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Introduction Cochlear implantation is well accepted as the treatment of choice for prelingual deafness in children [1]. However, the safety of routinely performing this procedure on an outpatient basis is debated. We aim to assess immediate postoperative complications that would affect a surgeon's decision to perform pediatric cochlear implantation on an outpatient basis. Methods A retrospective chart review was conducted which included all children 17 years old or younger who underwent cochlear implantation from 2004 to 2014 in a private neurotology practice. The immediate postoperative complication rates and types of complications were then examined. Results A total of 579 cochlear implants were placed in children ages 1–17 years old from 2004 to 2014. The most common complications were nausea/vomiting and dizziness/imbalance. The odds ratio of developing complications in the group ages 1–3 years old versus all other age patients was found to be statistically insignificant (OR 0.90, 95% CI 0.61 to 1.32, p = 0.58). The odds ratio of developing a complication after bilateral implantation compared to unilateral implantation was statistically significant (OR 1.96, 95% CI 1.18 to 3.28, p = 0.01). There was no difference in complication rates when comparing lateral wall and perimodiolar insertions. A total of 6 of 579 (1%) cochlear implants resulted in a complication requiring unplanned medical attention. Conclusions Overall, this series offers a decade of experience in pediatric cochlear implantation that shows a low incidence of the need for unplanned medical attention in the immediate postoperative period. The most common complication seen is Post-operative nausea and vomiting (PONV) that appears to be amenable to outpatient management even in the youngest populations. This supports providers routinely performing pediatric cochlear implantation on an outpatient basis.

The right planum temporale region is typically involved in higher-order auditory processing. After deafness, this area reorganizes to become sensitive to visual motion. This plasticity is thought to support compensatory enhancements to visual ability. In earlier work we showed that enhanced visual motion detection abilities in early-deaf people correlate with cortical thickness in a subregion of the right planum temporale. In the current study, we build on this earlier result by examining the relationship between enhanced visual motion detection ability and white matter structure in this area in the same sample. We used diffusion-weighted magnetic resonance imaging and extracted the measures of white matter structure from a region-of-interest just below the grey matter surface where cortical thickness correlates with visual motion detection ability. We also tested control regions-of-interest in the auditory and visual cortices where we did not expect to find a relationship between visual motion detection ability and white matter. We found that in the right planum temporale subregion, and in no other tested regions, fractional anisotropy, radial diffusivity, and mean diffusivity correlated with visual motion detection thresholds. We interpret this change as further evidence of a structural correlate of cross modal reorganization after deafness. (C) 2016 Elsevier B.V. All rights reserved.

This study aims to enhance understanding of the factors underlying variance in the reading comprehension skills of prelingually deaf individuals. Participants were 213 sixth through tenth graders with prelingual deafness recruited from four orthographic backgrounds (Hebrew, Arabic, English, and German) and allocated to three distinct reading profiles (levels). A sentence comprehension test manipulating the semantic plausibility of sentences and a word processing experiment requiring rapid determination of the semantic relationship between two real words or between a real word and a pseudohomophonic letter string were used to determine the factors distinguishing skilled from less skilled deaf readers. Findings point to deficits in structural (syntactic) knowledge and deficient knowledge structures, rather than differences in phonological processing skills, as making that distinction. Moreover, the acquisition of such knowledge seems to be modified by particularities of the read orthography.

This study aimed to determine the factors underlying the reading comprehension (RC) failure of prelingually deaf Turkish readers. Participants were 77 individuals with prelingual deafness and a hearing control of 78 normally developing hearing individuals selected from three distinct levels of education (3rd–4th graders = elementary; 6th–7th graders = middle; 9th–10th graders = high). We applied an experimental paradigm manipulating the semantic plausibility and syntactic complexity of sentences to compare RC at the sentence level. In line with findings reported for prelingually deaf individuals reading in other orthographies, the deaf participants tested in the present study manifested rather alarmingly impoverished RC skills in comparison to their hearing counterparts. The findings suggest that this reading failure is rooted in an apparent deficiency in the ability to recruit syntactic and prior knowledge to make sense of what they read. The evidence further suggests that such deficits prove strikingly resistant to years of formal oral education.

Mutations in Gjb2 and Gjb6 genes, coding for connexin26 (Cx26) and Cx30 proteins, respectively, are linked to about half of all cases of human autosomal non-syndromic prelingual deafness. Molecular mechanisms of the hearing impairments, however, are unclear. Most cochlear gap junctions (GJs) are co-assembled from Cx26 and Cx30 and deletion of either one of them causes deafness. Our previous studies have shown that normal hearing is possible in the absence of the Cx30 gene when Cx26 is over-expressed. To further test unique functional requirements for various types of connexins in the hearing, we investigated whether the hearing in the conditional Cx26 (cCx26) null mice could be rescued by genetically over-expressing Cx30. Multiple lines of control and experimental mouse models were used. Auditory brainstem response (ABR) measurements showed normal hearing in targeted gene deletion mice when the deleted Cx26 or Cx30 was transgenically expressed from integrated bacterial artificial chromosome (BAC), demonstrating the effectiveness of the BAC rescue approach. In contrast, severe hearing loss was found in cCx26 null mice in which Cx30 was over-expressed. Morphology observations were consistent with the ABR data. Cochleae of cCx26 null mice with and without the transgenic over-expression of Cx30 both showed the typical immature feature of postnatal cochlear development-the closed tunnel of Corti. Immunolabeling data and Western blot quantification indicated that the Cx26 protein expression preceded that of Cx30 during the early postnatal period in the cochlea. Null expression of Cx26 may therefore uniquely result in a transient period when a total elimination of GJs in functionally-important regions of the developing cochlea is possible. We conclude that Cx26 plays an essential role in the development of the auditory sensory epithelium and its unique developmental functions required for normal hearing is not replaceable by Cx30.

Summary Introduction: The majority of patients with hearing loss, including those with severe hearing loss, benefits from the use of hearing aids. The cochlear implant is believed to achieve better results in a child with hearing loss in cases where the severity of disability renders hearing aids incapable of providing adequate sound information, as they require sufficient cochlear reserve so that acoustic detention occurs. Objective: To assess if cochlear implants provide more benefit than conventional hearing aids in prelingually deaf patients. Summary of the findings: The study was a systematic review of scientific papers selected by a search of the SciELO, Cochrane, MEDLINE, and LILACS-BIREME databases. Among the 2169 articles found, 12 studies proved relevant to the issue and presented an evidence strength rating of B. No publications rated evidence strength A. Seven of the studies analyzed were prospective cohorts and 5 were cross-sectional studies. Conclusion: Based on several studies, cochlear implants were demonstrated to be the best current alternative for bilateral severe or profound hearing loss, achieving better results in speech perception and development in prelingual children when compared to conventional hearing aids.

