Multicenter US Clinical Trial With an Electric-Acoustic Stimulation (EAS) System in Adults: Final Outcomes.

OBJECTIVE: To demonstrate the safety and effectiveness of the MED-EL Electric-Acoustic Stimulation (EAS) System, for adults with residual low-frequency hearing and severe-to-profound hearing loss in the mid to high frequencies. STUDY DESIGN: Prospective, repeated measures. SETTING: Multicenter, hospital. PATIENTS: Seventy-three subjects implanted with PULSAR or SONATA cochlear implants with FLEX electrode arrays. INTERVENTION: Subjects were fit postoperatively with an audio processor, combining electric stimulation and acoustic amplification. MAIN OUTCOME MEASURES: Unaided thresholds were measured preoperatively and at 3, 6, and 12 months postactivation. Speech perception was assessed at these intervals using City University of New York sentences in noise and consonant-nucleus-consonant words in quiet. Subjective benefit was assessed at these intervals via the Abbreviated Profile of Hearing Aid Benefit and Hearing Device Satisfaction Scale questionnaires. RESULTS: Sixty-seven of 73 subjects (92%) completed outcome measures for all study intervals. Of those 67 subjects, 79% experienced less than a 30 dB HL low-frequency pure-tone average (250-1000 Hz) shift, and 97% were able to use the acoustic unit at 12 months postactivation. In the EAS condition, 94% of subjects performed similarly to or better than their preoperative performance on City University of New York sentences in noise at 12 months postactivation, with 85% demonstrating improvement. Ninety-seven percent of subjects performed similarly or better on consonant-nucleus-consonant words in quiet, with 84% demonstrating improvement. CONCLUSION: The MED-EL EAS System is a safe and effective treatment option for adults with normal hearing to moderate sensorineural hearing loss in the low frequencies and severe-to-profound sensorineural hearing loss in the high frequencies who do not benefit from traditional amplification.

Cochlear implantation in infants below 12 months of age.

OBJECTIVES: To provide safety and efficacy data on infants implanted below 12 months of age. METHODS: With the wide application of newborn hearing screening programs, infants with deafness are being identified at birth. When a hearing aid trial fails, cochlear implantation is the only option to restore hearing. Mounting evidence suggests that age at implantation is a strong predictor of language outcomes. Using the minimally invasive surgical technique we have employed for nearly two decades, a limited clinical trial was initiated in the year 2000 because this age limitation fell outside of FDA guidelines. The infants were initially assessed using the preferential listening paradigm to confirm that they could learn associations between speech sounds and objects. Sufficient time was allowed to pass to administer more traditional language measures. RESULTS: No surgical or anesthetic complications occurred in this group of infants. The pattern of listening skill development mirrored that seen in normal hearing infants. Long-term language assessments using the Peabody Picture Vocabulary Test (PPVT) and other measures have demonstrated that many of infants achieved age appropriate language skills. CONCLUSION: Cochlear implantation in children less than 12 months of age is safe and efficacious as demonstrated by long-term PPVT language data.

Brief report: sound output of infant humidifiers.

The sound pressure levels (SPLs) of common infant humidifiers were determined to identify the likely sound exposure to infants and young children. This primary investigative research study was completed at a tertiary-level academic medical center otolaryngology and audiology laboratory. Five commercially available humidifiers were obtained from brick-and-mortar infant supply stores. Sound levels were measured at 20-, 100-, and 150-cm distances at all available humidifier settings. Two of 5 (40%) humidifiers tested had SPL readings greater than the recommended hospital infant nursery levels (50 dB) at distances up to 100 cm. In this preliminary study, it was demonstrated that humidifiers marketed for infant nurseries may produce appreciably high decibel levels. Further characterization of the effect of humidifier design on SPLs and further elucidation of ambient sound levels associated with hearing risk are necessary before definitive conclusions and recommendations can be made.

Neurocognitive risk in children with cochlear implants.

