Prelingual Sensorineural Hearing Loss Research Articles

The Effects of Core Stabilization Exercises on Respiratory Muscle Strength, Respiratory Functions, and Postural Control in Children With Hearing Loss: A Randomized Controlled Trial.

To evaluate the effects of core stabilization training on respiratory muscle strength, respiratory functions, and postural control in children with hearing loss. We conducted a randomized controlled trial at Bezmialem Vakif University, Faculty of Health Sciences, Division of Physiotherapy and Rehabilitation. Thirty children with hearing loss who were diagnosed with prelingual sensorineural hearing loss were randomly allocated to the study (n = 15, 12.20 ± 1.69 years) and control (n = 15, 11.87 ± 2.20 years) groups. Core stabilization training protocol was performed by the study group for 8 weeks, 5 days/week, whereas the control group received no training. The primary outcome measure was respiratory muscle strength. The secondary outcome measures were: spirometry, postural control tests on Biodex Balance System, and Balance Error Scoring System (BESS). Exercise adherence was recorded via a diary for home sessions. Outcomes were assessed at baseline and the end of the 8 weeks. In the study group, a significant improvement was observed in force vital capacity, peak expiratory flow of the spirometry test, maximum inspiratory pressure/expiratory pressure (MEP) of the respiratory muscle strength test; postural stability test except "anterior/posterior stability index", limits of stability test except "backward" and "forward/right", all parameters of the Modified Clinical Test for Sensory Interaction and Balance (mCTSIB) test except "eyes closed firm surface" of the Biodex Balance System, and total score of BESS. Group × time interaction was found in MEP, "overall" stability index in postural stability test, "overall," "left," and "backward left" of limits of stability test, all parameters of the mCTSIB, and BESS total. Core stabilization exercises were effective in improving respiratory muscle strength, spirometry, and postural control in children with hearing loss.

Hearing and speech outcomes in children with cochlear nerve hypoplasia who underwent cochlear implantation

Background: Data on hearing and speech outcomes following cochlear implantation in prelingual sensorineural hearing loss (SNHL) children with cochlear nerve hypoplasia, especially in Indonesia, is still limited. Purpose: To evaluate the hearing and speech function of prelingual SNHL children who had undergone cochlear implantation. Method: A retrospective cross-sectional study was conducted on 28 children who had undergone cochlear implantation procedures at the Department of Otorhinolaryngology and Head and Neck Surgery, Dr. Cipto Mangunkusumo National General Hospital, Jakarta, Indonesia, for more than 12 months. The hearing and speech function of the subjects were assessed using the modified Categories of Auditory Performance (CAP-II) and the speech intelligibility rating (SIR) scores. Result: Children with cochlear nerve hypoplasia showed an increase in CAP-II and SIR scores after using cochlear implants for more than 12 months (versus pre-surgery, high CAP score [≥5]: 89.3% [25/28 children] vs 3.6% [1/28 children] and high SIR score [≥3]: 82.1% [23/28 children] vs 7.1% [2/28 children]; both p<0.001). Post-operative CAP-II and post-operative SIR scores were highly positively correlated (r=0.705, p<0.001). Type of cochlear nerve hypoplasia (unilateral vs bilateral) were not significantly associated with post-operative CAP-II and SIR scores (p=0.382 and p=0.459, respectively). Conclusion: Prelingual SNHL children with cochlear nerve hypoplasia may still get the benefit from cochlear implantation, as shown by a remarkable improvement in hearing and speech function as assessed with the CAP-II and SIR scores.
 Keywords: auditory perception, cochlear implants, cochlear nerve hypoplasia, prelingual sensorineural hearing loss, speech intelligibility

Communicative and Hearing Performance in Individuals with Cochlear Implants and Delayed Neuropsychomotor Development: A Longitudinal Analysis.

