Early Speech Perception Test Outcome in Children with Severe Sensorineural Hearing Loss with Unilateral Cochlear Implants Alone versus Bimodal Stimulation

Bilateral stimulation of the auditory system has clear advantages over unilateral hearing. Hearing-impaired children are, therefore, generally fitted with hearing aids in both ears so that they can have the benefits of binaural hearing. Children who use acochlear implant in one ear and no acoustic stimulation in the opposite ear are at a definite disadvantage. This study was undertaken to determine the advantages of bimodal stimulation in pediatric population especially in terms of speech recognition. This study comprised of 30 children between 3 and 6 years of age with profound bilateral sensorineural hearing loss with cochlear implant in one ear and fitted with digital hearing aid in non-implanted ear. Speech recognition performance was compared in unilateral cochlear implant only and with bimodal hearing stimulation in the same set of children. A statistically significant difference was found between speech reception scores in children with a unilateral cochlear implant only and those with a cochlear implant in one ear and a hearing aid in the non implanted ear in quiet surroundings. It is suggested that the use of bimodal fitting be considered as an effective management method to obtain the advantage of binaural hearing in children who undergo unilateral cochlear implantation.     

A Retrospective Clinical Audit of Outcomes of Cochlear Implantation in Children with Multiple Disabilities in Comparison with Normal Implantees: A South Indian Experience

AimsCochlear implantation (CI) is established as a standard remedy for children with congenital bilateral profound hearing loss to attain hearing perception and thereby develop speech and language. A subgroup includes children with multiple disabilities in whom the implant helps to improve their quality of life and also of their families via enhanced communication skills. Cochlear implants today form an integral part of their multi-handicap rehabilitation process.Material and MethodsA retrospective cohort study was carried out on children with and without multiple handicaps who have received cochlear implantation at the cochlear implant clinic of MERF, Chennai, India over the past decade. Category of Auditory Performance (CAP) scores, Speech Intelligibility Rating (SIR) scores, and also Meaningful Auditory-Integration Scale (MAIS) and Meaningful Use of Speech Scale (MUSS) scores were compared at set time frequencies of 6 months and 12 months post-implantation between the two groups of implanted children.ResultsAll the four CAP, SIR, MAIS and MUSS scores showed improvement over time with auditory and speech therapy in both groups of children as reflected by the improvement in their quality of life. The normative group of implantees showed better improvement compared to the group of children with multiple disabilities.ConclusionIntensive habilitation is essential especially for children with multiple disabilities who have received cochlear implantation in which their special needs are addressed individually and optimised for the best outcome. The study shows that restoration of the special sense of hearing helps as a remedy to alleviate their other multi-handicaps to a notable extent.     

The current status of cochlear implants

In the University of Sydney cochlear implant programmes, 109 adults and teenagers have received a 22 electrode cochlear implant (Cochlear? implant) since 1984; and 127 children have received a Cochlear? implant since 1987. The results were analysed when all patients were still using the MSP speech processors rather than the newer SPEAK processors. Seventy five percent of adults and teenagers deafened after learning speech for a period of less than 15 years were able to recognise some words by audition alone. Only 30 percent of adults and teenagers deafened for over 15 years regaining hearing were able to recognise any words by audition alone but most found the device very helpful in aiding lipreading. None of adults and teenagers who were born deaf who received a cochlear implant found they could recognise any sounds and half of them abandoned using the device. Children who were deafened after learning speech usually did extremely well with a cochlear implant and could remain in their regular school situation. Children who had done well with hearing aids were also very likely to succeed with a cochlear implant. Children who had learnt to communicate by gestures or signs who had reached an age of over 6 years did poorly with the cochlear implant with 73 percent unable to recognise speech by listening alone and unable to improve their speech production to an intelligible level. Children born deaf who received the implant early in life and were taught primarily through audition could be very successful with 52% recognising words by audition alone and gaining intelligible speech. Preliminary studies suggested that the younger the child received the cochlear implant the greater the possibility of success. Neural plasticity or the ability to the brain to learn or relearn tasks appear to be the most important factor affecting the selection of candidates for a cochlear implant. It appeared that after the age of 6 years, if a child had not utilised the auditory and motor areas of spech production within the brain, the neural plasticity remaining was insufficient to allow effective use of a cochlear implant. The cochlear implant is a device which can restore when a person is too deaf to be able to use a conventional hearing aid. The cochlear implant does not provide normal hearing but may provide sufficient information for the recipient to distinguish several words without the need for lipreading. Evidence will be presented which shows that the device can enable children suffering from congenital deafness to gain excellent speech and listening providing it is fitted at an early age and there is an appropriate training programme to teach the child.     

