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Introduction The World Health Organization (WHO) estimates that ∼ 32 million children worldwide are affected by hearing loss (HL). Cochlear implant is the first-line treatment for severe to profound sensorineural HL. It is considered one of the most successful prostheses developed to date. Objective To evaluate the oral language development of pediatric patients with prelingual deafness implanted in a reference hospital for the treatment of HL in southern Brazil. Methods We conducted a retrospective cohort study with a review of medical records of patients undergoing cochlear implant surgery between January 2009 and December 2018. Language development was assessed by reviewing consultations with speech therapy professionals from the cochlear implant group. Results A total of 152 children were included in the study. The mean age at cochlear implant surgery was of 41 months (standard deviation [SD]: ± 15). The patients were divided into six groups considering the type of language most used in their daily lives. We found that 36% of children use oral language as their primary form of communication. In a subanalysis, we observed that patients with developed or developing oral language had undergone cochlear implant surgery earlier than patients using Brazilian Sign Language (Língua Brasileira de Sinais, LIBRAS, in Portuguese) or those without developed language. Conclusion The cochlear implant is a state-of-the-art technology that enables the re-establishment of the sense of hearing and the development of oral language. However, language development is a complex process known to present a critical period to properly occur. We still see many patients receiving late diagnosis and treatment, which implies a delay and, often, the impossibility of developing oral communication. Level of Evidence Level 3 (cohort study). [ABSTRACT FROM AUTHOR]

The article discusses the reading process similarities and differences between deaf Arabic children in Israel and typical hearing readers in 2024. Topics covered include their similar processing strategies, but with less effectivity by deaf Arabic children, and the recognition of written words by deaf readers who use sign language for communication. Also noted are the knowledge categories of readers' prior knowledge and cognitive strategies, and their prediction of reading comprehension.

Objective: To evaluate the rate of loss to follow-up in a cochlear implant program from the public health system in Southern Brazil as well as the characteristics of hearing loss, sociodemographic, sociocultural and the development of oral language in children with prelingual deafness. Methods: Retrospective cohort study with children who underwent CI surgery between 2010 and 2020. Data was collected through of interviews and review of medical records. The language development assessment was performed using the MUSS, MAIS and IT-MAIS scales. For the classification of language development, we used as parameters the values (mean ± SD) found in a previous national study. From those values, the Z-score for each patient at each hearing age (time of experience with the cochlear implant) was calculated. Results: Of the 225 children implanted between 2010–2020, 129 were included in this study. The rate of loss to follow-up in the program was 42.6%. The mean age at first surgery was 40.5 (±16.9) months, with 77.5% of patients having received a unilateral implant. Language results below the expected for hearing age (

Introduction The World Health Organization (WHO) estimates that ∼ 32 million children worldwide are affected by hearing loss (HL). Cochlear implant is the first-line treatment for severe to profound sensorineural HL. It is considered one of the most successful prostheses developed to date.

Background: Profound hearing loss during childhood impacts the normal development of auditory and speech perception and language skills. Cochlear implants (CIs) have revolutionized the scenario of rehabilitation of profoundly deaf individuals, particularly the prelingual deaf. A prelingual deaf is congenitally deaf or has hearing loss before speech development. Objective: To perform a scoping review of available evidence for assessing the effects of CIs in prelingual deaf children on their auditory and speech perception and language development. Materials and Methods: A scoping review of research studies assessing the effects of CIs in prelingually deaf children was carried out in June 2022 using major databases such as PubMed, Medline, Scopus, and Web of Science using appropriate keywords. To be included in the review, the studies were required to be full‑text articles in the English language only, and the studies on adult populations were excluded. All the relevant studies found to be suitable for inclusion were classified under three subheadings: “Impact on Auditory perception,” “Impact on Speech perception and/or development,” and “Impact on “Language development.” Conclusion: CIs are a highly effective intervention in the treatment and rehabilitation of prelingually deaf children, although complex due to the interaction of variables that affect the implanted child’s performance. The factors found to influence it are the child’s age at the time of implantation, time of hearing sensorial deprivation, duration of use of a cochlear implant, and type of speech codification strategy used. More well‑designed longitudinal studies with greater follow‑up periods post‑implantation are required to better understand the implantation complexity and its impact on prelingual deaf children. [ABSTRACT FROM AUTHOR]

To investigate the postoperative long-term outcomes after an average of 9.2 years following cochlear implantation (CI) in prelingually deafened adults, along with preimplantation factors predicting postoperative outcomes. Twenty-six prelingually deafened adults who underwent CI at >18 years were compared with those who had undergone CI in childhood (<9 years) and were >10 years old. Outcome measures includedhearing thresholds, preoperative and postoperative aided hearing level (HL), speech discrimination score (SDS), and Categories of Auditory Performance (CAP) scores. Correlation analyses were performed on the following: SDS results, aided HL, school attendant status, implant manufacturers, and speech processor models. Improvement was achieved in the aided HL and SDS results, although these results were not better than those of the child group. CAP score was also statistically significantly improved after CI. Statistically significant correlation between the preoperative SDS and postoperative HL with CI results was observed. In other words, the better the preoperative SDS results, the better the postoperative SDS results. Prelingually deafened adults achieved considerable improvement through CI. It is important to understand that patients achieving better hearing with a well-fitted hearing aid and good SDS performance before surgery may be good candidates for CI. [ABSTRACT FROM AUTHOR]