Introduction Between 15% to 30% of individuals with bilateral prelingual sensorineural hearing loss present with associated disabilities. Cochlear implant (CI) is an alternative treatment that provides consistent access to environmental and speech sounds, which results in significant benefits regarding quality of life and auditory and language development. Objectives To study the auditory and communicative performance of individuals with CI and delayed neuropsychomotor development after a minimum of five years using the device. Methods A total of eight patients were included in the study. We collected the multidisciplinary clinical records of participants, as well as the answers for the questionnaires applied remotely, which included the Children with Cochlear Implants: Parental Perspectives (CCIPP), International Classification of Functioning, Disability and Health: Children and Youth Version (ICF-CY), and the Gross Motor Function Classification System (GMFCS). Results We found that throughout the years of CI use, the auditory threshold means improved significantly in all tested frequencies, as did the speech detection threshold and the language and hearing results. Regarding parental perception, parents evaluated aspects related to their children's social relations to be positive, and had worse perceptions regarding aspects related to their education. Conclusion We observed a progression in the participants' auditory and language skills throughout the years of CI use; even in the presence of other associated disabilities. Future multicentric studies with larger samples are needed to further the advancement of rehabilitation in patients with other associated disabilities.

Novel homozygous variant in the PDZD7 gene in a family with nonsyndromic sensorineural hearing loss

Hearing loss is the most common sensory neural disorder in humans, and according to a WHO estimation, 5.5% (466 million) of people worldwide have disabling hearing loss. In this study, a Chinese family with prelingual sensorineural hearing loss was investigated. The affected individuals showed moderately severe hearing loss at all frequencies. Using target genome enrichment and high-throughput sequencing, the homozygous variant c.2372del; p.(Ser791fs) was identified in PDZD7. This variant lies in exon 15 of PDZD7 and results in a frame shift followed by an early stop codon. It is classified as pathogenic according to the ACMG/AMP guidelines and ClinGen specifications. Our study expands the pathogenic variant spectrum of PDZD7 and strengthens the clinical importance of this gene in patients with moderately severe hearing loss.

SMS classification of inner ear malformations: our experience with implantation in type II anomalies.

To study and analyse the radiological and surgical findings of 24 cochlear implantees with SMS type II cochleovestibular malformation and to compare their outcomes in terms of hearing and speech gains pre- and postoperatively. Retrospective analysis of cochlear implanted candidates over a period of 8year 6months from 3 institutions was undertaken and 24 patients suffering from prelingual profound congenital sensori-neural hearing loss along with the presence of SMS type II cochleovestibular malformation were studied. Preoperative radiology, surgical difficulties and complication, and postoperative hearing and speech outcomes up to a period of 2years, using IT-MAIS and SIR scores were noted. Statistical comparison pre- and postimplantation was done using Wilcoxon signed rank test and a p value of < 0.05 was considered statistically significant. In 24 cases, we encountered four cases of SMS type IIa malformation with a smaller modiolus, while the rest 20 were type SMS type IIb with partially defective modiolus. Intraoperatively, CSF leak was observed in 11 patients. Complete electrode insertion and good electrical response (NRT) was detected in all. No facial nerve anomaly was encountered. IT-MAIS and SIR scores increased from a preoperative mean of 5.6 and 1.16-34.56 and 3.88 after 2 years postsurgery, respectively and this difference was found to be statistically significant. Our experience with SMS Type II malformations show promising and motivating results with less chances of complications. Proper evaluation and surgical planning preoperatively can lead to an uneventful surgery with good outcomes. A simple and uniform classification system of these anomalies is a must for appropriate prognostication and right decision making.

Beyond Audition: Psychosocial Benefits of Music Training for Children With Hearing Loss.