IMPORTANCE: Children who receive a cochlear implant (CI) for early severe to profound sensorineural hearing loss may achieve age-appropriate spoken language skills not possible before implantation. Despite these advances, reduced access to auditory experience may have downstream effects on fundamental neurocognitive processes for some children with CIs. OBJECTIVE: To determine the relative risk (RR) of clinically significant executive functioning deficits in children with CIs compared with children with normal hearing (NH). DESIGN, SETTING, AND PARTICIPANTS: In this prospective, cross-sectional study, 73 children at a hospital-based clinic who received their CIs before 7 years of age and 78 children with NH, with average to above average mean nonverbal IQ scores, were recruited in 2 age groups: preschool age (age range, 3-5 years) and school age (age range, 7-17 years). No children presented with other developmental, cognitive, or neurologic diagnoses. INTERVENTIONS: Parent-reported checklist measures of executive functioning were completed during psychological testing sessions. MAIN OUTCOMES AND MEASURES: Estimates of the RR of clinically significant deficits in executive functioning (≥1 SDs above the mean) for children with CIs compared with children with NH were obtained based on 2 parent-reported child behavior checklists of everyday problems with executive functioning. RESULTS: In most domains of executive functioning, children with CIs were at 2 to 5 times greater risk of clinically significant deficits compared with children with NH. The RRs for preschoolers and school-aged children, respectively, were greatest in the areas of comprehension and conceptual learning (RR [95% CI], 3.56 [1.71-7.43] and 6.25 [2.64-14.77]), factual memory ( 4.88 [1.58-15.07] and 5.47 [2.03-14.77]), attention (3.38 [1.03-11.04] and 3.13 [1.56-6.26]), sequential processing (11.25 [1.55-81.54] and 2.44 [1.24-4.76]), working memory (4.13 [1.30-13.06] and 3.64 [1.61-8.25] for one checklist and 1.77 [0.82-3.83] and 2.78 [1.18-6.51] for another checklist), and novel problem-solving (3.93 [1.50-10.34] and 3.13 [1.46-6.67]). No difference between the CI and NH samples was found for visual-spatial organization (2.63 [0.76-9.03] and 1.04 [0.45-2.40] on one checklist and 2.86 [0.98-8.39] for school-aged children on the other checklist). CONCLUSIONS AND RELEVANCE: A large proportion of children with CIs are at risk for clinically significant deficits across multiple domains of executive functioning, a rate averaging 2 to 5 times that of children with NH for most domains. Screening for risk of executive functioning deficits should be a routine part of the clinical evaluation of all children with deafness and CIs.

Profiles of verbal working memory growth predict speech and language development in children with cochlear implants.

PURPOSE: Verbal short-term memory (STM) and working memory (WM) skills predict speech and language outcomes in children with cochlear implants (CIs) even after conventional demographic, device, and medical factors are taken into account. However, prior research has focused on single end point outcomes as opposed to the longitudinal process of development of verbal STM/WM and speech-language skills. In this study, the authors investigated relations between profiles of verbal STM/WM development and speech-language development over time. METHOD: Profiles of verbal STM/WM development were identified through the use of group-based trajectory analysis of repeated digit span measures over at least a 2-year time period in a sample of 66 children (ages 6-16 years) with CIs. Subjects also completed repeated assessments of speech and language skills during the same time period. RESULTS: Clusters representing different patterns of development of verbal STM (digit span forward scores) were related to the growth rate of vocabulary and language comprehension skills over time. Clusters representing different patterns of development of verbal WM (digit span backward scores) were related to the growth rate of vocabulary and spoken word recognition skills over time. CONCLUSION: Different patterns of development of verbal STM/WM capacity predict the dynamic process of development of speech and language skills in this clinical population.

Verbal short-term memory development and spoken language outcomes in deaf children with cochlear implants.