Pediatric cochlear implantation-I : Candidacy

The cochlear implant is a successful means of intervention to facilitate the perception of sound and oral communication for children with severe to profound sensorineural hearing loss. Often, the cochlear implantation process is viewed as being limited to a surgical procedure, rather than an entire process of medical, audiological, psychosocial, educational and therapeutic, management. In India, cochlear implantation is available in only a few large cities. Here, the otolaryngologist will direct the cochlear implant programme. Besides determination of medical candidacy, device implantation and medical management, the otolaryngologist is responsible to ensure that other aspects of cochlear implant management are implemented. This paper, the first of two that describe the multidisciplinary, team approach of the Pediatric Cochlear Implant Program of The Children’s Hospital of Philadelphia (CHOP), in Pennsylvania, USA, discusses the non-medical aspects of cochlear implant candidacy. A second article will follow that covers post-implantation follow- up. The various speech tests used at CHOP for assessment are based on the English language. They may be translated into the regional Indian languages where the assessment and training can be carried out accordingly.     

Pediatric cochlear implantation — II: postoperative follow-up

A child with a cochlear implant is expected to achieve the successful outcome of facilitated perception of sound and more oral communication. To achieve this goal, ongoing intervention from a variety of professionals is required. These professionals may represent the disciplines of medicine, audiology, social work, education, and speech / language pathology. In India, cochlear implantation is available in only a few large cities. Here, the otolaryngologist will direct the cochlear implant program. Besides determination of medical candidacy, device implantation and medical management, the otolaryngologist is responsible to ensure that other aspects of cochlear implant management are implemented. This paper, the second of two that describe the multidisciplinary, team approach of the Pediatric Cochlear Implant Program of The Children’s Hospital of Philadelphia (CHOP), in Pennsylvania, USA, discusses the non-medical aspects of cochlear implant post-implantation follow-up. The first article, previously published, discussed cochlear implant candidacy. The various speech tests used at CHOP for assessment are based on the English language. They may be translated into the regional Indian languages where the assessment and training can be carried out accordingly.     

Electrophysiological Correlates of Behavioral Comfort Levels in Cochlear Implantees: A Prospective Study

Indications for cochlear implantation have expanded today to include very young children and those with syndromes/multiple handicaps. Programming the implant based on behavioral responses may be tedious for audiologists in such cases, wherein matching an effective MAP and appropriate MAP becomes the key issue in the habilitation program. In ‘Difficult to MAP’ scenarios, objective measures become paramount to predict optimal current levels to be set in the MAP. We aimed, (a) to study the trends in multi-modal electrophysiological tests and behavioral responses sequentially over the first year of implant use, (b) to generate normative data from the above, (c) to correlate the multi-modal electrophysiological thresholds levels with behavioral comfort levels, and (d) to create predictive formulae for deriving optimal comfort levels (if unknown), using linear and multiple regression analysis. This prospective study included ten profoundly hearing impaired children aged between 2 and 7 years with normal inner ear anatomy and no additional handicaps. They received the Advanced Bionics HiRes 90K Implant with Harmony Speech processor and used HiRes-P with Fidelity 120 strategy. They underwent, Impedance Telemetry, Neural Response Imaging, Electrically Evoked Stapedial Response Telemetry and Electrically Evoked Auditory Brainstem Response tests at 1, 4, 8 and 12 months of implant use, in conjunction with behavioral Mapping. Trends in electrophysiological and behavioral responses were analyzed using paired t test. By Karl Pearson’s correlation method, electrode-wise correlations were derived for NRI thresholds versus Most Comfortable Levels (M-Levels) and offset based (apical, mid-array and basal array) correlations for EABR and ESRT thresholds versus M-Levels were calculated over time. These were used to derive predictive formulae by linear and multiple regression analysis. Such statistically predicted M-Levels were compared with the behaviorally recorded M-Levels among the cohort, using Cronbach’s Alpha Reliability test method for confirming the efficacy of this method. NRI, ESRT and EABR thresholds showed statistically significant positive correlations with behavioral M-Levels, which improved with implant use over time. These correlations were used to derive predicted M-Levels using regression analysis. Such predicted M-Levels were found to be in proximity to the actual behavioral M-Levels recorded among this cohort and proved to be statistically reliable. When clinically applied, this method was found to be successful among subjects of our study group. Although there existed disparities of a few clinical units, between the actual and predicted comfort levels among the subjects, this statistical method was able to provide a working MAP, close to the behavioral MAP used by these children. The results help to infer that behavioral measurements are mandatory to program cochlear implantees, but in cases where they are difficult to obtain, this study method may be used as reference for obtaining additional inputs, in order to set an optimal MAP. The study explores the trends and correlations between electrophysiological tests and behavioral responses, recorded over time among a cohort of cochlear implantees and provides a statistical method which may be used as a guideline to predict optimal behavioral levels in difficult situations among future implantees. In ‘Difficult to MAP’ scenarios, following a protocol of sequential behavioral programming, in conjunction with electrophysiological correlates will provide the best outcomes.     