Introduction Studies on adults with congenital and prelingual deafness suggest very limited speech recognition gains. In addition, clinical experience shows that these adults are at high risk for device non-use. On the other hand, a growing number of studies suggest that despite limited auditory improvement, the majority of congenitally deaf adults continue to use their cochlear implant (CI); they also report benefits and satisfaction with the device (Chee et al., 2004; Kaplan et al., 2003). Thus, it appears that speech recognition performance does not fully capture the impact of CI in everyday life, particularly in this population. It is also important to examine personal and environmental factors that contribute to the impression of success (or nonsuccess) with the implant. Our research questions were the following: 1. What is the actual auditory (and speech production) performance of a group of early-deafened adults? 2. What are their impression of usefulness and level of satisfaction toward CI despite possible poor auditory results? 3. Are subjective benefits associated with speech recognition performance?

SummaryDeafness does not in itself cause emotional/behavioural or cognitive problems or psychiatric disorders. However, children with hearing impairment are at greater risk of developing emotional/behavioural problems and neurodevelopmental disorders. The incidence of both seems to be higher in deaf children from hearing families. Most prelingual deafness is caused by recessive genes; hence, most deaf individuals come from hearing families, the majority of whom do not use sign language. Numerous studies, in both hearing and deaf populations, show how the lack of access to language has an impact on the emotional development of children. This article focuses on the mechanisms by which early language deprivation mediates emotional/behavioural difficulties and consequent emotional dysregulation, and may produce behaviours and symptoms that can be misdiagnosed as neurodevelopmental disorders in deaf children and adolescents (from infancy to 18 years of age).

Purpose The purpose of this study was to examine the effect of cochlear implantation (CI) on prelingually deafened participants who were implanted as adults. The effect of the CI was examined with regard to the following variables: communication, family, social skills, education, and work satisfaction with one's life, loneliness, and self-esteem. Materials and methods Thirty-eight adults participated. Four self-report questionnaires were used at 2 points in time: before and after CI. Results The research findings show significant differences in the reports of most variables before and after implantation. The participants felt better with regard to communication, social skills, education, and work and satisfaction with one's life after implantation in comparison to their feelings before implantation. Furthermore, they felt less lonely after implantation. However, there were no significant differences before and after implantation regarding their feelings within the family and regarding their self-esteem. Conclusions The results demonstrated the need to evaluate the benefits resulting from the CI not only with traditional clinical measures but with additional measures as well. Furthermore, they demonstrated the benefit of the CI on the positive psychosociological implications of prelingually deafened adults.

This study focuses on similarities and differences in the processing of written text by individuals with prelingual deafness from different reading levels that used Hebrew as their first spoken language and Israeli Sign Language as their primary manual communication mode. Data were gathered from three sources, including (a) a sentence comprehension test, (b) a metalinguistic awareness (MLA) test, and (c) a word-processing experiment. Participants were 62 individuals who were prelingually deaf, of whom 36 were high school students (10th and 11th graders) and 26 were undergraduate or graduate university students. Findings imply that neither MLA nor word-processing efficiency distinguishes between skilled and less skilled readers with deafness. Rather, differences in reading comprehension skills seem to reflect variance in the ability to process text at the supralexical (sentence) level. Findings are discussed with regard to their implication for the reading instruction for students who are prelingually deaf.

Objective Recent advances in molecular biology of hearing and deafness have made genetic testing an option for deaf individuals and their families. In China, DNA microarray and other genetic testing method has been applied to rapid genetic diagnosis of non-syndromic hearing loss. However, there is no information about the interests in such testing in China. The purpose of this study is to document the attitudes of parents with normal hearing who have one or more deaf children toward diagnostic, carrier, and prenatal genetic testing for deafness. Methods A structured, self-completion questionnaire was given to delegates at a conference held at Hubei Rehabilitation Research Center for Deaf Children, Wuhan, China on March 3, 2010. Of 366 surveys distributed, 290 were completed and returned. Results Ninety-four percent of the respondents had a positive attitude toward genetic testing. Seventy-two percent stated that they were interested in genetic testing of deaf child. Of the individuals who were interested in such testing, 69% would consider having prenatal genetic testing for deafness. Conclusion The present study provided evidence of a predominantly positive attitude toward genetics. Appropriate genetic counseling can help parents to understand the risk, benefits, and limitations of genetic testing for prelingual deafness.

Objective Purpose of this paper is to analyse OTOF gene in a series of subjects affected by auditory neuropathy. Methods Four children showing mild to profound prelingual deafness, confirmed by the absence of a clear and detectable responses at auditory brainstem responses (ABR), associated with the presence of bilateral OAE, were enrolled in the study. Results and Conclusions Genetic analysis identified five new mutations (a nonsense, a small and a large deletion and two splicing site mutations), and one missense mutation (F1795C) previously described. These results further confirm the role of OTOF gene in auditory neuropathy. In the absence of a context of neurological syndrome, the combination of absent ABR and positive OAE responses should lead to an auditory neuropathy diagnosis and to a mutational screening in OTOF.

OBJECTIVE: The comparatively poor music appreciation in patients with cochlear implants might be ascribed to an inadequate exposure to music; however, the effect of training on music perception in prelingually deafened children with cochlear implants remains unknown. This study aimed to investigate whether previous musical education improves pitch perception ability in these children. METHODS: Twenty-seven children with congenital/prelingual deafness of profound degree were studied. Test stimuli consisted of 2 sequential piano tones, ranging from C (256 Hz) to B (495 Hz). Children were asked to identify the pitch relationship between the 2 tones (same, higher, or lower). Effects of musical training duration, pitch-interval size, current age, age of implantation, gender, and type of cochlear implant on accuracy of pitch perception were evaluated. RESULTS: The duration of musical training positively correlated with the correct rate of pitch perception. Pitch perception performance was better in children who had a cochlear implant and were older than 6 years than in those who were aged ≤6 years (ie, preschool). Effect of pitch-interval size was insignificant on pitch perception, and there was no correlation between pitch perception and the age of implantation, gender, or type of cochlear implant. CONCLUSIONS: Musical training seems to improve pitch perception ability in prelingually deafened children with a cochlear implant. Auditory plasticity might play an important role in such enhancement. This suggests that incorporation of a structured training program on music perception early in life and as part of the postoperative rehabilitation program for prelingually deafened children with cochlear implants would be beneficial. A longitudinal study is needed to show whether improvement of music performance in these children is measurable by use of auditory evoked potentials.

