Speech Recognition Performance in Children with Cochlear Implants Using Bimodal Stimulation

Cochlear implantees have considerably good speech understanding abilities in quiet surroundings. But, ambient noise poses significant difficulties in understanding speech for these individuals. Bimodal stimulation is still not used by many Indian implantees in spite of reports that bimodal stimulation is beneficial for speech understanding in noise as compared to cochlear implant alone and also prevents auditory deprivation in the un-implanted ear. The aim of the study is to evaluate the benefits of bimodal stimulation in children in an Indian cochlear implant clinic. A group of 14 children who have been using cochlear implants served as subjects in this study. They were fitted with advanced digital hearing aids in their un-implanted ears to provide bimodal stimulation. Results revealed that bimodal stimulation did not bring greater change in speech scores in quiet surroundings but have shown a noticeable improvement in noisy ambience. Hence the present study suggests that bimodal stimulation would benefit children with cochlear implants especially in adverse listening conditions.     

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.     

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.     

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.     

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.     

A Study of Effect of Consanguinity on Cochlear Morphology in Patients with Congenital Bilateral Profound Sensorineural Hearing Loss

Consanguinity has been considered as one of the risk factors predisposing to the development of congenital hearing loss. Effect of consanguinity on cochlear morphology has been subject of speculation, though many studies have provided insight into functional aspect of cochlea. This study was conducted to know the effect of consanguinity on cochlear morphology, if any. A prospective, observational study, including prelingually deaf children with bilateral profound sensorineural hearing loss who are registered as candidates for cochlear implantation at a tertiary care centre, was conducted. Seven children born out of consanguineous marriages and having bilateral congenital sensorineural hearing loss were imaged for their inner ears by MRI and various cochlear measurements were compared with that of seven control children born out of non-consanguineous marriages and who had congenital bilateral sensorineural hearing deafness. No statistically significant differences were noticed in the measurements of mean cochlear length, cross sectional diameter of cochlea at apex, middle turn and at basal turns; between children born of consanguineous and non-consanguineous marriages. Study revealed normal cochlear morphology in all the children born out of consanguineous marriages. Consanguinity, as a risk factor for development of sensorineural hearing loss, may not result in gross structural anomaly of cochlea. The genetic testing of these patients need not be extensive and can be limited to selective screening of genes responsible for functioning of cochlea rather than its anatomical development.     

Cochlear Implantation in Pierre Robin Syndrome

Hereditary hearing loss accounts for nearly 60% of deafness in developed countries and about 30% of them are syndromic. Pierre Robin Syndrome is one such condition. The patient with this syndrome usually presnts with triad of micrognathia, glossoptosis and cleft palate. Hearing loss is mostly conductive but there can be sensorineural hearing loss also. Here we present a case of Pierre Robin Syndrome who presented with congenital hearing loss. He also had bilateral serous otitis media. He underwent cochlear implant surgery and was prescribed antihistaminics and steroid spray for middle ear effusion. Therefore, proper clinical evaluation is required.     

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.     

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.     

Resolution of Sudden Sensorineural Hearing Loss Following a Roller Coaster Ride

We report a case of sudden unilateral sensorineural hearing loss of sudden onset during an aeroplane flight, which completely resolved during a roller coaster ride at Alton Towers theme park. A review of the literature concerning sudden idiopathic sensorineural hearing loss and spontaneous resolution are discussed. Initially, pure-tone audiometry showed a profound sensorineural hearing loss in the right ear and mild sensorineural hearing loss in the left ear (of note, the hearing was normal prior to the episode). Following resolution of the patient’s symptoms during a roller coaster ride, pure-tone audiometry showed normal hearing thresholds in both ears. Sudden sensorineural hearing loss is a symptom of cochlear injury and the mechanism of the patient’s symptoms was attributed to a patent cochlear aqueduct.     

Otoplast in the middle ear cleft — a rare complication of hearing aid fitting and its surgical management

Hearing aid fitment is a routine, usually safe procedure carried out by hearing aid technicians or even audiologists. Hearing aids are often considered the benign, non-surgical alternative to rehabilitate a patient who cannot be helped surgically. It is rare to have to resort surgery to manage a complication resulting from hearing aid fitting. We report here, a case of otoplast as a foreign body in the middle ear cleft (middle ear cavity and mastoid). The otoplast was used to prepare a mould for the hearing aid and the syringe carrying the otoplast burst splashing the material into the middle ear and the mastoid. This resulted in sudden excruciating pain, further loss of hearing and intractable otorrhea. After several attempts to remove the material elsewhere, our patient underwent a successful mastoid exploration to remove the otoplast resulting in a dry ear after seven years of persistent otorrhea. Patient’s professional requirement for good hearing necessitated a myringoplasty on the other (only hearing) ear. He is now happily rehabilitated with a dry but deaf left ear and a normally hearing right ear.     

