Bilateral Profound Hearing Loss Research Articles

Ambulatory Phonation Monitoring in Prelingual and Postlingual Deaf Patients after Cochlear Implantation

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.

The Effect of Age, Type of Noise, and Cochlear Implants on Adaptive Sentence-in-Noise Task.

Adaptive tests of sentences in noise mimic the challenge of daily listening situations. The aims of the present study were to validate an adaptive version of the HeBio sentence test on normal hearing (NH) adults; to evaluate the effect of age and type of noise on speech reception threshold in noise (SRTn); and to test it on prelingual adults with cochlear implants (CI). In Experiment 1, 45 NH young adults listened to two lists accompanied by four-talker babble noise (4TBN). Experiment 2 presented the sentences amidst 4TBN or speech-shaped noise (SSN) to 80 participants in four age groups. In Experiment 3, 18 CI adult users with prelingual bilateral profound hearing loss performed the test amidst SSN, along with HeBio sentences and monosyllabic words in quiet and forward digits span. The main findings were as follows: SRTn for NH participants was normally distributed and had high test–retest reliability; SRTn was lower among adolescents and young adults than middle-aged and older adults, and were better for SSN than 4TBN; SRTn for CI users was higher and more variant than for NH and correlated with speech perception tests in quiet, digits span, and age at first CI. This suggests that the adaptive HeBio can be implemented in clinical and research settings with various populations.

Parents’ experiences of remote microphone systems for children with hearing loss

Purpose Remote microphone systems improve intelligibility in difficult conditions when the performance of hearing aids/cochlear implants is insufficient. The purpose of this study was to explore parents’ experiences with remote microphone systems for their children with hearing loss and to determine the advantages and disadvantages as perceived by parents. Materials and methods Data were collected by means of semi-structured interviews with parents of children with moderate to profound bilateral hearing loss (2–19 years old). The open coding method and thematic analysis were used. The final sample consisted of 19 mothers and 9 fathers who had experience with remote microphone systems. Results Parents listed the advantages of remote microphone systems for their child, for themselves and for other carers, such as better hearing and understanding, a life more similar to that of their peers without hearing loss, safety in road traffic, lower fatigue, vocabulary acquisition, better school results. Some limitations were identified, namely low benefits, technical issues and a reluctance to use the device by children or teachers. Conclusions It is crucial to provide parents with information about assistive devices and the consequences of limited access to hearing speech. Professionals should motivate parents, children and teachers to use remote microphone systems even in situations when the benefit may not be obvious if there is a potential benefit for the child. IMPLICATIONS FOR REHABILITATION The main advantages of remote microphone systems perceived by parents are better hearing and understanding, a life more similar to peers without hearing loss, safety in traffic, lower fatigue, vocabulary acquisition and better school results. The main disadvantages are for parents: persisting hearing problems, low benefits, technical issues and a reluctance to use the device by children or teachers. Sufficient and accurate information is needed about remote microphone systems, but also about the consequences of hearing loss in general. The possibility to try wireless devices before buying and troubleshooting help is appreciated.

Cochlear implants evaluation for congenital rubella syndrome patients in Dr. Soetomo Academic Medical Center

Congenital rubella syndrome (CRS) is an infection caused by rubella virus that transmitted to the fetus during pregnancy which can cause congenital hearing loss. Cochlear implant is an effective therapy in children with severe to profound bilateral hearing loss. Categories of Auditory Performance (CAP) method is a receptive scale assessment of auditory stimulation that can be applied to all children regardless of age, before and after cochlear implant. Evaluating the results of cochlear implants using CAP-II in patients with CRS. A retrospective descriptive study were conducted to samples taken from medical records of CRS patients who had cochlear implants in 2011-2018 at ORL-HNS department of Dr. Soetomo Academic Medical Center. Evaluation of CAP-II results was performed 6 months, 12 months and 24 months after cochlear implant. Eleven CRS patient who underwent cochlear implant during 2011-2018. Six months after cochlear implant showed the highest scale was 6. At 12-months evaluation showed the highest scale was 7, while at 24-months evaluation showed the highest scale was 8. Cochlear implants provide good auditory performance based on CAP-II scale in sensorineural hearing loss of CRS patients.