Children with hearing loss tend to have poorer psychosocial and quality of life outcomes than their typical-hearing (TH) peers-particularly in the areas of peer relationships and school functioning. A small number of studies for TH children have suggested that group-based music activities are beneficial for prosocial outcomes and help develop a sense of belonging. While one might question whether perceptual limitations would impede satisfactory participation in musical activities, findings from a few studies have suggested that group music activities may have similar benefits for children with hearing loss as well. It is important to note that the effect of music on psychosocial outcomes has primarily been investigated at an anecdotal level. The objective of this study was to explore the effect of a music training program on psychosocial and quality of life outcomes for children with hearing loss. It was hypothesized that music training would provide benefits for domains centered upon peer relationships and prosocial measures. Fourteen children aged 6 to 9 years with prelingual sensorineural hearing loss (SNHL) participated in a 12-week music training program that consisted of group-based face-to-face music therapy supplemented by online music apps. The design was a pseudorandomized, longitudinal study (9 participants were waitlisted, initially serving as a passive control group). Psychosocial wellbeing and quality of life were assessed using a questionnaire battery comprised of the Strengths and Difficulty Questionnaire (SDQ), the Pediatric Quality of Life Inventory, the Hearing Environments and Reflection on Quality of Life (HEAR-QL), and the Glasgow Children's Benefit Inventory. For comparative purposes, responses were measured from 16 TH children that ranged in age from 6 to 9 years. At baseline, children with SNHL had poorer outcomes for internalizing problems, and all measures of the HEAR-QL compared with the TH children. There were no differences for general psychosocial and physical health. After music training, SDQ internalizing problems such as peer relationships and emotional regulation were significantly reduced for the children with SNHL. There were no changes for any outcomes for the passive control group. Additional benefits were noted for emotional and learning factors on the Glasgow Children's Benefit Inventory. However, there were no significant changes for any psychosocial and quality of life outcomes as measured by the Pediatric Quality of Life Inventory or HEAR-QL instruments. The present study provides initial evidence that music training has a positive effect on at least some psychosocial and quality of life outcomes for children with hearing loss. As they are at a greater risk of poorer psychosocial and quality of life outcomes, these findings are cause for cautious optimism. Children with hearing loss should be encouraged to participate in group-based musical activities.

Use of Cone Beam Computed Tomography to Assess Cochlear implant Electrode Position with Correlation to Performance.

Aim: Cone Beam Computed Tomography (CBCT) has been used in a variety of maxillodental applications and recently, it has proved to be effective in the otorhinolaryngology field. In this study, we used CBCT for assessment of scalar location, insertion length and distance between the cochlear implant electrode array and modiolus and correlate these factors to speech performance.Materials & Methods: This study was carried out on 30 children with bilateral severe to profound prelingual sensorineural hearing loss that had undergone cochlear implantation. Radiological assessment of electrode arrays position using CBCT was performed and correlated with speech recognition score..Results: CBCT revealed that the electrode array was inserted in the scala tympani in 26/30 children (86.66%) and showed better speech recognization score than scala vestibule insertion. The mean distance between the modiolus and electrode arrays was 0.53 ± 0.11 mm and the average length of insertion was 21.49 ± 3.65mm. Distance between the electrode array and modiolus was negatively correlated to audiological performance in all patients. Pearson test showed a statistically significant positive correlation between insertion length and speech recognition score. Conclusion: CBCT is very effective in localizing cochlear implant electrode arrays. Depth of insertion and closer distance to modiolus are associated with statistically better speech outcome.

Music Training for Children With Sensorineural Hearing Loss Improves Speech-in-Noise Perception.

Purpose A growing body of evidence suggests that long-term music training provides benefits to auditory abilities for typical-hearing adults and children. The purpose of this study was to evaluate how music training may provide perceptual benefits (such as speech-in-noise, spectral resolution, and prosody) for children with hearing loss. Method Fourteen children aged 6-9 years with prelingual sensorineural hearing loss using bilateral cochlear implants, bilateral hearing aids, or bimodal configuration participated in a 12-week music training program, with nine participants completing the full testing requirements of the music training. Activities included weekly group-based music therapy and take-home music apps three times a week. The design was a pseudorandomized, longitudinal study (half the cohort was wait-listed, initially serving as a passive control group prior to music training). The test battery consisted of tasks related to music perception, music appreciation, and speech perception. As a comparison, 16 age-matched children with typical hearing also completed this test battery, but without participation in the music training. Results There were no changes for any outcomes for the passive control group. After music training, perception of speech-in-noise, question/statement prosody, musical timbre, and spectral resolution improved significantly, as did measures of music appreciation. There were no benefits for emotional prosody or pitch perception. Conclusion The findings suggest even a modest amount of music training has benefits for music and speech outcomes. These preliminary results provide further evidence that music training is a suitable complementary means of habilitation to improve the outcomes for children with hearing loss.

