Compliance with cochlear implantation in children subsequently diagnosed with autism spectrum disorder.

OBJECTIVE: To assess the compliance with cochlear implantation (CI) in children subsequently diagnosed with autism spectrum disorder (ASD). METHODS: This was a retrospective case review and survey performed at a tertiary referral centre. Children meeting the criteria for CI who were implanted between 1989 and 2015 and who subsequently received a diagnosis of ASD were included. The primary outcome measure was to assess compliance with CI in children subsequently diagnosed with ASD. Secondary outcome measures included assessment of pre-CI risk factors that may have identified children at higher risk of a subsequent diagnosis of ASD, as well as the benefit obtained by these children following CI. RESULTS: 1050 children were implanted between 1989 and 2015. Of these, 22 children were diagnosed with ASD after receiving their CI. The average age at implantation was 2.6 years (median 3, range 1-8 years). The average age for diagnosis of ASD was 5 years, approximately 2 years (median 22 months, range 2-85 months) following CI. Of these, 16/22 (712.7%) regularly use their CI. 6/22 (27.2%) children became non-users of their implant. Some degree of verbal communication was used by 13/22 (59%) of our studied group. CONCLUSION: There is a range of level of disabilities in ASD, with some relatively minor social communication difficulties through to severe language, cognitive, and behavioural difficulties. Compliance with CI is variable and appears to correlate with the severity of the ASD. Preoperative counselling should include information about the possible impact of later diagnosed disabilities such as ASD on performance.

Bone-anchored hearing aids in conductive and mixed hearing losses: why do patients reject them?

This study aimed to report the bone-anchored hearing aid uptake rate and the reasons for their rejection by patients with conductive and mixed hearing losses. A retrospective review was performed of 113 consecutive patients with unilateral or bilateral conductive or mixed hearing loss referred to the Greater Manchester bone-anchored hearing aid (BAHA) programme between September 2008 and August 2011. 98 (86.7 %) patients were deemed audiologically suitable for BAHA implantation. Of these, 38 (38.8 %) had BAHA implanted; 60 (61.2 %) patients declined. Of those who declined, 27 (45 %) cited anxiety over surgery, 18 (30 %) cited cosmetic reasons, 16 (26.7 %) perceived limited benefit from the device and six (10 %) preferred conventional hearing aids. Our study highlights a 38.8 % BAHA uptake rate in audiologically suitable patients. The main reasons cited for rejection of BAHA were anxiety over surgery and cosmetic concerns. It is important that clinicians address these early during consultation with prospective BAHA recipients and avoid rushing to implant these patients with a bone-anchored hearing aid.

Cochlear implantation in adolescents: Factors influencing compliance.

OBJECTIVES: To quantify rates of non- and partial-use of cochlear implants (CIs) in adolescent patients implanted in adolescence and childhood and identify factors influencing compliance. METHODS: A retrospective case note review undertaken at The Manchester Auditory Implant Centre. Adolescents were defined as young people aged 11-18 years. Individuals implanted in adolescence were defined as Group 1, individuals implanted in childhood under the age of 3 years and currently adolescents were defined as Group 2 and individuals implanted between the age of 3 and11 years and currently adolescents were defined as Group 3. Non-use was defined as not using the CI at all and partial use was defined as consistently using the CI less than full-time, or fluctuating periods of full and less than full-time use. RESULTS: In Group 1 there was 1 non-user (1.3%) and 11 partial-users (13.9%), with an overall non-compliance rate of 15.2%. In Group 2 there was one non-user (1.9%) and one partial-user (1.9%) with an overall non-compliance rate of 3.8%. In Group 3 there were no non-users and eight partial-users (9%), with an overall non-compliance rate of 9%. The factors influencing compliance differed between groups with the most common factor in Group 1 being a preference for the auditory input gained from the contralateral hearing aid (50%). In Groups 2 and 3 the main factors influencing compliance were behavioural and related to wearing the implant only at school (50 and 75%, respectively). CONCLUSIONS: Patients implanted during adolescence have higher rates of non- and partial-use compared with their adolescent counterparts who have been implanted during childhood. It is important to investigate factors influencing non-compliance so appropriate support may be provided to the patient and their family.

Hearing preservation cochlear implantation in adolescents.

