Long-term Quality of Life Following Vestibular Schwannoma Excision Via the Translabyrinthine Approach.

Objective: To assess postoperative quality of life (QOL) and other patient-reported outcomes following surgery for vestibular schwannoma.

Study Design: Cross-sectional retrospective case review using postal questionnaires.

Setting: Tertiary referral center.

Auditory Brainstem Implantation in Neurofibromatosis Type 2: Experience From the Manchester Programme.

Objective: To describe the experience of auditory brainstem implantation (ABI) in patients with Neurofibromatosis type 2 (NF2).

Study Design: Retrospective case review.

Setting: Tertiary referral centre.

Subjective outcomes of auditory brainstem implantation.

Aim: To assess the long-term subjective benefits of auditory brainstem implants (ABIs) and investigate the extent to which ABI users perceive it to be a useful device.

Methods: Using the Manchester database, all 31 living patients registered as ABI users were identified and sent the ABI performance questionnaire. Data regarding daily duration of use, auditory fatigue, ability to differentiate between speech and environmental sounds, and subjective usefulness of the ABI in different listening conditions were collected.

Clinical and radiological guidance in managing facial nerve schwannomas.

Objective: To present a review of all patients diagnosed with a facial nerve schwannoma (FNS) managed in our center over almost two decades, and suggest guidelines for their classification and management.

Study Design: Retrospective case review

Setting: Tertiary referral center

Patients: Twenty-eight patients with a facial nerve schwannoma

Intervention: Conservative or surgical management depending on clinical and radiological features

Main Outcome Measure: Patient demographics, site of tumor, and clinical symptoms, including facial nerve function (House-Brackmann score) at baseline and follow-up. In those managed surgically, operative approach and surgical outcomes were also recorded.

Surgical outcomes in cystic vestibular schwannoma versus solid vestibular schwannoma.

Objective: To review the postoperative surgical outcomes of cystic vestibular schwannomas (CVSs), especially facial nerve outcomes, and compare these results with those from matched solid vestibular schwannomas (SVS) resected during the same period at a tertiary referral center.

Study Design: Retrospective case series.

Methods: One hundred thirty-one surgically managed patients with cystic vestibular schwannomas (CVSs) were age, sex, and tumor size matched to 131 surgically managed patients with solid vestibular schwannomas (SVSs).

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.

History of cochlear implantation.

The evolution of cochlear implantation from early experimentation with electrical stimulation of the ear in the 18th century to the sophisticated multichannel device that we now routinely implant has been remarkable. It is a tribute to the perseverance of a number of dedicated scientists in many disciplines who, in the face of serious, but ultimately unfounded, theoretical objections and ethical challenges, were eventually able to restore useful hearing to that large group of profoundly deaf adults and children, who were hitherto untreatable. The transformation in the lives of prelingually deaf children, who are now able to acquire speech and language and fulfil their full educational potential has been particularly spectacular.

Ipsilateral cochlear implantation after cochlear nerve preserving vestibular schwannoma surgery in patients with neurofibromatosis type 2.

Objective: To investigate the outcomes from ipsilateral simultaneous or sequential cochlear implantation in patients with neurofibromatosis type 2 (NF2) after vestibular schwannoma removal with cochlear nerve preservation.

Study Design: Retrospective case series.

Setting: Single tertiary referral NF2 center.

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).

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.

Vestibular schwannomas occur in schwannomatosis and should not be considered an exclusion criterion for clinical diagnosis.

Schwannomatosis is a recently delineated inherited condition that has clinical overlap with neurofibromatosis type 2 (NF2). Diagnostic criteria have been developed to distinguish schwannomatosis from NF2, but the existence of mosaic NF2, which may closely mimic schwannomatosis, makes even these criteria problematic. In particular, it is not clear why there is a relative sparing of the cranial nerves from schwannomas in schwannomatosis.

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.

Empirical development of improved diagnostic criteria for neurofibromatosis 2.

Purpose: Four sets of clinical diagnostic criteria have been proposed for neurofibromatosis 2, but all have low sensitivity at the time of initial clinical assessment for the disease among patients with a negative family history who do not present with bilateral vestibular schwannomas. We have empirically developed and tested an improved set of diagnostic criteria that uses current understanding of the natural history and genetic characteristics of neurofibromatosis 2 to increase sensitivity while maintaining very high specificity.

Methods: We used data from the UK Neurofibromatosis 2 Registry and Kaplan-Meier curves to estimate frequencies of clinical features at various ages among patients with or without unequivocal neurofibromatosis 2.