Application of the new SMS system of cochleovestibular anomalies: our experience with nine cases of type III anomaly.

To present and analyse the radiological findings, surgical findings, pre-implantation and post-implantation speech and hearing outcomes of nine children with SMS Type III cochleovestibular malformation. Retrospective case series of nine children with pre-lingual profound sensorineural hearing loss who underwent cochlear implantation (Jan 2012 to July 2019). These children had been classified as Type III malformation according to theSMS Classification of cochleovestibular anomalies. Facial nerve anomalies, CSF leaks and any other significant surgical finding were noted. Meaningful Auditory Integration Scale (MAIS) was used to report the child's pre-operative and two-year post-implantation auditory and speech abilities. Any significant improvement was assessed using the Wilcoxon signed rank test. P value < 0.05 was considered significant. Out of nine patients, five patients sustained CSF gushers, while three patients had mild CSF leak, which were plugged adequately. No facial nerve anomalies were encountered. Post-op course was uneventful for all nine patients. MAIS scores at a two-year follow-up showed significant statistical improvement (P < 0.05) when compared to pre-operative scores. Cochlear implant is the treatment of choice for children with type III cochleovestibular malformation. There are significant auditory and speech improvements expected. However, the surgeon should bear in mind the risk of CSF leak and subsequent meningitis.

Quality of life and parental care burden in cochlear implanted children: A case–control study

ObjectivesCochlear implantation is a widely accepted and effective surgical method used to treat severe hearing loss. What's more, it affects the lives of both cochlear implanted children and their parents. This study aims to compare cochlear-implanted children (CIC) and their parents with healthy counterparts and their parents in terms of the quality of life (QOL) and parental care burden (CB). MethodsThis study was conducted between February and December 2018 in Turkey after receiving approval from the ethics committee. The Case Group included 34 children between 3 and 7 years of age, who received a CI due to bilateral prelingual sensorineural hearing loss and were using it for at least 1 year, and their parents. The Control Group consisted of 68 healthy children and their parents. The data were collected using disease and age-specific quality of life scales and burden interview. Normally distributed variables were analysed using parametric tests while non-normally distributed variables were analysed using nonparametric tests. The odds ratio (OR) and confidence interval (95%) were also calculated. Results were evaluated at significance level of p < 0.05. ResultsThe mean age of the children and their parents in both Case and Control Groups was 63.9 months and 33.8 years, and 61.3 months and 36.6 years, respectively. There was a positive correlation between PPQ social relationship subscale and KINDL subscale scores in CIC (p < 0.05). The Case Group obtained lower mean scores from the subscales of KINDL and WHOQOL-BREF (p < 0.001, p < 0.01). The ZBI mean scores of the parents in Case and Control Groups were 43.18 and 27.54, respectively (p = 0.000). ConclusionIt was determined that the QOL of the Case Group was lower than QOL of the Control Group, while the CB of the Case Group was higher than CB of the Control Group.

A novel mutation in TRIOBP gene leading to congenital deafness in a Chinese family

