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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 FLEX24 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.

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. Keywords: Infants, Cochlear implantation, Treatment outcome

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.

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.

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

A b s t r ac t 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 phosphorylationspecific 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.)

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.

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.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 for2 years.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.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.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.

The benefit of early cochlear implantation (CI) to later speech perception outcomes in prelingually deaf (PLD) children is well established and implantation of infants has become more prevalent. The aim of this study was to determine whether or not deaf infants could discriminate audiovisual non-words shortly after CI and whether their attention to speech and non-speech audiovisual stimuli was similar to infants with normal hearing (NH). Three groups of participants were tested: PLD infants tested prior to CI (pre-CI), PLD infants tested post-implantation (post-CI), and a group of age-matched NH infants. A novel version of the visual habituation (VH) procedure was used. Infants were presented repetitions of an audiovisual non-word until their looking-time decreased to a predetermined criterion. They were then presented two types of test trials: repetitions of the old word (non-alternating (NA) trials) and repetitions of a novel non-word alternating with the old word (alternating (A) trials). Longer lookin...

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.

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.

Profound sensorineural hearing loss secondary to cochlear dysplasia presents a number of surgical challenges during cochlear implantation. The standard transmastoid-facial recess approach can be performed in the majority of cases. In cases of common cavity deformity, the transmastoid labyrinthotomy approach has a number of advantages. A high incidence of CSF gushers occurs in this population but can be managed by creating a small cochleostomy and tightly sealing the cochleostomy with connective tissue. Acceptable postoperative speech perception results can be expected.

Objectives/Hypothesis: Individual speech and language outcomes of deaf children with cochlear implants (CIs) are quite varied. Individual differences in underlying cognitive functions may explain some of this variance. The current study investigated whether behavioral inhibition skills of deaf children were related to performance on a range of audiologic outcome measures. Design: Retrospective analysis of longitudinal data collected from prelingually and profoundly deaf children who used CIs. Methods: Behavioral inhibition skills were measured using a visual response delay task that did not require hearing. Speech and language measures were obtained from behavioral tests administered at 1-year intervals of CI use. Results: Female subjects showed higher response delay scores than males. Performance increased with length of CI use. Younger children showed greater improvement in performance as a function of device use than older children. No other subject variable had a significant effect on response delay score. A series of multiple regression analyses revealed several significant relations between delay task performance and open set word recognition, vocabulary, receptive language, and expressive language scores. Conclusions: The present results suggest that CI experience affects visual information processing skills of prelingually deaf children. Furthermore, the observed pattern of relations suggests that speech and language processing skills are closely related to the development of response delay skills in prelingually deaf children with CIs. These relations may reflect underlying verbal encoding skills, subvocal rehearsal skills, and verbally mediated self-regulatory skills. Clinically, visual response delay tasks may be useful in assessing behavioral and cognitive development in deaf children after implantation.

We investigated relations between sustained visual attention, behavioral inhibition skills, and speech–language outcomes in prelingually deaf children who use cochlear implants (CIs) using two computerized continuous performance tasks (CPTs). One test measured their ability to sustain visual attention to a string of numbers and another test measured their ability to delay a behavioral response. Performance on latter task was related to postimplant scores on tests of vocabulary knowledge, language skills, and speech intelligibility. We conclude that behavioral inhibition skills of prelingually deaf children are related to several audiological outcome measures in deaf children with CIs. Our findings suggest that further investigation is warranted into executive functions and subvocal rehearsal skills of deaf children with CIs.

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

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.

Objectives In this investigation, we report the outcomes of 14 adults (age >18 y) and 9 children (age