OBJECTIVE: This study describes our experience of cochlear implantation (CI) with hearing preservation in adolescents. Our aim was to determine if hearing preservation is successful in this population, if the preserved hearing is maintained, and what the potential benefit of preserving hearing in this population is. PATIENTS: Fourteen profoundly deaf adolescents with preservation of low-frequency hearing (125, 250, and 500 Hz). INTERVENTION: Twelve adolescents had a single-sided CI, and two had bilateral CI. All were having their first implantation, and all patients had hearing preservation surgery (soft surgery). MAIN OUTCOME MEASURES: Hearing preservation was measured with preoperative and postoperative pure-tone audiograms. Speech audiometry was performed before implantation and at subsequent follow-up appointments. RESULTS: Hearing preservation (measurable hearing thresholds) was achieved in 13 of 14 patients. Average follow-up was 2 years 10 months (range, 4 mo-4 yr 9 mo). Three of 13 patients with initial successful hearing preservation had deterioration of their hearing at subsequent follow-up. The addition of naturally preserved hearing to the cochlear implant improved speech audiometry scores compared with using the implants in isolation. CONCLUSION: This study demonstrates that residual hearing can be consistently preserved and maintained in adolescents during the short-/medium-term using a soft surgical technique to insert standard-length electrodes. The potential benefit of preserving residual low-frequency hearing seems to be improvement in speech discrimination in challenging hearing conditions, although larger studies are required.

Cochlear implantation in children with syndromic deafness.

OBJECTIVE: To examine the outcome of cochlear implantation in children with syndromic deafness, who are increasingly being considered for cochlear implants and who represent a unique challenge to the cochlear implant team. METHOD: In this retrospective case series in a tertiary referral cochlear implant centre, we describe a series of 38 children with a clinical syndrome causing deafness who have undergone cochlear implantation. The outcome measures are Bench-Kowal-Bamford (BKB) speech reception score (range 0-100%) and speech perception ability using the Geers and Moog Speech Reception Score (SRS) (range from 0; no speech perception, to 6; open set recognition of words). RESULTS: The syndromes identified were Waardenburg syndrome (n = 10), Usher syndrome (n = 9), Pendred syndrome (n = 7), Jervell and Lange-Nielsen syndrome (n = 5), CHARGE syndrome (n = 2), and 1 each of Stickler, CINCA (Chronic Infantile Neurological Cutaneous and Articular), Bartter, Down, and Donnai-Barrow syndromes. After a minimum of 19 months following implantation, BKB was measurable in 20 of 38 patients, and ranged from 46 to 100% in quiet (median 87%, mean 81%). Eighteen children (55%) achieved a SRS at level six, and a further 8 (24%) achieved level five. There was significant variation of outcome between and within syndrome groups. CONCLUSIONS: Additional disabilities are frequently encountered when considering children for cochlear implantation, and may be part of a recognised syndrome. Outcome is often excellent but can be variable even within the same syndrome group, and such children are therefore assessed on an individual basis to ensure a realistic expectation.

Hearing preservation after cochlear reimplantation.

OBJECTIVE: The combination of electrical and acoustical hearing (EAS) is the aim of successful hearing preservation in patients with low-frequency residual hearing who receive a cochlear implant. With adequate surgical treatment and electrode arrays designed for hearing preservation, partial hearing preservation can nowadays be achieved in the majority of patients. Over recent years, the number of patients with EAS has increased, and device failures within this group are a problem that will need to be addressed. It remains unclear how reliably hearing can be preserved during revision surgery. The outcome of 3 subjects requiring cochlear reimplantation after surgery for hearing preservation is presented and discussed. Our aim was to investigate the influence of electrode reinsertion on hearing preservation. PATIENTS: Three patients with measurable residual hearing were implanted with a flexible, free-fitting electrode array in 3 different centers. INTERVENTIONS: Two subjects received a 31.5-mm array inserted 24 mm into the cochlea, whereas a third was treated with a 24-mm array, which was inserted 21 mm into the cochlea. In all cases, hearing was preserved at the initial operation. All of these subjects subsequently represented with device problems, and reimplantation was performed. MAIN OUTCOME MEASURES: Hearing preservation was measured using preoperative and postoperative pure tone audiograms. In addition, speech perception with the implant was evaluated before and after reimplantation surgery. RESULTS: Reimplantation was feasible in all subjects also in cases where a slightly deeper reinsertion was performed. Speech understanding scores after reimplantation were comparable to those seen after the first intervention. CONCLUSION: Revision surgery in patients with preserved hearing after cochlear implantation does not necessarily lead to loss of natural residual hearing, and patients can continue to benefit from the combination of electric and acoustic hearing. Even deeper insertion is possible without hearing loss within residual frequencies.