BackgroundThe autosomal recessive non-syndromic deafness DFNB28 is characterized by prelingual sensorineural hearing loss. The disease is related with mutations in TRIOBP (Trio- and F-actin-Binding Protein) gene, which has three transcripts referred to as TRIOBP-5, TRIOBP − 4 and TRIOBP-1. Among them, TRIOBP-5/− 4 are expressed in the inner ears and crucial for maintaining the structure and function of the stereocilia.MethodsThe proband is a 26-year-old Chinese female. She and her younger brother have being suffered from severe deafness since birth, whereas her parents, who are cousins, have normal communication ability. Hearing impairment of the two siblings was determined by pure tone audiometry. Whole Exome Sequencing (WES) was performed on the genomic DNA of the proband and Sanger sequencing was conducted on the DNA samples of the four family members.ResultsTests of pure tone hearing thresholds showed a severe to profound symmetric hearing loss for the proband and her younger brother. Moreover, a novel TRIOBP c.1342C > T (p.Arg448*) variant was identified by WES in the DNA sample of the proband and confirmed by Sanger sequencing in DNA of the family members.ConclusionsThe TRIOBP c.1342C > T (p.Arg448*) variant is predicted to disrupt TRIOBP-5 and TRIOBP-4, which may lead to the congenital deafness. The results will broaden the spectrum of pathogenic variants in TRIOBP gene. The characteristics of deafness in the family imply that marriage between close relatives should be avoided.

Prelingual Sensorineural Hearing Loss Caused by a Novel GJB2 Dominant Mutation in a Chinese Family.

Background GJB2 mutation is the most common cause of genetic deafness. Many pathogenic variations have already been identified, and thus, fewer and fewer novel pathogenic variations remain to be identified. Here, we describe a novel pathogenic variation associated with dominant hereditary deafness in a Chinese family. Methods In this study, we examined four generations of a Chinese family (M127) with hearing loss. Temporal CT scan, complete physical examination (including skin and hair), and audiological tests were performed. Targeted next-generation and Sanger sequencing were used to identify pathogenic mutations in affected individuals. Results All patients exhibited prelingual nonsyndromic sensorineural hearing loss, with severity ranging from moderate to severe. A novel dominant pathogenic variant c.205T > C (p.Phe69Leu) was identified in all patients in this family. Conclusions c.205T > C (p.Phe69Leu) was identified as a novel dominant pathogenic variant of GJB2 associated with prelingual nonsyndromic sensorineural hearing loss.GJB2 mutation is the most common cause of genetic deafness. Many pathogenic variations have already been identified, and thus, fewer and fewer novel pathogenic variations remain to be identified. Here, we describe a novel pathogenic variation associated with dominant hereditary deafness in a Chinese family.

Delineation of Homozygous Variants Associated with Prelingual Sensorineural Hearing Loss in Pakistani Families.

Hearing loss is a genetically heterogeneous disorder affecting approximately 360 million people worldwide and is among the most common sensorineural disorders. Here, we report a genetic analysis of seven large consanguineous families segregating prelingual sensorineural hearing loss. Whole-exome sequencing (WES) revealed seven different pathogenic variants segregating with hearing loss in these families, three novel variants (c.1204G>A, c.322G>T, and c.5587C>T) in TMPRSS3, ESRRB, and OTOF, and four previously reported variants (c.208C>T, c.6371G>A, c.226G>A, and c.494C>T) in LRTOMT, MYO15A, KCNE1, and LHFPL5, respectively. All identified variants had very low frequencies in the control databases and were predicted to have pathogenic effects on the encoded proteins. In addition to being familial, we also found intersibship locus heterogeneity in the evaluated families. The known pathogenic c.226C>T variant identified in KCNE1 only segregates with the hearing loss phenotype in a subset of affected members of the family GCNF21. This study further highlights the challenges of identifying disease-causing variants for highly heterogeneous disorders and reports the identification of three novel and four previously reported variants in seven known deafness genes.