A group of 20 patients with large vestibular aqueduct syndrome was identified at the Indiana University School of Medicine. The major objective of this study was to explore the improvements in quality of life associated with cochlear implantation in patients with large vestibular aqueduct syndrome, as well as the cost-utility of cochlear implantation in this group.A total of 70 patients were identified with large vestibular aqueduct syndrome through analysis of thin-section computed tomography of the temporal bone over the past 6 years at this medical center. Data collected from the medical records for each patient included demographic data, hearing-related statistics, implantation data, and audiometric data. Sixteen children and adults with large vestibular aqueduct syndrome had undergone cochlear implantation before the beginning of this study, and the remaining 54 children and adults were identified as undergoing treatment of progressive or fluctuant sensorineural hearing loss. Health utility indexes used in this analysis were taken through the use of the Ontario Health Utility Index, Mark III. The range of costs used for cost-utility analysis was derived from the costs of cochlear implantation at this medical center, as well as from costs associated with implantation published in the medical literature.Participants were selected from the total population of patients with large vestibular aqueduct syndrome at this center who were postlingually deafened and who currently had severe hearing loss. Two groups were formed. These groups comprised either cochlear implant patients with large vestibular aqueduct syndrome or patients with large vestibular aqueduct syndrome currently using hearing aids. Ten of the 16 cochlear implant patients and 10 of the remaining 54 patients with large vestibular aqueduct syndrome met these criteria. Mark III health utility indexes were distributed to patients in each group and scored. Those health utility indexes not completed by the patients were scored by proxy, using the audiologist at this center who was the most familiar with the patient. Changes in quality of life associated with cochlear implantation were derived by comparison of the health utility index results of the two groups. Cost-utility measures were then made using discounted costs per quality-of-life years (QALYs) (5%), and a sensitivity analysis was performed that evaluated changes in scoring done by proxy. The cost-utility results were then compared with the cost-utilities derived from similar studies and associated with other disease states.Although both groups of patients had significant hearing loss, the hearing aid group had a better mean pure-tone average. The mean pure-tone average for the hearing aid group was 70.8 dB (SD 24.4), and the mean pure-tone average for the cochlear implant group was 107.0 dB (SD 21.7). Seven of the 20 health utility indexes were scored by proxy. Results from the base case indicate a 0.20 gain in health utility from cochlear implantation (hearing aid = 0.62, cochlear implant = 0.82, p = 0.037), resulting in a discounted cost per QALY of $12,774. Sensitivity analysis of the proxy scoring indicated that decreasing the hearing score one level on the health utility index resulted in a gain in health utility with cochlear implantation of 0.15, resulting in a discounted cost per QALY of $17,832. A decrease of the hearing score by two levels on the health utility survey resulted in no significant gain in quality of life with cochlear implantation.This study found an improvement in quality of life associated with cochlear implantation in postlingually deafened patients with large vestibular aqueduct syndrome. By weighing this improvement in quality of life against the significant difference noted between the pure-tone averages of each group, further strength can be given to this conclusion. This gain in quality of life, as well as the results derived for the cost-utility of cochlear implantation, was similar to that in previous published studies of cochlear implantation in all types of patients. These results also indicate a favorable cost-utility when compared with published data about other disease states. As patients with large vestibular aqueduct syndrome progress to profound levels of hearing loss, these results indicate that cochlear implantation can be offered as a beneficial, life-improving therapy.

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.

Cochlear implants are electronic prostheses that provide a high quality sense of hearing to severely and profoundly deaf children and adults. As improvements in surgical technique and device performance have occurred, indications for implantation have expanded.

Although cochlear implants improve the ability of profoundly deaf children to understand speech, critics claim that the published literature does not document even a single case of a child who has developed a linguistic system based on input from an implant. Thus, it is of clinical and scientific importance to determine whether cochlear implants facilitate the development of English language skills. The English language skills of prelingually deaf children with cochlear implants were measured before and after implantation. We found that the rate of language development after implantation exceeded that expected from unimplanted deaf children (p < .001) and was similar to that of children with normal hearing. Despite a large amount of individual variability, the best performers in the implanted group seem to be developing an oral linguistic system based largely on auditory input obtained from a cochlear implant.

DFNA9 is an autosomal dominant, nonsyndromic, progressive sensorineural hearing loss with vestibular pathology. Here we report three missense mutations in human COCH (previously described as Coch5b2), a novel cochlear gene, in three unrelated kindreds with DFNA9. All three residues mutated in DFNA9 are conserved in mouse and chicken Coch, and are found in a region containing four conserved cysteines with homology to a domain in factor C, a lipopolysaccharide-binding coagulation factor in Limulus polyphemus. COCH message, found at high levels in human cochlear and vestibular organs, occurs in the chicken inner ear in the regions of the auditory and vestibular nerve fibres, the neural and abneural limbs adjacent to the cochlear sensory epithelium and the stroma of the crista ampullaris of the vestibular labyrinth. These areas correspond to human inner ear structures which show histopathological findings of acidophilic ground substance in DFNA9 patients.

This study examined the development of visual attention in 5- to 13-year-olds who differed in their access to sound. Hearing children, deaf children with cochlear implants, and deaf children without implants participated in a task in which they were to respond to some visual signals and not others. The results of Experiment 1 indicated that the timing of developmental changes in visual selective attention was similar for all 3 groups, occurring around 8 years. The magnitude of age-related change differed among groups; hearing children and older deaf children using a cochlear implant reached higher levels of performance with age than did deaf children without enhanced access to sound. The results of Experiment 2 suggest that the developmental differences between deaf children with and without cochlear implants begin prior to 8 years and may be related to their use of environmental sounds to organize visual attention.