Cochlear Implant in Prelingually Deaf Children: Our Experience

Retrospective study of the prelingual cochlear implantation programme under government scheme done at medical college hospital in central India. Forty-two prelingually deaf children screened and sent for cochlear implantation at our centre From March 2015 to Feb 2018 were reviewed with respect to their age, sex, preimplantation hearing aid use, surgical technique for cochlear implantation, type of FDA (USA) approved cochlear implant, post operative speech therapy and its outcome with respect to categories of auditory perception and speech intelligibility scoring were compared for children younger than 4 years and older than 4 years. For outcome measurement non parametric statistical method was used for any significance between the two groups. There was a wide range of children implanted ranging from 2 to 7 years. Both varia and mastoidectomy and posterior tympanotomy method of cochlear implantations were done with good rate complete insertion and electrode activation. There was no significant difference between the two group with regard to CAP and SIR outcomes after 1 year. In order to get better outcomes with respect to the speech language development, there is need to strengthen the early identification and cochlear implantation before 4 years of age in government approved schemes.     

Auditory brainstem implantation: The first Indian experience

Multichannel auditory brainstem implants (ABI) are currently indicated for patients with neurofibromatosis type II (NF2) involving both vestibulocochlear nerves. The ABI helps bypass the damaged cochlear nerves and restores a level of auditory sensation via the electrical stimulation of the cochlear nucleus. The implant is usually placed in the lateral recess of the fourth ventricle at the time of tumor resection to stimulate the cochlear nucleus. We report a case of ABI done on a 15-year-old girl with bilateral vestibular schwannomas.     

Biological and genetic landscape of breast implant-associated anaplastic large cell lymphoma (BIA-ALCL)

Breast implant–associated anaplastic large cell lymphoma (BIA-ALCL) is an uncommon form of non-Hodgkin lymphoma (cancer of the immune system) that can develop around breast implants. Breast implants are among the most commonly used medical devices for cosmetic or reconstructive purposes. In the past few years, the number of women with breast implants diagnosed with anaplastic large cell lymphoma (ALCL) has increased, and several studies have suggested a direct association between breast implants and an increased risk of this disease. Although it has been hypothesized that chronic stimulation of the immune system caused by implant materials and biofilms as well as a possible genetic predisposition play an important role in this disease, the cellular and molecular causes of BIA-ALCL are not fully understood. This review aims to describe the current understanding around the environmental and molecular drivers of BIA-ALCL as well as the genetic and chromosomal abnormalities identified in this disease to date.     

Trans-Aditus Approach: An Alternative Technique for Cochlear Implantation

The objective of this study was to report our preliminary experience with an alternative technique for cochlear implantation. Twenty patients underwent cochlear implantation via a trans-aditus ad antrum approach to the round window. The main steps involved in the surgical procedure are cortical mastoidectomy, elevation of tympanomeatal flap, incudostapedial joint dislocation, incus removal, preparation of a bed for the implant, cochleostomy via the external auditory canal, and finally insertion of the electrode into the cochlea via the aditus. Twenty-five implants were performed on 20 patients, 18 children (mean age of 3.2 years) and 2 adults. Twelve patients were males and eight were females. All the children were pre-lingual while the adults were post-lingual. Nucleus freedom cochlear implant system (Cochlear, Lane Cover, NSW, Australia) was used in four patients and a cochlear Nucleus 5 was used in six patients. A Med-el SONATA implant (MED-EL, Innsbruck, Austria) was used in 15 patients. The minimum follow-up was 5 months. Here, we describe a new alternative technique for cochlear implantation and report our preliminary results. The procedure has advantages over the existing techniques and avoids the potential complications of posterior tympanotomy, transcanal, and transmeatal techniques.     