Recent studies have suggested that speech perception outcomes after cochlear implantation (CI) in prelingually deafened adults have improved with advances in CI technology and speech processing strategies. However, the outcomes vary from case to case. Communication mode has been reported in many studies as the factor that related to the post CI outcomes. This study aimed at investigating the post CI outcomes and the progress during 2 years for each communication mode. The subjects were 17 prelingually deafened adults undergoing CI at our hospital between April 2013 and March 2019. We investigated preoperative factors affecting post CI outcomes. Also we analyzed post CI outcomes for each communication mode and compared preoperative factors for each communication mode. Communication mode and preoperative discrimination score were the factor affecting on postoperative discrimination score. The speech perception score after CI improved significantly in the oral and lip-reading group and total communication group. The speech perception scores in postlingually deafened adults improved significantly during the first six months and became to plateau after CI. On the other hand, the scores of prelingually deafened adults tended to improve gradually after six months postoperatively. Furthermore, the degree of improvement and progress differed by each communication mode. The communication mode is important factors in predicting outcomes in prelingually deafened adults after CI. Long-term auditory training is important for prelingually deafened adults who use visual information as their preoperative method of communication. [ABSTRACT FROM AUTHOR]

Introduction: Hearing loss is known to play a fundamental role in voice production due to a lack of auditory feedback. In this study, we evaluated both fundamental frequency (F0) and loudness of voice on adult deaf patients subjected to cochlear implantation, and we analyzed these results according to the prelingual or postlingual onset of the deafness. Methods: The study population, balanced in terms of sex, consisted of 32 adults who had undergone cochlear implantation due to severe or profound bilateral hearing loss (16 with prelingual deafness and 16 with postlingual deafness) and their outcomes were compared with a control group of 32 normal hearing (NH) subjects. All subjects were asked to utter the sustained vowel /a/ for at least 5 s and then to read an Italian phonetically balanced text. Voice recordings were performed by means of an ambulatory phonation monitoring (APM 3200). Measurements were performed without cochlear implant (CI), then with CI switched on, both in quiet condition and with background noise. Results: Compared to NH subjects, deaf individuals were overall characterized by higher F0 and loudness values, especially in the vowel task than the reading. In the sustained vowel task, no patients demonstrated significant voice changes after switching on the CI; contrarily, in the reading task, the use of the CI reduced both loudness and F0 up to values comparable to NH subjects, although only in males. There was no significant difference in speech parameters between prelingual and postlingual deafness, although overall lower values were evident in case of postlingual deafness. The use of the CI showed a significant reduction of F0 in males with postlingual deafness and of loudness, both for patients with prelingual and postlingual deafness. Finally, there was a positive correlation between postoperative hearing thresholds and overall speech loudness, highlighting how subjects with better hearing outcomes after CI positioning generally speak with a lower loudness and therefore a reduced vocal effort and load. Discussion/Conclusion: We found similar speech performances between prelingual and postlingual deafness, both in the vowel /a/ phonation and in the reading, providing a further suggestion that prelingual adult patients may benefit from cochlear implantation in phonation as well, in addition to the known excellent hearing outcomes. Overall, these results highlight the ability of the CI to adjust in everyday speech certain phonatory aspects such as F0 and loudness by restoring the auditory feedback. [ABSTRACT FROM AUTHOR]

Introduction: Cochlear implantation is an effective treatment method for severe to profound hearing loss. Many factors that may influence cochlear implantation success have been explained in previous studies. Apart from those, minor differences in size of normal cochlear nerves may affect postoperative performance. Objective: To investigate whether the minor differences in cochlear nerve size in normal cochlear nerves affect postoperative cochlear implant performance. Methods: 30 pediatric prelingually deaf patients who were treated with cochlear implantation were included in this study. From the reconstructed parasagittal magnetic resonance images, the diameter and cross-sectional area of the cochlear nerve on the ipsilateral and contralateral side were measured. Auditory evaluations were performed 1, 3, 6 and 12 months following the first fitting. All the analysis was performed by using EARS®, evaluation of auditory responses to speech tool. Correlation between cochlear nerve diameter, cross-sectional area and postoperative auditory perception was analyzed to determine whether variation in cochlear nerve size contributes to postoperative auditory performance. Results: The mean diameter of the cochlear nerve on the ipsilateral side was 718.4 μm (504.5 − 904.3 μm) and mean cross sectional area was 0.015 cm2 (0.012 − 0.018 cm2). On the contralateral side the mean cochlear nerve diameter was 714.4 μm (502.6 − 951.4 μm) and mean cross sectional area was 0.014 cm2 (0.011 − 0.019 cm2). The correlation between the diameter and cross-sectional area of the ipsilateral and contralateral cochlear nerve revealed no significance. Mean score at first month monosyllable-trochee-polysyllable test, MTP1, was 0.17 (0.08 − 0.33), at 6th month with 6 words test, 6th month MTP6 was 0.72 (0.39 − 1.0), at 6th month with 12 words, 6th month MTP 12 was 0.46 (0.17 − 0.75) and at 12th month with 12 words, 12th month MTP12 was 0.73 (0.25 − 1.0). There was no correlation between the monosyllable-trochee-polysyllable test, values at any time with the diameter of the ipsilateral cochlear nerve. However, the first month MTP, 6th month MTP6 and 12th month MTP12 correlated with the cross-sectional area of the ipsilateral cochlear nerve. Conclusion: Measuring the cross sectional area of the normal- appearing cochlear nerve may give important prognostic knowledge on cochlear implant outcomes. In patients with a larger cross sectional area the auditory performance was better and faster. Although normal appearing, slight differences on cross sectional area of the cochlear nerve may affect performance. Measuring the size of the cochlear nerve on parasagittal magnetic resonance images may provide beneficial information on the postoperative rehabilitation process.

