Postimplantation Speech Perception Research Articles

Speech Perception Performance in Cochlear Implant Recipients Correlates to the Number and Synchrony of Excited Auditory Nerve Fibers Derived From Electrically Evoked Compound Action Potentials.

Many studies have assessed the performance of individuals with cochlear implants (CIs) with electrically evoked compound action potentials (eCAPs). These eCAP-based studies have focused on the amplitude information of the response, without considering the temporal firing properties of the excited auditory nerve fibers (ANFs), such as neural latency and synchrony. These temporal features have been associated with neural health in animal studies and, consequently, could be of importance to clinical CI outcomes. With a deconvolution method, combined with a unitary response, the eCAP can be mathematically unraveled into the compound discharge latency distribution (CDLD). The CDLD reflects both the number and the temporal firing properties of excited ANFs. The present study aimed to determine to what extent the CDLD derived from intraoperatively recorded eCAPs is related to speech perception in individuals with CIs. This retrospective study acquired data on monosyllabic word recognition scores and intraoperative eCAP amplitude growth functions from 124 adult patients with postlingual deafness that received the Advanced Bionics HiRes 90K device. The CDLD was determined for each recorded eCAP waveform by deconvolution. Each of the two Gaussian components of the CDLD was described by three parameters: the amplitude, the firing latency (the average latency of each component of the CDLD), and the variance of the CDLD components (an indication of the synchronicity of excited ANFs). Apart from these six CDLD parameters, the area under the CDLD curve (AUCD) and the slope of the AUCD growth function were determined as well. The AUCD was indicative of the total number of excited ANFs over time. The slope of the AUCD growth function indicated the increases in the number of excited ANFs with stimulus level. Associations between speech perception and each of these eight CDLD-related parameters were investigated with linear mixed modeling. In individuals with CIs, larger amplitudes of the two CDLD components, greater AUCD, and steeper slopes of the AUCD growth function were all significantly associated with better speech perception. In addition, a smaller latency variance in the early CDLD component, but not in the late, was significantly associated with better speech recognition scores. Speech recognition was not significantly dependent on CDLD latencies. The AUCD and the slope of the AUCD growth function provided a similar explanation of the variance in speech perception (R 2 ) as the eCAP amplitude, the slope of the amplitude growth function, the amplitude, and variance of the first CDLD component. The results demonstrate that both the number and the neural synchrony of excited ANFs, as revealed by CDLDs, are indicative of postimplantation speech perception in individuals with a CI. Because the CDLD-based parameters yielded a higher significance than the eCAP amplitude or the AGF slope, the authors conclude that CDLDs can serve as a clinical predictor of the survival of ANFs and that they have predictive value for postoperative speech perception performance. Thus, it would be worthwhile to incorporate the CDLD into eCAP measures in future clinical applications.

Does Auditory Environment Predict Speech Perception Outcomes in Elderly Cochlear Implant Patients?

Introduction: Cochlear implantation (CI) is a reliable and safe means by which sensorineural hearing loss can be ameliorated in the elderly population. However, a high degree of variation exists in postimplantation hearing outcomes for which some modifiable factors of the daily natural auditory environment may be contributory. In this study, we analyze the relationship between cochlear implant patient age, natural auditory environment, and postimplantation speech perception among older adults. Methods: Data log from automatic environment classification enabled sound processors of postlingually deafened CI recipients ≥50 years old (n = 115) were obtained retrospectively and analyzed for time spent (hours per day) in listening environment and loudness (SPL dB). Speech perception testing was assessed in a subset of patients (n = 27) using open-set word recognition in quiet Consonant-Nucleus-Consonant in the short and intermediate postoperative period. Results: The mean subject age was 70 years (range, 53–99 years). Average daily implant use was 10.8 h and was not significantly correlated with age (p = 0.23, Spearman’s rho). Age was positively correlated with the percentage of hours spent at <40 and 40–50 dB and negatively correlated to proportional CI use at higher volume (60–70, 70–80, and >80 dB; r_s = 0.21, 0.20, −0.20, −0.35, −0.43; p = 0.021, 0.036, 0.033, <0.001, <0.001, respectively). Age was positively correlated with CI use in the quiet scene (r_s = 0.26, p = 0.006) and negatively correlated with scenes containing speech and noise (r_s = −0.19, −0.25; p = 0.046, 0.007). Total hours of device use and time spent at <40, 40–50 dB, and quiet environments were significantly correlated with improved CNC word scores (r_s = 0.48, 0.48, 0.51; p = 0.01, 0.01, <0.01, Spearman’s rho). While all speech (speech in noise + speech) was not significantly correlated to improvements in speech perception, a medium effect size was observed (r_s = 0.37, p = 0.057). Discussion/Conclusion: This study supports a relationship between auditory environment and age, with older CI recipients spending a greater proportion of time in quiet. Older CI users demonstrated greater improvements in speech perception with longer daily device use. Additional examination of the relationship between auditory environment and speech perception is necessary to conclusively guide future auditory rehabilitation efforts.