Cochlear implantation in children with Jervell and Lange-Nielsen syndrome - a cautionary tale.

OBJECTIVE AND IMPORTANCE: Jervell and Lange-Nielsen (JLN) syndrome is a rare cause of congenital profound hearing loss associated with a prolonged QT interval on the electrocardiogram. Children presenting for cochlear implantation with this condition may be asymptomatic but are at risk of sudden death. SCREENING AND SUBSEQUENT: careful management is therefore required to ensure a successful outcome. We present our experience of cochlear implantation in children with JLN syndrome, including two who died unexpectedly, and suggest a protocol for management of such cases. Clinical presentation Four cases of cochlear implantation in JLN syndrome are described. None had any previous cardiological family history. Two were diagnosed pre-operatively but, despite appropriate management under a cardiologist, died from cardiac arrest; the first in the perioperative period following reimplantation for infection, and the second unrelated to his cochlear implant surgery. The other two patients were diagnosed only subsequent to their implantation and continue to use their implants successfully. CONCLUSION: These cases highlight the variation in presentation of JLN syndrome, and the spectrum of disease severity that exists. Our protocol stresses the importance of careful assessment and counselling of parents by an experienced implant team.

Cochlear implantation in a patient with sensori-neural deafness secondary to Charcot-Marie-Tooth disease.

OBJECTIVE AND IMPORTANCE: Charcot-Marie-Tooth (CMT) disease is the most common hereditary motor and sensory neuropathy and can result in profound sensori-neural hearing loss with deficiency in speech perception out of proportion to that which would be expected if the loss was cochlear in origin. This study investigates whether the reintroduction of auditory synchrony by means of cochlear implantation will improve speech perception in those with dys-synchrony related to impairment of temporal processing abilities secondary to CMT. CLINICAL PRESENTATION: A 67-year-old male presented with a gradual but significant decrease in his hearing as part of a slowly progressing demyelinating peripheral neuropathy. On open-set speech discrimination he scored 0%. INTERVENTION: A Med-el Flex(SOFT) cochlear implant (CI) was fully inserted into the left ear with no surgical complications. The CI speech processor was fitted 1 month post-implantation and standardized speech assessments conducted at 1 week, 3 months, 9 months, and 21 months following initial fitting, gave open-set speech discrimination scores of 0, 0, 53, and 54%, respectively. CONCLUSION: This report demonstrates that cochlear implantation is an option to rehabilitate severe-to-profound hearing loss in adults with auditory dys-synchrony secondary to CMT disease. Progress post-implantation is likely to be slower than for the average CI user.

15 minute consultation: a structured approach to the management of facial paralysis in a child.

OBJECTIVE: To present a structured approach for an outpatient consultation of a child with facial paralysis. METHOD: Review of literature and description of approach followed in our unit. CONCLUSION: A focused history and examination is key to establish the cause and draw a management plan for paediatric facial paralysis.

Cochlear implantation in children with cerebral palsy.

OBJECTIVE: Few studies have looked at the outcomes of children with complex needs following cochlear implantation. Increasing evidence supports the case for implantation in these children. To date there is very little evidence available evaluating the role of cochlear implantation in children with cerebral palsy. In this paper we look at the Manchester Cochlear Implant Programme's experience of implantation in 36 children with cerebral palsy. METHODS: A retrospective review of prospectively collected data for all children with cerebral palsy was undertaken. Cognitive and physical disability was scored by members of the cochlear implant team. A modified version of Geers and Moogs 1987 Speech Reception Score was used to assess outcome. Data was analysed looking at the relationship between cognitive and physical impairment, age at implantation and the SRS outcomes. RESULTS: This study demonstrated that children with cerebral palsy and a mild cognitive impairment do significantly better following implantation than those with a severe impairment (p=0.008). Children with mild physical impairment did not appear to do significantly better than those with moderate or severe impairments (mild versus severe p=0.13). Age at implantation was not a significant prognostic factor in this study group. CONCLUSIONS: Children with complex needs are increasingly being referred for consideration of cochlear implantation. Further research is required to help guide candidacy, but each case must be considered individually. Higher functioning does appear to be the most important prognostic indicator regarding outcome but the effect of modest improvement in sound perception should not be underestimated.