Benefits of using a contralateral hearing aid in cochlear implanted children with bilateral pre-lingual profound sensorineural hearing loss on language development and auditory perception performance

Objective: To evaluate the benefits of a contralateral hearing aid on expressive and receptive language development and auditory perception performance over a 36-month post-surgical period in children implanted on a single side with a cochlear device for bilateral pre-lingual profound sensorineural hearing loss. Methods: Seventy-five patients with idiopathic profound sensorineural hearing loss were included. The cases were divided into two groups: cochlear implant users (50 patients, “CI group”) and cochlear implant plus hearing aid users (25 patients, “CI+HA group”). Language development and auditory performance were compared in the two groups during the first 3 years following cochlear implant surgery. The Pre-school Language Scale-4 was used to assess language development and the LittlEars® Auditory Survey, Meaningful Auditory Integration Scale and Meaningful Use of Speech Scale were employed to assess auditory perception performance. Results: Language development in the CI+HA group was superior to that in the CI group at 6 months post-surgery, in terms of receptive and expressive language development; auditory perception performance was also superior in the CI+HA group, compared to the CI group. Conclusion: The use of a contralateral hearing aid in cochlear implanted children with prelingual sensorineural hearing loss positively contributes to language development and auditory perception performance.

Comprehensive Analysis of Factors Leading to Poor Performance in Prelingual Cochlear Implant Recipients.

To comprehensively analyze the prognostic factors responsible for affecting outcomes following cochlear implantation in prelinguals. Retrospective cohort study from June 2004 to November 2015. Tertiary care center. Patients who had undergone cochlear implantation during June 2004 to November 2015 for prelingual sensorineural hearing loss with a minimum follow-up of 2 years. Patients were evaluated for the presence of 20 risk factors possibly influencing postimplantation outcomes using a questionnaire. Assessment for speech and auditory function was done at the last follow-up with speech intelligibility ratings (SIR) and categories of auditory performance (CAP) scores, respectively. One hundred fifty-one patients were included in this study. On univariate analysis for CAP, age at implantation, noncompliance to pre and postimplantation auditory and speech habilitation, poor parental motivation, socioeconomic status and literacy were found to be associated with lower scores (p < 0.05). Whereas, for SIR, additionally, attention deficit hyperactivity disorder and inner ear malformation were statistically significant negative predictors on univariate analysis. Finally, factors responsible for low CAP scores on multivariate analysis were poor parental literacy, poor socioeconomic status, irregular pre/postimplantation rehabilitation, and attention deficit hyperactivity disorder. While for SIR, additionally age at implantation was also a significant negative predictor. Increasing IQ and duration of implant use were associated with higher CAP (univariate) and SIR scores (univariate and multivariate) (p < 0.05). We were able to demonstrate negative impact of higher age at implantation, minimal cognitive disorder, adverse parental/socioeconomic profile, and poor compliance to pre/postimplantation auditory verbal habilitation on auditory and speech outcomes.

Oxidative stress in prelingual sensorineural hearing loss and the effects of cochlear implant application on serum oxidative stress levels

ObjectivesThe aim of this study is to investigate oxidative stress conditions in patients with prelingual profound sensorineural hearing loss (SNHL) and the effects of cochlear implant application on oxidative stress. In addition, we also aimed to evaluate the correlation between pre- and post-surgery oxidative stress markers and Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS), Meaningful Use of Speech Scale (MUSS) and Free-Field Audiometry (FFA). MethodsThis is a prospective controlled study of the Ear-Nose-Throat Department of the Harran University Faculty of Medicine between April 2017 and January 2018. The study included 25 patients with prelingual profound SNHL diagnosis and cochlear implant surgery and 25 healthy control groups. Total thiol, native thiol, disulphide levels and LOOH levels were studied in serum samples taken from the patient group 2 weeks before and 6 months after the cochlear implant surgery and the healthy control group. In addition, IT-MAIS, MUSS and FFA tests, which were used to evaluate the personal development of patients with cochlear implants, were performed at preoperative and postoperative 6th month. ResultsIn our study, we found that the levels of native thiol (p < 0.01) and total thiol (p < 0.01) were significantly lower and LOOH (p < 0.04) values were significantly higher in preoperative SNHL patients than control group. We found that native thiol, total thiol values increased and LOOH values decreased in the postoperative with respect to preoperative values. However, the difference between preoperative and post operative values was not statistically significant. The correlation between pre- and post-surgery biochemical data and IT-MAIS, MUSS and FFA tests were found not to be significant. ConclusionsOur study suggests that patients with prelingual profound SNHL are under oxidative stress and post-CI surgery data show that there is no significant relief in patients. This study can shed light on the biologic mechanisms between prelingual profound SNHL and oxidative stress.