The transmastoid facial recess approach has become the standard technique for cochlear implantation. Although this approach has been used for implantation in patients with common cavity deformities, it is not without increased risk to the facial nerve. Using a direct approach to the common cavity that circumvents the facial recess, we have successfully implanted four patients with common cavity deformities. An aberrant facial nerve in one patient would have precluded placement of the electrode array using standard cochlear implant techniques. As demonstrated in these four patients, the direct approach to the common cavity is an effective approach for placement of the electrode array, minimizes risk to the facial nerve, and may decrease the likelihood of postoperative cerebrospinal fluid leaks. Intraoperative video footage demonstrates the feasibility and facility of this approach in patients with common cavity deformities.

Background Current experience with carotid body tumors suggesting a high prevalence of associated cervical paragangliomas prompted this review. Patients and methods An 8-year retrospective study of patients with carotid body tumors was undertaken, detailing presentation, diagnosis, and treatment. Results Eleven patients harboring 17 carotid body tumors were discovered. All patients had a neck mass. Seven patients (64%) had bilateral carotid body tumors. Six (55%) reported a positive family history-4 were first-generation relatives, 5 had bilateral tumors, and 3 had other head and neck paragangliomas. Angiography documented 4 associated vagal and 2 glomus jugulare paragangliomas in addition to the carotid body tumors. Precise surgical care limited blood loss to an average of 590 cc. The carotid artery was repaired during 5 resections (29%). Cranial nerve injury occurred in 3 cases, all following vagal body or glomus jugulare resection. Every patient is currently alive, stroke free, and functioning without major disability. Conclusions Patients with carotid body tumors have a propensity for multiple head and neck paragangliomas. Angiography is diagnostic. The need for associated paraganglioma resection dramatically increases the risk of cranial nerve injury.

The field of pediatric cochlear implantation has changed tremendously since the early 1980s when single‐channel cochlear implants were first provided to postlingually deafened children in the United States. Although implantation in children was at first restricted to older children with postlingually acquired hearing loss, it is now common for prelingually deafened children as young as two years of age to receive a cochlear implant. Furthermore, the introduction of multichannel cochlear implants and improved signal processing strategies have resulted in far greater speech perception benefits than originally thought possible. This presentation will review the major developments in pediatric implantation and summarize the results to date. [Work supported by NIDCD Grant Nos. DC00064 and DC00423.]

The acquisition of speech perception and speech production skills emerges over a protracted time course in congenitally deaf children with multichannel cochlear implants (CI). Only through comprehensive, longitudinal studies can the full impact of cochlear implantation be assessed. In this study, the performance of CI users was examined longitudinally on a battery of speech perception measures and compared with subjects with profound hearing loss who used conventional hearing aids (HA). The average performance of the multichannel cochlear implant users gradually increased over time and continued to improve even after 5 years of CI use. Speech intelligibility was assessed from recordings of the subjects' elicited speech and played to panels of listeners. Intelligibility was scored in terms of percentage of words correctly understood. The average scores for subjects who had used their CI for 4 years or more exceeded 40%.

This study investigated the perception and production of Thai tones by an adult, native speaker of Thai with a severe to-profound sensorineural hearing impairment. Five Thai words minimally distinguished by tone were presented for identification in both natural speech and synthetic speech versions. The same five words were produced by the hearing-impaired speaker. Results indicated that both tone perception and production were impaired, but that the magnitude of the deficit was greater in perception.

We investigated the predictive relations between pre-implant visuomotor integration ability and subsequent oral speech/language outcomes in prelingually deaf children who use cochlear implants (CIs). Prior to implantation, children were given a task that tested their accuracy in copying geometric forms. Performance on this task predicted speech perception, sentence comprehension, and speech intelligibility outcomes over 3 years of CI use. We conclude that individual differences in visuomotor integration ability are predictive of some audiological outcome measures in deaf children with CIs.

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.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).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.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 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.

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.

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.Experiment utilizing an infant-controlled visual preference procedure.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.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.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.

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. Key Words: ChildrenVCochlear implantsVInfantsVSensitive periodsVSpeech perceptionV Word learning.

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.

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.

