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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↗

Laterality in visual speech perception.

The lateralization of visual speech perception was examined in 3 experiments. Participants were presented with a realistic computer-animated face articulating 1 of 4 consonant-vowel syllables without sound. The face appeared at 1 of 5 locations in the visual field. The participants' task was to identify each test syllable. To prevent eye movement during the presentation of the face, participants had to carry out a fixation task simultaneously with the speechreading task. In one study, an eccentricity effect was found along with a small but significant difference in favor of the right visual field (left hemisphere). The same results were found with the face articulating nonlinguistic mouth movements (e.g., kiss). These results suggest that the left-hemisphere advantage is based on the processing of dynamic visual information rather than on the extraction of linguistic significance from facial movements.

Adolescent↗

Changes in educational placement and speech perception ability after cochlear implantation in children.

OBJECTIVE: The purpose of this study was to evaluate the effect and relationship of paediatric cochlear implantation on educational placement and speech perception ability and to determine the effect of a multilingual background on educational placement and speech perception ability after cochlear implantation. DESIGN: This study consisted of a retrospective chart review of 83 implanted patients and a questionnaire sent to parents of 80 implanted children. SETTING: This study was conducted in a cochlear implant program at a tertiary care centre. METHODS AND OUTCOME MEASURES: From the chart review, the educational placements and speech perception scores of implanted children were determined before and after implantation. In the questionnaire, parents were asked about their preferred educational placement for their child, the language spoken at home, and their impressions of the chosen school program for their child. RESULTS: Of the children who were in nonmainstream school programs at implantation (n = 30), 50% moved toward mainstream with 9 (30%) reaching mainstream placement. Of the children who were preschool at implantation (n = 34), 24 (70%) were placed or planned to be placed in mainstream after implantation. The rate of improvement in speech perception ability was significantly higher in those children who moved toward or remained in mainstream than those who stayed at the same nonmainstream educational placement or moved away from mainstream. Children from a multilingual background were able to achieve similar educational placements and similar rates of progress of speech perception outcome as the only English-speaking children. CONCLUSIONS: Children with cochlear implants have increased educational opportunities, with those children in mainstream and those who have moved toward mainstream demonstrating improved progress in speech perception ability.

Child↗

Improvements in speech perception by children with profound prelingual hearing loss: effects of device, communication mode, and chronological age.

The present investigation expanded on an earlier study by Miyamoto, Osberger, Todd, Robbins, Karasek, et al. (1994) who compared the speech perception skills of two groups of children with profound prelingual hearing loss. The first group had received the Nucleus multichannel cochlear implant and was tested longitudinally. The second group, who were not implanted and used conventional hearing aids, was tested at a single point in time. In the present study, speech perception scores were examined over time for both groups of children as a function of communication mode of the child. Separate linear regressions of speech perception scores as a function of age were computed to estimate the rate of improvement in speech perception abilities that might be expected due to maturation for the hearing aid users (n=58) within each communication mode. The resulting regression lines were used to compare the estimated rate of speech perception growth for each hearing aid group to the observed gains in speech perception made by the children with multichannel cochlear implants. A large number of children using cochlear implants (n=74) were tested over a long period of implant use (m=3.5 years) that ranged from zero to 8.5 years. In general, speech perception scores for the children using cochlear implants were higher than those predicted for a group of children with 101-110 dB HL of hearing loss using hearing aids, and they approached the scores predicted for a group of children with 90-100 dB HL of hearing loss using hearing aids.

Age Factors↗

Neurophysiology of cochlear implant users II: comparison among speech perception, dynamic range, and physiological measures.