Younger Age at Cochlear Implant Activation Results in Improved Auditory Skill Development for Children With Congenital Deafness.

The U.S. Food and Drug Administration indications for cochlear implantation in children is currently 9 months of age and older for children with bilateral profound sensorineural hearing loss (SNHL). Studies have shown that earlier activation of a cochlear implant (CI) can lead to better spoken language outcomes. As auditory skills are a precursor to the development of spoken language, this study was developed to investigate the influence of age at CI activation on auditory skill acquisition in young children. A secondary aim was to describe the auditory skills of children implanted prior to 9 months of age as compared to children with older ages of activation. Functional Listening Index (FLI) scores obtained during routine clinical visits were reviewed for 78 pediatric CI recipients with congenital bilateral profound hearing loss who were activated before 2 years of age. A linear mixed-effects model assessed the effect of age at CI activation on cumulative FLI scores over time. There was a significant interaction between age at activation and chronological age at the time of evaluation, indicating that children with earlier access to sound achieved a greater number of auditory skills than those with later CI activations when measured at the same chronological age. Children activated before the age of 9 months approximated scores expected of children with typical hearing, whereas children activated between 9 and 24 months of age did not. Younger age at CI activation is associated with increased auditory skills over time. Children who undergo cochlear implantation and CI activation before 9 months achieve more auditory skills by 4 years of age than children who are activated at later ages. These data suggest that reducing the approved age at cochlear implantation for children with congenital bilateral profound SNHL may support optimal auditory skill acquisition.

Sequential bilateral cochlear implant: long-term speech perception results in children first implanted at an early age

PurposeThe study aims to assess the benefit of sequential bilateral cochlear implantation in children with congenital bilateral profound hearing loss, submitted to the first implant at an early age.MethodsWe enrolled all the bilateral sequential cochlear implanted children who received the first implant within 48 months and the second within 12 years of age at our Institution. The children were submitted to disyllabic word recognition tests and Speech Reception Threshold (SRT) assessment using the OLSA matrix sentence test with the first implanted device (CI1), with the second implanted device (CI2), and with both devices (CIbil). Furthermore, we measured the datalogging of both devices. Then we calculated the binaural SRT gain (b-SRTgain) and checked the correlations between speech perception results and the b-SRTgain with the child’s age at CI1 and CI2, DELTA and the datalogging reports.ResultsWith the bilateral electric stimulation, we found a significant improvement in disyllabic word recognition scores and in SRT. Moreover, the datalogging showed no significant differences in the time of use of CI1 and CI2. We found significant negative correlations between speech perception abilities with CI2 and age at CI2 and DELTA, and between the SRT with CI1 and the b-SRTgain.ConclusionsFrom this study we can conclude that in a sequential CI procedure, even if a short inter-implant delay and lower ages at the second surgery can lead to better speech perception with CI2, children can benefit from bilateral stimulation independently of age at the second surgery and the DELTA.

Inner Ear Anomalies among Cochlear Implant Candidates in Erbil City, Iraq

Background and objective: To study the frequency of each type of inner ear abnormalities in cochlear implant candidates in Erbil city, Iraq. Methods: This is a case series study of high resolution computed tomography and magnetic resonance images of temporal bone in 111 candidates for cochlear implantation in Erbil city between June 2015 and December 2017; who had congenital bilateral profound hearing loss. Results: In total, 111 cases of congenital sensorineural hearing loss were included. Of these, 101 patients had a normal inner ear pattern. Ten patients identified to have inner ear anomalies (9%). unilateral anomaly seen in one child, so the total number of ears with inner ear anomalies was 19. Among these 19 abnormal ears, 7 were classified as incomplete partition type II (36.8%), 4 as incomplete partition type I (21.1%), 4 as a common cavity (21.1%), 2 as cochlear aplasia (10.5%), 1 as cochlear hypoplasia (5.3%) and 1 as Michel deformity (5.3%). Conclusions: The most common type of anomaly was incomplete partition type II “Mondini” deformity followed by common cavity and incomplete partition type I deformities. The incidence of congenital hearing loss with inner ear anomalies is similar to the results of such studies worldwide.