Cochlear implant (CI) users with prelingual deafness (hearing impairment started before language development was completed) show variable speech-in-noise (SIN) understanding. The present study aimed to assess cortical activation patterns to speech-in-quiet (SIQ) and SIN in prelingual CI users and compared to individuals with normal hearing (NH), using functional Near-Infrared Spectroscopy (fNIRS). Participants included 15 NH who listened to natural speech, 15 NH who listened via 8-channel noise-excited vocoder, and 14 prelingual CI users. fNIRS data were collected in a block design that included three conditions: SIQ, SIN in a signal-to-noise ratio of 0 dB, and noise. Speech reception thresholds in noise (SRTn) were also assessed. Results revealed different patterns of activation between the NH and CI participants in channels covering mainly the right and left middle temporal gyrus (MTG), depending on the SRTn of the CI users. Specifically, while the NH group showed large response to SIQ and SIN in the MTG areas, prelingual CI users with poor SRTn showed significantly smaller response to SIQ, and inversed response (a reduction in activation) to SIN in the same brain areas. These novel findings support the notion that the MTG can serve as a neural marker for speech understanding in CI patients. [ABSTRACT FROM AUTHOR]

The aim of our study is to compare the outcomes in unilateral and bilateral cochlear implants in pediatric age and also between simultaneous and sequential cochlear implant surgery. This retrospective study was carried out with 83 children aged between 12 months to 2.5 years which included 41 children with bilateral Cochlear implants and 42 with unilateral implants. Out of these 41 children, 21 were simultaneous and 20 were sequential cochlear implant. All the children were operated at civil hospital Gandhinagar, Gujarat, India. CAP, SIR, localization, traffic noise and speech in noise scores were assessed at regular intervals over the period of 4 years. Also the drug administration time, surgical time, operating room time were assessed for simultaneous and sequential cochlear implant surgery. Children with bilateral simultaneous implants fared significantly better with CAP, SIR, localization, speech noise and traffic noise scores than sequential bilateral implants and unilateral implants with a significant difference of means t tests between the two groups. Simultaneous cochlear implant surgery is associated with reduced surgical time, operating room time, it shortens the total in patient stay. There is less of drug administration and bilateral ones are stimulated simultaneously. Bilateral cochlear implants perform better with respect to auditory perception skills and spontaneous speech when compared with unilateral implants, but simultaneous surgery is better and safe option for pediatric cochlear implantation. [ABSTRACT FROM AUTHOR]

Purpose: This review discusses the assessment of psychotic disorders in prelingually deaf patients and the related challenges. Views: Although prelingually deaf patients are often diagnosed with psychotic disorders, the clinical presentation of this group is complex and no clear guidelines regarding the assessment process can be formulated due to the lack of valid, reliable research. Sign language dynamics or language dysfluency may be falsely recognized as disorganized thinking. Some of the symptoms indicating disorganized thinking in the case of spoken languages may fulfill specific functions when used in sign language (for example object chaining). Furthermore, deaf individuals experience multiple stressors that may be considered as risk factors for developing delusions, especially for those growing up in hearing, non-signing families. Nevertheless, it is important to acknowledge that some seemingly delusional beliefs shared by prelingually deaf people may be associated with the lack of assisted learning, gullibility, low level of confidence in healthcare or deaf communities' social norms. The question concerning the sensory modality of hallucinations experienced by deaf individuals remains unresolved. Patients' accounts suggest that hallucinations described as auditory may be perceived as lip-reading without identifying the speaker's face, among others. However, for those who provide help it is far more important to recognize the function of hallucinations and to differentiate them from normative experience. Conclusions: It is essential to expand our knowledge regarding the clinical presentation of psychotic disorders in deaf individuals in order to develop assessment guidelines and tools and, above all, increase the well-being of those suffering from psychotic disorders, as well as of those whose diagnosis remains unclear. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the rate of loss to follow-up in a cochlear implant program from the public health system in Southern Brazil as well as the characteristics of hearing loss, sociodemographic, sociocultural and the development of oral language in children with prelingual deafness., Methods: Retrospective cohort study with children who underwent CI surgery between 2010 and 2020. Data was collected through of interviews and review of medical records. The language development assessment was performed using the MUSS, MAIS and IT-MAIS scales. For the classification of language development, we used as parameters the values (mean ± SD) found in a previous national study. From those values, the Z-score for each patient at each hearing age (time of experience with the cochlear implant) was calculated., Results: Of the 225 children implanted between 2010-2020, 129 were included in this study. The rate of loss to follow-up in the program was 42.6%. The mean age at first surgery was 40.5 (±16.9) months, with 77.5% of patients having received a unilateral implant. Language results below the expected for hearing age (

Cochlear implant (CI) users with prelingual deafness (hearing impairment started before language development was completed) show variable speech-in-noise (SIN) understanding. The present study aimed to assess cortical activation patterns to speech-in-quiet (SIQ) and SIN in prelingual CI users and compared to individuals with normal hearing (NH), using functional Near-Infrared Spectroscopy (fNIRS). Participants included 15 NH who listened to natural speech, 15 NH who listened via 8-channel noise-excited vocoder, and 14 prelingual CI users. fNIRS data were collected in a block design that included three conditions: SIQ, SIN in a signal-to-noise ratio of 0 dB, and noise. Speech reception thresholds in noise (SRTn) were also assessed. Results revealed different patterns of activation between the NH and CI participants in channels covering mainly the right and left middle temporal gyrus (MTG), depending on the SRTn of the CI users. Specifically, while the NH group showed large response to SIQ and SIN in the MTG areas, prelingual CI users with poor SRTn showed significantly smaller response to SIQ, and inversed response (a reduction in activation) to SIN in the same brain areas. These novel findings support the notion that the MTG can serve as a neural marker for speech understanding in CI patients.