Preimplant Hearing Aid Fittings and Aided Audibility for Pediatric Cochlear Implant Recipients.

Audibility of speech for children with hearing loss (HL) depends on the degree of HL and the fitting of the hearing aids (HAs) themselves. Many studies on cochlear implant (CI) users have demonstrated that preimplant hearing is associated with postimplant outcomes, but there have been very few reports on the fitting of HAs before surgery. The aims of this study were to characterize HA fittings and aided audibility of speech for pediatric HA users with severe to profound HL and to examine the relation between preimplant aided audibility and postimplant speech perception. A descriptive/observational and correlational study. Audiologic records of pediatric CI participants involved in a larger study examining the effects of early acoustic hearing were analyzed retrospectively; when available, these records included HA verification and speech recognition performance. The CI participants were enrolled in audiology centers and oral schools for the deaf across the United States. To determine whether deviations from prescribed DSL target were significantly greater than zero, 95% confidence intervals of the mean deviation were calculated for each frequency (250, 500, 1000, 2000, and 4000 Hz). Correlational analyses were used to examine the relationship between preimplant aided Speech Intelligibility Indices (SIIs) and postimplant speech perception in noise. Correlational analyses were also used to explore the relationship between preimplant aided SIIs and demographic data. T-tests were used to compare preimplant-aided SIIs of HAs of listeners who later became users of either sequential CIs, simultaneous CIs, or bimodal devices. Preimplant fittings of HAs were generally very close to prescriptive targets, except at 4000 Hz for those HAs with active frequency-lowering processing, and preimplant SIIs, albeit low, were correlated with postimplant speech recognition performance in noise. These results suggest that aided audibility should be maximized throughout the HA trial for later speech recognition purposes. It is recommended that HA fittings be optimized to support speech audibility even when considering implantation. In addition to the age at which HA use begins, the aided audibility itself is important in determining CI candidacy and decisions regarding bimodal HA use.

Early Postimplant Speech Perception and Language Skills Predict Long-Term Language and Neurocognitive Outcomes Following Pediatric Cochlear Implantation.

We sought to determine whether speech perception and language skills measured early after cochlear implantation in children who are deaf, and early postimplant growth in speech perception and language skills, predict long-term speech perception, language, and neurocognitive outcomes. Thirty-six long-term users of cochlear implants, implanted at an average age of 3.4 years, completed measures of speech perception, language, and executive functioning an average of 14.4 years postimplantation. Speech perception and language skills measured in the 1st and 2nd years postimplantation and open-set word recognition measured in the 3rd and 4th years postimplantation were obtained from a research database in order to assess predictive relations with long-term outcomes. Speech perception and language skills at 6 and 18 months postimplantation were correlated with long-term outcomes for language, verbal working memory, and parent-reported executive functioning. Open-set word recognition was correlated with early speech perception and language skills and long-term speech perception and language outcomes. Hierarchical regressions showed that early speech perception and language skills at 6 months postimplantation and growth in these skills from 6 to 18 months both accounted for substantial variance in long-term outcomes for language and verbal working memory that was not explained by conventional demographic and hearing factors. Speech perception and language skills measured very early postimplantation, and early postimplant growth in speech perception and language, may be clinically relevant markers of long-term language and neurocognitive outcomes in users of cochlear implants. https://doi.org/10.23641/asha.5216200.

Factors Affecting Speech Perception Improvement Post Implantation in Congenitally Deaf Adults.