Vestibular Imaging and Function in Patients With Inner Ear Malformation Presenting With Profound Hearing Loss.

ObjectiveVestibular impairment has been observed in patients with congenital hearing loss, but little is known about the vestibular anatomy and function of those in this group with inner ear malformations. This study aims to investigate the association between vestibulocochlear anatomy and vestibular function test results in children with inner ear malformations.Study DesignCase series with chart review.SettingPediatric patients with inner ear malformations presenting with bilateral profound hearing loss at a tertiary hospital from 1999 to 2017.MethodsEars were classified into subgroups based on anatomic abnormalities seen on computed tomography imaging. Cervical vestibular evoked myogenic potential (cVEMP), rotatory chair, and caloric test results were obtained and collated. Descriptive and inferential statistics were calculated.ResultsOf 82 ears, 29.3% had incomplete partition type II malformation, the most common type. The second-most common type was isolated vestibular organ anomaly (20.7%), which is not included in currently accepted categories. Most ears exhibited abnormal vestibular function. Abnormal vestibule volume was associated with a nonreactive cVEMP (P < .001). Radiologically abnormal lateral semicircular canals were associated with abnormal caloric and rotatory chair results (P < .001).ConclusionWith a relatively large number of cases of isolated vestibular organ anomaly not only in our study but also in previous publications, we suggest that this category be added to the subsets of inner ear malformations. Abnormal vestibule volume was significantly associated with a nonreactive cVEMP finding. The majority of patients with hearing loss secondary to inner ear malformations have abnormal vestibular function test results.

Efficacy of endoscopic repair for CSF otorrhoea in children with recurrent meningitis due to incomplete partition type 1

ObjectiveTo review the efficacy of endoscopic management with or without cochlear implantation in a pediatric population presenting with recurrent meningitis due to inner ear incomplete partition type 1 (IP-1). Materials and methodsA multicentric report of 6 patients with a history of recurrent meningitis due to IP-1 was done. Radiological evaluation was performed to confirm the diagnosis. Transcanal endoscopic repair of the defect was performed in 3 cases, 2 cases underwent endoscope-assisted repair followed by cochlear implantation in the contralateral ear later, and 1 patient underwent endoscopic repair along with simultaneous cochlear implantation. ResultsRadiology confirmed the diagnosis and identified the site of the leak. A leak was detected in all cases from the stapes footplate. Two patients with unilateral deformity had a CSF-filled cyst protruding through a defect in the stapes footplate, and the rest of the four had a high flow CSF leak from the footplate. Five patients who underwent repair have had no further episodes of meningitis. One patient who received simultaneous implantation and repair developed postoperative meningitis managed successfully with antibiotics. ConclusionHearing loss due to inner ear deformity can be easily missed as a cause for recurrent meningitis, especially if unilateral. A high index of suspicion, audiological screening and radiology are essential to clinch the diagnosis. In such cases, a pure endoscopic or endoscopic assisted transcanal approach to repair CSF otorrhoea is an effective alternative to more radical procedures like subtotal petrosectomy with obliteration. In patients with bilateral profound hearing loss, simultaneous cochlear implantation and repair of the defect can be performed successfully.

Effect of Serious Gaming on Speech-in-Noise Intelligibility in Adult Cochlear Implantees: A Randomized Controlled Study.