Abstract Introduction Cervical vestibular-evoked myogenic potentials (cVEMPs) are biphasic, short latency potentials, which represent the inhibition of the contraction of the sternocleidomastoid muscle (SCM) mediated by the saccule, the inferior vestibular nerve, the vestibular nuclei and the medial vestibular spinal tract. Objective To evaluate the response of cVEMPs in individuals with profound prelingual bilateral cochlear hearing loss. Methods A prospective case-control study. A total of 64 volunteers, divided into a study group (31 patients with profound prelingual sensorineural hearing loss) and a control group (33 subjects matched for age and gender with psychoacoustic thresholds of ≤ 25 dB HL between 500 and 8,000 Hz) were submitted to the cVEMP exam. The causes of hearing loss were grouped by etiology and the involved period. Results The subjects of the study group are more likely to present changes in cVEMPs compared to the control group (35.5% versus 6.1% respectively; p = 0.003), with an odds ratio (OR) of 8.52 (p = 0.009). It means that they had 8.52-fold higher propensity of presenting altered cVEMP results. There were no statistically significant differences between the latencies, the interamplitude and the asymmetry index. Regarding the etiology, there was a statistically significant difference when the cause was infectious, with an OR of 15.50 (p = 0.005), and when the impairment occurred in the prenatal period, with an OR of 9.86 (p = 0.009). Conclusion The present study showed abnormalities in the sacculocolic pathway in a considerable portion of individuals with profound prelingual sensorineural hearing loss due to infectious and congenital causes, as revealed by the cVEMP results.

Our study was a prospective, non-randomised, single tertiary care centre study involving 50 children below 10 years, all implanted with Nucleus 24™ cochlear implants to find the relationship between intra-operative NRT (Neural Response Telemetry) values and post-operatively behaviourally obtained Threshold (T) and Comfort (C) levels. NRT threshold values were obtained intra-operatively by using Custom Sound EP Software V 4.4™ (Cochlear Corporation). At switch-on after three weeks, behavioural T and C levels were measured based on behavioural responses given by patients using Custom Sound Version 4.4 software™ (Cochlear Corporation). NRT values were also measured at switch-on, 2 months, 3 months and 6 months. Intra-operative NRT levels (187.96 ± 12.48) were higher than both T (160.63 ± 22.69) and C (181.21 ± 22.41) levels obtained after 3 weeks. In addition, there was a weak correlation of NRT values with T (P value: 0.05, r = 0.391) and C (P value: 0.05, r = 0.390) levels. NRT is a quick and non-invasive tool to confirm cochlear implant integrity. There is a weak correlation of NRT values with both T and C levels and therefore, intra-operative NRT is a weak predictor for setting both T and C levels at switch-on. [ABSTRACT FROM AUTHOR]

Background: The spread of excitation (SOE) and auditory nerve recovery function (REC) are objective measures recorded by neural response telemetry and may interfere in cochlear implant (CI) stimulation. Objective: To analyze and correlate SOE with the refractory periods in subjects with pre- and postlingual deafness implanted with different electrode arrays. Methods: This was a retrospective study of 323 ears separated by perimodiolar or straight arrays and by pre- or postlingually deaf recipients. Measures were collected intraoperatively on electrode 11. The SOE width was measured in millimeters at the 0.75 point of the curve, and the relative (tau) and absolute (t0) refractory periods were measured in microseconds. Results: There was a statistical correlation between the SOE and the t0 in the patients with postlingual deafness implanted with the perimodiolar array. The SOE width was statistically different between the straight and perimodiolar arrays and between the pre- and postlingual groups in the perimodiolar array. Tau was statistically different between the pre- and postlingual groups with the straight array and the t0, between the pre- and postlingual groups with the perimodiolar array. Neural response threshold and amplitude of the neural response were not statistically different among groups. Conclusion: There was a correlation between SOE width and t0 only in patients with acquired deafness. The findings suggest that different factors influence SOE and REC, considering SOE is different according to the electrode array and REC being different according the onset of deafness. [ABSTRACT FROM AUTHOR]

OBJECTIVES: Aim of present study is to compare audiological and surgical outcomes in prelingual deaf children with Mondini's dysplasia (MD) and those with normal inner ear anatomy. MATERIALS and METHODS: Retrospective data was collected from Jan 2008 to Dec 2016. Children with bony IEM other than MD, syndromic association, multiple disabilities, those lost to follow up, and perilingual or postlingual deafness were excluded from study. Audiological outcomes for auditory perception (CAP score) and speech intelligibility (SIR score) was noted for a follow up period of 1 year. RESULTS: Mean age at implantation was 2.8 years (Range of 2 to 6 years). 2 patients had intraoperative CSF ooze which was controlled intraoperatively by conservative measures. Post operative facial nerve function was normal in all patients. None of the patient in either group had any complications at one year of follow up period. There was statistically significant improvement in CAP & SIR score in Group A at 6 & 12 months compared to pretreatment. There was no statistically significant difference between the 2 groups in terms of CAP & SIR score at 6 & 12 months. CONCLUSION: The study stresses the fact that cochlear implantation can be safely performed in children with MD although there is a risk of intraoperative CSF leak which can be controlled intraoperatively. Cochlear implantation in children with MD has good surgical, auditory and speech outcomes at par with children with normal bony inner ear anatomy. [ABSTRACT FROM AUTHOR]

