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ABSTRACT: OBJECTIVE: Describe the long-term benefits of early cochlear implantation. Provide a comprehensive description of outcomes, including: language, speech production, and speech perception. Compare the communication outcomes for the early implanted children to those of normally hearing children and children who received a cochlear implant at a comparatively older age. METHOD: Retrospective review of the communication development of 35 children implanted between 6 and 12 months of age and 85 children implanted between 13 and 24 months of age. Audiologic assessments included unaided and aided audiograms, auditory brainstem response (ABR), auditory steady state response (ASSR), and otoacoustic emissons (OAEs). Formal language, speech production, and speech perception measures were administered, preimplant and at 1, 2, 3, and 5 years postimplant. RESULTS: The children who received their cochlear implant by 12 months of age demonstrated language growth rates equivalent to their normally hearing peers and achieved age appropriate receptive language scores 3 years postimplant. The children who received their cochlear implant between 13 and 24 months demonstrated a significant language delay at 3 years postimplant. Speech production development followed a similar pattern to that of normal-hearing children, although was delayed, for both groups of children. Mean open-set speech perception scores were comparable with previous reports for children and adults who use cochlear implants. CONCLUSION: Children implanted by 12 months of age demonstrate better language development compared with children who receive their cochlear implant between 13 and 24 months. This supports the provision of a cochlear implant within the first year of life to enhance the likelihood that a child with severe-to-profound hearing impairment will commence elementary school with age-appropriate language skills.
Otology & neurotology: official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology 02/2013; · 1.44 Impact Factor
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ABSTRACT: OBJECTIVE: The relative impact of early intervention approach on speech perception and language skills was examined in these 3 well-matched groups of children using cochlear implants. STUDY DESIGN: Eight children from an auditory verbal intervention program were identified. From a pediatric database, researchers blind to the outcome data, identified 23 children from auditory oral programs and 8 children from bilingual-bicultural programs with the same inclusion criteria and equivalent demographic factors. PATIENTS: All child participants were male, had congenital profound hearing loss (pure tone average >80 dBHL), no additional disabilities, were within the normal IQ range, were monolingual English speakers, had no unusual findings on computed tomography/magnetic resonance imaging, and received hearing aids and cochlear implants at a similar age and before 4 years of age. MAIN OUTCOME MEASURES: Open-set speech perception (consonant-nucleus-consonant [CNC] words and Bamford-Kowal-Bench [BKB] sentences) and the Peabody Picture Vocabulary Test (PPVT) were administered. RESULTS: The mean age at cochlear implant was 1.7 years (range, 0.8-3.9; SD, 0.7), mean test age was 5.4 years (range, 2.5-10.1; SD, 1.7), and mean device experience was 3.7 years (range, 0.7-7.9; SD, 1.8). Results indicate mean CNC scores of 60%, 43%, and 24% and BKB scores of 77%, 77%, and 56% for the auditory-verbal (AV), aural-oral (AO), and bilingual-bicultural (BB) groups, respectively. The mean PPVT delay was 13, 19, and 26 months for AV, AO, and BB groups, respectively. CONCLUSION: Despite equivalent child demographic characteristics at the outset of this study, by 3 years postimplant, there were significant differences in AV, AO, and BB groups. Results support consistent emphasis on oral/aural input to achieve optimum spoken communication outcomes for children using cochlear implants.
Otology & neurotology: official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology 02/2013; · 1.44 Impact Factor
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ABSTRACT: This study investigated the effect of low to moderate electrical stimulation rates (275, 350, 500 and 900 pps/ch) on modulation detection ability of cochlear implant subjects, and the relationship between modulation detection and speech perception as a function of rate.
A repeated ABCD experimental design for the four rate conditions was employed. A sinusoidally amplitude modulated acoustic signal was presented to the audio input of a research processor. Stimuli were presented at an acoustic level that produced electrical levels close to the subjects' most comfortable level (MCL) of stimulation and at an acoustic level 20 dB below this.
Ten postlingually deaf adult users of the Nucleus CI24 cochlear implant participated.
Acoustic modulation detection thresholds (MDTs), averaged across the subject group, were significantly better for rates of 500 pps/ch compared to the other rates examined for stimuli presented at MCL. In addition, there was a significant relation between speech perception in noise and acoustic MDTs at MCL.
The benefits obtained in speech perception and modulation detection as a function of rate were attributed to an increased electrical dynamic range as a function of stimulation rate, at least for rates up to 500 pps/ch.