OBJECTIVE: The overall objective of this study was to relate electrically evoked potentials recorded from different levels of the auditory pathway with behavioral measures obtained from adult cochlear implant subjects. The hypothesis was that adult recipients of cochlear implants who have open-set speech perception and those recipients with no open-set speech perception would differ in their neurophysiologic responses recorded at one or more levels of the auditory pathway. DESIGN: The subjects were 11 adults implanted with the Clarion cochlear implant. The electrical auditory brainstem response (EABR, Wave V), electrical auditory middle latency response (EAMLR, Na-Pa complex), and the electrical late auditory response (ELAR, N1-P2 complex), were recorded from three intra-cochlear electrodes. The stimuli used to record the evoked potentials varied in rate and amplitude. Behavioral measures (between threshold and upper limit of comfortable loudness) were used to define the subject's dynamic range at the different stimulus rates. Word and sentence recognition tests evaluated subjects' speech perception in quiet and noise. Evoked potential and behavioral measures were examined for statistical significance using analysis of variance for repeated measures and correlational analyses. RESULTS: Subjects without open-set speech recognition demonstrated 1) poorly formed or absent evoked potential responses, 2) reduced behavioral dynamic ranges, 3) lack of change in the size of the dynamic range with a change in stimulus rate, and 4) longer periods of auditory deprivation. The variables that differentiated the best performers included 1) presence of responses at all three levels of the auditory pathway, with large normalized amplitudes for the EAMLR, 2) lower evoked potential thresholds for the Na-Pa complex, 3) relatively large dynamic ranges, and 4) changes in the size of the dynamic range with changes in stimulus rate. CONCLUSIONS: In this study, the inability to follow changes in the temporal characteristics of the stimulus was associated with poor speech perception performance. Results also illustrate that variability in speech perception scores of cochlear implant recipients relates to neurophysiologic responses at higher cortical levels of the auditory pathway. Presumably, limited neural synchrony for elicitation of electrophysiologic responses underlies limited speech perception. Results confirm that neural encoding with electrical stimulation must provide sufficient physiologic responses of the central nervous system to perceive speech through a cochlear implant.

Adult↗

Speech perception, lexicality, and reading skill.

This study examined the interaction between speech perception and lexical information among a group of 7-year-old children, of which 26 were poor readers and 36 were good readers. The children's performance was examined on tasks assessing reading skill, phonological awareness, pseudoword repetition, and phoneme identification. Although good readers showed clearly defined categorical perception in the phoneme identification task for both the /bif/-/pif/ and the /bis/-/pis/ continua, the category boundary for /bif/-/pif/ was at longer VOTs than the boundary for /bis/-/pis/, which characterizes the classic lexicality effect. Poor readers showed less sharply defined categorical perception on both continua. Although poor readers did not show the classic lexicality effect, lexicality did affect the overall rate with which phonemes were identified as /b/ or /p/ at each VOT. These findings suggest that the lexicon may operate as a compensatory mechanism for resolving ambiguities in speech perception. Furthermore, statistical correction for group differences in phoneme identification made group differences in phoneme deletion disappear, suggesting that deficits in speech perception may play a causal role in the phonological core deficit associated with reading failure.

Awareness↗

Audiovisual gating and the time course of speech perception.

The time course of audiovisual information in speech perception was examined using a gating paradigm. VCVs that evoked the McGurk effect were gated visually and auditorily. The visual gating yielded a McGurk effect that increased in strength as a linear function of amount of visual stimulus presented. The acoustic gating revealed a more nonlinear function in which the VC information was considerably weaker than the CV portion of the VCV. The results suggest that the flow of cross-modal information in quite complex during audiovisual speech perception.

Humans↗

Use of audiovisual information in speech perception by prelingually deaf children with cochlear implants: a first report.