Objectives: To demonstrate the feasibility of continuing cochlear implantation during the coronavirus disease 2019 crisis and to report on trends of referrals via the neonatal hearing screening programme. Methods: A prospective case series was conducted on children who underwent cochlear implantation during the coronavirus disease 2019 crisis in the UK and a sample of referrals via the neonatal hearing screening programme. A step-by-step description of peri-operative management is included. Results: Regionally, between February and May 2020, 106 babies were referred via the neonatal hearing screening programme to paediatric audiology. Eleven children were operated on during the coronavirus disease 2019 study period. None of the 11 children developed coronavirus symptoms. Discussion: It is widely recognised that the demands of managing the current pandemic may compromise screening, clinical assessment and elective surgery. Time-sensitive issues such as cancer management have gained prominence, but a similar need exists for timely paediatric cochlear implantation. Conclusion: Implantation in the paediatric population during the coronavirus disease 2019 pandemic is feasible with careful planning. [ABSTRACT FROM AUTHOR]

The broad spectrum of causal variants in the newly discovered GIPC3 gene is well reflected in worldwide studies. Except for one missense variant, none of the reported variants had reoccurred, thus reflecting the intragenic heterogeneity. We screened all the six coding exons of GIPC3 gene in a large cohort of 177 unrelated prelingual hearing impaired after excluding the common GJB2, GJB6 nuclear and A1555G mitochondrial variants. We observed a single homozygous pathogenic frameshift variant c.685dupG (p.A229GfsX10), accounting for a low incidence (0.56%) of GIPC3 variants in south Indian population. GIPC3 being a rare gene as a causative for deafness, the allelic spectra perhaps became much more diverse from population to population, thus resulting in a minimal recurrence of the variants in our study, that were reported by authors from other parts of the globe. [ABSTRACT FROM AUTHOR]

Children with hearing loss are more likely to suffer from voice and speech disorders than with normal hearing due to their poor and less robust auditory feedback mechanisms. Prelingually deaf children are not able to supervise their own voice due to lack of auditory feedback. Cochlear implanted children achieve better hearing and consequently enhanced speech intelligibility. Various parameters of voice improve with continous usage of cochlear implant. However all the vocal parameters do not show similar degree of change. Previous studies have assessed the voice quality of cochlear implantees and have given inconsistent results. The aim of the study is to compare the voice quality of cochlear implant children with normal subjects using objective and subjective methods. The study was undertaken after obtaining clearance from the Institutional Ethics committee. Written informed consent for carrying out voice analysis was taken from the parents. The voice analysis of 42 normal and 42 cochlear implant children were done using the Dr Voice software by Tiger Inc. All the children were age and sex matched. The parameters assessed were fundamental frequency, maximum phonation time, jitter%, shimmer% and harmonics to noise ratio. The findings of both the group were compared. The cochlear implanted children showed significant deviation of all the measured parameters as compared to the normal children. The voice quality of the implanted children is poor and deviant from the normal. Even after continuous usage of cochlear implant for 1 year the voice quality of these children did not reach to the levels of their normal hearing peers. [ABSTRACT FROM AUTHOR]

Introduction Cervical vestibular-evoked myogenic potentials (cVEMPs) are biphasic, short latency potentials, which represent the inhibition of the contraction of the sternocleidomastoid muscle (SCM) mediated by the saccule, the inferior vestibular nerve, the vestibular nuclei and the medial vestibular spinal tract. Objective To evaluate the response of cVEMPs in individuals with profound prelingual bilateral cochlear hearing loss. Methods A prospective case-control study. A total of 64 volunteers, divided into a study group (31 patients with profound prelingual sensorineural hearing loss) and a control group (33 subjectsmatched for age and gender with psychoacoustic thresholds of ≤ 25 dB HL between 500 and 8,000 Hz) were submitted to the cVEMP exam. The causes of hearing loss were grouped by etiology and the involved period. Results The subjects of the study group aremore likely to present changes in cVEMPs compared to the control group (35.5% versus 6.1% respectively; p = 0.003), with an odds ratio (OR) of 8.52 (p = 0.009). Itmeans that they had 8.52-fold higher propensity of presenting altered cVEMP results. There were no statistically significant differences between the latencies, the interamplitude and the asymmetry index. Regarding the etiology, there was a statistically significant difference when the cause was infectious, with an OR of 15.50 (p = 0.005), and when the impairment occurred in the prenatal period, with an OR of 9.86 (p = 0.009). Conclusion The present study showed abnormalities in the sacculocolic pathway in a considerable portion of individuals with profound prelingual sensorineural hearing loss due to infectious and congenital causes, as revealed by the cVEMP results. [ABSTRACT FROM AUTHOR]