This study seeks to provide new insight into the phonemic, orthographic, and syntactic awareness of individuals with prelingual deafness and the way those contribute to reading. Two tests were used: one designed for the assessment of phonemic/orthographic awareness (PO/OA) and another examining reading comprehension (RC) in contexts where prior knowledge was either helpful or not. Participants were 83 prelingually deaf individuals (DIs): 21 primary school, 36 high school, and 26 university students. The control group consisted of 85 hearing individuals (HIs) from parallel education levels (29 primary school, 29 high school, 27 university). Contrary to predictions made by current reading theories, findings imply that the failure of DIs to develop sensitivity to the phonological properties of words may not underlie their reading difficulties. Rather, this weakness seems to reflect a processing deficit at the supra-lexical (sentence) level where the final meaning of single words is elaborated by its integration based upon syntactic (structural) knowledge.

Background: The acquisition of voice onset time (VOT) in plosive consonants among hearing-impaired individuals has long been of interest to many researchers especially following the use of a hearing device such as the cochlear implant. The aim of the study was to study acquisition of voice onset time in Hindi speaking children with cochlear implant. Methods: A total of 15 CI subjects were chosen who were attending the speech and language therapy in Hindi and age matched 15 normal hearing children were selected for the study. Subjects were divided into 3 age groups according to hearing experience. Results: The present study investigated the VOT values of voiced and voiceless plosives produced by Hindi-speaking prelingually deafened CI children with duration of CI experience ranged between 4 to 10 years. The results were then compared to a group of NH children with chronological age similar to the hearing experience of the CI children. Conclusions: A longitudinal study is recommended to continue monitoring the CI children acquisition of the voicing contrast to determine at what hearing age the difference would be insignificant between the CI and NH groups and whether similar developmental trend would continue.

The present study was designed to further clarify whether impoverished phonological skills of prelingually deaf readers undermine their ability to process written words. The word reading skills of forty students with prelingual deafness recruited from two levels of education (primary school, mid/high school) and a chronologically age-matched hearing control group—all of them reading Modern Standard Arabic (MSA) in a diglossic context—were examined with a research paradigm that required the categorization of written real words and paralleling pseudo-homophones. In general, findings suggest that despite severely impaired phonological decoding skills, prelingually deaf MSA readers eventually succeed to develop word reading skills that sustain word recognition with hearing-comparable efficiency. Linguistic representations underlying these skills seem to be of a nonphonological, most likely orthographic nature. Findings are discussed with reference to the validity of reading theories that assign a central role to phonology in the explanation of reading failure in individuals with prelingual deafness.

The purpose of this study was to investigate the musical experiences of preschool cochlear implant users. Research objectives were to examine: (1) musical, social and emotional responses to activities; and (2) whether length of experience with the implant influenced responses. Participants were three prelingually deafened children, age 4, attending a Deaf school in the Northeastern USA. Cochlear implant experience of Child A, Child B and Child C was three years, one year and one month, respectively. Qualitative data including lesson and video transcripts and researcher field notes were coded into musical, social and emotional response categories. Data analyses revealed participants’ ample musical responses and that cochlear implant experience had an impact upon participants’ singing. Music educators must consider the individual differences of the prelingually deafened child modifying lesson design and content to meet their various needs.

Background: Inherited hearing impairment affects about 1 in 1000 newborns. Up to 50 percent of all patients with autosomal recessive nonsyndromic prelingual deafness in many populations have mutations in the gene encoding the gap junction protein connexin 26 (GJB2) at locus DFNB1 (autosomal recessive nonsyndromic deafness) on chromosome 13q11-12. In East Asia, there is a common mutation (235delC) of connexin 26 (GJB2) gene mutation, and a common GJB2 gene mutation (V37I) in Singapore and Malaysia with congenital deafness. No connexin 26 gene study was done in Indonesia. In this preliminary study, we analyzed 40 Indonesian patients with prelingual nonsyndromic sensorineural hearing loss. Objective: To detect the common frameshift mutation (235delC) of connexin 26 (GJB2) gene in Indonesian patients by using the PCR-RFLP (Polymerase Chain Reaction-Restriction Fragment Length Polymorphism ) analysis. Materials and Methods: Forty patients with prelingual nonsyndromic sensorineural hearing loss in Makassar Indonesia were identified and genomic DNAs were extracted from peripheral leukocyte blood of each subject. The important region for connexon-connexon interaction of GJB2 gene in the exon 2 was amplified by using PCR. RFLP analysis (ApaI enzyme) was performed to detect the 235delC mutation in all subjects. Result: The target fragment (722bp) of exon 2 in 40 subjects of prelingual nonsyndromic sensorineural hearing loss were amplified by using PCR method. No 235delC mutation of connexin 26 (GJB2) gene was identified in our RFLP (ApaI enzyme) analysis. Conclusion: No 235delC of connexin 26 (GJB2) gene mutation was found in our preliminary study in 40 Indonesian patients with prelingual nonsyndromic sensorineural hearing loss.

The main objective of this study was to unveil similarities and differences in the word reading strategies of orally raised individuals with prelingual deafness and hearing individuals. Relevant data were gathered by a computerized research paradigm asking participants to make rapid same/different judgments for words. There were three distinct study conditions: (a) a visual condition manipulating the visual-perceptional properties of the target word pairs, (b) a phonological condition manipulating their phonological properties, and (c) a control condition. Participants were 31 high school and postgraduate students with prelingual deafness and 59 hearing students (the control group). Analysis of response latencies and accuracy in the three study conditions suggests that the word reading strategies the groups relied upon to process the stimulus materials were of the same nature. Evidence further suggests that prelingual deafness does not undermine the efficiency with which readers use these strategies. To gain a broader understanding of the obtained evidence, participants' performance in the word processing experiment was correlated with their phonemic awareness-the hypothesized hallmark of proficient word reading-and their reading comprehension skills. Findings are discussed with reference to a reading theory that assigns phonology a central role in proficient word reading.