Listening in noise remains challenging for adults with cochlear implants (CI) even after prolonged experience. Personalized auditory training (AT) programs can be proposed to improve specific auditory skills in adults with CI. The objective of this study was to assess serious gaming as a rehabilitation tool to improve speech-in-noise intelligibility in adult CI users. Thirty subjects with bilateral profound hearing loss and at least 9 months of CI experience were randomized to participate in a 5-week serious game-based AT program (n = 15) or a control group (n = 15). All participants were tested at enrolment and at 5 weeks using the sentence recognition-in-noise matrix test to measure the signal-to-noise ratio (SNR) allowing 70% of speech-in-noise understanding (70% speech reception threshold, SRT70). Thirteen subjects completed the AT program and nine of them were re-tested 5 weeks later. The mean SRT70 improved from 15.5 dB to 11.5 dB SNR after 5 weeks of AT (p < 0.001). No significant change in SRT70 was observed in the control group. In the study group, the magnitude of SRT70 improvement was not correlated to the total number of AT hours. A large inter-patient variability was observed for speech-in-noise intelligibility measured once the AT program was completed and at re-test. The results suggest that serious game-based AT may improve speech-in-noise intelligibility in adult CI users. Potential sources of inter-patient variability are discussed. Serious gaming may be considered as a complementary training approach for improving CI outcomes in adults.

Bypassing conventional anaesthetics: Dexmedetomidine sedation in MEGDEL syndrome

Sir, 3-Methylglutaconic aciduria (MEG) with sensori-neural deafness (D), encephalopathy (E), and neuroradiological evidence of Leigh-like disease (L) (MEGDEL) syndrome is one of the rare mitochondrial disorders with a prevalence of 0.09:100,000. Clinical features include progressive myopathy, hypoglycaemia, feeding difficulty, developmental delay, failure to thrive, acute liver failure, arrhythmias and cardiac failure.[1] We report a case of a 1-year-old female child, weighing 8 kg, scheduled for magnetic resonance imaging (MRI) and computerised tomography (CT) under anaesthesia. The child was diagnosed to have MEGDEL syndrome on the basis of genetic and biochemical testing in the neonatal period. She presented with global developmental delay, bilateral profound hearing loss and bilateral pyramidal signs. There was no history of seizure disorder. A careful anaesthesia plan was chalked out considering the concerns of using conventional anaesthetic agents like propofol and sevoflurane for sedation. Since both these agents are contraindicated in a child with mitochondrial dysfunction, we planned to use dexmedetomidine infusion to maintain sedation during the MRI procedure. The child was admitted on the morning of the procedure and was kept fasting as per American Society of Anesthesiologists guidelines. Intravenous (IV) maintenance fluid with dextrose normal saline at the rate of 25 ml/hour was started 2 hours prior to the procedure and administered till the induction of anaesthesia. Pre-induction blood glucose level was 70 mg/dl and a bolus of 15 ml of dextrose normal saline was also administered prior to induction. Sedation was induced with IV ketamine 0.25 mg/kg, IV dexmedetomidine 0.5 μg/kg and IV midazolam 0.1 mg/kg, in titrated bolus doses. Maintenance of sedation was done with IV dexmedetomidine infusion at the rate of 0.5 μg/kg/hour. The baseline core temperature was 36.4°C and the child was carefully wrapped in warm clothes prior to shifting inside the MR gantry. The imaging period was uneventful and was completed in 35 minutes. The child was next shifted to the CT room with ongoing sedation and the scan was completed in 4 minutes duration. The vital signs of heart rate, respiratory rate, and saturation were continuously monitored and were within normal limits. Following the completion of MRI and CT, dexmedetomidine infusion was discontinued, and the child was monitored for awakening, vital signs, temperature and glucose. The child woke up after 15 minutes and vital signs remained stable during the recovery period. Post-procedure period in the ward was uneventful and the child was discharged the next day. In this report, we want to highlight the use of dexmedetomidine in a patient with MEGDEL syndrome. Dexmedetomidine was used instead of propofol or sevoflurane based anaesthesia, to avoid the development of malignant hyperthermia due to inhaled anaesthetics or mitochondrial dysfunction due to propofol infusion and inhaled anaesthetics. The reduced ability to generate adenosine triphosphate and use oxygen can result in these patients being at more risk of developing rhabdomyolysis and anaesthesia induced malignant hyperthermia. Propofol, though considered safe in malignant hyperthermia, has the same risk as inhaled anaesthetics in causing mitochondrial dysfunction.[2] Centrally acting alpha-adrenergic agonists like dexmedetomidine can be used for sedation as an alternative to propofol. Dexmedetomidine does not depress respiration, protects airway reflexes, provides good anxiolysis and sedation.[3] It has been reported to be safe in patients with mitochondrial disease since the mechanism of action in the central nervous system differs from commonly used general anaesthetics. Successful trials have been conducted using non-triggering anaesthesia with dexmedetomidine in patients with mitochondrial disease.[45] We also made efforts to reduce the metabolic burden of the patient which includes avoidance of prolonged fasting and hypoglycaemia, prevention of hypovolaemia and maintenance of normothermia.[6] In remote location anaesthesia like an MRI suite, dexmedetomidine is a safer alternative to propofol or sevoflurane for children with mitochondrial dysfunction undergoing sedation. In addition, strategies to decrease the metabolic burden in the perioperative period should be stringently employed. Post-anaesthesia monitoring in a hospital setting is prudent for the first 24 hours. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Consanguinity as a Determinant of Profound Bilateral Sensory Neural Hearing Loss Disease among Deaf Children in Specified Rehabilitation Schools at Karachi, Pakistan