Cochlear implants are expensive surgeries. It was expected that by the end of the year 2021 about 80,000 cochlear implants will be shipped worldwide. Alone in India 500 cochlear implants are placed in a year. Patients for these procedures are mostly young children with prelingual deafness. They often pose challenges like anxiety, post operative nausea and vomiting, haemorrhage and CSF leak or flap necrosis. Parent child separation is another concern in a deaf paediatric patient. A meticulous anaesthetic management provides comfortable induction and a stable intra operative hemodynamics to give favourable outcomes. [ABSTRACT FROM AUTHOR]

Background: Congenital cytomegalovirus (cCMV) infection is a major cause of sensorineural hearing loss in children. Objective of review: The objective of this systematic review was to compare performance in paediatric cochlear implant users with SNHL caused by cCMV compared to non‐cCMV implantees. Type of review: Systematic review Search strategy: PubMed, EMBASE and the Cochrane databases were searched from inception up to 15 May 2017 for children, cochlear implant, performance and their synonyms. Evaluation methods: Titles, abstracts and full texts were screened for eligibility. Directness of evidence and risk of bias were assessed. From the included studies, study characteristics and outcome data (speech perception, speech production, receptive language and auditory performance of cCMV groups and non‐cCMV groups) were extracted. Results: A total of 5280 unique articles were screened of which 28 were eligible for critical appraisal. After critical appraisal, 12 studies remained for data extraction. Seven of 12 studies showed worse performance after cochlear implantation in cCMV children compared to non‐cCMV children. Worse performance in cCMV children was attributed to cCMV‐related comorbidities in six of these studies. Available data on asymptomatic cCMV children compared to non‐cCMV children did not reveal an unfavourable effect on cochlear implant performance. Conclusions: The available evidence reveals that cCMV children often have worse cochlear implant performance compared to non‐cCMV children, which can be attributed to cCMV related comorbidities. We urge physicians to take into account the cCMV related comorbidities in the counselling of paediatric CI users deafened by cCMV. [ABSTRACT FROM AUTHOR]

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Objectives: It is known that early-deafened cochlear implant (CI) users are a very heterogeneously performing group. To gain more insight into this population, this study investigated (1) postoperative changes in auditory performance over time based on various outcome measures, focusing on poor performers, (2) self-perceived outcomes, (3) relations between auditory and self-perceived outcomes, and (4) preimplantation factors predicting postoperative outcomes.Methods: Outcomes were assessed prospectively in a group of 27 early-deafened, late-implanted CI users, up to 3 years after implantation. Outcome measures included open-set word and sentence recognition, closed-set word recognition, speech tracking and a questionnaire on self-perceived outcomes. Additionally, the relative influence of 8 preimplantation factors on CI outcome was assessed with linear regression analyses.Results: Significant improvements were found for auditory performance measures and most of the questionnaire domains. Significant changes of the closed-set word test, speech tracking and questionnaire were also found for a subgroup of poor performers. Correlations between auditory and self-perceived outcomes were weak and nonsignificant. Preoperative word recognition and preoperative hearing thresholds, both for the implanted ear, were significant predictors of postoperative outcome in the multivariable regression model, explaining 63.5% of the variation.Conclusions: Outcome measurement in this population should be adjusted to the patients' individual performance level and include self-perceived benefit. There is still a need for more knowledge regarding predictors of CI outcomes in this group, but the current study suggests the importance of the preoperative performance of the ear to be implanted. [ABSTRACT FROM AUTHOR]

Background: Individuals with early-onset severe--profound bilateral hearing loss (S/PHL) manifest diverse levels of benefit and satisfaction with hearing aids (HAs), even with prescriptive HA fitting. Such fittings incorporate normal loudness values, but little is known about aided loudness outcomes in this population and how those outcomes affect benefit or satisfaction. Purpose: To describe aided loudness growth and satisfaction with aided listening in experienced adult HA users with S/PHL. Research Design: The Contour Test of loudness perception was administered to listeners with S/PHL in the aided sound field using broadband speech, band-limited speech, and warble tones. Patterns and slopes of resultant loudness growth functions were referenced to sound field results from listeners with normal hearing (NH). S/PHL listeners also rated their aided listening satisfaction. It was expected that (1) most S/PHL listeners would demonstrate steeper than normal aided loudness growth, (2) loudness normalization would be associated with better high-frequency detection thresholds and speech recognition, and (3) closer approximation to normal would yield greater satisfaction. Study Sample: Participants were paid college-student volunteers: 23 with S/PHL, long-term aided listening experience, and new HAs; 15 with NH. Data Collection and Analysis: Participants rated loudness on four ascending runs per stimulus (5-dB increments) using categories defined in 1997 by Cox and colleagues. The region between the 10th and 90th percentiles of the NH distribution constituted local norms against which location and slope of the S/ PHL functions were examined over the range from Quiet to Loud-but-OK. S/PHL functions were categorized on the basis of their configurations (locations/slopes) relative to the norms. Results: Pattern of aided loudness was normalized or within 5 dB of the normal region on 37%of trials with sufficient data for analysis. Only one of the 23 S/PHL listeners did not demonstrate Normal/Near-normal loudness on any trials. Four nonnormal patterns were identified: Steep (recruitment-like; 38% of trials); Shifted right, with normal growth rate (10%); Hypersensitive, with most intensities louder than normal (10%); and Shallow, with decreasing growth rate (7%). Listeners with high-frequency average thresholds above 100 dB hearing loss or no phonemic-based speech-discrimination skill were less likely to display normalized loudness. Slope was within norms for 52% of S/PHL trials, most also having a Normal/Near-normal growth pattern. Regardless of measured loudness results, all but four listeners with S/PHL reported satisfactory hearing almost always or most of the time with their HAs in designated priority need areas. Conclusions: The variety of aided loudness growth patterns identified reflects the diversity known to characterize individuals with early-onset S/PHL. Loudness rating at the validation stage of HA fit with these listeners is likely to reveal nonnormal loudness, signaling need for further HA adjustment. High satisfaction, however, despite nonnormal loudness growth, suggests that listeners with poor auditory speech recognition may benefit more from aided loudness that supports pattern perception (via the time-intensity waveform of speech), different from most current-day prescription fits. [ABSTRACT FROM AUTHOR]