OBJECTIVES: Cochlear implants (CIs) help many deaf children achieve near-normal speech and language (S/L) milestones. Nevertheless, high levels of unexplained variability in S/L outcomes are limiting factors in improving the effectiveness of CIs in deaf children. The objective of this study was to longitudinally assess the role of verbal short-term memory (STM) and working memory (WM) capacity as a progress-limiting source of variability in S/L outcomes after CI in children. DESIGN: Longitudinal study of 66 children with CIs for prelingual severe-to-profound hearing loss. Outcome measures included performance on digit span forward (DSF), digit span backward (DSB), and four conventional S/L measures that examined spoken-word recognition (Phonetically Balanced Kindergarten word test), receptive vocabulary (Peabody Picture Vocabulary Test ), sentence-recognition skills (Hearing in Noise Test), and receptive and expressive language functioning (Clinical Evaluation of Language Fundamentals Fourth Edition Core Language Score; CELF). RESULTS: Growth curves for DSF and DSB in the CI sample over time were comparable in slope, but consistently lagged in magnitude relative to norms for normal-hearing peers of the same age. For DSF and DSB, 50.5% and 44.0%, respectively, of the CI sample scored more than 1 SD below the normative mean for raw scores across all ages. The first (baseline) DSF score significantly predicted all endpoint scores for the four S/L measures, and DSF slope (growth) over time predicted CELF scores. DSF baseline and slope accounted for an additional 13 to 31% of variance in S/L scores after controlling for conventional predictor variables such as: chronological age at time of testing, age at time of implantation, communication mode (auditory-oral communication versus total communication), and maternal education. Only DSB baseline scores predicted endpoint language scores on Peabody Picture Vocabulary Test and CELF. DSB slopes were not significantly related to any endpoint S/L measures. DSB baseline scores and slopes taken together accounted for an additional 4 to 19% of variance in S/L endpoint measures after controlling for the conventional predictor variables. CONCLUSIONS: Verbal STM/WM scores, process measures of information capacity, develop at an average rate in the years after cochlear implantation, but were found to consistently lag in absolute magnitude behind those reported for normal-hearing peers. Baseline verbal STM/WM predicted long-term endpoint S/L outcomes, but verbal STM slopes predicted only endpoint language outcomes. Verbal STM/WM processing skills reflect important underlying core elementary neurocognitive functions and represent potential intervention targets for improving endpoint S/L outcomes in pediatric CI users.

Effects of simultaneous speech and sign on infants' attention to spoken language.

OBJECTIVES: To examine the hypothesis that infants receiving a degraded auditory signal have more difficulty segmenting words from fluent speech if familiarized with the words presented in both speech and sign compared to familiarization with the words presented in speech only. STUDY DESIGN: Experiment utilizing an infant-controlled visual preference procedure. METHODS: Twenty 8.5-month-old normal-hearing infants completed testing. Infants were familiarized with repetitions of words in either the speech + sign (n = 10) or the speech only (n = 10) condition. Infants were then presented with four six-sentence passages using an infant-controlled visual preference procedure. Every sentence in two of the passages contained the words presented in the familiarization phase, whereas none of the sentences in the other two passages contained familiar words. RESULTS: Infants exposed to the speech + sign condition looked at familiar word passages for 15.3 seconds and at nonfamiliar word passages for 15.6 seconds, t (9) = -0.130, p = .45. Infants exposed to the speech only condition looked at familiar word passages for 20.9 seconds and to nonfamiliar word passages for 15.9 seconds. This difference was statistically significant, t (9) = 2.076, p = .03. CONCLUSIONS: Infants' ability to segment words from degraded speech is negatively affected when these words are initially presented in simultaneous speech and sign. The current study suggests that a decreased ability to segment words from fluent speech may contribute towards the poorer performance of pediatric cochlear implant recipients in total communication settings on a wide range of spoken language outcome measures.

The ear is connected to the brain: some new directions in the study of children with cochlear implants at Indiana University.

Since the early 1980s, the DeVault Otologic Research Laboratory at the Indiana University School of Medicine has been on the forefront of research on speech and language outcomes in children with cochlear implants. This paper highlights work over the last decade that has moved beyond collecting speech and language outcome measures to focus more on investigating the underlying cognitive, social, and linguistic skills that predict speech and language outcomes. This recent work reflects our growing appreciation that early auditory deprivation can affect more than hearing and speech perception. The new directions include research on attention to speech, word learning, phonological development, social development, and neurocognitive processes. We have also expanded our subject populations to include infants and children with additional disabilities.

Word learning in deaf children with cochlear implants: effects of early auditory experience.

Word-learning skills were tested in normal-hearing 12- to 40-month-olds and in deaf 22- to 40-month-olds 12 to 18 months after cochlear implantation. Using the Intermodal Preferential Looking Paradigm (IPLP), children were tested for their ability to learn two novel-word/novel-object pairings. Normal-hearing children demonstrated learning on this task at approximately 18 months of age and older. For deaf children, performance on this task was significantly correlated with early auditory experience: Children whose cochlear implants were switched on by 14 months of age or who had relatively more hearing before implantation demonstrated learning in this task, but later implanted profoundly deaf children did not. Performance on this task also correlated with later measures of vocabulary size. Taken together, these findings suggest that early auditory experience facilitates word learning and that the IPLP may be useful for identifying children who may be at high risk for poor vocabulary development.

