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Cochlear implantation has become a standard therapy for children with bilateral profound hearing loss, resulting in substantial and sustainable benefits for the development of expressive and receptive and expressive language skills and cognition. During the last few years, audiologic and otologic criteria for cochlear implantation have been expanded. Recently, patients with profound single-sided deafness with or without tinnitus have received cochlear implants despite normal to near-normal hearing on the contralateral side. This indication, however, has thus far been restricted to adult patients. Although it is known that unilateral hearing has an impact on social-emotional development in children, otologic surgeons have been reluctant to treat children with single-sided deafness with a cochlear implant. We report here on a case of successful cochlear implantation in an 8-year-old boy with acute single-sided deafness due to a lateral skull-base fracture, after an MRI showed signs of imminent fibrosis of the inner ear with possible prevention of cochlear implantation at a later stage. There was normal hearing in the contralateral ear. The child showed rapid development of speech discrimination in the implanted ear, improvements in sound localization and speech perception in noise, and a high degree of patient satisfaction. This experience may encourage using this therapeutic approach in children with chronic profound single-sided deafness.

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Recently it has been postulated that there is an atypical facioscapulohumeral muscular dystrophy (FSHD) phenotype with isolated axial myopathy. Involvement of paraspinal and limb muscles was evaluated in six patients with molecularly proven FSHD and a predominant bent spine phenotype. Consistent with the camptocormia phenotype, the most severely affected muscles in all six patients were the thoracic and lumbar spinal tract together with hamstrings. MRI disclosed severe axial muscle degeneration but mostly subclinical involvement of limb muscles. The involvement of hip extensor muscles in FSHD might considerably contribute to the clinical phenotype of camptocormia due to axial muscle involvement.

Invasive aspergillosis (IA) originating from the paranasal sinuses can cause an intracranial growth mainly along the skull base and larger vessels. This study reports our experience in the diagnosis and treatment of a series of patients with IA. A retrospective chart review of four patients with chronic invasive intracranial aspergillosis was performed. Clinical signs, physical examinations, radiographs, histological samples, and outcome were demonstrated. The patients demonstrated different symptoms like exophthalmus, ophthalmoplegia, loss of vision, and hypaesthesia of the ophthalmic and maxillary nerve. Computed tomography and MRI revealed extensive sino-orbital and skull base lesions. The patients were treated with aggressive endonasal debridement, intravenous antifungal agents and daily irrigations with antimycotic suspensions. Furthermore, we applied hyperbaric oxygenation. Two patients died from complications due to subarachnoidal hemorrhage and accompanied complications respectively. Despite the high mortality rate patients with an invasive aspergillosis can be effectively treated in some cases by an early and rigorous treatment schedule using all surgical and conservative therapeutic options.