Consanguinity as a Determinant of Profound Bilateral Sensory Neural Hearing Loss Disease among Deaf Children in Specified Rehabilitation Schools at Karachi, Pakistan

Aplicação clínica da Classificação Internacional de Funcionalidade, Incapacidade e Saúde (CIF) em crianças e adolescentes de um serviço público de reabilitação auditiva

ABSTRACT Purpose: to develop an International Classification of Functioning, Disability and Health (ICF) Checklist for clinical application in the rehabilitation of children and adolescents presented with hearing loss, to describe the biopsychosocial profile of participants and present the results of the use of ICF by speech therapists. Methods: a descriptive cross-sectional observational research, with quantitative and qualitative analyses. The study was conducted with five speech therapists, eight children aged 7 to 10 years and eight adolescents between 11 and 18 years old, diagnosed with moderate to profound bilateral hearing loss. Data collection included interviews, analysis of medical records and a questionnaire for the speech therapists. Exploratory data analysis was performed using summary measures and descriptive analysis of the profile of participants. Results: an ICF Checklist for clinical use in Hearing Rehabilitation was created. With its application, deficiencies related to hearing, speech and language functions were observed, with considerable difficulties in expressive and receptive language skills, learning and communication and access barriers related to communication and health services. Conclusion: the use of the Checklist expands the aspects observed by professionals, allows identification of the subjects' needs, and enables the recording and monitoring of worsening, stabilization or progress of the biopsychosocial aspects in the rehabilitation process.

Well Diving is Not Well for Hearing: Sudden Sensory Neural Hearing Loss-A Case Report

Background: Sudden sensory neural hearing loss can be caused by many factors. It may range from minimal to profound degree of hearing loss. Barotrauma is a type of ear damage caused by pressure difference between the atmosphere and the inner ear. This may result in permanent sensory neural hearing loss. Objective: The aim of the study was to use behavioral and electrophysiological assessments to analyze the hearing of a patient who experienced sudden hearing loss following a dive in well. Case Report: A 31 year old male adult who reported sudden hearing loss following a dive in the well was evaluated. Hearing assessment was done. Pure tone audiometry revealed bilateral profound hearing loss. Immittance audiometry showed ‘A’ type tympanogram in both ears and acoustic reflexes absent in both ears. In ABR click stimulus test, no responses were observed till 105dBnHL in both ears. DPOAE’s were also absent in both ears. Vestibular assessments (subjective and objective) were also done. Conclusion: The current case study verifies the importance of audiological assessment for divers and importance of safety measures to be taken.