International journal of audiology 11/2010; 50(2):123-32. · 1.34 Impact Factor
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ABSTRACT: CT and MRI scans for 48 children with cochlear and/or vestibular abnormality were classified in decreasing severity; common cavity, Mondini plus enlarged vestibular aqueduct, Mondini dysplasia alone and enlarged vestibular aqueduct alone. No significant relationship between degree of cochlea abnormality and surgical issues (cerebrospinal fluid gusher, depth of insertion, number of electrodes) or speech perception/language outcomes was found. A significant relationship was observed between cerebrospinal fluid gusher and partial electrode insertion, fewer active electrodes and poorer sentence understanding. Optimum language outcomes were associated with younger age at implant.
Audiology and Neurotology 10/2010; 16(4):222-32. · 2.46 Impact Factor
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ABSTRACT: Friedreich's ataxia (FRDA) is an inherited ataxia with a range of progressive features including axonal degeneration of sensory nerves. The aim of this study was to investigate auditory perception in affected individuals.
Fourteen subjects with genetically defined FRDA participated. Two control groups, one consisting of healthy, normally hearing individuals and another comprised of subjects with sensorineural hearing loss, were also assessed. Auditory processing was evaluated using structured tasks designed to reveal the listeners' ability to perceive temporal and spectral cues. Findings were then correlated with open-set speech understanding.
Nine of 14 individuals with FRDA showed evidence of auditory processing disorder. Gap and amplitude modulation detection levels in these subjects were significantly elevated, indicating impaired encoding of rapid signal changes. Electrophysiologic findings (auditory brainstem response, ABR) also reflected disrupted neural activity. Speech understanding was significantly affected in these listeners and the degree of disruption was related to temporal processing ability. Speech analyses indicated that timing cues (notably consonant voice onset time and vowel duration) were most affected.
The results suggest that auditory pathway abnormality is a relatively common consequence of FRDA. Regular auditory evaluation should therefore be part of the management regime for all affected individuals. This assessment should include both ABR testing, which can provide insights into the degree to which auditory neural activity is disrupted, and some functional measure of hearing capacity such as speech perception assessment, which can quantify the disorder and provide a basis for intervention.
Audiology and Neurotology 11/2009; 15(4):229-40. · 2.46 Impact Factor
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ABSTRACT: The objective of this study was to explore cochlear implant users' speech perception performance in quiet and in noise for low to moderate stimulation rates. Eight postlingually deaf adult users of the Nucleus CI24 cochlear implant (contour electrode array) using the ESPrit 3G speech processor participated in this study. Monosyllabic word recognition in quiet and sentence perception in noise was evaluated for low to moderate stimulation rates of 275, 350, 500, and 900 pulses-per-second/channel (pps/ch). All four stimulation rate programs were balanced for loudness. A repeated ABCD experimental design was employed. Take home practice was provided with each stimulation rate. Subjects also responded to a comparative questionnaire to examine their rate preference for a variety of listening situations. Results for six of the eight subjects showed no significant effect of rate for monosyllables in quiet. However, results for the sentence test in noise demonstrated improvements with 500 or 900 pps/ch stimulation rates in seven out of the eight subjects. Although there was not a close relationship between each subject's subjective preference and the rate program that provided best speech perception, most subjects indicated a preference for 500 pps/ch rate in noise.
International journal of audiology 08/2009; 48(8):561-7. · 1.34 Impact Factor
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The Lancet 10/2007; 370(9589):719-20. · 38.28 Impact Factor
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ABSTRACT: To evaluate the effect of background noise on speech perception in children with auditory neuropathy/dys-synchrony (AN/AD) type hearing loss.
Open and closed-set speech perception abilities were assessed in 12 school-age children who had been diagnosed with AN/AD in infancy. Data were also obtained from a cohort of subjects with sensorineural (SN) hearing loss and from a group of normal-hearing children.
Closed-set speech understanding was more affected by the presence of a competing signal in the hearing impaired than in the normal-hearing subjects. The mean S/N ratio required to identify a spondee in noise was -11.5 +/- 2.0 dB for the normal group, whereas the ratio required for the SN group was -5.4 +/- 5.1 dB and for the AN/AD group was -2.5 +/- 4.7 dB. Closed-set perception in noise was not significantly different for the AN/AD children and their SN counterparts although there was a trend toward poorer performance in the AN/AD group. The effect of background noise on open-set speech perception was also similar across hearing-impaired subjects although again, the AN/AD cohort tended to show greater difficulties in noise than their SN peers.
Listening in background noise was more difficult for our group of children with AN/AD-type hearing loss than for their normal-hearing peers. However, the noise effects were not consistent across subjects and some children demonstrated reasonable perceptual ability at low signal-to-noise ratios. The ways in which speech understanding is affected by competing signals may be different for different types of hearing deficit, but the results of this investigation indicate that significant perceptual disruption occurs both in children with auditory neuropathy/dys-synchrony and sensorineural type hearing loss.
