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.).

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.

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.

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.

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.

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.

Enhanced survival of bone-marrow-derived pluripotent stem cells in an animal model of auditory neuropathy.

OBJECTIVE: The loss of spiral ganglion neurons (SGNs) is one of the major causes of profound sensorineural hearing loss (SNHL). Stem cell replacement therapy, which is still in its infancy, has the potential to treat or cure those who suffer from an array of illnesses and degenerative neurologic disorders, including sensorineural deafness (SNHL). Little is known about the potentials of mesenchymal stem cells (MSCs) and their ability to take on properties of SGNs. The two main purposes of this study were to evaluate the survival of mouse MSCs transplanted into normal and ouabain-treated gerbil cochleae and to determine the migratory patterns of MSCs with two differing injection methods. SUBJECTS: Thirty-two Mongolian gerbils, 3 to 4 months old, were used as recipients, and four 6-week-old TgN(ACTbEGFP) mice that ubiquitously express green fluorescent protein (GFP) were used as donors. DESIGN: The animals were deafened by ouabain, which damaged SGNs while leaving hair cell systems intact. After 4 weeks of recovery, the animals received an intraperilymphatic transplantation of 1.0x10(6) GFP-positive undifferentiated MSCs via two different injection methods: scala tympani injection and modiolar injection. Seven days after the transplantation, the survival of MSCs was evaluated by microscopic examination of frozen sections cut through the cochleae of the recipient animals. The number of profiles was counted on the five most central modiolar sections. One-way analyses of variance (ANOVA) were used to determine any significantdifferences among mean profile counts across the experimental conditions. RESULTS: Our findings indicated that undifferentiated MSCs were able to survive in the modiolus both in the control and the ouabain-treated cochleae. The average number of profiles found in the modiolus was greater in the ouabain-treated cochleae than in the control cochleae. This difference was statistically significant (P<.01) as determined using a one-way ANOVA and an ad hoc Tukey-Kramer's test. With the scala tympani injection, there were no profiles found in the modiolus either in the control or ouabain-treated cochleae. This finding may indicate that donor MSCs need to be directly injected into the modiolus to replace injured SGNs. Finally, there was no evidence of hyperacute rejection in any of the gerbils despite the use of xenotransplantation. CONCLUSIONS: These findings may have important clinical implications as a means of delivering MSCs in the cochlea for stem-cell replacement therapy. Survival of transplanted MSCs into the modiolus of the cochlea may result in regeneration of damaged SGNs.

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.

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.

Divergence of fine and gross motor skills in prelingually deaf children: implications for cochlear implantation.

OBJECTIVE: The objective of this study was to assess relations between fine and gross motor development and spoken language processing skills in pediatric cochlear implant users. STUDY DESIGN: The authors conducted a retrospective analysis of longitudinal data. METHODS: Prelingually deaf children who received a cochlear implant before age 5 and had no known developmental delay or cognitive impairment were included in the study. Fine and gross motor development were assessed before implantation using the Vineland Adaptive Behavioral Scales, a standardized parental report of adaptive behavior. Fine and gross motor scores reflected a given child's motor functioning with respect to a normative sample of typically developing, normal-hearing children. Relations between these preimplant scores and postimplant spoken language outcomes were assessed. RESULTS: In general, gross motor scores were found to be positively related to chronologic age, whereas the opposite trend was observed for fine motor scores. Fine motor scores were more strongly correlated with postimplant expressive and receptive language scores than gross motor scores. CONCLUSIONS: Our findings suggest a disassociation between fine and gross motor development in prelingually deaf children: fine motor skills, in contrast to gross motor skills, tend to be delayed as the prelingually deaf children get older. These findings provide new knowledge about the links between motor and spoken language development and suggest that auditory deprivation may lead to atypical development of certain motor and language skills that share common cortical processing resources.

In vivo and in vitro characterization of bone marrow-derived stem cells in the cochlea.