The Auditory Brainstem Responses in Patients with Unilateral Cochlear Hearing Loss

The aim of our study is to analyze changes occurring in the auditory brainstem response (ABR) according to stimulus parameters in unilateral cochlear hearing loss cases. Twenty-nine cases (14 male, 15 female) with unilateral sensorineural hearing loss (SNHL) were investigated. All cases had cochlear SNHL on one side whereas normal hearing on the other side. All cases underwent ABR testing with varying stimulus intensity levels and stimulus repetition rates (SRRs). Results were compared and their correlation with audiogram shapes investigated. As stimulus intensity levels decreased on both ears, latencies expanded and amplitudes decreased in all traces of ABR. Latencies of ears with cochlear hearing loss were observed to be longer than those in normal ears. Responses to SRR increases were similar on both ears. Audiogram shapes should be taken into consideration while performing ABR in order to address asymmetric SNHL. The interpretation of ABR changes with various stimulus levels may provide a better understanding of cochlear pathologies associated with hearing loss in the future.     

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.     

Early neuro-otologic effects of three-dimensional irradiation in children with primary brain tumors

PURPOSE: Central nervous system (CNS) irradiation can cause sensorineural hearing loss. The relationship between the dose to the cochlea and the development of hearing loss is unknown. Conformal radiation therapy (CRT) techniques facilitate accurate cochlear dosimetry. We modeled hearing threshold levels (HTL) after CRT in children with localized primary brain tumors (ependymoma, low- or high-grade astrocytoma, craniopharyngioma, or CNS germinoma) by using cochlear dose and clinical variables. PATIENTS AND METHODS: We evaluated 72 children (median age, 9.5 years) with audiograms before and every 6 months after CRT (median follow-up, 16.6 months; range, 4.3-42.6 months). We used a mixed-effects model to predict change in hearing for each ear as a function of time, cochlear dose, and clinical variables. RESULTS: Hearing was affected the greatest in patients with CSF shunts and pre-CRT ototoxic chemotherapy, enhanced by cochlear dose, and was more prominent on the right side. Hearing impairment after CRT alone occurred at low and intermediate frequencies in patients with shunts and supratentorial tumors when the cochlear dose exceeded 32 Gy. Patients with shunts and central supratentorial tumors developed intermediate-frequency hearing loss after CRT alone regardless of dose. CONCLUSIONS: Hearing loss during the first 4 years after CRT alone is uncommon, although patients with shunts and supratentorial tumors appear to be at increased risk for low- and intermediate-frequency effects. CSF shunting and increased cochlear dose enhance the effect of ototoxic chemotherapy. If possible, the average cochlear dose should be <32 Gy over a 6-week course of treatment until more specific dose data become available.     

Cochlear implantation in branchio-oto-renal syndrome — A surgical challenge

Branchio-oto-renal syndrome (Melnick-Fraser Syndrome) is a rare Autosomal Dominant disorder characterized by the syndromic association of branchial cysts or fistulae along with external, middle & inner malformations and renal anomalies. Incomplete penetrance and variable expressivity are common with the phenotypic variation ranging from mild to severe forms & consisting of various eye, ear, oral and craniofacial abnormalities. Mutations in the EYA1 gene on chromosomal site 8q13.3 are identified as the primary cause of BOR syndrome. We present a 3year old child with BOR syndrome, who came to us with bilateral low set, malformed ears & profound cochlear hearing loss along with bilateral branchial fistulae & unilateral renal agenesis. This child underwent successful cochlear implantation recently. The clinical presentation, pre-operative investigations, intra-operative findings & post-op habilitation status are presented with special highlights on the unique facial nerve course along with middle and inner ear anomalies which posed a surgical challenge during cochlear implantation.     

Deafness,a Social Stigma: Physician Perspective

Hearing is an essential sensory sense of an individual for development of speech which is crucial for verbal communication and personality development. It is the second most common form of disability after loco motor disability in India. Disabling hearing loss is more than 40 dB hearing loss in better ear in a person more than 15 years of age and greater than 30 dB hearing loss in better hearing ear below 14 years of age. WHO estimated 360 million individuals in the world with disabling hearing loss, out of which 91 % are adults and only 9 % are children. Early and accurate identification of birth asphyxia, hyperbilirubinemia, auditory neuropathy Presbyacusis and avoiding noise pollution and discouraging use of mobile phone, tobacco chewing/smoking, in those who are prone to deafness, an intervention is a must to decrease deafness from our society. Deafness prevention can only be possible with mutual cooperation with dedication of different medical and non-medical personnel and also by helping the persons with deafness. We have to focus not only on the children but also on senior citizens as most alarming, up to 40 %, incidence of deafness is in senior citizens above the age of 75 years. Timely cure and preventive measures are essential for better socio-economic state of the country. By helping the persons with deafness, we will not only be doing a great service to the Nation but also to the society at large.     

Current practice of paediatric chochlear implantation in nottingham

The number of integrated paediatric cochlear implantation programme are very few in the world. In U.K. this pioneering integrated paediatric chochlear implantation programme has been started in the year 1989 under the supervision of group of dedicated paediatric personnel comprising of ENT surgeons, neuro-radiologist, audiologist, speech therapist, orthophonists, teachers of the deaf and others associated with the deaf children. Only the surgical procedure of cochlear implantation is not the answer of a profound sensorineural deaf child who can not be helped by conventional hearing aids. Proper selection of the cases, pre-operative counselling, pre-operative audiological and non-audiological investigations are of immense value. Similarly the programme following surgery as a team approach is equally important for proper rehabilitation of profound sensorineural deaf child who can not be helped by conventional hearing aids.     

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.