To identify factors preimplantation associated with postimplantation speech perception improvement in the adult congenitally deaf population. Forty-four adult cochlear implant (CI) patients who had a severe to profound hearing loss from birth were identified from this centre's database. Eight preimplantation factors, speech intelligibility, preimplantation hearing levels, communication mode, preimplantation speech perception scores, progression of hearing loss, age at implantation, hearing aid use preimplantation, and gender, were recorded during the CI assessment process. These factors were investigated to determine their effect on speech perception improvement postimplantation. The outcome measures were the improvement in scores for the BKB Sentence test and CUNY Sentence test with lipreading after implantation. In the final analysis, 26 patients were included in the CUNY analysis, and 30 patients were included in the BKB analysis. Speech intelligibility rating, preimplantation hearing levels, and communication mode were shown to be significantly associated with improvements in speech perception postimplantation. Three factors were identified that affected speech perception improvement postimplantation: speech intelligibility, preimplantation hearing levels, and communication mode. These factors can be used to counsel CI patients regarding potential speech perception improvements from cochlear implantation, although these are based on average data and may not reflect individual performance.

Intelligibility of the Patient's Speech Predicts the Likelihood of Cochlear Implant Success in Prelingually Deaf Adults.

The objective of this study was to determine the validity and clinical applicability of intelligibility of the patient's own speech, measured via a Vowel Identification Test (VOW), as a predictor of speech perception for prelingually deafened adults after 1 year of cochlear implant use. Specifically, the objective was to investigate the probability that a prelingually deaf patient, given a VOW score above (or below) a chosen cutoff point, reaches a postimplant speech perception score above (or below) a critical value. High predictive values for VOW could support preimplant counseling and implant candidacy decisions in individual patients. One hundred and fifty-two adult cochlear implant candidates with prelingual hearing impairment or deafness took part as speakers in a VOW; 149 speakers completed the test successfully. Recordings of the speech stimuli, consisting of nonsense words of the form [h]-V-[t], where V represents one of 15 vowels/diphthongs ([(Equation is included in full-text article.)]), were presented to two normal-hearing listeners. VOW score was expressed as the percentage of vowels identified correctly (averaged over the 2 listeners). Subsequently, the 149 participants enrolled in the cochlear implant selection procedure. Extremely poor speakers were excluded from implantation, as well as patients who did not meet regular selection criteria as developed for postlingually deafened patients. From the 149 participants, 92 were selected for implantation. For the implanted group, speech perception data were collected at 1-year postimplantation. Speech perception score at 1-year postimplantation (available for 77 of the 92 implanted participants) correlated positively with preimplant intelligibility of the patient's speech, as represented by VOW (r = 0.79, p < 0.00001): the more intelligible the patient's speech, the higher the predicted postimplant speech perception score. This correlation is explained by the hypothesis that the two variables have a common driving force, i.e., (in)adequacy of auditory speech input in the earliest years of life. With a 60% cutoff point, VOW can discriminate between individuals with "above-chance" postimplant speech perception and those with "chance level" postimplant speech perception with sensitivity and specificity of 0.84 and 0.86, respectively. The probability that a patient with a VOW score ≥ 60% achieves "above-chance" speech perception after implantation is 0.91. Conversely, the probability that a patient with VOW < 60% reaches "above-chance" speech perception is 0.25. For prelingually deaf adults, intelligibility of the patient's speech-as represented by VOW-is a valid predictor of postimplant speech perception. A patient with a VOW score above a preset cutoff is much more likely to develop acceptable speech perception after implantation than a patient with a VOW score below that cutoff. The binary classification based on VOW and the associated probabilities of cochlear implant success in terms of speech perception can be used-in addition to existing criteria-to support the clinician in guiding patient expectations and in considering implant candidacy for individual patients.

FMRI as a Preimplant Objective Tool to Predict Postimplant Oral Language Outcomes in Children with Cochlear Implants.