GJB2 Mutations in Non Syndromic Hearing Loss in the Republic of MacedoniaHearing impairment is a common sensori-neural disorder with the incidence of profound deafness in one per 1,000 births. Non syndromic recessive deafness (NSHL), accounts for approximately 80% of cases of hereditary deafness. It is extremely heterogeneous genetically with over 130 gene loci. Mutations in the GJB2 and GJB6 genes for DFNB1 locus (13q12) are responsible for about half of all cases of autosomal recessive prelingual hearing loss.We determined the prevalence and mutations in the GJB2 gene, and the presence of delD13S1830 in the GJB6 gene in DNA samples from 33 unrelated Macedonian families with recesive NSHL and 200 normal hearing individuals using single-strand conformation polymorphism (SSCP) analysis followed by direct sequencing and specific polymerase chain reaction (PCR).We found mutations in the GJB2 gene in 12 patients, but no delD13S1830 in the GJB6 gene. In 22 mutated chromosomes, 15 (68.2%) had the 35delG mutation, four (6.1%) W24X, two (3.0%) V37I and one (1.5%) R127H.Because of the high mutation rate (36.4%) in the GJB2 gene in NSHL patients, testing should be performed in all cases with prelingual deafness.

Objectives/Hypothesis: The aim of this study was to 1) determine the prevalence of DFNB1 in a cohort of children with prelingual nonsyndromic sensorineural hearing loss (HL), 2) study phenotype/genotype correlations, and 3) establish guidelines for genetic counseling of DFNB1. Study Design: Prospective cohort study. Methods: A total of 119 unrelated children (107 sporadic and 12 familial cases) with prelingual nonsyndromic HL underwent mutational screening for DFNB1 in the noncoding and coding exons of GJB2, in addition to the del(GJB6-D13S1830) mutation of GJB6. Information regarding demographics, HL, developmental milestones, inner ear high resolution computed tomography, hearing habilitation, and associated phenotypic manifestations were collected in probands with biallelic pathogenic mutations. Results: The prevalence of DFNB1 in cases of prelingual nonsyndromic HL was 26% (25% in sporadic and 50% in familial cases). In regards to ethnicity, 19 probands were white and 12 probands of Hispanic ancestry had a mixed racial origin (black, Native-American, white). Greater allelic heterogeneity was shown with Hispanics of mixed descent exhibiting 10 of 12 GJB2 allelic variants, whereas whites had 4 of 10 allelic variants (Fisher exact test, P = .033); both ethnic groups had theGJB6 deletion. The frequency of deaf carriers of the most commonly found mutation (c.35delG) was 8% and higher than that of expected for the general population (Fisher exact test, P = .015). The hearing phenotype was variable in terms of degree of impairment (from mild to profound),onset, symmetry and progression, and there was no correlation with any specific genotype class. DFNB1 probandshad normal gross motor development, and the frequency of computed tomography abnormalities of the inner ear was low at 8%. No other specific associated phenotypic manifestations were identified. Conclusions: DFNB1 is the most common identifiable etiology of nonsyndromic prelingual deafness both in sporadic and familial cases in this cohort with ethnic diversity. The greater allelic variability observed in Hispanics and the high frequency of deaf probands carrying a single allelic variant of DFNB1 support extending the screening to noncoding regions of GJB2 and to the remaining DFNB1 locus. Most probands have a congenital HL that is stable, symmetrical and without associated manifestations, but the audiometric profile should not be the only criteria for offering mutational screening of DFNB1 because of the observed variability. These data can be applied to direct the clinical evaluation and effectively counsel families of children with DFNB1.

The indication of cochlear implants for patients with pre-lingual loss, both adolescents and adults, was always very controversialdue to the fact that most studies show little or no benefit in the open set speech recognition. It is necessary to review thisconcept, reorganizing pre-implant evaluation, emphasizing language evaluation, social insertion and expectation. This work aims toevaluate the benefits young adults with prelingual deafness have after cochlear implant, considering not only the results in speechperception tests, but also the subjective impressions of patients in relation to the changes in quality of life. This is a transversal retrospectiveclinical study. We evaluated the results of speech perception tests and the subjective perception of quality of life after theconstant use of cochlear implants of eleven young adults with prelingual deafness and submitted to surgery for implantation. Only twoindividuals presented an open-site speech recognition at a rate of 50% or more. All individuals were able to recognize their own namesand to discriminate suprasegmental aspects. Some patients with long-standing prelingual deafness develop auditory abilities and havea good performance in open-set speech recognition. However, even those not capable of reaching these results say to be satisfied withthe device and recognize an improvement in quality of life.

Summary Objective Infants are diagnosed with severe to profound hearing loss at an earlier age due to the advent of universal newborn hearing screening. This offers the opportunity to provide intervention in the form of cochlear implantation at an earlier age than was previously possible. The purpose of this investigation is to evaluate the risk of cochlear implant surgery in children less than 12 months of age. Design Retrospective review of children who underwent cochlear implantation before 12 months of age. Setting Patients were identified from a database of pediatric cochlear implant patients at a tertiary care center. All patients were diagnosed with severe to profound hearing loss by otoacoustic emission and auditory brainstem response. Follow-up ranged from 2 months to 5 years. Results Fourteen of 15 patients had full insertions of the electrode hardware. Less than full insertion and post-operative CSF otorrhea occurred in one patient with severe cochlear abnormalities. There were no other perioperative surgical complications. The average speech detection threshold was 27.6 dB (20–45 dB) at approximately 1–3 months post-stimulation and 25 dB (15–30 dB) at approximately 5–7 months. Conclusion In our experience, we feel cochlear implantation is safe for infants as young as 6 months of age. The current standard at our institution is to implant by 7 months of age for prelingual deafness as opposed to waiting additional time until 12 months of age before the brain is presented with speech.

The outcome of cochlear implantation in patients with deafness of prelingual onset is largely unpredictable due to high individual variability. This study evaluated speech perception performances in a group of 18 prelingually deafened subjects (aged 13–30 years) which was homogeneous with respect to duration of deafness, hearing aid use before cochlear implantation, mode of communication and administration of auditory-oral speech therapy. Word discrimination length, word and sentence identification, phoneme identification and word and sentence recognition were tested before cochlear implantation and at 6 months, 1, 2 and 3 years of cochlear implant use. Scores on all tests significantly improved after cochlear implantation, although mean values were lower compared to those achieved by postlingually deafened patients. Speech performances on both word and sentence recognition continued to increase over time also beyond 1 year after cochlear implantation. Moreover, scores on sentence recognition tests were significantly higher compared to disyllabic words at 3 years of cochlear implant use. The presence of an auditory input delivered by hearing aids before cochlear implantation associated with auditory-oral therapy and a good level of education may positively influence the cochlear implant outcome in prelingually deafened adults.

