Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology
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To describe a unilateral progressive conductive hearing loss caused by incus discontinuity (without erosion of the long process of the incus), and otosclerosis with fixation of the stapedial footplate. ⋯ This is the third reported case of an unusual combination of otosclerosis and ossicular discontinuity, and the first such case report in a patient without head trauma. In addition, it adds a unique item to the differential diagnosis of the pathologic features implicated in an ear with a conductive deficit and normal tympanogram.
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Cochlear implantation in patients with neurofibromatosis type 2 and bilateral vestibular schwannoma.
To investigate the results of cochlear implantation in patients with neurofibromatosis Type 2 (NF2) and bilateral vestibular schwannoma. ⋯ In selected cases of deafness in patients with NF2 where there has been anatomic preservation of the auditory nerve after acoustic neuroma resection or radiation therapy, cochlear implantation may offer some improvement in communication skills, including the possibility of open-set speech communication in some patients. These results compare favorably to the auditory brainstem implant offering an alternative for hearing rehabilitation in patients with NF2.
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Delivery of the gene encoding X-linked inhibitor of apoptosis (XIAP) using an adeno-associated viral (AAV) vector can protect against cisplatin-mediated ototoxicity. ⋯ XIAP delivery to the cochlea can protect against the audiometric changes and hair-cell loss associated with cisplatin ototoxicity. The efficacy, specificity, and duration of the protective effects make this a potentially attractive therapeutic paradigm.