OBJECTIVE: Although there has been a great deal of recent empirical work and new theoretical interest in audiovisual speech perception in both normal-hearing and hearing-impaired adults, relatively little is known about the development of these abilities and skills in deaf children with cochlear implants. This study examined how prelingually deafened children combine visual information available in the talker's face with auditory speech cues provided by their cochlear implants to enhance spoken language comprehension. DESIGN: Twenty-seven hearing-impaired children who use cochlear implants identified spoken sentences presented under auditory-alone and audiovisual conditions. Five additional measures of spoken word recognition performance were used to assess auditory-alone speech perception skills. A measure of speech intelligibility was also obtained to assess the speech production abilities of these children. RESULTS: A measure of audiovisual gain, "Ra," was computed using sentence recognition scores in auditory-alone and audiovisual conditions. Another measure of audiovisual gain, "Rv," was computed using scores in visual-alone and audiovisual conditions. The results indicated that children who were better at recognizing isolated spoken words through listening alone were also better at combining the complementary sensory information about speech articulation available under audiovisual stimulation. In addition, we found that children who received more benefit from audiovisual presentation also produced more intelligible speech, suggesting a close link between speech perception and production and a common underlying linguistic basis for audiovisual enhancement effects. Finally, an examination of the distribution of children enrolled in Oral Communication (OC) and Total Communication (TC) indicated that OC children tended to score higher on measures of audiovisual gain, spoken word recognition, and speech intelligibility. CONCLUSIONS: The relationships observed between auditory-alone speech perception, audiovisual benefit, and speech intelligibility indicate that these abilities are not based on independent language skills, but instead reflect a common source of linguistic knowledge, used in both perception and production, that is based on the dynamic, articulatory motions of the vocal tract. The effects of communication mode demonstrate the important contribution of early sensory experience to perceptual development, specifically, language acquisition and the use of phonological processing skills. Intervention and treatment programs that aim to increase receptive and productive spoken language skills, therefore, may wish to emphasize the inherent cross-correlations that exist between auditory and visual sources of information in speech perception.

Acoustic Stimulation↗

Speech perception deficit in dyslexic adults as measured by mismatch negativity (MMN).

Deficits in phonological processing are known to play a major role in the aetiology of dyslexia, and speech perception is a prerequisite condition for phonological processing. Significant group differences between dyslexics and controls have been found in the categorical perception of synthetic speech stimuli. In a previous work, we have demonstrated that these group differences are already present at an early pre-attentive stage of signal processing in dyslexic children: the late component of the MMN elicited by passive speech perception was attenuated in comparison to a control group. In this study, 12 dyslexic adults and 13 controls were assessed using a passive oddball paradigm. Mismatch negativity (MMN) was determined for both tone and speech stimuli. The tone stimuli yielded two MMN components, but no group differences. Three components were found for the speech stimuli. Multivariate testing for group differences yielded a significant result, and univariate P values revealed significant differences between dyslexics and controls in two of the three time windows. This suggests that speech perception as measured on an early, pre-attentive level plays a major role in dyslexia not only in children (as shown in our previous study) but also in adults.

Acoustic Stimulation↗

Separating contributions of hearing, lexical knowledge, and speech production to speech-perception scores in children with hearing impairments.

Open-set word and sentence speech-perception test scores are commonly used as a measure of hearing abilities in children and adults using cochlear implants and/or hearing aids. These tests are usually presented auditorily with a verbal response. In the case of children, scores are typically lower and more variable than for adults with hearing impairments using similar devices. It is difficult to interpret children's speech-perception scores without considering the effects of lexical knowledge and speech-production abilities on their responses. This study postulated a simple mathematical model to describe the effects of hearing, lexical knowledge, and speech production on the perception test scores for monosyllabic words by children with impaired hearing. Thirty-three primary-school children with impaired hearing, fitted with hearing aids and/or cochlear implants, were evaluated using speech-perception, reading-aloud, speech-production, and language measures. These various measures were incorporated in the mathematical model, which revealed that performance in an open-set word-perception test in the auditory-alone mode is strongly dependent on residual hearing levels, lexical knowledge, and speech-production abilities. Further applications of the model provided an estimate of the effect of each component on the overall speech-perception score for each child.

Adolescent↗

Speech perception in noise with BICROS hearing aids.

The speech perception in noise abilities of 14 asymmetrically hearing-impaired subjects who had been fitted with Bilateral Contralateral routing of Signal (BICROS) hearing aids was evaluated. Speech tests were administered with subjects wearing both conventional monaural amplification and their own BICROS aids. Test procedures involved the use of sentence lists from the Speech Perception in Noise (SPIN) test and the Synthetic Sentence Identification (SSI) test, presented in a background of recorded four-talker babble. The performance of the subjects was found to be significantly better with the BICROS aids than with monaural amplification alone.

Acoustic Stimulation↗

Cortical bases of speech perception: evidence from functional lesion studies.