Despite the positive effects of cochlear implantation, postimplant variability in speech perception and oral language outcomes is still difficult to predict. The aim of this study was to identify neuroimaging biomarkers of postimplant speech perception and oral language performance in children with hearing loss who receive a cochlear implant. The authors hypothesized positive correlations between blood oxygen level-dependent functional magnetic resonance imaging (fMRI) activation in brain regions related to auditory language processing and attention and scores on the Clinical Evaluation of Language Fundamentals-Preschool, Second Edition (CELF-P2) and the Early Speech Perception Test for Profoundly Hearing-Impaired Children (ESP), in children with congenital hearing loss. Eleven children with congenital hearing loss were recruited for the present study based on referral for clinical MRI and other inclusion criteria. All participants were <24 months at fMRI scanning and <36 months at first implantation. A silent background fMRI acquisition method was performed to acquire fMRI during auditory stimulation. A voxel-based analysis technique was utilized to generate z maps showing significant contrast in brain activation between auditory stimulation conditions (spoken narratives and narrow band noise). CELF-P2 and ESP were administered 2 years after implantation. Because most participants reached a ceiling on ESP, a voxel-wise regression analysis was performed between preimplant fMRI activation and postimplant CELF-P2 scores alone. Age at implantation and preimplant hearing thresholds were controlled in this regression analysis. Four brain regions were found to be significantly correlated with CELF-P2 scores. These clusters of positive correlation encompassed the temporo-parieto-occipital junction, areas in the prefrontal cortex and the cingulate gyrus. For the story versus silence contrast, CELF-P2 core language score demonstrated significant positive correlation with activation in the right angular gyrus (r = 0.95), left medial frontal gyrus (r = 0.94), and left cingulate gyrus (r = 0.96). For the narrow band noise versus silence contrast, the CELF-P2 core language score exhibited significant positive correlation with activation in the left angular gyrus (r = 0.89; for all clusters, corrected p < 0.05). Four brain regions related to language function and attention were identified that correlated with CELF-P2. Children with better oral language performance postimplant displayed greater activation in these regions preimplant. The results suggest that despite auditory deprivation, these regions are more receptive to gains in oral language development performance of children with hearing loss who receive early intervention via cochlear implantation. The present study suggests that oral language outcome following cochlear implant may be predicted by preimplant fMRI with auditory stimulation using natural speech.

Receptive language as a predictor of cochlear implant outcome for prelingually deaf adults

Objective: This study investigated outcomes and predictive factors, specifically language skills, for a group of prelingually hearing-impaired adults who received a cochlear implant. Design: Speech perception data, demographic information, and other related variables such as communication mode, residual hearing, and receptive language abilities were explored. Pre- and post-implant speech perception scores were compared and multiple regression analysis was used to identify significant predictive relationships. Study sample: The study included 43 adults with a prelingual onset of hearing loss, who proceeded with cochlear implantation at the Royal Victorian Eye and Ear Hospital in Melbourne, Australia. Results: The majority of patients experienced benefit from their cochlear implants, with 88% demonstrating significant improvement in speech perception performance. Volunteers achieved better post-operative speech perception scores if they had a shorter duration of severe-to-profound hearing loss, better language skills, and used an exclusively oral communication mode. Conclusions: Although post-operative speech perception performance is significantly poorer for prelingually hearing-impaired adults compared to postlingually hearing-impaired patients, the study group demonstrated significant benefit from their cochlear implants. The variability in post-operative outcomes can be predicted to some extent from the hearing history and language abilities of the individual patient.

Factors that influence outcomes in cochlear implantation in adults, based on patient-related characteristics - a retrospective study.

Outcomes in speech perception following cochlear implantation in adults vary widely. Many studies have been carried out to identify and quantify factors that influence outcomes. This study adds a new dimension to pre-existing literature. Single-centre retrospective cohort study. University Medical Center Utrecht, the Netherlands. A total of 428 adults with bilateral severe-to-profound sensorineural hearing loss, unilaterally implanted between February 1988 and March 2014. Univariable and multivariable linear regression analyses were carried out to identify factors that may influence outcome after cochlear implantation. Consonant-vowel-consonant word scores were recorded pre- and post-implant and were used as outcome measure in two groups of patients (prelingually and postlingually deafened adults). As an added dimension, multiple imputation was implemented and evaluated to tackle 4% (17/407) missing data. For postlinguals, pre-implant speech perception score and age at onset of deafness are positive predictors and meningitis and otosclerosis as cause of deafness are negative predictors of post-implant speech perception. This model accounted for 26% of variance. For prelinguals, pre-implant speech perception score is the only strong positive predictor (β 0.524; P < 0.001). This model accounted for 31% of variance. Age at implantation was not a significant predictor in either group. Speech perception is predicted by pre-implant speech perception, age at onset of deafness and aetiology (meningitis and otosclerosis) for postlinguals and solely pre-implant speech perception for prelinguals. Age at implantation is of lesser importance in predicting speech perception outcome post-implant. Multiple imputation is a useful statistical technique when analysing incomplete data sets.

