A consensus conference on neurofibromatosis 2 (NF2) was held in 2002 at the request of the United Kingdom (UK) Neurofibromatosis Association, with particular emphasis on vestibular schwannoma (VS) surgery. NF2 patients should be managed at specialty treatment centres, whose staff has extensive experience with the disease. All NF2 patients and their families should have access to genetic testing because presymptomatic diagnosis improves the clinical management of the disease. Some clinical manifestations of NF2, such as ocular abnormalities, can be detected in infancy; therefore, clinical screening for at-risk members of NF2 families can start at birth, with the first magnetic resonance (MRI) scan at 10-12 years of age. Minimal interference, maintenance of quality of life, and conservation of function or auditory rehabilitation are the cornerstones of NF2 management, and the decision points to achieve these goals for patients with different clinical presentations are discussed.

Facial paralysis is the loss of voluntary muscle movement of one side of the face. A quantitative, objective, and reliable assessment system would be an invaluable tool for clinicians treating patients with this condition. This paper presents a novel framework for objective measurement of facial paralysis. The motion information in the horizontal and vertical directions and the appearance features on the apex frames are extracted based on the local binary patterns (LBPs) on the temporal-spatial domain in each facial region. These features are temporally and spatially enhanced by the application of novel block processing schemes. A multiresolution extension of uniform LBP is proposed to efficiently combine the micropatterns and large-scale patterns into a feature vector. The symmetry of facial movements is measured by the resistor-average distance (RAD) between LBP features extracted from the two sides of the face. Support vector machine is applied to provide quantitative evaluation of facial paralysis based on the House-Brackmann (H-B) scale. The proposed method is validated by experiments with 197 subject videos, which demonstrates its accuracy and efficiency.

Fifty-six patients with osteogenesis imperfecta underwent clinical and audiological assessment. They completed questionnaires regarding their physical and otological disabilities, and attended various centres for audiological testing. It was found that 31 patients had a hearing loss. Hearing loss began in the second and third decades as a conductive loss, and progressed to a mixed loss. Eight patients, found in all age groups, had a pure sensorineural loss in one or both ears. Patients with osteogenesis imperfecta congenita suffered more fractures, became more physically disabled and had more severe hearing loss than those with osteogenesis imperfecta tarda. Tympanometry was found to give unpredictable results and was unhelpful in assessing middle ear function in these patients.