OBJECTIVES/HYPOTHESIS: Stem cell replacement therapy has the potential to treat or cure an array of degenerative neurologic disorders, including sensorineural deafness. However, little is known about the potential for marrow-derived stem cells (MSCs) to take on properties of spiral ganglion neurons. The main purpose of this prospective animal study was to evaluate the survival of MSCs transplanted into the gerbil cochlea. METHODS: Eight 3- to 4-month-old Mongolian gerbils were used as recipients. The animals received an intraperilymphatic transplantation of 100,000 green fluorescent protein (GFP)-positive MSCs with scala tympani injection and modiolar injection. Seven days after transplantation, MSC survival was evaluated by microscopic examination of frozen sections cut through the cochleae of the recipient animals. RESULTS: MSCs isolated from the TgN (ACTbEGFP) mouse line used in this study exhibited bright green florescence after five to seven passages in vitro. Seven days after postoperatively, most transplanted MSCs were found in the scala tympani and scala vestibule and only a small number located in the scala media in animals that received both forms of injection. There were no GFP-positive MSCs in the modiolus in animals with scala tympani injection. In contrast, the mean profile count in animals with modiolar injection was 28, which was the highest in all regions. Although MSCs have the potential to migrate, the anatomic barrier between the perilymphatic space and the modiolus might account for the absence of GFP-positive MSCs in this region. CONCLUSION: These findings may have important clinical implications as a means of delivering MSCs in the cochlea for cell replacement therapy.

Cochlear implantation in deaf infants.

OBJECTIVES: With the application of universal newborn hearing screening programs, a large pool of newly identified deaf infants has been identified. The benefits of early intervention with cochlear implants (CI) is being explored. Mounting evidence suggests that age at implantation is a strong predictor of language outcomes. However, new behavioral procedures are needed to measure speech and language skills during infancy. Also, procedures are needed to analyze the speech input to young CI recipients. STUDY DESIGN: Cohort-sequential. METHODS: Thirteen infants with profound hearing loss who were implanted between the ages of 6 to 12 months of age participated in this study. Eight participated in two new behavioral methodologies: 1) the visual habituation procedure to assess their discrimination of speech sounds; 2) the preferential looking paradigm to assess their ability to learn associations between speech sounds and objects. Older implanted infants and normal-hearing infants were also tested for comparison. The pitch of mothers' speech to infants was analyzed. RESULTS: Patterns of looking times for the very early implanted infants were similar to those of normal hearing infants. Mothers' speech to infants with CIs was similar in pitch to normal-hearing infants who had the same duration of experience with sounds. CONCLUSIONS: No surgical or anesthetic complications occurred in this group of infants, and the pattern of listening skill development mirrors that seen in normal-hearing infants. Mothers adjust their speech to suit the listening experience of their infants.

Behavioral assessment of prelingually deaf children before cochlear implantation.

OBJECTIVES/HYPOTHESIS: To determine whether scores from a behavioral assessment of prelingually deafened children who present for cochlear implant surgery are predictive of audiological outcomes. STUDY DESIGN: Retrospective review of longitudinal data collected from 42 children with prelingual hearing loss who presented for a cochlear implant before age 5 years. METHODS: The Vineland Adaptive Behavioral Scales (VABS) was administered during the preimplant workup. Standardized scores reflect daily living skills, socialization, and motor development compared with a normative sample. Regression analyses were conducted to determine whether any subject variables were related to VABS scores. Mixed-model analyses were computed to determine whether preimplant VABS scores were predictive of longitudinal spoken-language data obtained after cochlear implant surgery. RESULTS: Motor scores tended to be higher than nonmotor scores. Nonmotor scores were significantly lower than the normative mean and decreased with testing age. Children with acquired deafness demonstrated lower motor scores than children with congenital causes. Children with higher motor scores demonstrated significantly higher performance on language, vocabulary, and word recognition tests than children with lower motor scores. Nonmotor domains were not as robustly related to spoken-language measures, although similar trends were observed. CONCLUSION: Profound deafness and language delay may confound the assessment of daily living skills and socialization in the population studied. Motor development appears to proceed normally in prelingually deafened children and is a preimplant predictor of spoken-language outcome in young infants and children with cochlear implants, a finding consistent with the large body of work establishing links between perceptual-motor and language development.

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.

PET imaging of differential cortical activation by monaural speech and nonspeech stimuli.