Summary Introduction This study examines the ability and development in the comprehension of abstract words with hearing impaired children. The ability to understand abstract words is quite important for their academic learning and adaptation in their school life. Here, we qualitatively and quantitatively analyzed the development of abstract vocabulary in hearing impaired children using The Standardized Comprehension Test for Abstract Words (SCTAW). Subjects and methods We examined 75 hearing impaired children (hearing aid users, 61; cochlear implant users, 14; 1st to 10th grade) and 188 children with normal hearing (1st to 6th grade) using the Picture Vocabulary Test (PVT) and SCTAW. Results The PVT and SCTAW results closely correlated ( r = 0.87). The SCTAW scores of the hearing impaired group were lower than those of their peers with normal hearing, but the scores improved as their school grade advanced. In particular, their abstract ability began to catch up from the fifth grade. The error trends of abstract vocabulary in the two groups did not significantly differ. Conclusions The SCTAW was useful as an abstract lexical evaluation of hearing impaired children. The development of an abstract vocabulary did not qualitatively differ between children with or without a hearing impairment.

Objectives: To evaluate sound and speech perception and quality of life in prelingually deafened adults implanted with state of the art devices. To investigate which patient factors influence postoperative performance. Study Design: Prospective intervention study. Methods: Eight prelingually deafened subjects (with onset of severe hearing impairment before the age of 4 years and functioning in an oral-aural setting) participated in this study. Subjects were implanted at a mean age of 36 (range, 21–55) years with a CII or 90 K cochlear implant (Advanced Bionics Corp.). All subjects completed standard speech perception tests as well as quality of life measures (Health Utility Index Mark-II, Nijmegen Cochlear Implant Questionnaire, visual analogue scale for subject's hearing and health) at different points in time. Postoperative scores were compared with each other and with the baseline preoperative scores. The relationship between nine patient variables and the postoperative consonant-vowel-consonant (CVC) phoneme score was also investigated. Results: Significant improvement was measured for CVC word and phoneme scores and several quality of life measures. Postoperative speech perception correlated with a new and promising factor named quality of a patient's own speech production (QoSP). Conclusion: With state of the art implants, speech perception and quality of life do improve in prelingually deafened adults. More importantly, the prognostic value of QoSP should be investigated further.

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.

In order to evaluate the genetic epidemiology of deafness in Turkey, we first analyzed the pedigree data obtained from 2,169 families whose children were students of the schools for hearing loss/deafness in 31 cities of Turkey. Single major locus segregation analysis was performed after families were grouped according to hearing status of the parents. The results showed that sporadic phenocopies, autosomal dominant, and autosomal recessive transmission account for 18.2%, 4.9%, and 76.9% of the cases respectively, after exclusion of probands with unequivocal evidence for environmental etiologies. The high frequency of autosomal recessive transmission of this study differs from those of previous ones in Western populations. We subsequently analyzed the data from a subset of 574 unrelated families that were evaluated clinically, including mutation analysis of the GJB2 gene in 406 probands. Biallelic mutations were detected in 22.4% of all probands. They were present in 68.8% of probands whose parents were both deaf, yet in only 9.3% when both parents were hearing and consanguineous without a family history of deafness. Our study shows that GJB2 is the major gene for deafness in Turkey and was amplified in deaf by deaf matings, since assortative mating preferentially affects common genes. Deafness in the remaining families appears to result from mutations at many loci that are less frequent causes of deafness, because consanguinity has a proportionally greater effect on rare genes. Conclusions of this study may be relevant to other populations where consanguineous or assortative mating is present with various frequencies.

The GJB2 gene (connexin 26) has been shown to be responsible for DFNB1 and DFNA3. We screened the GJB2 gene in 488 patients with prelingual deafness(Group 1), 124 with postlingual deafness(Group 2), and 117 normal hearing subjects (Group 3). We found that, in Group 1, 65 patients (13.32%) were homozygotes or compound heterozygotes and 51 patients (10.45%) carried a single pathogenic mutation. The 235delC mutation was the most frequent mutation, accounting for 73.22% of the known pathogenic alleles in Group 1. No homozygotes or compound heterozygotes were detected in Group 2 or Group 3. Some postlingual deaf patients (2.42%) and normal hearing subjects(4.27%) were 235delC carriers. Our preliminary data indicate that 235delC, the most frequent mutation identified in this study, is a major cause for prelingual deafness.

This study seeks to clarify the relation between the phonological skills of both dyslectic readers and prelingually deafened readers and their ability to conceptually process written stimuli. Data was gathered by means of a test designed for the assessment of orthographic and phonemic awareness, and a categorization paradigm designed for the examination of written word processing skills. Twenty individuals with diagnosed dyslexia (grade = 9.05), 11 individuals with prelingual deafness (grade = 8.18), and 25 normally-developing hearing readers (n = 25, grade = 9.00) participated in the study. In general, findings indicate that the reading disorders manifested by dyslectic and deaf readers have different origins none of which is directly related to their phonological abilities. It is suggested that in prelingually deafened readers, but maybe also in dyslectic readers, teachers should foster the development of orthographic knowledge as the basis for proficient reading without making such development contingent on the processing of the phonology of written words.

Purpose The purpose of this review article is to synthesize evidence from the fields of developmental linguistics and cochlear implant technology relevant to the production and perception of Mandarin lexical tone in cochlear implant users with prelingual deafness. The aim of this review was to identify potential factors that determine outcomes for tonal-language speaking cochlear implant users and possible directions for further research. Method A computerized database search of MEDLINE, CINAHL, Academic Search Premier, Web of Science, and Google Scholar was undertaken in June and July 2014. Search terms used were lexical tone AND tonal language, speech development AND/OR speech production AND/OR speech perception AND cochlear implants, and pitch perception AND cochlear implants, anywhere in the title or abstract. Conclusion Despite the demonstrated limitations of pitch perception in cochlear implant users, there is some evidence that typical production and perception of lexical tone is possible by cochlear implant users with prelingual deafness. Further studies are required to determine the factors that contribute to better outcomes to inform rehabilitation processes for cochlear implant users in tonal-language environments.

Early hearing detection and intervention (EHDI) is critical for achievement of age-appropriate speech perception and language development in hearing-impaired children. It has been 15 years since newborn hearing screening (NHS) was introduced in Japan, and its effectiveness for language development in hearing-impaired children has been extensively studied. Moreover, after over 20 years of cochlear implantation in Japan, many of the prelingual cochlear implant (CI) users have reached school age, and the effect of CI on language development have also been assessed. To identify prognostic factors for language development, audiological/language test scores and demographic factors were compared among prelingual severe-to-profound hearing-impaired children with CI divided into subgroups according to age at first hearing aid (HA) use and whether they received NHS.Prelingual severe-to-profound deafened children from the Research on Sensory and Communicative Disorders (RSCD) project who met the inclusion criteria were divided into groups according to the age (in months) of HA commencement (before 6 months: group A, after 7 months: group B), and the presence or absence of NHS (groups C and D). Language development and socio-economic data were obtained from audiological/language tests and a questionnaire completed by caregivers, respectively.In total, 210 children from the RSCD project participated in this study. Group A (n=49) showed significantly higher scores on comprehensive vocabulary and academic achievement (p0.05) than group B (n=161), with no difference in demographics except for significantly older age in group B. No differences in language scores were observed between group C (n=71) and group D (n=129), although participants of group D was significantly older and had used CIs longer (p0.05).Early use of HAs until the CI operation may result in better language perception and academic achievement among CI users with prelingual deafness. A long-term follow-up is required to assess the usefulness of NHS for language development.