Current criteria for selecting cochlear implant in deaf patients: a review

Cochlear implantation is indicated in patients with severe to profound hearing loss that cannot be adequately treated by other auditory rehabilitation measures. The definitive indication of cochlear implantation is made on the basis of an extensive interdisciplinary clinical, audiological, radiological, and psychological diagnostic work-up. There are numerous changes are happening in cochlear implant candidacy. These have been associated with concomitant changes in surgical techniques, which enhanced the utility and safety of cochlear implantation. Currently, cochlear implants are approved for individuals with severe to profound unilateral hearing loss rather than previously needed for bilateral profound hearing loss. Studies have begun using the short electrode arrays for shallow insertion in patients with low-frequency residual hearing loss. The advancement in designs of the cochlear implant along with improvements in surgical techniques reduce the complications and result in the safety and efficacy of the cochlear implant which further encourages the use of these devices. This review article aims to discuss the new concepts in the candidacy of the cochlear implant, cochlear implant in younger children and hearing preservation, a cochlear implant for unilateral deafness, bilateral cochlear implant, and cochlear implant with neural plasticity and selection of patients for the cochlear implant.

The Tietz syndrome associated with cardiac malformation: a case report with literature review

BackgroundTietz syndrome is a very rare clinical entity characterized by the association of profound bilateral congenital sensorineural deafness and generalized hypopigmentation of skin, eyes, and integuments (snow white appearance). It is an autosomal dominant syndrome due to a mutation in the melanocyte inducing transcription factor (MITF) gene. The association of a heart malformation has never been reported in this syndrome.Case presentationWe report two cases of two cousins aged 5 years and 20 months respectively with a history of first degree consanguineous parents. Both girls presented with diffuse hypopigmentation of the skin, blond hair, blue eyes, and bilateral diffuse retinal hypopigmentation at ocular fundus exam. Bilateral profound sensorineural hearing loss was confirmed by auditory brainstem response in both cases. Echocardiography revealed a cardiac malformation such as interventricular communication in the older cousin and interatrial communication in the younger cousin. The family investigation did not reveal a similar case among ancestors. The diagnosis of Tietz syndrome was based on clinical criteria and pedigree. The older cousin underwent a total optical correction and a right unilateral cochlear implantation followed by speech therapy with a satisfactory result after a follow-up of two years. Unfortunately, the little cousin died following a head trauma.ConclusionsTietz syndrome is a rare autosomal dominant genetic disorder, characterized by generalized albinism with bilateral profound hearing loss. It results from a nontruncating mutation in the basic domain of in the MITF gene. Its management must include, in addition to hearing and ophthalmic rehabilitation, the research and treatment of cardiac malformations which may be life-threatening.

Combined translabyrinthine, anterior petrosal approach for resection of collision vestibular schwannoma and petrous apex meningioma in neurofibromatosis type 2, with auditory brainstem implant placement.

The natural history of neurofibromatosis type 2 (NF2) is profound bilateral hearing loss. The decision to pursue microsurgery may be more complicated in NF2 than with sporadic tumors. Schwannomas in NF2 often occur with other skull base tumors. Treatment should be tailored to preserve auditory perception for as long as possible. The authors present the case of a man with NF2 and a vestibular schwannoma who has poor hearing on the same side as a large petrous apex meningioma, both opposite to a well-hearing ear. This case highlights surgical decision-making and technical nuances during resection of collision tumors in NF2.The video can be found here: https://stream.cadmore.media/r10.3171/2021.7.FOCVID21130