Developmental trajectories of forward and backward digit spans in deaf children with cochlear implants.

BACKGROUND: Accounting for outcome variability among pediatric cochlear implant (CI) recipients is an enduring clinical challenge. Short-term memory and working memory (STM/WM), as indexed by digit span forward (DSF) and digit span backward (DSB), have been shown to be strongly correlated with speech and language (S/L) outcomes. The enormous variability observed in conventional outcome measures of S/L may reflect individual differences in STM/WM. METHODS: Repeated measure auditory digit spans were obtained from 110 children (age 3-15 years; mean 7.2 years) with at least 2 years of CI use. Growth curves were computed for each child, and linear functions were fit to both DSF and DSB. Slopes and intercepts were used as parameters in mixed-models to assess relations between STM/WM capacity change over time and S/L outcome measures including vocabulary (PPVT), open-set set spoken word recognition (PBK), and sentence perception (HINT-C). RESULTS: For DSF, the percent of the sample more than 1 SD below the norm at each age ranged from 54 to 90% (mean = 66.5%). For DSB, the percent of the sample more than 1 SD below the norm at each age ranged from 23 to 42% (mean = 34.5%) at ages where there were at least five children. Four subgroups within our CI sample emerged: (Subgroup 1) children demonstrating age-appropriate growth in both DSF and DSB scores over time (49/110, 44.55%); (Subgroup 2) children demonstrating age appropriate growth in DSF over time but below average growth in DSB over time (23/110, 20.91%); (Subgroup 3) children demonstrating below average growth in DSF over time but age-appropriate growth in DSB over time (19/110, 17.27%); and (Subgroup 4) children demonstrating below average growth in both DSF and DSB over time (19/110, 17.27%). For all tests except CELF-3, Subgroup 4 demonstrated the poorest performance among the four DS slope subgroups. Significant differences were observed between Subgroup 1 and Subgroup 4 on last visit PBK-Word (P = 0.029), PPVT (P = 0.018), and HINT-C in Quiet (P = 0.001), but not CELF-3 (P = 0.433). CONCLUSION: The findings from this longitudinal study suggest that differences in the rate of development of STM/WM may influence S/L outcomes in children with CIs. The clinical implications of these findings are significant because they indicate that the rate of development of STM/WM, and not just the actual level of STM/WM at a single time point, predicts later S/L development in this clinical population. Targeted interventions to improve developmental rate of verbal STM/WM may hold promise for enhancing S/L skills in children with CIs.

A mosaic activating mutation in AKT1 associated with the Proteus syndrome.

BACKGROUND: The Proteus syndrome is characterized by the overgrowth of skin, connective tissue, brain, and other tissues. It has been hypothesized that the syndrome is caused by somatic mosaicism for a mutation that is lethal in the nonmosaic state. METHODS: We performed exome sequencing of DNA from biopsy samples obtained from patients with the Proteus syndrome and compared the resultant DNA sequences with those of unaffected tissues obtained from the same patients. We confirmed and extended an observed association, using a custom restriction-enzyme assay to analyze the DNA in 158 samples from 29 patients with the Proteus syndrome. We then assayed activation of the AKT protein in affected tissues, using phosphorylation-specific antibodies on Western blots. RESULTS: Of 29 patients with the Proteus syndrome, 26 had a somatic activating mutation (c.49G→A, p.Glu17Lys) in the oncogene AKT1, encoding the AKT1 kinase, an enzyme known to mediate processes such as cell proliferation and apoptosis. Tissues and cell lines from patients with the Proteus syndrome harbored admixtures of mutant alleles that ranged from 1% to approximately 50%. Mutant cell lines showed greater AKT phosphorylation than did control cell lines. A pair of single-cell clones that were established from the same starting culture and differed with respect to their mutation status had different levels of AKT phosphorylation. CONCLUSIONS: The Proteus syndrome is caused by a somatic activating mutation in AKT1, proving the hypothesis of somatic mosaicism and implicating activation of the PI3K-AKT pathway in the characteristic clinical findings of overgrowth and tumor susceptibility in this disorder. (Funded by the Intramural Research Program of the National Human Genome Research Institute.).