Neurofibromatosis type 2.

Neurofibromatosis type 2 (NF2) is an autosomal-dominant inherited tumour predisposition syndrome caused by mutations in the NF2 gene on chromosome 22. Affected individuals develop schwannomas characteristically affecting both vestibular nerves leading to hearing loss and eventual deafness. Rehabilitation with brain stem implants and in some cases cochlear implants is improving this outcome.

Cranial meningiomas in 411 neurofibromatosis type 2 (NF2) patients with proven gene mutations: clear positional effect of mutations, but absence of female severity effect on age at onset.

Background: Meningiomas have been reported to occur in approximately 50% of neurofibromatosis type 2 (NF2) patients. The NF2 gene is commonly biallelically inactivated in both schwannomas and meningiomas. The spectrum of NF2 mutations consists mainly of truncating (nonsense and frameshift) mutations.

Brain stem responses evoked by stimulation of the mature cochlear nucleus with an auditory brain stem implant.

Objectives: The Nucleus auditory brain stem implant (ABI) has been used in the hearing rehabilitation of totally deaf individuals for whom a cochlear implant is not an option such as in the case of neurofibromatosis type 2 (NF2). Intraoperative electrically evoked auditory brain stem responses (EABRs) are recorded to assist in the placement of the electrode array over the dorsal and ventral cochlear nuclei in the lateral recess of the IVth ventricle of the brain stem. This study had four objectives: (1) to characterize EABRs evoked by stimulation with an ABI in adolescents and adults with NF2, (2) to evaluate how the EABR morphology relates to auditory sensations elicited from stimulation by an ABI, (3) to establish whether there is evidence of morphology changes in the EABR with site of stimulation by the ABI, and (4) to investigate how the threshold of the EABR relates to behavioral threshold and comfortably loud sensations measured at initial device activation.

Brain stem responses evoked by stimulation with an auditory brain stem implant in children with cochlear nerve aplasia or hypoplasia.

Objectives: The inclusion criteria for an auditory brain stem implant (ABI) have been extended beyond the traditional, postlingually deafened adult with Neurofibromatosis type 2, to include children who are born deaf due to cochlear nerve aplasia or hypoplasia and for whom a cochlear implant is not an option. Fitting the ABI for these new candidates presents a challenge, and intraoperative electrically evoked auditory brain stem responses (EABRs) may assist in the surgical placement of the electrode array over the dorsal and ventral cochlear nucleus in the brain stem and in the postoperative programming of the device. This study had four objectives: (1) to characterize the EABR by stimulation of the cochlear nucleus in children, (2) to establish whether there are any changes between the EABR recorded intraoperatively and again just before initial behavioral testing with the device, (3) to establish whether there is evidence of morphology changes in the EABR depending on the site of stimulation with the ABI, and (4) to investigate how the EABR relates to behavioral measurements and the presence of auditory and nonauditory sensations perceived with the ABI at initial device activation.

Outcome from surgery for vestibular schwannomas in children.

Object: A review of sporadic and NF2-related vestibular schwannoma surgery in children (under 18 years of age) with a specific interest in resection rates, recurrence, facial nerve outcomes, hearing preservation, hearing rehabilitation and genetic analysis.

Methods: A retrospective analysis of prospectively collected data of 35 consecutively operated vestibular schwannomas in 29 paediatric patients that underwent 38 operations between 1992 and 2007. Pre- and post-operative radiology, facial nerve function, pure tone audiogram and speech discrimination tests were performed with a mean follow-up of 4.

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.

Does Meningitis Stop CSF Rhinorrhea following Lateral Skull Base Surgery?

In cases of cerebrospinal fluid (CSF) rhinorrhea following lateral skull base surgery, fibrosis and fibrin formation resulting from meningitis has been postulated as a mechanism of spontaneously resolving the CSF leak. This study was undertaken to explore any possible relationship between the cessation of CSF leak and meningitis. A retrospective study at a tertiary referral center of 232 consecutive patients was performed.

What are the implications in individuals with unilateral vestibular schwannoma and other neurogenic tumors?

Objectives: Individuals who develop a unilateral vestibular schwannoma (VS) and other neurogenic tumors are at high risk of having the inherited condition neurofibromatosis Type 2 (NF2). The risk of bilateral disease and transmission risk to offspring are important in surgical planning and counseling. The authors have attempted to resolve these risks.