Hearing Screening of Newborns using Distortion Product Otoacoustic Emissions

Introduction The present study was devised to estimate the prevalence of neonatal hearing loss and document the importance of using DPOAE as a screening tool for identifying hearing loss in newborns. Materials and Methods This hospital based descriptive study was conducted from August 2018 to August 2019. A total of 928 newborn babies were included in the study. These newborn babies were subjected to hearing screening by distortion product otoacoustic emissions (DPOAE) at 24-72 hrs after birth. For pass cases, no further testing was done. For refer cases, repeat testing with DPOAE was done within 15-30 days. Newborns with refer result on repeat DPOAE testing were subjected to Brainstem evoked response audiometry (BERA) within 3 months to confirm hearing loss. Results Nine hundred and twenty eight newborn babies were screened by DPOAE. 851 newborns passed the first DPOAE hearing screening and 77 newborns gave refer result. 21 newborns were lost to follow-up. 56 newborns underwent repeat DPOAE testing and 5 newborns were referred for BERA. Amongst the 5 newborns who underwent BERA testing, one newborn was diagnosed with bilateral profound hearing loss. Hence, the prevalence of hearing loss of 1.08 per thousand newborn babies was estimated in this study. Conclusion Hearing screening of newborns using DPOAE followed by BERA in refer cases to confirm hearing loss is useful for early detection followed by timely intervention and rehabilitation.

The Use of a Robot to Insert an Electrode Array of Cochlear Implants in the Cochlea: A Feasibility Study and Preliminary Results

Introduction: Cochlear implants (CIs) are commonly used for the rehabilitation of profound bilateral hearing loss. However, patients with substantial residual acoustic hearing are potential CI candidates. Because of both improvements in technology and advancements in surgical techniques, it may be possible to preserve hearing to some extent. For more than a decade, it has been suggested that robots are used to perform middle ear surgery. We evaluated the use of the RobOtol® otologic robot specifically to insert CI electrodes into the inner ear. Methods: CI surgery with the conventional approach was performed under general anesthesia. The MED-El Flex 24-electrode array was inserted using RobOtol®. Video recordings were used to calculate the speed of insertion. The positions of the electrodes were evaluated using a cone beam CT. All subjects underwent pure-tone audiometry tests before and after surgery, and the pure-tone average (PTA) was calculated from 250 to 4,000 Hz. Results: The robot inserted implants in 5 patients, and complete insertion of the electrode array was achieved. The speed of insertion of the electrode array was 0.88 ± 0.12 mm/s. The mean loss of the PTA for 5 frequencies (250, 500, 1,000, 2,000, and 4,000 Hz) was 13.60 ± 7.70 dB. Only 1 patient showed a loss of the PTA by >20 dB. For these 5 patients, the cone beam CT findings showed that all the electrode arrays were in the tympanic ramp and had a grade of 0. The results were compared with those obtained from a cohort of 17 patients who underwent manual implantation of a MED-El Flex 24-electrode array. Conclusion: To minimize disturbance to the cochlea while atraumatic electrode arrays are inserted, electrodes can be inserted at a constant, slow speed in the inner ear with the assistance of the RobOtol® robot in a normal clinical surgical setting.

Importance of TORCH Profile in Hearing Impaired Paediatric Population Planned for Cochlear Implant: A Study From an Eastern India Tertiary Care Centre.

TORCH group of infections are one of the most common causes of bilateral profound hearing loss in a developing country like ours. Seroprevalance is quite high in eastern part of our country. Screening for TORCH infections in children's with profound hearing loss has significant prognostic, planning and policy forming implications. To evaluate the seroprevalance of TORCH infections in prospective cochlear implant children and its significance. Ours is a retrospective study conducted from 2017 to 2018 on 50 children with bilateral profound hearing loss attending the Department of ENT at AIIMS, Patna. Thorough clinical and audiological assessment of the patients was done using objective tests like OAE (otoacoustic emission), ABR (auditory brainstem response) and subjective tests like BOA(behavioural audiometry) and PTA (puretone audiometry) wherever feasible. Blood samples were collected and serotesting was done using ELISA for Toxoplasma, Rubella, Cytomegalovirus (CMV) and Herpes Simplex Virus (HSV) (TORCH). We found that IgM was negative for all patients. Seroprevalance for IgG was 16.3% for toxoplasma, 74.4% for rubella, 69.8% for CMV and 20.9% for HSV. All the children had bilateral severe to profound loss on ABR and bilateral REFER on OAE. As prevalence of TORCH infection is quite common in India and is an established risk factor for sensorineural hearing loss with multisystem involvement screening for the same will help in early identification and in decision making for cochlear implantation thus improving the prognosis and also aid in policy making.