Abstract: Mutations in CDH23 are known to cause autosomal‐recessive nonsyndromic hearing loss (DFNB12). Until now, there was only one study describing its frequency in Indian population. We screened for CDH23 mutations to identify prevalent and recurring mutations among South Indian assortative mating hearing‐impaired individuals who were identified as non‐DFNB1 (GJB2 and GJB6). Whole‐exome sequencing was performed in individuals found to be heterozygous for CDH23 to determine whether there was a second pathogenic allele. In our study, 19 variants including 6 pathogenic missense mutations were identified. The allelic frequency of pathogenic mutations accounts to 4.7% in our cohort, which is higher than that reported previously; three mutations (c.429+4G>A, c.2968G>A, and c.5660C>T) reported in the previous Indian study were found to recur. DFNB12 was found to be the etiology in 3.4% of our cohort, with missense mutation c.2968G>A (p.Asp990Asn) being the most prevalent (2.6%). These results suggest a need to investigate the possibility for higher proportion of CDH23 mutations in the South Indian hearing‐impaired population. [ABSTRACT FROM AUTHOR]

• Satisfaction with life in prelingually deaf adults using cochlear implant(s), who were highly competent Polish speakers, was at the average level according to Polish norms as established by the Satisfaction With Life Scale. • Psychosocial functioning, assessed in terms of positive or negative self-perception and self-acceptance as a deaf person, was related to satisfaction with life, with a significant effect of depressive symptoms. • Marginal deaf identity was associated with low life satisfaction, and at the same time also with difficulties in socio-cultural identification (as deaf, hearing, or both). • Satisfaction with life did not depend on subjectively perceiving the benefit of a CI, at least in the areas of auditory perception and speech production. Instead, higher life satisfaction was positively related to self-esteem, fewer limitations in activity, and social interactions. • Life satisfaction appeared to be higher in CI users who, prelingually, were profoundly deaf than in those whose prelingual deafness had been rated as severe. This study investigated the level of satisfaction with life (SWL) in a group of cochlear implant (CI) users who had been prelingually deaf but were orally educated. They had received one or two CIs (as a child, adolescent, or adult) and were highly competent Polish speakers. This study looked at three factors that may affect SWL – psychosocial, deafness/hearing and communication related, and sociodemographic. The participants were prelingually deaf CI users who had learned highly competent spoken Polish as their primary language. They had been educated in mainstream or integrated schools (not schools for the deaf), and had no other disability or severe illness. Measurements were done with 5 questionnaires: the Satisfaction With Life Scale (SWLS), the I–Others Questionnaire , the Patient Health Questionnaire (PHQ-9), the Deaf Identity Development Scale (DIDS), and the Nijmegen Cochlear Implant Questionnaire (NCIQ). The SWL level of the group was similar to that of the standard Polish population. SWL was positively related to positive self-perception, acceptance of oneself as a deaf person, and to perceiving the benefits of having a CI (as measured by three NCIQ domains: self-esteem, activity limitations, and social interactions). On the other hand, negative self-perception, marginal deaf identity, and depressive symptoms were negatively related to SWL. There was no relationship between SWL and knowledge of sign language. Lower depressive symptoms and greater hearing loss were both significant predictors of SWL, although those who used two CIs generally had a lower SWL. Prelingually deaf CI users with low SWL require psychological support in many spheres, including working through problems of deaf identity, self-acceptance, and depression. Additional research should involve diverse DHH CI users, including those with limited spoken Polish competency or sign language skills, as well as members of the Polish Deaf community. [ABSTRACT FROM AUTHOR]

Objectives An effect of audio-visual (AV) integration is observed when the auditory and visual stimuli are incongruent (the McGurk effect). In general, AV integration is helpful especially in subjects wearing hearing aids or cochlear implants (CIs). However, the influence of AV integration on spoken word recognition in individuals with bilateral CIs (Bi-CIs) has not been fully investigated so far. In this study, we investigated AV integration in children with Bi-CIs. Methods The study sample included thirty one prelingually deafened children who underwent sequential bilateral cochlear implantation. We assessed their responses to congruent and incongruent AV stimuli with three CI-listening modes: only the 1st CI, only the 2nd CI, and Bi-CIs. The responses were assessed in the whole group as well as in two sub-groups: a proficient group (syllable intelligibility ≥80% with the 1st CI) and a non-proficient group (syllable intelligibility < 80% with the 1st CI). Results We found evidence of the McGurk effect in each of the three CI-listening modes. AV integration responses were observed in a subset of incongruent AV stimuli, and the patterns observed with the 1st CI and with Bi-CIs were similar. In the proficient group, the responses with the 2nd CI were not significantly different from those with the 1st CI whereas in the non-proficient group the responses with the 2nd CI were driven by visual stimuli more than those with the 1st CI. Conclusion Our results suggested that prelingually deafened Japanese children who underwent sequential bilateral cochlear implantation exhibit AV integration abilities, both in monaural listening as well as in binaural listening. We also observed a higher influence of visual stimuli on speech perception with the 2nd CI in the non-proficient group, suggesting that Bi-CIs listeners with poorer speech recognition rely on visual information more compared to the proficient subjects to compensate for poorer auditory input. Nevertheless, poorer quality auditory input with the 2nd CI did not interfere with AV integration with binaural listening (with Bi-CIs). Overall, the findings of this study might be used to inform future research to identify the best strategies for speech training using AV integration effectively in prelingually deafened children. [ABSTRACT FROM AUTHOR]