Cochlear Implantation in Patients With Superficial Siderosis: Seven Cases and Systematic Review of the Literature.

To date, there have been less than 30 cases of cochlear implantation (CI) in patients with superficial siderosis (SS) reported in the literature. The primary objective of the current study is to evaluate CI outcomes in six additional patients (seven ears) with SS and sensorineural hearing loss (SNHL) and to perform a systematic review of the literature. Case series and systematic review of the literature. Two tertiary academic CI centers. All patients with SS who underwent CI between 2007 and 2014. Cochlear implantation. Pre- and post-implantation speech perception scores and durability of benefit. A total of seven ears (four males; median age 52 yr) with SS and SNHL met inclusion criteria. All patients developed progressive bilateral SNHL that was no longer amenable to conventional hearing aids. Additional presenting symptoms included vestibulopathy (n = 4), cerebellar ataxia (n = 3), mild dementia (n = 1), and myelopathy (n = 1). All patients underwent uncomplicated CI, and intraoperative device telemetry revealed normal responses in all electrodes. The median postoperative auditory threshold average was 32.5 dB HL (range 16-36 dB) and the median postoperative CNC word score was 51% (range 46-64%). The median duration of follow-up was 15.5 months (range 3-64 mo). All patients demonstrated initial improvement in speech perception testing. Two patients had performance decline and worsening dementia resulting from progressive SS. Cochlear implantation is a viable strategy for auditory rehabilitation in patients with SS and associated SNHL. Most individuals enjoy benefit from CI; however, patients should be counseled regarding the risks of performance decline with progressive SS.

Delayed-onset hearing loss in pediatric candidates for cochlear implantation.

The objective of this study was to evaluate the clinical significance of delayed-onset hearing loss in children. Seventy-three children who underwent cochlear implantation (CI) were included. They were divided into a congenital hearing loss group (n = 50) and a delayed-onset hearing loss group (n = 23). The age at diagnosis of hearing loss, age at the beginning of auditory habilitation, the age at CI, and the postimplant speech perception abilities were compared between the two groups. Children in the congenital hearing loss group were confirmed to have hearing loss at a mean age of 0.3 years, and those in the delayed-onset hearing loss group were diagnosed with hearing loss at a mean age of 2.0 years. Auditory habilitation began at a mean age of 0.4 and 2.0 years, and CI was performed at a mean age of 1.4 and 2.6 years, respectively. Children in the congenital hearing loss group had better scores on speech perception tests than those in the delayed-onset hearing loss group, but the differences were not significant. About half of the children with delayed-onset hearing loss (57 %) had risk factors associated with delayed-onset hearing loss. A high prevalence of delayed-onset hearing loss was noted in the group of children who underwent CI. Risk factors for hearing loss were not found in 43 % of children with delayed-onset hearing loss. Universal screening for delayed-onset hearing loss needs to be performed during early childhood.

A New Classification of Cochleovestibular Malformations and Implications for Predicting Speech Perception Ability after Cochlear Implantation