Positron emission tomography imaging was used to investigate the brain activation patterns of listeners presented monaurally (right ear) with speech and nonspeech stimuli. The major objectives were to identify regions involved with speech and nonspeech processing, and to develop a stimulus paradigm suitable for studies of cochlear-implant subjects. Scans were acquired under a silent condition and stimulus conditions that required listeners to press a response button to repeated words, sentences, time-reversed (TR) words, or TR sentences. Group-averaged data showed activated foci in the posterior superior temporal gyrus (STG) bilaterally and in or near the anterior insula/frontal operculum across all stimulus conditions compared to silence. The anterior STG was activated bilaterally for speech signals, but only on the right side for TR sentences. Only nonspeech conditions showed frontal-lobe activation in both the left inferior frontal gyrus [Brodmann's area (BA) 47] and ventromedial prefrontal areas (BA 10/11). An STG focus near the superior temporal sulcus was observed for sentence compared to word. The present findings show that both speech and nonspeech engaged a distributed network in temporal cortex for early acoustic and prelexical phonological analysis. Yet backward speech, though lacking semantic content, is perceived as speechlike by engaging prefrontal regions implicated in lexico-semantic processing.

Cochlear implantation in adults with prelingual deafness. Part I. Clinical results.

OBJECTIVES/HYPOTHESIS: To examine the hypothesis that the newer generations of cochlear implants could provide considerable speech understanding to late-implanted, prelingually deaf adult patients. STUDY DESIGN: Retrospective review. METHODS: Speech perception scores of 103 patients with long-term prelingual deafness obtained from the recent clinical trials were compared with other previously published results. RESULTS: Unlike patients receiving implants in early childhood, the audiologic performance of most patients with long-term prelingual deafness rapidly approached asymptotic levels within 1 year after implantation. Beginning at 3 months postimplantation, statistically significant improvement was noted in their speech perception scores compared with their preimplant levels. However, the average performance plateau achieved by this group of patients was significantly below the levels published for postlingual patients. No statistically significant differences were observed between the test scores at any testing interval among patients with different devices. CONCLUSIONS: Most patients with long-term prelingual deafness achieve their performance plateau within 1 year after implantation. The results suggest that patient characteristics, rather than device properties, are likely to be the major contributing factor responsible for the observed audiologic outcomes.

Cochlear implantation in adults with prelingual deafness. Part II. Underlying constraints that affect audiological outcomes.

OBJECTIVES/HYPOTHESIS: To discuss the underlying physiological and anatomical constraints on audiological performance of late-implanted prelingually deafened adult cochlear implant patients. STUDY DESIGN: Retrospective review. METHODS: Published literature on the topic of auditory pathway responses to prolonged congenital deafness was reviewed. In particular, the authors sought to identify the anatomical and physiological changes that take place in both the peripheral and central auditory pathways in response to prolonged deafness, as well as how they are altered by chronic electrical stimulation. RESULTS: The currently available evidence suggests that the colonization of the auditory cortex by other sensory modalities is the main limiting factor in postimplantation performance, not the pathological degenerative changes of the auditory nerve, cochlear nucleus, or auditory midbrain. CONCLUSION: The reviewed evidence, although circumstantial, suggests that emphasizing aurally based educational programs before (with hearing aids) and after cochlear implantation could reduce the cortical colonization phenomenon and potentially improve postimplantation audiological performance of patients with long-term prelingual deafness.

Cochlear implantation with large vestibular aqueduct syndrome.

OBJECTIVES: In this investigation, we report the outcomes of 14 adults (age >18 y) and 9 children (age <18 y) with radiographically proven large vestibular aqueduct syndrome (LVAS) who received cochlear implants at Indiana University School of Medicine. STUDY DESIGN: This is a retrospective case-control study detailing the outcomes of 23 patients with LVAS and 46 control patients implanted with Nucleus (Cochlear Corp., Englewood, CO), Clarion (Advanced Bionics Corp., Sylmar, CA), or Med-El (MED-EL Corp., Innsbruck, Austria) cochlear implants. METHODS: Performance data on pure-tone averages, speech detection thresholds, and a variety of auditory and speech recognition tasks from these patients with LVAS were compared with performance data obtained from a matched group of 46 cochlear implant users who did not have LVAS. Specific patient characteristics used in matching included the age of the patient, the age at implant of the patient, and whether the patient was pre- or postlingually deafened. Data for the adult group was analyzed using the Student t test, and data for the pediatric patient group was compared using a chi2 test. RESULTS: The results indicated positive outcomes for both pediatric and adult groups. With both adult and pediatric groups, auditory and speech recognition performance did not differ significantly between those patients with LVAS and control subjects. CONCLUSIONS: This study adds further support for the use of cochlear implantation in patients with LVAS. Cochlear implantation is beneficial and can be offered as an eventual treatment of LVAS if hearing loss progresses to profound levels in these patients.