Cochlear implantation in Donnai-Barrow syndrome.

OBJECTIVE AND IMPORTANCE: Donnai-Barrow syndrome is a rare autosomal recessive disorder associated with severe sensorineural hearing loss (SNHL). Several ocular abnormalities have also been described in this syndrome, including hypertelorism, down-slanting palpebral fissures, myopia and retinal detachment. The condition is also associated with diaphragmatic hernia, exomphalos, absent corpus callosum and developmental delay. We describe the first recorded case of cochlear implantation in this rare disorder. CLINICAL PRESENTATION: This case of Donnai-Barrow syndrome was identified from our paediatric cochlear implant database. A case-note review was performed identifying patient demographics, operative findings and surgical outcome. In addition, pre- and post-operative auditory and communication performance was analysed. INTERVENTION: The child initially received a right cochlear implant at 39 months of age, but unfortunately the device failed 4 years post-operatively. Subsequently, bilateral simultaneous cochlear implantation was performed. BKB scores were 98% for bilateral implant use when tested 2 years post-operatively. Individual testing revealed a score of 94% for the right ear alone, and 80% for the left. The child achieved a 100% score on sound localisation testing using 3 speakers. CONCLUSIONS: Donnai-Barrow syndrome is a rare cause of SNHL compounded by the potential for visual impairment. To our knowledge this is the first report of cochlear implantation to rehabilitate hearing loss in this condition.

Outcome of cochlear implantation in asymptomatic congenital cytomegalovirus deafened children.

OBJECTIVES/HYPOTHESIS: Congenital cytomegalovirus (cCMV) infection is a common cause of sensorineural hearing loss (SNHL). The incidence of SNHL is higher in symptomatic cCMV infants and is usually identified early. By contrast, the incidence of SNHL is lower in children with asymptomatic cCMV, and the hearing loss can be delayed in onset and progressive. The objective was to compare the outcome of cochlear implantation in children deafened by cCMV with a control group of children with implants who do not have the condition. STUDY DESIGN: Retrospective review of case notes and data base. METHODS: Retrospective review of 14 children with asymptomatic cCMV who underwent cochlear implantation. Their outcome measures were compared with those of a matched population by using standard assessment tools. RESULTS: In the study group, the Modified Categories of Auditory Performance (M-CAP) score (range, 1-7) ranged from 2 to 7 (mean, 4.2). In the control group, the M-CAP ranged from 5 to 7 (mean, 6.0). In the study group, the Manchester Spoken Language Development Scale (MSLDS) score (range, 1-10) ranged from 1 to 9 (mean, 5.4). In the control group, the MSLDS ranged from 3 to 10 (mean, 8.1). CONCLUSIONS: Children with asymptomatic deafness caused by cCMV benefit from cochlear implantation but perform less well than a comparable group of children with implants who do not have cCMV. There is a range of performance in the cCMV group that may relate to the degree of motor or cognitive disabilities.

Neural plasticity in blind cochlear implant users.

This study, using positron emission tomography, investigates the cortical activation generated by auditory stimulation in two congenitally blind cochlear implant users. In the patient with a relatively short history of deafness, activity increased in both auditory cortices and fell in the visual cortices. The patient with a longer period of deafness had greater activation of the visual cortices than the auditory cortices. A similar pattern of activity was seen when this patient subsequently had a second cochlear implant inserted into the opposite ear. The neural pathways formed after the restoration of auditory input in the congenitally blind can activate either the auditory or visual cortices. We suggest that the visual cortical activation demonstrated is of functional significance.

Auditory cortical activation and speech perception in cochlear implant users: effects of implant experience and duration of deafness.

This study aimed to investigate the relationship between outcome following cochlear implantation and auditory cortical activation. It also studied the effects of length of implant use and duration of deafness on the auditory cortical activations. Cortical activity resulting from auditory stimulation was measured using [(18)F]FDG positron emission tomography. In a group of 18 experienced adult cochlear implant users, we found a positive correlation between speech perception and activations in both the primary and association auditory cortices. This correlation was present in a subgroup of experienced implant users but absent in a group of new implant users with similar speech perception abilities. There was a significant negative correlation between duration of deafness and auditory cortical activation. This study gives insights into the relationship between implant speech perception and auditory cortical activation and the influence of duration of preceding deafness and implant experience.