Objectives: The aims of this study were to introduce a new classification of cochleovestibular malformation (CVM) and to investigate how well this classification can predict speech perception ability after cochlear implantation in children with CVM. Methods: Fifty-nine children with CVM who had used a cochlear implant for more than 3 years were included. CVM was classified into 4 subtypes based on the morphology of the cochlea and the modiolus on temporal bone computed tomography (TBCT): normal cochlea and normal modiolus (type A, n = 16), malformed cochlea and partial modiolus (type B, n = 31), malformed cochlea and no modiolus (type C, n = 6), and no cochlea and no modiolus (type D, n = 6). Speech perception test scores were compared between the subtypes of CVM using analysis of covariance with post hoc Bonferroni test. Univariate and multivariate regression analyses were used to identify the significant predictors of the speech perception test scores. Results: The speech perception test scores after implantation were significantly better in children with CVM type A or type B than in children with CVM type C or type D. The test scores did not differ significantly between the implanted children with CVM type A or type B and those without CVM. In univariate regression analysis, the type of CVM was a significant predictor of the speech perception test scores in implanted children with CVM. Multivariate regression analysis revealed that the age at cochlear implantation, cochlear nerve size and preimplantation speech perception test scores were significant predictors of the postimplantation speech perception test scores. The chance of cochlear nerve deficiency increased progressively from CVM type A to type D. Conclusion: The new classification of CVM based on the morphology of the cochlea and the modiolus is simple and easy to use, and correlated well with postimplantation speech perception ability and cochlear nerve status. This simple classification of CVM using TBCT with cochlear nerve assessment by magnetic resonance imaging is helpful in the preoperative evaluation of children with CVM.

WYNIKI ZASTOSOWANIA STANDARDOWYCH IMPLANTÓW ŚLIMAKOWYCH U PACJENTÓW Z OPADAJĄCYM NIEDOSŁUCHEM

BackgroundThis study examined the speech perception outcomes for postlingually deafened adults using cochlear implants who preoperatively had steeply sloping hearing loss and in whom there was no attempt at electroacoustic stimulation. The aims were firstly to determine whether patients with sloping loss (SL) who received a standard-length cochlear implant electrode would show significant benefit; and secondly to compare the degree of benefit to a matched group of cochlear implant users with preoperative profound hearing loss.Material and MethodsA retrospective analysis of pre- and post-implant speech perception scores of 27 adults with sloping hearing loss and a matched group of 27 adults with profound hearing loss was conducted. Matching was based on age at implant and duration of loss. All were implanted with a Nucleus Freedom (CA) or a Nucleus 5 implant.ResultsPostoperative open-set speech perception testing demonstrated significant improvement compared to pre-implant for both groups. Speech perception outcomes were better in the SL group; however, there was no significant difference between the groups in the degree of improvement pre- to post-operatively under either the condition of implant alone or binaurally.ConclusionsThis study demonstrates that postlingually deafened adults with sloping hearing loss have the potential to gain significant benefit from cochlear implants, and achieve equivalent improvement in speech perception to implant recipients with profound loss. The results achieved in this group, without any attempt at hearing preservation, support the use of newer standard-length electrodes for both hearing preservation and optimal electric stimulation in patients with sloping hearing loss.

Cochlear Implantation in Patients Profoundly Deafened After Head Injury

There is very little information in the literature regarding outcomes of cochlear implantation in patients profoundly deafened after head injury. The aim of this study was to assess outcomes in this group of patients. Retrospective case review. The Manchester Cochlear Implant Programme, University of Manchester. Profoundly deafened patients after head injury who proceeded to cochlear implantation for auditory rehabilitation. Mean age and duration of deafness at implantation. Preimplantation and postimplantation speech perception outcomes were measured using Bench Kowel Bamford (BKB) sentences in quiet and noise, City University of New York sentences with lip reading and Arthur Boothroyd words scoring the percentage phonemes correct. Twenty patients received 23 cochlear implants. Mean age at implantation was 51 years (standard deviation, 12 yr). Mean duration of deafness at implantation was 12 years (range, 1-30 yr).Preimplantation BKB score in quiet of 0%. Mean postimplantation BKB score in quiet was 64% (range, 0%-100%) and in noise was 61% (range, 0%-97%). Three were nonusers, and 1 required reimplantation. There was a moderately negative correlation between outcome and age at implantation (r = -0.41, p < 0.05) and between outcome and duration of deafness (r = -0.52, p < 0.05). Cochlear implantation is an effective method for hearing rehabilitation in profoundly deafened patients after head injury. However, negative factors, such as significant injury to the central auditory pathway, basal turn obliteration, long duration of deafness, and nonauditory stimulation, should be considered in the preoperative assessment of these patients.