Objective Visual take-over of the auditory cortex in prelingual deaf children has been widely reported. However, there have been few studies on visual cortex plasticity after cochlear implantation (CI). In this study, we investigated the hypothesis that extrinsic auditory stimulation following CI in prelingual deafened children can induce visual cortex plasticity. Method Visual evoked potentials (VEPs) were recorded in 37 CI children (4 groups with different use times) and 8 control subjects, in response to sound and nonsound stimuli. Latency and amplitude were analyzed for the P1, N1 and P2 components on the Oz electrode. Comparisons of VEP were conducted between the sound and nonsound stimuli and among different groups in order to view evidence of visual cortex reorganization. Results The latency of the P2 component was significantly longer at the occipital site (Oz) in CI 0M than those in the other four groups. After the effect of age was excluded, a significant negative correlation was found between CI usage and P2 latency of nonsound stimuli. Occipital P1N1 latency and P1 amplitude were not affected by group or stimulus category. However, the N1 and P2 amplitudes were significantly larger in response to a sound stimulus than to a nonsound stimulus. Conclusion Our findings suggest that P2 latency develops with CI usage and may be a biomarker of visual cortex plasticity. [ABSTRACT FROM AUTHOR]

The objectives of this study were to assess: (i) patient expectations met as a measure of outcome in early-deafened, late-implanted (non-traditional) cochlear implant recipients and (ii) pre-implantation predictive factors for postoperative speech perception. The notes of 13 recipients were retrospectively reviewed. The mean age at onset of profound deafness was 1.5 years (range 0-6). The mean age at implantation was 37 years (range 22-51 years). Patient expectations were assessed pre-operatively and 1 year after implantation. They were met or exceeded in 129/140 (92%) domains overall. A higher Speech Intelligibility Rating and audiovisual City University of New York sentence score before implantation were found to be positive predictive factors for improved speech discrimination after cochlear implantation. [ABSTRACT FROM AUTHOR]

Objective: This study investigated the hypotheses that (1) prelingually deafened CI users do not have perfect electrode discrimination ability and (2) the deactivation of non-discriminable electrodes can improve auditory performance. Design: Electrode discrimination difference limens were determined for all electrodes of the array. The subjects' basic map was subsequently compared to an experimental map, which contained only discriminable electrodes, with respect to speech understanding in quiet and in noise, listening effort, spectral ripple discrimination and subjective appreciation. Study Sample: Subjects were six prelingually deafened, late implanted adults using the Nucleus cochlear implant. Results: Electrode discrimination difference limens across all subjects and electrodes ranged from 0.5 to 7.125, with significantly larger limens for basal electrodes. No significant differences were found between the basic map and the experimental map on auditory tests. Subjective appreciation was found to be significantly poorer for the experimental map. Conclusions: Prelingually deafened CI users were unable to discriminate between all adjacent electrodes. There was no difference in auditory performance between the basic and experimental map. Potential factors contributing to the absence of improvement with the experimental map include the reduced number of maxima, incomplete adaptation to the new frequency allocation, and the mainly basal location of deactivated electrodes. [ABSTRACT FROM AUTHOR]

Limiares auditivos de crianças surdas pré-linguais usuárias de implante coclear foram avaliados com estimulação elétrica em um dos eletrodos mediais. A avaliação empregou um procedimento operante do tipo go/no go para ensinar uma discriminação simples, evidenciada por uma resposta motora, entre presença e ausência do estímulo auditivo. Estabelecida a linha de base, a manipulação na intensidade do estímulo foi implementada de acordo com o método psicofísico de escada modificado, começando por uma seqüência descendente. Os sete participantes do estudo mostraram perda da precisão no responder sob controle do estímulo quando a intensidade diminuía além de um certo valor e a precisão era recuperada quando a intensidade era novamente aumentada, o que permitiu a identificação de limiares individuais. Os resultados sugerem que o método psicofísico combinado com o procedimento operante pode ser uma alternativa viável para avaliar limiar auditivo de pessoas sem linguagem em situação clínica de regulagem do implante coclear.Auditory thresholds of prelingually deaf children who received cochlear implants were evaluated for the electrical stimulation to one of the medial electrodes. A go/no go operant procedure was used to teach a simple discrimination, indicated by a motor response, between the presence versus the absence of an auditory stimulus. Simple discrimination was used as a baseline upon which the electrical stimulus's intensity was manipulated in a decreasing sequence followed by an increasing sequence, according to a modified psychophysical staircase method. The accuracy of responding in the presence of the electrical stimulus was reduced for all seven participants when the stimulus intensity decreased bellow a certain value and was recovered when the stimulus intensity was increased to the previous level. The experimental design allowed reliable identification of individual thresholds. The results suggest that the psychophysical paradigm in combination with the operant procedure might be useful for the evaluation of the auditory threshold for purposes of cochlear implant fitting in clinical settings.