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.

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.

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).

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.

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).

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.

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.

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.

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.

Hearing preservation via a cochleostomy approach and deep insertion of a standard length cochlear implant electrode.

Objective: The suggestion that the depth of insertion of the electrode into the cochlea is critical to hearing preservation has led to the development of a generation of short electrodes designed to minimize intracochlear trauma and avoid contact with the apical region of the cochlea. This study aims to describe our experience of hearing preservation surgery using a deeply inserted standard length electrode array covering the region of residual hearing.

Study Design: A retrospective case note review was performed identifying cases of attempted hearing preservation using standard length electrodes.

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.

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.

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.

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.

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.