Speech Intelligibility as a Predictor of Cochlear Implant Outcome in Prelingually Deafened Adults

Among adult patients with prelingual deafness, interindividual variability in speech perception outcome after cochlear implantation is generally large. It was hypothesized that the intelligibility of the patient's own speech may be predictive of speech perception with a cochlear implant. The objectives were (1) to provide a validation of a new test battery as a measurement tool for intelligibility using a group of prelingually deafened patients; (2) to investigate the validity of the test battery as a predictor of postimplant speech perception, based on preliminary data with a cochlear implant from a subgroup of patients; and (3) to investigate the validity of the test battery as a predictor of postimplant health-related quality of life (HRQoL) for the same subgroup of patients. Twenty-five adult cochlear implant candidates with prelingual deafness participated in this study. Average age at onset of deafness was 8 mos (range 0 to 4 yrs). Speech samples from the participants were recorded and presented to two normal-hearing listeners. Results from the seven intelligibility tests in the battery were subjected to analyses of reliability and validity. Furthermore, the multiple test outcomes were submitted to a Principal Component Analysis to investigate the possibility of summarizing the data. Subsequently, from the group of 25 participants, 9 participants with above-average intelligibility were selected for implantation. Speech perception data with a cochlear implant from the nine implantees were collected at 12 mos postimplantation, as well as HRQoL data. Predictive validity of the intelligibility test battery was determined, using the postimplant data as the criterion. Results from the 25 participants averaged over listeners showed that all tests in the battery had good reliability and validity as measures of intelligibility. Principal Component Analysis showed that the multiple test outcomes could be summarized by a single underlying variable. Despite the early age at onset of deafness, the subgroup of nine participants who received a cochlear implant included several good performers in terms of speech perception with the implant. The intelligibility test battery summary score had good validity as a predictor of postimplant outcome: the more intelligible the participant's speech, the better his or her speech perception outcome with the cochlear implant. Availability of effective auditory input in early life may be the fundamental factor underlying the potential for speech perception with a cochlear implant in later life. The intelligibility test battery can be reduced to a single test to minimize testing time without negatively affecting its predictive validity. Predictive validity of the intelligibility test battery can be generalized to HRQoL outcomes of cochlear implantation, provided these outcomes are concerned with speech processing abilities. The new test battery (or its reduced version), used as a measure of intelligibility, is a promising tool for guiding cochlear implant candidacy decisions and counseling for individual patients with prelingual deafness. Because intelligibility has superior predictive power in comparison to age at onset of deafness, the latter should be discarded as an exclusion criterion for cochlear implantation.

Detection of the GJB2 Mutation in Iranian Children with Hearing Loss Treated with Cochlear Implantation

The 35delG mutation in the gap junction protein, β2, 26kDa (GJB2) gene is the most common mutation that has been found in children with non syndromic hearing loss. Testing for the GJB2 gene mutation is simple and can directly answer the concerns of the parents about cause of the disorder and prognosis for their children. Cochlear implantation (CI) is one of the methods of hearing rehabilitation in patients with complete hearing loss. The present study was designed for genetic assessment of children who were referred for CI.Connexin 26 (Cx26) gene analyses were performed on 42 children with non syndromic hearing loss who were referred to the Baqiyatallah Hospital, Tehran, Iran for genetic consultation and CI. Clinical history was obtained and an examination conducted on each individual. Genomic DNA was extracted from peripheral blood and mutation identification of the Cx26 gene was performed by polymerase chain reaction (PCR) amplification and direct sequencing of the coding sequence of the gene. Cochlear implantation was performed for all patients and treatment response was assessed for all of them based on speech intelligibility rating (SIR) before and after CI.We found six patients (14.3%) with the 35delG mutation on the Cx26 gene, two homozygotes and four heterozygotes. No other mutation was detected. Treatment response in children with the homozygous 35delG mutation was better than in heterozygous patients, and treatment response in children with the mutation was better than in children with no mutation.Mutation screening for finding deafness causing mutations in the GJB2 gene is a useful predictor of post-implantation speech perception. We suggest microarray or other advanced mutation detection methods for assessment of other genes that might be responsible for non syndromic deafness.