Registering event-related auditory potentials (P300) in patients with cochlear implants

Event-related auditory evoked potentials can contribute to the evaluation of discrimination abilities of cochlear implant users. Auditory P300 potentials to a frequency contrast were obtained in six post-lingually deaf adults using a cochlear implant and in a control group of normal hearing subjects. The aim of this study was to investigate how these potentials were determined by the stimulation pattern of the cochlear implant. To visualise these stimulation patterns colour-coded plots (stimulograms) were calculated based on the stimulus and the fitting file of the individual subject as inputs. These stimulograms were used to evaluate the influence of various stimulation parameters on the stimulation contrast used in an oddball paradigm. The influence of discrimination difficulty on the P300 response is demonstrated.     

Cochlear implants: response to therapeutic irradiation

PURPOSE: To determine the response of cochlear implants ("bionic ears") to therapeutic irradiation. METHODS AND MATERIALS: A patient with a cochlear implant was referred for palliative cranial irradiation. As there were no published or manufacturer's data available regarding the response to radiation, implants were tested for functional changes following irradiation. Cochlear implants were supplied by Cochlear Ltd. Two units each of models CI22M, CI22M (with the second generation integrated circuit) and CI24M were irradiated with 4 MV X-rays, and an unirradiated unit of each model was used as a control. The implants were irradiated initially with 25 daily fractions to 50 Gy. To determine the response at higher doses, 10 Gy fractions were delivered to the same implants to 100 Gy, followed by a final fraction of 50 Gy (total dose 150 Gy). The implants were tested after each 10 Gy, up to 1100 Gy, and at 150 Gy. Several indicators of functionality were assessed, including RF (radio frequency) link range, and stimulator output current. The radiation shielding effect of the implants was also assessed. RESULTS: Within the dose range < or = 50 Gy, the stimulator output current of the CI22M units was the only parameter to change. At higher doses (to 150 Gy), changes in current output continued, and gradual loss of RF link range occurred in the CI22M units. The CI24M units showed changes in output current to 100 Gy, and large changes at 150 Gy. Dose attenuation by the implants was measured at 6% for ipsilateral single field 4 MV X-rays. CONCLUSION: Our results suggest that patients with these cochlear implants can receive cranial irradiation with a low risk of implant failure. Changes in stimulator output current can be compensated simply by reprogramming the speech map after the course of radiation treatment.     

New SMS Classification of Cochleovestibular Anomalies: Our Experience with 25 Cases of Type I Anomaly

PurposeTo study and analyse the radiological and surgical findings of 25 cochlear implantees with SMS type—I cochleovestibular malformation and to compare their outcomes in terms of hearing and speech gains pre- and post-operatively.MethodsRetrospective analysis of cochlear implanted candidates over a period of 8 year from 3 institutions was undertaken and 25 patients suffering from pre-lingual profound congenital sensori-neural hearing loss along with presence of SMS type I cochleovestibular malformation were studied. Pre-operative radiology, surgical difficulties and complication, and post-operative hearing and speech outcomes upto a period of 2 years, using IT-MAIS scores were noted. Statistical comparison pre- and post-implantation was done using Wilcoxon signed rank test and a p-value of < 0.05 was considered significant.ResultsIn 25 cases, we encountered 2 posterior SCC dysplasia, 15 enlarged vestibular aqueducts (LVA), 2 dilated vestibules, 4 superior SCC dysplasia, 2 cases with both posterior and superior SCC dysplasia and of those 1 had a dilated vestibule also. Intra-operatively, CSF leak was observed in 10 patients. Complete electrode insertion and good electrical response (NRT) was detected in all. No facial nerve anomaly was encountered. IT-MAIS scores increased from a pre-operative mean of 4.32 to 34.56 two years post-operatively and this difference was found to be statistically significant.ConclusionOur experience with SMS Type I malformations show promising and motivating results with less chances of complications, similar to implant candidates with normal anatomy. The outcomes in this group of patients are definitely not affected by any malformations in semicircular canals, vestibule or vestibular aqueduct.     