The central aim of this study was to clarify whether sign language (SL) nativeness is a significant factor in determining prelingually deaf individuals' reading skills and whether its contribution is modified by the reader's orthographic background. A second aim was to elucidate similarities and differences between native and nonnative signers in processing written information at different processing levels in order to understand how SL nativeness sustains the reading process, if at all. Participants were 176 students with prelingual deafness recruited from two education levels (6th-7th graders and 9th-10th graders) and three orthographic backgrounds (Hebrew, German, and Turkish). Sixty-six students were native and the remainder nonnative signers. They were tested with a battery of eight experimental paradigms, each assessing their information processing skills in a specific reading-related or reading-unrelated domain. Findings corroborate SL nativeness enhancing the reading process in some regard. However, its contribution was not found to scaffold the structural processing of a written text to turn reading into a tool for learning. Rather, gains were restricted to facilitating processing written words from a perceptual to a conceptual level. Evidence suggests that compared with other determining factors, the contribution of SL nativeness to proficient reading may be rather marginal.

Zeinu Hizkuntza (ZH) da Gor kulturaren bereizgarri nagusiena, baita mundu entzulearekin komunikatzeko traba handiena suposatzen duena. Gorrek hainbat arazo aurkitzen dituzte Osasun Sisteman entzuleekin komunikatzeko, nahiz eta zerbitzu publikoek komunikazio erraztasunak ahalbidetu behar dizkietela araututa egon. Helburua: Gorren ezaugarri kulturalak ezagutu, Osasun Sisteman dituzten zailtasunak identifikatu eta erizainek Gorren beharrei erantzuteko estrategia eraginkorrak zehaztu. Metodologia: Errebisio bibliografiko honetan, PIO estruktura erabili da. Barneratze eta baztertze irizpideak finkatuta, Biblioteca Virtual de la Salud, PubMed eta Scopus-en burutu da bilaketa, 2005-2010 urte tarteko artikuluak aztertuz. Emaitzak: Gortasuna ikuspuntu medikotik bereizten du Gor kulturak, izan ere, beraien ezaugarri naturaltzat dute. Gorrek komunitate bat eratzen dute, beraien artean elkar identifikatu eta kulturaren garapenean laguntzen dutelarik. Hizkuntza ezberdina darabiltenez, Osasun Sisteman zailtasunak aurkitzen dituzte, honek ezaguera ezera eta gaixotasunak pairatzeko arrisku handiagora eramanez. Arrazoietako bat, profesionalek kultura hau ezezagun izatea da, heziketa gabeziagatik, batik bat. Gainera, ez dute pazientziarik, honek Gorretan konfiantza eza eta beldurra eraginez. Horrenbestez, funtsezkoa da erizainak Gor kulturaren inguruan heztea, pertsona hauekin eraginkorki komunikatzeko nahiz arreta kalitatea ahalik eta hoberena izateko. Halaber, ezinbestekoa da Gorrentzako informazioa egokitzea. Ondorioak: Osasun Sisteman komunikazio zailtasunak dituzte Gorrek eta ondorioz, osasun gaietan ezaguera baxuagoa dute, gaixotasunak pairatzeko arrisku handiagoarekin bat. Horregatik, sentikorra den informazioa ahalbidetu behar zaio biztanleria honi. Halaber, profesionalek Gor kulturaren inguruko heziketa jaso behar dute, baita komunikazio eraginkorrerako erraztasunak ahalbidetu ere.

Summary Introduction The purpose of this investigation was to describe the correlation between vocal and hearing development by longitudinal analysis of sound spectrograms, as a basic system for evaluating progress in vocal development. Subjects and methods Two school-aged children with prelingual deafness were evaluated diachronically to assess speech perception and speech intelligibility after cochlear implantation. One child had non-syndromic hearing impairment without any known neurological deficit except for hearing loss, while the other had hearing impairment accompanied by mild mental retardation and attention deficit disorder. Their voices were recorded for monthly follow-up after cochlear implantation; these were used for formant analysis and compared with their mother's voice, and alteration of the formant data was also compared with monosyllable speech perception. Results Formant analysis demonstrated high concordance was observed between monosyllable speech perception and speech intelligibility. F1–F2 forms of the patients more closely resembled those of their mothers after 1 year's follow-up. The time point at which speech development altered was very similar in both cases although the final outcomes were different. Conclusion Fair improvement of articulation after cochlear implant was demonstrated by the F1–F2 gram analysis. This procedure can be used for data sharing and cooperation between medical and educational specialists.

The objective of this study was to clarify the nature and efficiency of the word recognition skills of prelingually deafened Hebrew readers and the way these skills relate to their reading comprehension level. Relevant data were gathered by means of a research paradigm demanding the same/different categorization of phonologically or formationally manipulated word pairs and by a sentence comprehension test (SCT). Participants were prelingually deafened individuals (n=22, mean grade=8.7) who attested to using sign language as their preferred means for communication, and a task-matched hearing control group (n=40, mean grade=8.9). In general, findings suggest that both hearing readers and readers with prelingual deafness rely upon orthographic knowledge rather than upon their primary language for mediating the processing of written words in the working memory (WM). As predicted, in comparison to their hearing counterparts, the reading comprehension of the prelingually deafened participants, as a group, was significantly impaired. Comparative analyses of quantitative and qualitative aspects of the same/different categorizations of word pairs by the participant groups, and the way these correlated with performance on the SCT clearly indicated, however, that this weakness is not rooted in poor word recognition skills but probably reflects a lack of syntactic knowledge crucial for the adequate, post-lexical processing of recognized words. Finally, the findings convincingly show that, despite their profound hearing loss, some individuals who were prelingually deaf acquired rule-based knowledge for the adequate processing of written text.