Functional lesion studies have yielded new information about the cortical organization of speech perception in the human brain. We will review a number of recent findings, focusing on studies of speech perception that use the techniques of electrocortical mapping by cortical stimulation and hemispheric anesthetization by intracarotid amobarbital. Implications for recent developments in neuroimaging studies of speech perception will be discussed. This discussion will provide the framework for a developing model of the cortical circuitry critical for speech perception.

Amobarbital↗

Effects of insertion depth of cochlear implant electrodes upon speech perception.

OBJECTIVE: To investigate whether the insertion depth of a cochlear implant array affects postoperative speech perception. DESIGN: The subjects were 48 postlingually deaf adults who received either the Nucleus 22 or the Nucleus 24 cochlear implant with a straight array. A postoperative radiograph of the cochlear electrode was used to estimate insertion depth, as either the angle of the electrode tip (angle) or the intracochlear length of the electrode (length). Other estimates of insertion depth included the numbers of active electrodes and channels used by the speech processor. Electrode depth, together with the duration of deafness, hearing aid usage, pre-operative speech perception score and pre-operative pure-tone averages were independent variables in a forward stepwise multiple regression analysis, where the dependent variables were postoperative CNC words and CNC phonemes. RESULTS: Duration of deafness and insertion depth (angle, insertion length or active electrodes) were the predictive variables for CNC words or CNC phonemes. Angle was the best 'depth-related' predictor of postoperative speech perception. An even clearer relationship was found between CUNY sentences in noise and angle, in a subset of 26 patients. CONCLUSION: Depth of electrode insertion affects postoperative speech perception.

Adult↗

The effect of intensity on pitch in electric hearing and its relationship to the speech perception performance of cochlear implantees.

This study investigated the effect of intensity on pitch in electric hearing and its relationship to the speech perception ability of cochlear implantees. Subjects were 13 adult users of the Nucleus 22 cochlear implant system, using either the Spectra22 or ESPrit22 speech processor and the SPEAK speech processing strategy. A multidimensional scaling technique was employed. Speech perception was measured using sentences and vowels. All measurements were performed in a soundfield condition, and subjects wore their own speech processors with their normally used settings. Results showed a significant correlation between the degree of deviation of the subjects' stimulus spaces from the "ideal" space and subjects' performance with the sentences, but not with the vowels. A significant correlation was found between subjects' response variability in performing the multidimensional scaling task and their speech perception measures, suggesting that spectral smearing or underlying cognitive abilities might affect implantees' speech perception performance.

Acoustic Stimulation↗

Speech perception outcomes in older children who use multichannel cochlear implants: older is not always poorer.

Speech perception outcomes for early-deafened children who undergo implantation as teenagers or young adults are generally reported to be poorer than results for young children. It is important to provide appropriate expectations when counseling adolescents and their families to help them make an informed choice regarding cochlear implant surgery. The considerable variation of results in this group makes this process more difficult. This study considered a number of factors in a group of 25 children who underwent implantation in Melbourne between the ages of 8 and 18 years. Each subject completed open-set speech perception testing with Bamford-Kowal-Bench sentences before and after implantation and preoperative language testing with the Peabody Picture Vocabulary Test. Data were collected regarding the type of hearing loss, age at implantation, age at hearing aid fitting, audiometric details, and preoperative and postoperative communication mode. Results were submitted to a stepwise multiple linear regression analysis with postoperative open-set sentence scores as the dependent variables. The analysis suggested that 3 factors have a significant predictive value for speech perception after implantation: preoperative open-set sentence score, duration of profound hearing loss, and equivalent language age. These 3 factors accounted for 66% of the variance in this group. The results of this study suggest that children who have useful speech perception before implantation, and higher age-equivalent scores on language measures, would be expected to do well with a cochlear implant. Consistent with other studies, a shorter duration of profound hearing loss is also advantageous. The mean sentence score for this group, 47%, was not significantly different from the mean result across all children in the Melbourne program.

Adolescent↗

Short electrode insertion in cochlear implantation: speech perception performance of Cantonese-speaking subjects.