Microstructural Alterations in the Brains of Adults With Prelingual Sensorineural Hearing Loss: a Diffusion Kurtosis Imaging Study.

To investigate the alterations in microstructural gray matter (GM) and white matter (WM) in adults with prelingual sensorineural hearing loss (SNHL) using diffusion kurtosis imaging (DKI) and explore whether the clinical data are associated with the whole brain structure changes in SNHL subjects. Prospective case study. Tertiary class A teaching hospital. Eighty adults with prelingual SNHL. Diagnostic. DKI-derived metrics, including mean kurtosis, radial kurtosis, axial kurtosis, fractional anisotropy, mean diffusivity, radial diffusivity, and axial diffusivity were applied to explore the differences in GM and WM between the groups across the whole brain with voxel-based analysis. Compared with the control group, adults with prelingual SNHL exhibited significantly decreased fractional anisotropy, mean kurtosis, radial kurtosis, and axial kurtosis in the bilateral superior temporal gyrus in both the GM and WM. No correlations were detected between these alterations and clinical data. These findings suggest that DKI can provide comprehensive characteristics to demonstrate reductions in the auditory cortical GM and hypomyelination of WM fibers in the auditory pathway caused by auditory deprivation early in life.

The Relationship between the Etiology of Profound Prelingual Sensorineural Hearing Loss and the Results of Vestibular-Evoked Myogenic Potentials.

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.

Auditory development in 1－5 years prelingual hearing loss children after cochlear implantation

Objective:To study the early prelingual auditory development in the pediatric cochlear implant（CI） recipients from 1 years old to 5 years old. Method:The study was designed as retrospectively. A total of 103 children with profound prelingual sensorineural hearing loss were included in this study. All participants were divided into four groups as 1－2 yr，＞2－3 yr，＞3－4 yr and ＞4－5 yr，according to the age of cochlear implantation. The scores of IT-MAIS/MAIS（total scores，sound detection scores and sound recognition scores）before implantation（or at switch-on）and 3,6,12 months after implantation were analyzed with Mixed linear model. Result:The scores of IT-MAIS/MAIS improved notably after cochlear implantation in all four groups as time prolonged（P<0.01).There were no significant difference in total scores，sound detection scores and sound recognition scores among those four different implant age groups（P>0.05）after switch-on. As the rehabilitation duration，the abilities of overall auditory，sound detection and sound recognition for those CI recipients showed a similar increasing trend in normal hearing pediatrics,and there were no significant difference among the four groups（P>0.05）. Conclusion:The early prelingual auditory abilities of pediatric CI recipients implanted at 1-year-old up to 5-year-old developed considerably within the first year after cochlear implantation. What's more, the implant age has no obvious influence on their early prelingual auditory abilities after implant surgery.

Audit of Bilateral Simultaneous Cochlear Implantation in Pediatric Population: South Indian Study.

To conduct a medical audit of bilateral simultaneous cochlear implantation (CI) in patients with severe prelingual sensorineural hearing loss (SNHL). A medical audit of a tertiary care ear, nose, and throat center in Southern India was conducted on data collected from January 2007 to December 2014. All cochlear implantees <6 years of age with severe bilateral SNHL who underwent bilateral simultaneous CI were included in the present study. The exclusion criteria were children >6 years, sequential bilateral CI, revision cases, abnormal or malformed cochlea, and children with global developmental delay in milestones. Subjective outcome scores used were Category of Auditory Performance (CAP) and Speech Intelligibility Rating (SIR). The CAP and SIR results showed that 20% of implantees achieved peak scores of 7 and 5, respectively. Mean CAP and SIR scores at 12 months were 5.4 and 3.1, respectively. The present study supports the claim that bilateral CI in severe prelingual bilateral SNHL is better than unilateral and recommends that bilateral CI should be the standard of care in children.