This paper studies the linguistic input attended by a deaf child exposed to cued speech (CS) in the final part of her prelinguistic period (18-24 months). Subjects are the child, her mother, and her therapist. Analyses have provided data about the quantity of input directed to the child (oral input, more than 1,000 words per half-an-hour session; cued ratio, more than 60% of oral input; and attended ratio, more than 55% of oral input), its linguistic quality (lexical variety, grammatical complexity, etc.), and other properties of interaction (child attention and use of spontaneous gestures). Results show that both adults provided a rich linguistic input to the child and that the child attended most of the input that the adults cued. These results might explain the positive linguistic development of children exposed early to CS.

Summary Objectives Recent advances in genetic research indicate that about 50% of congenital deaf patients have a genetic background, with mutations in the Connexin-26 gene being the most frequent one. Screening methods for the genetic cause of deafness have so far mostly been based on the use of peripheral whole blood as DNA source. The use of buccal smears for the genetic screening of deaf patients presents an interesting alternative to drawing blood, especially in young children. In order to validate this method, we compared results from buccal smears from very young deaf children (age ≤ 3 years) and deaf patients older than 3 years with the results from blood samples deriving from the same patients. Methods The detection of the 35delG mutation in the Connexin-26 gene was chosen to demonstrate the method's feasibility. Blood and buccal smears were collected for genetic analysis from 29 very young deaf children (Group 1: age ≤ 3 years) and 31 deaf patients older than 3 years (Group 2) during their clinical pre-evaluation for cochlear implantation. Genomic DNA was isolated from blood as well as from buccal smears. Yield of both sources was determined by photometric evaluation of the isolated DNA concentration. Genomic DNA isolated from blood and buccal smears was then submitted to PCR-mediated site-directed mutagenesis followed by BS1 YI restriction and electrophoresis. The results for 35delG detection in DNA originating from blood were compared to those of buccal smears. Results Quantitative DNA analysis showed that both sources provided adequate amounts of DNA for PCR. No significant difference was found between Group 1 and Group 2 considering either the DNA amount isolated from blood or from buccal smears. In all 60 patients, DNA isolated from blood revealed the same results concerning the presence of the 35delG mutation as DNA originating from buccal smears. Conclusions This study demonstrates that buccal smears are an adequate, reliable source of genomic DNA providing material for genetic tests that can especially help to avoid drawing blood from very young children for the genetic screening of deafness.

The functional status of central neural pathways, in particular their susceptibility to plasticity and functional reorganization, may influence speech performance of deaf cochlear implant users. In this paper, we sought to determine how brain metabolic activity measured before implantation relates to cochlear implantation outcome, that is, speech perception. In 22 prelingually deaf children between 1 and 11 years, we correlated preoperative glucose metabolism as measured by F-18 fluorodeoxyglucose positron emission tomography with individual speech perception performance assessed 3 years after implantation, while factoring out the confounding effect of age at implantation. Whereas age at implantation was positively correlated with increased activity in the right superior temporal gyrus, speech scores were selectively associated with enhanced metabolic activity in the left prefrontal cortex and decreased metabolic activity in right Heschl's gyrus and in the posterior superior temporal sulcus. These results reinforce the notion that implantation should be performed as early as possible to prevent cross-modal takeover of auditory regions and suggest that rehabilitation strategies may be more efficient if they capitalize on general cognitive functions instead of only targeting specialized circuits dedicated to auditory and audiovisual pattern recognition.

Hearing loss, mainly due to recurrent otitis media, has been reported in approximately 40% of individuals with Niikawa-Kuroki (Kabuki) syndrome (NKS). Sensorineural hearing loss leading to congenital or prelingual deafness has been described rarely. We have identified two unrelated individuals with Niikawa-Kuroki syndrome among 535 probands who have severe to profound sensorineural deafness. Bilateral absence of the cochlea with dilated dysplastic vestibule and unilateral enlarged vestibule were demonstrated in these two individuals. In conclusion, Niikawa-Kuroki syndrome should be kept in mind when evaluating an individual with congenital deafness and a wide spectrum of inner ear abnormalities occurs in this syndrome.

The present experiment represents an attempt to learn from the comparison of dyslectic readers, prelingually deafened readers, and a control group of typically developing hearing readers, in four particular areas, about factors determining their reading skills.Twenty-three students with diagnosed dyslexia (mean grade level 9.7 [1.53]), 20 students with prelingual deafness (mean grade level 8.9 [1.33]), and a control of 41 typically developing hearing students (mean grade level 8.9 [1.43]) participated in the study.Four distinct areas were examined which included: (a) the rapid, repetitive execution of two simple, familiar motor actions; (b) the rapid, consecutive judgment of the identicalness of two digit stimuli; (c) the rapid, consecutive, perceptual processing of word pairs; and (d) the rapid, consecutive, conceptual processing of word pairs. Findings obtained from th comparison of the three participant groups in the four tested areas indicate that the reading difficulties of dyslectic readers and prelingually deafened readers have differentsources, neither of which are directly, causally related to a phonological processing deficit.

The relation between partial or absent hearing and control of the voicing contrast has long been of interest to investigators, in part because speakers who are born deaf characteristically have great difficulty mastering the contrast and in part for the light it can cast on the role of hearing in the acquisition and maintenance of phonological contrasts in general. One of the phonetic characteristics that distinguish voiced from voiceless plosives in English (p/b, t/d, k/g) is voice onset time (VOT): the interval from plosive release to the onset of voicing of the following vowel. This article first reviews research on VOT anomalies in the speech production of prelingually and postlingually deaf speakers. Then it turns to studies of the mechanisms in speech breathing, phonation and articulation that underlie those anomalies. In both populations of speakers, there is a tendency for the difference between voiced and voiceless VOT to be reduced, to the point for many speakers that there is in effect a substitution of the voiced for the voiceless cognate. The separation of the cognate VOTs can be enhanced when some hearing is restored with a cochlear implant. Both populations also present anomalies in speech breathing that can hinder the development of intraoral pressures and transglottal pressure drops that are required for the production of the VOT contrast. Its successful management further requires critical timing among phonatory and articulatory gestures, most of which are not visible, rendering the VOT contrast a particular challenge in the absence of hearing.

This study investigated the effects of age at time of testing, hearing history (age at hearing loss) and hearing status on melody recognition. Four groups were compared: children with normal hearing thresholds, and three groups of cochlear implant recipients (children with prelingual deafness, children with postlingual deafness, adults with postlingual deafness). Participants were tested for recognition of familiar melodies (no lyrics) in a closed-set task. Groups differed in accuracy in the following rank order (most to least accurate): children with normal hearing thresholds, adult cochlear implant recipients, children with postlingual deafness, children with prelingual deafness. Melody recognition scores were correlated with age, variables regarding hearing history, musical background/experience and speech perception scores. Copyright © 2005 John Wiley & Sons, Ltd.