CONCLUSIONS: Subjects with cochlear ossification derived benefits in terms of speech performance similar to those of the non-ossified group. It is thus recommended that the insertion of short electrode arrays should be considered an alternative choice for patients with cochlear ossification. OBJECTIVE: Cochlear ossification has been recognized as a major obstacle to the full insertion of a multichannel cochlear implant electrode array. To alleviate the technical difficulty of placing a standard electrode array and the possibility of causing undesirable trauma to the cochlea, a newly designed electrode array with the same number of electrodes compressed into a shorter length has been made available. The aim of the present study was to examine the speech perception performance of patients implanted with the MED-EL C40+S compressed electrode array and to compare their results with those of matched groups implanted with the MED-EL C40+standard electrode array. MATERIAL AND METHODS: One pre-lingually and two post-lingually deaf subjects using short electrode arrays were matched with three groups of subjects using standard electrode arrays. The pre- and postoperative speech perception scores were evaluated. RESULTS: All three subjects using compressed electrode arrays achieved speech perception scores comparable to those of matched subjects using standard electrode arrays. There was also a tendency for the subjects to show similar patterns of speech perception scores as a function of the difficulty of the tests.

Adult↗

Relationships among speech perception, production, language, hearing loss, and age in children with impaired hearing.

Eighty-seven primary-school children with impaired hearing were evaluated using speech perception, production, and language measures over a 3-year period. Forty-seven children with a mean unaided pure-tone-average hearing loss of 106 dB HL used a 22-electrode cochlear implant, and 40 with a mean unaided pure-tone-average hearing loss of 78 dB HL were fitted with hearing aids. All children were enrolled in oral/aural habilitation programs, and most attended integrated classes with normally hearing children for part of the time at school. Multiple linear regression was used to describe the relationships among the speech perception, production, and language measures, and the trends over time. Little difference in the level of performance and trends was found for the two groups of children, so the perceptual effect of the implant is equivalent, on average, to an improvement of about 28 dB in hearing thresholds. Scores on the Peabody Picture Vocabulary Test (PPVT) and the Clinical Evaluation of Language Fundamentals showed an upward trend at about 60% of the rate for normally hearing children. Rates of improvement for individual children were not correlated significantly with degree of hearing loss. The children showed a wide scatter about the average speech production score of 40% of words correctly produced in spontaneous conversations, with no significant upward trend with age. Scores on the open-set Consonant-Nucleus-Consonant (CNC) monosyllabic word test and the Bench-Kowal-Bamford (BKB) sentence test were strongly related to language level (as measured by an equivalent age on the PPVT) and speech production scores for both auditory-visual and auditory test conditions. After allowing for differences in language, speech perception scores in the auditory test condition showed a slight downward trend over time, which is consistent with the known biological effects of hearing loss on the auditory periphery and brainstem. Speech perception scores in the auditory condition also decreased significantly by about 5% for every 10 dB of hearing loss in the hearing aid group. The regression analysis model allows separation of the effects of language, speech production, and hearing levels on speech perception scores so that the effects of habilitation and training in these areas can be observed and/or predicted. The model suggests that most of the children in the study will reach a level of over 90% sentence recognition in the auditory-visual condition when their language becomes equivalent to that of a normally hearing 7-year-old, but they will enter secondary school at age 12 with an average language delay of about 4 or 5 years unless they receive concentrated and effective language training.

Age Factors↗

Atypical cortical responses underlie poor speech perception in children using cochlear implants.

Variability in speech perception abilities after years of cochlear implant use could reflect differences in central auditory processing of the electrical input provided. Cortical responses were measured in 23 experienced pediatric cochlear implant users who were 12.3+/-3.1 years of age at testing and had used their implants for 6.0+/-2.9 years. All had prelingual onset of deafness. An observer identified blind three types of cortical waveforms ranging from those similar to previous reports to more atypical responses. Children displaying atypical types of responses were implanted at a wide range of ages and had significantly poorer behavioral speech perception scores (P<0.05) than their peers with expected waveforms. Results suggest a persistent immaturity and/or abnormal organization in the auditory cortex in some children.

Age Factors↗