Auditory neuropathy : Diagnostic and therapeutic challenge report of first series of four cases from india

Auditory neuropathy (AN), a recently described clinical entity, is a sensorineural disorder where the patient has hearing loss with impaired word discrimination out of proportion to pure tone loss in the pressence of abnormal / absent auditory brain stem responses, and normal outer hair cell as measured by otoacoustic emissions and / cochlear microphonics. It is essential that the practicing, ENT surgeon have a high degree of suspicion of AN in patients complaining of difficulty in understanding speech with hearing loss and audiological evidence of dissociation between pure-tone and speech audiometry. Appropriate newer diagnostic tests of ABR and OAE and /or CM for confirmation of AN is essential. We present a series of four patients with auditory neuropathy from a tertiary care teaching hospital. This is the first Series of 4 cases of this clinical entity from the Indian subcontinent.     

Pediatric Cochlear Implantation—Why the Delay

The aim of the study was to evaluate the factors that act as barriers and delay the process of cochlear implantation in children with congenital profound sensorineural hearing loss. This is a cross sectional observational study in which 154 children with profound sensorineural deafness attending ENT outdoor from Jan 2013 to June 2014 at Sri Aurobindo Institute of Medical College and PG Institute, Indore, were included in the study. Information was taken from the parents of the candidates regarding the reasons for the delay in reporting, the delay in intervention and a detailed history was also taken to evaluate the possible reason for the hearing loss with the help of open ended questions. The most common cause for the delay in reporting was lack of information about the availability of technique and procedure for cochlear implant. Financial constrain was the most common cause for the delay in getting the cochlear implant surgery.     

Bilateral Simultaneous Cochlear Implantation in Children: Report of a Case and Review of Literature

The benefits of bilateral cochlear implant in adults are well established. Auditory ability in children is also substantially improved by binaural hearing. We report the first case of bilateral simultaneous paediatric cochlear implant in India and discuss the merits of bilateral implant.     

Assessment of Auditory and Psychosocial Handicap Associated with Unilateral Hearing Loss Among Indian Patients

In order to assess psychosocial and auditory handicap in Indian patients with unilateral sensorineural hearing loss (USNHL), a prospective study was conducted on 50 adults with USNHL in the ENT Outpatient clinic of a tertiary care centre. The hearing handicap inventory for adults (HHIA) as well as speech in noise and sound localization tests were administered to patients with USNHL. An equal number of age-matched, normal controls also underwent the speech and sound localization tests. The results showed that HHIA scores ranged from 0 to 60 (mean 20.7). Most patients (84.8 %) had either mild to moderate or no handicap. Emotional subscale scores were higher than social subscale scores (p = 0.01). When the effect of sociodemographic factors on HHIA scores was analysed, educated individuals were found to have higher social subscale scores (p = 0.04). Age, sex, side and duration of hearing loss, occupation and income did not affect HHIA scores. Speech in noise and sound localization were significantly poorer in cases compared to controls (p < 0.001). About 75 % of patients refused a rehabilitative device. We conclude that USNHL in Indian adults does not usually produce severe handicap. When present, the handicap is more emotional than social. USNHL significantly affects sound localization and speech in noise. Yet, affected patients seldom seek a rehabilitative device.     

Our Experience with Split Electrode Array Implant for Obliterated Cochlea

To study the outcomes of split electrode array cochlear implantation in ossified cochlea using the CAP scoring system. Retrospective case study. Tertiary referral center. Six cochleae in three adult and three pediatric patients with ossification. Intervention(s): All Patients underwent cochlear implantation with a split electrode array system. Major outcome parameter(s): Number of electrodes inserted during surgery, number of functioning electrodes on follow-up and auditory performance evaluation using the CAP score—Category of Auditory Perception [TSC Revised Version, based on Nottingham CI Program, 1995]. Six patients (three children and three adults) had insertion of split electrode array system. The mean number of electrodes inserted were 18.3 (range 15–21) and functioning electrodes at follow-up were mean of 14.3 (range 7–21). Auditory performance was measured using CAP score at 1 year post implant follow up, mean score in children was six and that in adult was eight. One pt had facial nerve twitching which was corrected by switching off the concerned electrode. No complications in sort of facial palsy or vestibular disorder were observed. Patients of ossified cochlea having profound deafness do well with split electrode array cochlear implantation as evaluated with CAP scoring system. The split electrode array results in more number of electrodes within the cochlear lumen. Retro graded apical array insertion has less chances of facial nerve stimulation as it is placed away from the nerve.