Prelingual deafness and developmental dyslexia have confounding developmental effects on reading acquisition. Therefore, standard reading assessment methods for diagnosing dyslexia in hearing people are ineffective for use with deaf people. Recently, Samar, Parasnis, and Berent (2002) reported visual evoked potential evidence that deaf poor readers, compared to deaf good readers, have dorsal stream visual system deficits like those previously found for hearing dyslexics. Here, we report new psychometric and psychophysical evidence that deficits in dorsal stream function, likely involving extrastriate area MT, are associated with relatively poor reading comprehension in deaf adults. Poorer reading comprehension within a group of 23 prelingually deaf adults was associated with lower scores on the Symbol Digit Modality Test, a perceptual speed test commonly used to help identify dyslexia in hearing people. Furthermore, coherent dot motion detection thresholds, which reflect the functional status of area MT, correlated negatively with reading scores in each visual quadrant. Elevated motion thresholds for deaf poor readers were not due to general cognitive differences in IQ but were specifically correlated with poor perceptual speed. With IQ controlled, a highly reliable right visual field advantage for coherent motion detection was found. Additional analyses suggested that the functional status of dorsal stream motion detection mechanisms in deaf people is related to reading comprehension, but the direction and strength of lateralization of those mechanisms is independent of reading comprehension. Our results generally imply that dyslexia is a hidden contributor to relatively poor reading skill within the deaf population and that assessment of dorsal stream function may provide a diagnostic biological marker for dyslexia in deaf people.

Introducción: Los implantes cocleares en niños se aprobaron desde la década de los ochenta. Las investigaciones del desarrollo de la vía auditiva sugieren que la colocación temprana de un implante coclear en niños con sorderas prelinguales, brindaría beneficios mayores en discriminación del lenguaje. Este trabajo busca establecer la relación entre la edad de implantación de niños que han sufrido hipoacusia prelingual y la discriminación del lenguaje, tanto en contexto cerrado como abierto, así como describir cómo se alcanza en los diferentes grupos el 50% de discriminación del lenguaje en contexto abierto, considerando este punto como un umbral funcional para el entendimiento adecuado de un mensaje oral.Métodos: Se realizó un estudio descriptivo retrospectivo con pacientes implantados en la Fundación Santa Fé de Bogotá y el Hospital de San José, desde 1992 hasta 2001, incluyendo 76 pacientes con hipoacusia prelingual y seguimiento mínimo de 36 meses en pruebas para contexto abierto y cerrado.Resultados: Los pacientes se dividieron en cuatro grupos según la edad de implantación en años cumplidos. Grupo I de O a 3 años, grupo II de 4 a 6 años, grupo III de 7 a 9 años y grupo IV de 10 a 12 años. Los I y II presentaron mejor desempeño en la discriminación del lenguaje en contexto abierto y el primero en alcanzar el 50% fue el grupo I, realizándolo hacia los 20 meses. En el contexto cerrado no se hallaron diferencias importantes. Conclusión: Los resultados indican que implantar tempranamente a un paciente con hipoacusia prelingual, podría brindar la ventaja de estimular la vía auditiva en la etapa de oro, en la cual aún está presente el fenómeno de plasticidad neural y maduración activa de la misma. Este estudio sugiere que la edad ideal de implantación se halla por debajo de los tres años.

In an animal model of prelingual deafness, we examined the anatomical and physiological effects of prolonged deafness and chronic electrical stimulation on temporal resolution in the adult central auditory system. Maximum following frequencies ( Fmax) and first spike latencies of single neurons responding to electrical pulse trains were evaluated in the inferior colliculus of two groups of neonatally deafened cats after prolonged periods of deafness (>2.5 yr): the first group was implanted with an intracochlear electrode and studied acutely (long-deafened unstimulated, LDU); the second group (LDS) received a chronic implant and several weeks of electrical stimulation (pulse rates ≥300 pps). Acutely deafened and implanted adult cats served as controls. Spiral ganglion cell density in all long-deafened animals was markedly reduced (mean max, longer response latencies) as compared with control animals. In contrast, chronic stimulation in LDS animals led to a significant increase in temporal resolution. Changes in temporal resolution after long-term deafness and chronic stimulation occurred broadly across the entire ICC and were not correlated with its tonotopic gradient. These results indicate that chronic electrical stimulation can reverse the degradation in temporal resolution in the auditory midbrain after long-term deafness and suggest the importance of factors other than peripheral pathology on plastic changes in the temporal processing capabilities of the central auditory system.

Maternal ratings of self-efficacy and involvement in early intervention were compared across two groups of mothers with young children with prelingual deafness: 24 mothers of children with hearing aids and 30 mothers of children with a cochlear implant. Although mothers of children with cochlear implants rated their child's early intervention program lower in quality, they perceived themselves as more efficacious in the care and maintenance of their child's sensory device and their involvement in developing their child's speech-language skills. Mothers of children with hearing aids had lower self-ratings of self-efficacy related to their child's sensory device and involvement in their child's early intervention program. Implications for early interventionists are discussed.

Background: The speech recognition performances in postlingually deaf patients, which had a long duration of deafness, seem to be poorer than in patients with a short duration of deafness. The reason could be a functionalreorganization in the auditory cortex. A long time of auditory deprivation may decrease neuronal activity in the auditory related cortices. As the late acoustically evoked potentials are generated in this region, we compared the speech recognition performances and also the late acoustically evoked potentials with the individual duration of deafness in a series of postlingually deaf patients. Methods: In 9 patients (cochlear implant (CI), MED EL) with postlingual deafness the late acoustically evoked potentials and the HSM-set-test on the quiet were measured. Additionally 1 patient with prelingual deafness was examined. The Cl-operation was done, when he was 14 years old. The relations between the late acoustically evoked potentials and the speech recognition performances in dependence of the duration of deafness were examined. Results: Typical late acoustically evoked potentials could be measured in 3 patients only. The speech recognition in these patients was very good. The longest time of deafness in these patients was 6 years. In 6 patients the minimum duration of deafness was 12 years. Here were measured the N1-potentials only, the P2- and the N2-potentials were absent. In these patients the scores of the HSM-set-test were lower than in the others. In the 15-years old patient with prelingual deafness the speech recognition was absent. The late acoustically evoked potentials of this patient showed an atypical form. Conclusions: The results of this study showed a correlation between the duration of deafness, the forms of the late acoustically evoked potentials and the speech perception. The duration of deafness preceding Cl-implantation should be very small in deaf children and in postlingually deaf patients.