Effects of early auditory experience on word learning and speech perception in deaf children with cochlear implants: implications for sensitive periods of language development.

HYPOTHESIS: That early word learning and speech perception skills have different sensitive periods and that very early implantation may affect later vocabulary outcomes more than speech perception outcomes. BACKGROUND: Several studies have found that deaf children who receive cochlear implants before 3 years of age tend to have better speech perception outcomes than children implanted later. Recent studies have not found age-at-implantation effects on speech perception or central auditory processing among children implanted younger than 2 years, suggesting that there may be a sensitive period for speech perception skills that closes by around 3 years of age. There has been very little work investigating possible sensitive periods for other language skills, such as the ability to learn words. Recent work suggests the possibility that the development of word-learning skills may have an earlier sensitive period than the development of speech perception skills. METHODS: Assess speech perception and vocabulary outcomes in children implanted before 13 months of age and in children implanted between 16 and 23 months of age. RESULTS: Children implanted during the first year of life had better vocabulary outcomes than children implanted during the second year of life. However, earlier implanted children did not show better speech perception outcomes than later implanted children. CONCLUSION: There may be an earlier sensitive period for developing the ability to associate the sound patterns of words to their referents than for developing speech perception and central auditory processing skills.

Effects of congenital hearing loss and cochlear implantation on audiovisual speech perception in infants and children.

PURPOSE: Cochlear implantation has recently become available as an intervention strategy for young children with profound hearing impairment. In fact, infants as young as 6 months are now receiving cochlear implants (CIs), and even younger infants are being fitted with hearing aids (HAs). Because early audiovisual experience may be important for normal development of speech perception, it is important to investigate the effects of a period of auditory deprivation and amplification type on multimodal perceptual processes of infants and children. The purpose of this study was to investigate audiovisual perception skills in normal-hearing (NH) infants and children and deaf infants and children with CIs and HAs of similar chronological ages. METHODS: We used an Intermodal Preferential Looking Paradigm to present the same woman's face articulating two words ("judge" and "back") in temporal synchrony on two sides of a TV monitor, along with an auditory presentation of one of the words. RESULTS: The results showed that NH infants and children spontaneously matched auditory and visual information in spoken words; deaf infants and children with HAs did not integrate the audiovisual information; and deaf infants and children with CIs initially did not initially integrate the audiovisual information but gradually matched the auditory and visual information in spoken words. CONCLUSIONS: These results suggest that a period of auditory deprivation affects multimodal perceptual processes that may begin to develop normally after several months of auditory experience.

Cochlear implants and spoken language processing abilities: review and assessment of the literature.

Cochlear implants (CIs) process sounds electronically and then transmit electric stimulation to the cochlea of individuals with sensorineural deafness, restoring some sensation of auditory perception. Many congenitally deaf CI recipients achieve a high degree of accuracy in speech perception and develop near-normal language skills. Post-lingually deafened implant recipients often regain the ability to understand and use spoken language with or without the aid of visual input (i.e. lip reading). However, there is wide variation in individual outcomes following cochlear implantation, and some CI recipients never develop useable speech and oral language skills. The causes of this enormous variation in outcomes are only partly understood at the present time. The variables most strongly associated with language outcomes are age at implantation and mode of communication in rehabilitation. Thus, some of the more important factors determining success of cochlear implantation are broadly related to neural plasticity that appears to be transiently present in deaf individuals. In this article we review the expected outcomes of cochlear implantation, potential predictors of those outcomes, the basic science regarding critical and sensitive periods, and several new research directions in the field of cochlear implantation.

Large asymptomatic pneumocephalus developing years after middle cranial fossa surgery--a case report.

Whereas acute pneumocephalus has been well characterized in the literature: typically seen postoperatively after neurosurgical and neurotological procedures, a chronic pneumocele developing several years after surgery is highly unusual. Most otogenic pneumocephali develop in an acute or subacute fashion, presenting with focal neurological symptoms, headache or signs of meningitis secondary to translocation of bacteria into the cavity. We describe a patient with a supra-auricular soft-tissue swelling as the only presenting symptom of a large chronic epidural pneumocele with extension into the extracranial subcutaneous tissues. It presented several years after surgical resection of a meningioma that involved the temporal bone.