Case-Control Analysis of Cochlear Implant Performance in Elderly Patients

To characterize speech perception performance in elderly cochlear implant users compared with younger adult users. Case-control retrospective analysis from January 1, 1999, to January 28, 2008. Tertiary care, academic practice cochlear implant program. Medical records for 78 patients with age at implantation of 65 years or older were analyzed for ear-specific preimplantation speech perception performance, length of deafness, age at implantation, and 1-year postimplantation speech perception performance. A subset of 28 elderly patients with complete data was matched to 28 younger adult patients (age at implantation, 18-64 years) for preimplantation performance using the Hearing in Noise Test-Quiet scores (mean, 22% and 23%, respectively). One-year postimplantation performance on word and sentence testing. Within the elderly cohort, the Consonant-Nucleus-Consonant and Hearing in Noise Test-Quiet scores were not affected by age. The Hearing in Noise Test-Noise scores trended downward with increasing age but did not reach statistical significance (P = .052). Of the matched older and younger patients, 55 of 56 showed improvement in their 1-year postimplantation compared with preimplantation Hearing in Noise Test-Quiet scores, with better preimplantation performance predictive of better postimplantation performance, independent of age at implantation (P = .02). Group comparisons, however, revealed poorer postimplantation scores overall for the elderly patients compared with the younger ones for the Hearing in Noise Test-Quiet (70% vs 83%; P = .02) and the Consonant-Nucleus-Consonant test (38% vs 53%; P = .02). Elderly patients benefit significantly from cochlear implantation. Compared with a younger cohort matched for preimplantation performance, however, their postimplantation scores are significantly lower on some measures. These results may provide guidelines for candidacy and counseling regarding elderly patients with cochlear implants.

Cochlear implantation in children with cognitive impairments

Cochlear implantation in children with cognitive impairments

Cochlear implantation in children with cerebral palsy. A preliminary report

Objectives The aim of this study is to assess the post-implantation speech perception and intelligibility of speech produced by five profoundly deaf children with cerebral palsy. Methods This study is derived by a review of a prospectively maintained data collection on all patients entering the cochlear implant program. Five children with cerebral palsy who underwent cochlear implantation participated in this study. Functional outcome was assessed using the Speech Perception Categories and the Speech Intelligibility Rating scale. The follow-up of the series ranged from 12 to 45 months. Results At the last follow-up, two children who were placed into speech perception category 1 (detection of a speech signal) preoperatively progressed to category 6 (open-set word recognition with familiar words) postoperatively. Two children moved from preoperative category 2 (pattern perception) to postoperative category 6. One child placed into category 0 (no detection of speech) preoperatively progressed to category 4 (word identification) postoperatively. Before implantation, three children had connected speech unintelligible, and two subjects had connected speech intelligible to a listener who concentrates and lip-reads. At the last follow-up, one child had connected speech unintelligible, two children had connected speech intelligible to a listener who concentrate and lip-reads, one child had connected speech intelligible to a listener who has little experience of a deaf person's speech, and one child had connected speech intelligible to all listeners. Conclusions Cochlear implantation allowed these patients to dramatically improve their quality of life, increasing their self-confidence, independence and social integration.

Speech Perception Outcome in Multiply Disabled Children Following Cochlear Implantation: Investigating a Predictive Score

Children with multiple disabilities account for a small percentage of implantees in a cochlear implant program, but they remain the most challenging group for which to predict benefit from the implant and for cooperation with habilitation postoperatively. To assess the relationship of pre-implant functional disabilities with postoperative speech perception scores and determine the feasibility of predicting outcome with a cochlear implant in a multiply disabled pediatric population. Retrospective cohort study. Sixty-six children with a cochlear implant and at least one additional disability. We retrospectively examined the relationship between pre-implant Graded Profile Analysis (GPA) scores and postimplant speech perception scores. A pre-implant functional disability score (based on the Battelle developmental screen) was applied to the same cohort of patients and its association with postimplant speech perception scores was examined. The functional disability score significantly predicted high (k > 24) and low (k < 7) speech perception scores (p < 0.001 and p < .0001) and had excellent discrimination ability (c statistic = 0.88 and 0.93 respectively). The GPA score was not significantly associated with speech perception scores (p = 0.519 and p = 0.146) and demonstrated no ability to discriminate postimplant speech perception scores in this implant population (c statistic = 0.49 and c = 0.57). Prediction of outcomes following cochlear implantation in multiply disabled children can be facilitated using this newly developed functional disability score as an adjunct to traditional candidacy assessments.