The present results demonstrated that all 3 factors --lexical difficulty, stimulus variability, and word length--significantly influenced spoken word recognition by children with multichannel cochlear implants. Lexically easy words were recognized significantly better than lexically hard words, regardless of talker condition or word length of the stimuli. These results support the earlier findings of Kirk et al(12) obtained with live-voice stimulus presentation and suggest that lexical effects are very robust. Despite the fact that listeners with cochlear implants receive a degraded speech signal, it appears that they organize and access words from memory relationally in the context of other words. The present results concerning talker variability contradict those previously reported in the literature for listeners with normal hearing(7,11) and for listeners with mild-to-moderate hearing loss who use hearing aids.(14) The previous investigators used talkers and word lists different from those used in the current study and found that word recognition declined as talker variability increased. In the current study, word recognition was better in the multiple-talker condition than in the single-talker condition. Kirk(15) reported similar results for postlingually deafened adults with cochlear implants who were tested on the recorded word lists used in the present study. Although the talkers were equally intelligible to listeners with normal hearing in the pilot study, they were not equally intelligible to children or adults with cochlear implants. It appears that either the man in the single-talker condition was particularly difficult to understand or that some of the talkers in the multiple-talker condition were particularly easy to understand. Despite the unexpected direction of the talker effects, the present results demonstrate that children with cochlear implants are sensitive to differences among talkers and that talker characteristics influence their spoken word recognition. We are conducting a study to assess the intelligibility of each of the 6 talkers to listeners with cochlear implants. Such studies should aid the development of equivalent testing conditions for listeners with cochlear implants. There are 2 possible reasons the children in the present study identified multisyllabic words better than monosyllabic words. First, they may use the linguistic redundancy cues in multisyllabic words to aid in spoken word recognition. Second, multisyllabic words come from relatively sparse lexical neighborhoods compared with monosyllabic tokens. That is, multisyllabic words have fewer phonetically similar words, or neighbors, competing for selection than do monosyllabic stimuli. These lexical characteristics most likely contribute to the differences in identification noted as a function of word length. The significant lexical and word length effects noted here may yield important diagnostic information about spoken word recognition by children with sensory aids. For example, children who can make relatively fine phonetic distinctions should demonstrate only small differences in the recognition of lexically easy versus hard words or of monosyllabic versus multisyllabic stimuli. In contrast, children who process speech using broad phonetic categories should show much larger differences. That is, they may not be able to accurately encode words in general or lexically hard words specifically. Further study is warranted to determine the interaction between spoken word recognition and individual word encoding strategies.

The speech perception abilities of 37 children with cochlear implants (single-channel or multichannel) were examined as a function of age at onset of deafness. There was no significant difference in the speech perception abilities of implanted children who were born deaf and those of implanted children who lost their hearing during the first 3 years of life. In contrast, the performance of children whose age at onset of deafness was 5 years or later was significantly better than that of the children with congenital or early-acquired deafness on tests of stress pattern categorization, closed-set word identification, open-set identification of common phrases, and lipreading enhancement.

The speech perception abilities of deaf children with a single- or multi-channel cochlear implant are compared with those of deaf children who derive substantial benefit from conventional hearing aids. The children with hearing aids have unaided pure-tone thresholds ranging from 90- to 110-dB HL through at least 2000 Hz, and aided thresholds of 30- to 60-dB HL. The group data show that the speech perception scores of the subjects with hearing aids were significantly higher than those of the subjects with implants on a range of speech perception measures. Although a few subjects with implants achieved scores as high as those who used hearing aids, the majority did not. Even though the children with implants receive substantial benefit from their devices, they continue to have limited auditory perception abilities relative to their peers who derive benefit from conventional hearing aids. The data highlight the importance of establishing hearing aid benefit in potential candidates for implant.

ProblemThere is a paucity of instruments available to assess speech discrimination in normal-hearing (NH) and hearing-impaired (HI) infants. We assess the utility of a hybrid visual habituation paradigm (HVHP) in assessing speech discrimination and predicting later language ability.MethodsNH infants were tested with the HVHP on an easier vowel contrast or a more difficult fricative contrast. Parents completed Macarthur Communicative Development Inventories (CDI) when infants were 14,18,24, and 30 months old to assess correlations between discrimination abilities and later language ability. Similarly, HVHP and CDI measures were obtained from HI infants at following hearing aid (HA) or cochlear implant (CI) use.Results29% of NH infants significantly discriminated the vowel contrast. 6% of NH infants significantly discriminated the fricative contrast. Effect size of fricative discrimination at 9 months correlated with early gestures (n=10), r=.73 p=.02, and later gestures (n=10, r=.81 p = .01), at 14 months ...

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.