilateral, chiefly profound, sensorineural hearing loss. INTERVENTIONS: The subject underwent cochlear implantation as treatment of bilateral, chiefly profound sensorineural hearing loss. MAIN OUTCOME MEASURES: Outcome measures include results of electrical auditory brainstem response testing, electrical compound action potential measures, and speech recognition scores. RESULTS: Results of testing revealed expected latencies of waves II, III, and V of the electrical auditory brainstem response, measurable compound action potentials across virtually the entire length of the electrode array, and high levels of open-set, auditory-only speech understanding. CONCLUSIONS: This is the first case report describing the outcome of cochlear implantation in a patient with neurofibromatosis type 1. Composite test results support the absence of VIIIth nerve degeneration or other anomalies, indicating a cochlear site of lesion for the hearing loss in this patient. Based on the results of this investigation, standard cochlear implantation should be considered a viable option and the first line of therapy for the treatment of bilateral, severe to profound sensorineural hearing loss in patients with neurofibromatosis type 1 and normal, VIIIth nerve radiographic findings.

Adult↗

Case of recurrent, reversible, sudden sensorineural hearing loss in a child.

This paper describes audiologic, electrophysiologic, and medical test results for a now 10-year-old girl who has had 45 episodes of reversible, sudden sensorineural hearing loss over the last 8 years. Episodes have lasted from 6 to 72 hours and often have been accompanied by a mild illness. Acoustic immittance measures have been consistent with normal middle-ear function with the exception of absent ipsilateral and contralateral acoustic reflexes. Mechanically evoked perioral reflex activity was markedly asymmetric following lower lip stimulation. The asymmetry of R1 activation between right and left side lower lip inputs raises questions about the integrity of central connections within the brain stem, including internuncial pathways coursing between trigeminal sensory relay nuclei and the facial motor nucleus. An electrocochleographic evaluation revealed cochlear microphonic but absent or markedly abnormal whole nerve action potentials. Auditory brainstem responses (ABR) have been either absent or poorly formed and significantly delayed, regardless of hearing sensitivity. Middle and late auditory evoked potentials were essentially normal. Both transient-evoked and distortion-product otoacoustic emissions were present regardless of peripheral auditory sensitivity. All medical tests have been essentially normal. Although no definitive diagnosis has been reached, beta blockers have been used with some success. Taken together, these data document a very unusual case of fluctuating hearing loss. The electrocochleographic and otoacoustic emission data suggest that the outer hair cells are functioning normally and that the loss is not cochlear in origin.(ABSTRACT TRUNCATED AT 250 WORDS)

Audiometry, Evoked Response↗

Previous experience as a confounding factor in comparing cochlear-implant processing schemes.

It is of great importance to compare the relative merits of different cochlear-implant speech-processing strategies. Some groups have compared different strategies within single subjects, but usually the subject has prior experience with one strategy, and no allowance is made for this prior experience. We show in the present study that this is inappropriate. We tested one subject using the Melbourne (Cochlear Corp.) multichannel implant with the device set to process sounds in two different ways. In the first processing scheme, the device functioned normally, extracting information about voicing frequency, amplitude and second-formant frequency. This information activated the 21-channel device, determining pulse rate, pulse amplitude and electrode position (respectively). In the second processing scheme, a single electrode (with the largest dynamic range) was activated. This electrode coded overall amplitude and voicing frequency. The subject was tested on an audiovisual test of a 14-choice consonant recognition in the form /iCi/ over a period of over 4 months. During this time the subject used the 21-channel processor outside of the laboratory. Upon initial connection, there was little difference between the results obtained with the two schemes when tested in sound alone or in sound plus vision. However, after about 4 months, scores obtained with the 21-channel processor in sound plus vision were superior to the scores obtained with the one channel. This advantage came from a superiority in the features of voicing and nasality, but not place. Scores for sound-alone conditions between the two processing schemes remained similar for the 4-month period.(ABSTRACT TRUNCATED AT 250 WORDS)

Auditory Threshold↗

Temporally jittered speech produces performance intensity, phonetically balanced rollover in young normal-hearing listeners.

This study investigates whether temporally jittered stimuli will produce performance-intensity, phonetically balanced (PI-PB) rollover in young adults with normal hearing. Although not yet explicitly stated in the literature, there is clinical and theoretical evidence to suggest that PI-PB rollover, such as that found in cases of acoustic neuroma, is caused by neural dyssynchrony in the auditory system. Sixteen participants were tested with intact and temporally jittered word lists in quiet at 40, 55, 65, and uncomfortable listening level -5 dB HL. The results show significant rollover in the jittered but not the intact conditions. The results are consistent with the existing evidence that suggests that neural PI-PB rollover is caused by decreased neural synchrony and support the claim that temporal jitter simulates neural dyssynchrony. Furthermore, these results are consistent with the hypothesis that synchrony coding plays an important role in the perception of high-level speech.

Adolescent↗

Conservation of residual hearing with cochlear implantation.

HYPOTHESIS: The goals of this work were (a) to review the pre- and postsurgical auditory thresholds in a consecutive sample of cochlear implant recipients to determine the fate of residual hearing, and (b) to obtain preliminary indication of the value of a multicenter longitudinal study of residual hearing in implant patients. BACKGROUND: Indications for cochlear implantation have been expanded to include severely hearing impaired (SHI) adults and may someday include SHI children. Implantation of individuals with more residual hearing is a concern owing to the possible development of better devices that may make use of residual hearing within the lifetime of implanted children if not that of adults. Preservation of residual hearing would be a desirable outcome of implant surgery; however, conventional thought is that implantation destroys any remaining hearing. This study was undertaken to assess if and how often conservation of hearing occurred after implantation in a sample of multichannel implant recipients. METHODS: Records of 50 profoundly hearing impaired consecutively implanted patients were examined for pre- and post-surgical audiometric results. Standard audiometric techniques were used for all testing procedures. Forty patients were considered to have some hearing conserved based on a response obtained at any one of the three speech frequencies prior to implantation. The most recent postimplantation audiometric results were used providing data from users with 1-41 months of use. RESULTS: Twenty-one of 40 implanted subjects were found to have responses in at least one of the speech frequencies both pre- and postsurgery, with the majority of those displaying responses at all three frequencies. In this preliminary retrospective study, it did not appear that duration of cochlear implant use, gender, level of preoperative hearing, or length of electrode insertion were related to outcome. There were insufficient data to draw conclusions on individual devices. CONCLUSIONS: Conservation of hearing occurred in approximately half of the subjects reviewed. There is no indication of what factors contributed to the preservation of hearing in those with postsurgical residual hearing or if that hearing is usable. The study does suggest that a larger multicenter longitudinal study would be of value of determine what factors may be related to conserved hearing in implanted patients.

Adult↗