Building a cathedral for your soul: generosity as a virtue and a practice.

OBJECTIVE: To evaluate the virtue of generosity as it relates to how we are what we are. STUDY DESIGN: Systematic review of the literature. RESULTS: Generosity has to do with our very being and our behaving, with our living as caring, respectful, courteous, grace-giving people. Authentic generosity is rooted in our unique sense of calling. Generosity is an interactive, interpersonal two-way relationship of trust and respect between doctor and patient, student and teacher. How do we nurture generosity? Generous people are reflective people who take inventory of their souls. CONCLUSION: Building a cathedral for your soul has to do with tomorrow as well as with today, with how we choose to be remembered, and with the kind of spiritual legacy that we are crafting by the way we live.

Outcomes in bilateral cochlear implantation.

OBJECTIVE: The objective of this study was to explore improvements in quality of life and the cost-utility of bilateral cochlear implantation. STUDY DESIGN AND SETTING: A prospective case-control study was conducted on 23 bilateral cochlear implant patients with the Mark III health utility index. RESULTS: Results indicate a 0.48 mean gain in health utility after bilateral cochlear implantation and a discounted cost per quality adjusted life year of $24,859 in this cohort of patients. With a comparison of patient scores for unilateral and bilateral use, improvements in the domains of hearing, speech, emotion, and cognition were noted, resulting in a mean gain in health utility of 0.11. CONCLUSIONS: This study found an improvement in quality of life and a favorable cost-utility associated with bilateral cochlear implantation in patients with profound hearing loss. These patients showed additional improvements in quality of life after they received their second implant. CLINICAL SIGNIFICANCE: This is the first study that showed improvements in quality of life and a favorable cost-utility after bilateral cochlear implantation in patients with profound hearing loss.

Language skills of profoundly deaf children who received cochlear implants under 12 months of age: a preliminary study.

CONCLUSION: This study demonstrated that children who receive a cochlear implant below the age of 2 years obtain higher mean receptive and expressive language scores than children implanted over the age of 2 years. OBJECTIVE: The purpose of this study was to compare the receptive and expressive language skills of children who received a cochlear implant before 1 year of age to the language skills of children who received an implant between 1 and 3 years of age. SUBJECTS AND METHODS: Standardized language measures, the Reynell Developmental Language Scale (RDLS) and the Preschool Language Scale (PLS), were used to assess the receptive and expressive language skills of 91 children who received an implant before their third birthday. RESULTS: The mean receptive and expressive language scores for the RDLS and the PLS were slightly higher for the children who were implanted below the age of 2 years compared with the children who were implanted over 2 years old. For the PLS, both the receptive and expressive mean standard scores decreased with increasing age at implantation.

Visual-motor integration skills of prelingually deaf children: implications for pediatric cochlear implantation.

OBJECTIVE: To investigate visual-motor integration (VI) skills of prelingually deaf (PLD) children before and after cochlear implantation (CI) and investigate correlations with spoken-language and related processing measures. DESIGN: Study 1 was a longitudinal study in which VI was tested preimplant. Study 2 was a cross sectional study of school-age children who used a CI for >2 years. METHOD: In study 1, a standardized design-copying task was administered preimplant, and spoken-language data were obtained at intervals up to 4 years postimplantation. Analyses were conducted to determine if preimplant VI scores were predictive of various spoken-language measures. In study 2, standardized design copying and speeded maze tracing tasks were administered along with speech perception, vocabulary, and related processing measures. RESULTS: Whereas preimplant VI scores for children in study 1 fell within the typical range based on age-equivalent norms, postimplant VI standard scores in study 2 were low compared to the normative sample. Postimplant VI scores were inversely related to age at implantation. Preimplant VI scores were robustly predictive of most, but not all, spoken-language outcome scores. Postimplant design copying scores were also correlated with spoken-language and related processing measures whereas maze-tracing scores were less robustly related to these measures. CONCLUSIONS: Early auditory and linguistic experience may impact the development of VI skills. VI is a preimplant predictor of later spoken language outcomes. Design copying and speeded maze tracing tasks appear to tap different sets of cognitive resources in PLD children with CIs.