Ear and Hearing 07/2007; 28(3):351-60. · 2.58 Impact Factor
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ABSTRACT: 1. To examine whether speech perception after implantation is correlated with the total duration of deafness, the duration of deafness in the implanted ear, or age at implantation. 2. To examine whether the rate of facial nerve stimulation postoperatively is correlated with the type of electrode used.
Retrospective case note review.
Tertiary referral center.
Fifty-nine adults with profound postlingual sensorineural hearing loss due to otosclerosis.
Cochlear implantation with the Nucleus device using either a straight (n = 35) or Contour (n = 29) electrode array.
Speech perception scores for patients at 3, 6 and 12 months postimplantation were correlated against duration of deafness in the implanted ear, duration of total deafness, and age at implantation. Data on facial nerve stimulation rates postoperatively were collected.
Implantation in the shortest deafened ear conferred an initial advantage for speech perception 3 months after surgery; however, this effect was lost by 6 months. There were no significant correlations between the duration of bilateral deafness and hearing outcomes. Age at implantation was negatively correlated with outcome at 3 months, but not at 6 and 12 months. Fourteen of 35 patients with straight electrodes and 0 of 24 patients with Contour electrodes experienced facial nerve stimulation during mapping sessions (p < 0.005, chi).
Patients with otosclerosis are not disadvantaged in the long term by implantation in the longest deafened ear. Increasing age at implantation did not predict poorer outcomes. A perimodiolar design of electrode should be used in otosclerotic patients when possible to reduce the risk of facial nerve stimulation.
Ontology & Neurotology 07/2007; 28(4):438-46. · 1.90 Impact Factor
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ABSTRACT: Cochlear implant technology continues to advance, placing new challenges on physicians, audiologists, speech-language pathologists, and deaf educators to properly determine cochlear implant candidacy. This panel addressed the topics of new technology applied to elderly and to very young children. Six panelists were selected to represent varied regions of the world, prompting interesting discussion and interaction with the audience.
Ear and Hearing 05/2007; 28(2 Suppl):128S-129S. · 2.58 Impact Factor
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ABSTRACT: 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.
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.
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.
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.
Ear and Hearing 07/2002; 23(3):239-53. · 2.58 Impact Factor
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ABSTRACT: The aim of this study was to develop guidelines, based on the unaided pure-tone audiogram, for recommending a cochlear implant (CI) for infants and young children with residual hearing. As awareness of the benefits of early implantation increases and age at diagnosis decreases, an increasing number of infants are presenting for consideration of implantation with significant residual hearing in one or both ears. Determining the likelihood that these infants will have an improved speech and language outcome if they receive a CI is a challenge.
Subjects were 142 hearing impaired children (ages 4.6 to 16.2 yrs) with unaided three-frequency pure-tone average (PTA; at 500, 1000, and 2000 Hz) ranging from 28 to 125 dB HL: 62 used conventional amplification (analog or digital hearing aids [HAs]) and the remaining 80 used a Nucleus 24 (N24) or Freedom CI. Open-set monosyllabic word (Phonetically Balanced Kindergarten or Consonant-Nucleus-Consonant words) and sentence (Bamford-Kowal-Bench sentences) testing was administered audition alone to both groups of children.
Comparison of means for sentence testing showed that the children using CIs performed significantly better than their peers with profound hearing loss (PTA >90 dB HL) using HAs and not significantly differently to those with severe (PTA 66 to 90 dB HL) or moderate (PTA <66 dB HL) hearing loss. Comparison of means for monosyllabic word testing showed that the children using CIs performed significantly better than their peers with severe and profound hearing loss and not significantly differently to those with moderate hearing loss. Regression analysis was used to determine the equivalent unaided PTA values that corresponded to the median and first quartile scores for the children using CIs on speech perception testing. For open-set words, scored for phonemes correct, the equivalent unaided PTAs were 46 and 56 dB HL, respectively. For sentence testing, the equivalent unaided PTAs were 63 and 72 dB HL, respectively.
Results suggest that recommendation for implantation can be made confidently for children presenting with bilateral profound hearing loss. For children with unaided PTA hearing levels in the range of 75 to 90 dB HL, a recommendation for implantation can also be made, provided that a 75% chance of improvement in hearing outcome is an acceptable level of benefit to the family and clinician. Children presenting with PTA hearing levels better than 75 dB HL should be encouraged to continue with binaural HA use.
Ear and hearing 32(3):313-22. · 2.06 Impact Factor
