1/73. bone marrow transplantation corrects osteopetrosis in the carbonic anhydrase ii deficiency syndrome.carbonic anhydrase ii (CAII), found in renal tubules, brain, and osteoclasts, is critical in acid-base homeostasis and bone remodeling. Deficiency of CAII gives rise to a syndrome of osteopetrosis, renal tubular acidosis (RTA), and cerebral calcification with associated developmental delay. It is inherited in an autosomal recessive fashion and found most frequently in the mediterranean region and the middle east. We report 2 related Irish families with clinically severe CAII deficiency in whom the gene mutation has been fully elucidated. Two children, one from each family, have undergone allogeneic bone marrow transplantation because of severe progressive visual and hearing loss. The older 2 children had already developed cerebral calcification and marked visual loss at the time of diagnosis and were treated symptomatically. Post-transplantation evaluation at 2 and 3 years demonstrates histologic and radiologic resolution of their osteopetrosis with stabilization of hearing and vision. Both children remain developmentally delayed and continue to have RTA, and the older child has now developed cerebral calcification. Allogeneic bone marrow stem cell replacement cures the osteoclast component of CAII deficiency and retards the development of cerebral calcification, but it appears to have little or no effect on the renal lesions. (blood. 2001;97:1947-1950)- - - - - - - - - - ranking = 1keywords = stem (Clic here for more details about this article) |
2/73. Influence of otitis media on hearing and development.otitis media affects nearly every child at some time; many children have mild-to-moderate hearing losses for prolonged periods. The effects of these losses on language and educational development may be significant; possible mechanisms are discussed. Especially in suboptimal listening situations, speech perception may be impaired by even a mild hearing loss. Patterns of imperception are predicted by comparison of composite audiometric data from children with middle ear effusions with speech power data, and by analysis of sound pressure waveforms of speech filtered to simulate the typical hearing loss of these patients. A new method of analysis of brain stem evoked responses, yielding response components attributable to binaural interaction, is reported. This and other evoked response techniques may be able to identify objective changes in auditory nervous system function attributable to relative sensory deprivation during development. Finally, directions for further research in this area are discussed.- - - - - - - - - - ranking = 52.839750499168keywords = brain stem, stem (Clic here for more details about this article) |
3/73. Otologic injuries from airbag deployment.Airbags became available as an optional passive restraint system in motor vehicles in 1973. The National Highway Traffic safety Administration mandated placement of driver and right passenger airbags in all passenger vehicles and light trucks beginning in model year 1997. An estimated 2.1 million airbags have been deployed from the late 1980s until the present. There have been several case reports of hearing loss after exposure to airbag deployments in drivers and passengers since 1995. Members of the American Academy of otolaryngology-head and neck Surgery submitted case reports on 71 patients with otologic symptoms after airbag deployment.- - - - - - - - - - ranking = 1keywords = stem (Clic here for more details about this article) |
4/73. Meniere's disease in the elderly.OBJECTIVES: To assess the prevalence, presentation, treatment, and evolution of Meniere's disease in elderly patients (> or = 65 yr old). STUDY DESIGN: Retrospective clinical study and case report. SETTING: A neuro-otology referral center at the university hospital in Bern, switzerland, and a neurotologic practice in Geneva, switzerland. patients: patients were selected from the hospital clinic and otolaryngologic practice registers. Among 8423 neurotologic checkups performed between 1988 and 1998, 432 (5.1%) patients had definite Meniere's disease and 66 (15.3%) of these patients were > or = 65 years old. INTERVENTION: The files of these 66 patients were analyzed. RESULTS: Meniere's disease in the elderly had 2 modes of presentation: reactivation of longstanding Meniere's disease (40.9%) and de novo Meniere's disease (59.1%), where the first dizzy spells, tinnitus, and hearing loss appeared after 65 years of age. Drop attacks occurred in 11.1 and 25.6% of the cases, respectively, and could be responsible for misdiagnosis of strokes of the brainstem. The patients were treated by mild antivertiginous drugs (betahistine, cinnarizine). Twenty-seven patients (41%) underwent surgery: transtympanic ventilation tubes (19 patients), sacculotomy (4 patients), vestibular neurectomy (3 patients), surgical labyrinthectomy (1 patient). The vertigo spells disappeared in 73.3 to 100% of the cases according to the type of surgery performed. CONCLUSION: Our study shows that Meniere's disease in the elderly is not at all uncommon. It can appear as a de novo disease or as a reactivated longstanding disease. Drop attacks are more frequent than reported in general patient populations with Meniere's disease and can mimic a stroke of the brainstem. Medical and surgical treatments have to be cautious because many of these elderly patients are fragile.- - - - - - - - - - ranking = 2keywords = stem (Clic here for more details about this article) |
5/73. Severe hearing loss in Pallister-Killian syndrome.Pallister-Killian syndrome is a rare disorder characterised by a specific combination of anatomic anomalies, mental retardation and lack of speech acquisition due to tetrasomy 12p. hearing loss does not seem to be characteristic for this syndrome, although it was reported in several cases. We present the case of a girl first seen in our department at the age of 6 months. A severe sensory hearing loss was confirmed by subjective and objective audiometry. The child was successfully equipped with hearing aids. In the literature almost all children with Pallister-Killian syndrome are described as not developing verbal speech. Surprisingly their hearing abilities were not examined systematically. We advise audiological testing of children with Pallister-Killian syndrome.- - - - - - - - - - ranking = 1keywords = stem (Clic here for more details about this article) |
6/73. Vestibular schwannoma with contralateral facial pain - case report.BACKGROUND: Vestibular schwannoma (acoustic neuroma) most commonly presents with ipsilateral disturbances of acoustic, vestibular, trigeminal and facial nerves. Presentation of vestibular schwannoma with contralateral facial pain is quite uncommon. CASE PRESENTATION: Among 156 cases of operated vestibular schwannoma, we found one case with unusual presentation of contralateral hemifacial pain. CONCLUSION: The presentation of contralateral facial pain in the vestibular schwannoma is rare. It seems that displacement and distortion of the brainstem and compression of the contralateral trigeminal nerve in Meckel's cave by the large mass lesion may lead to this atypical presentation. The best practice in these patients is removal of the tumour, although persistent contralateral pain after operation has been reported.- - - - - - - - - - ranking = 1keywords = stem (Clic here for more details about this article) |
7/73. Subtonsillar placement of auditory brainstem implant.A 26-year-old female with NF2 underwent removal of an acoustic schwannoma via extended retrosigmoid approach with subtonsillar placement of the auditory brainstem implant. The patient had already shown palsy of the lower cranial nerves on the opposite side due to previous surgery. Differing from conventional routes such as the translabyrinthine and the retrosigmoid, the subtonsillar approach enabled us to observe the entire cochlear nucleus and avoid injury to the 9th and 10th cranial nerves when applying the electrode. Hearing in pure tone average was maintained almost at the preoperative level. We present a new use of this approach in placing the electrode and discuss its advantages.- - - - - - - - - - ranking = 5keywords = stem (Clic here for more details about this article) |
8/73. radiation-induced schwannomas of the nervous system. Report of five cases and review of the literature.radiation therapy has important delayed effects on the central nervous system. Prominent among these effects is radiation necrosis of nervous tissue, but an oncogenic effect is also recognized. Both benign and malignant intracranial tumors can develop in irradiated fields, particularly in children. Most of these tumors are sarcomas, meningiomas or gliomas and only occasionally schwannomas. We report 5 cases of postirradiation acoustic nerve schwannoma observed in our Department.- - - - - - - - - - ranking = 5keywords = stem (Clic here for more details about this article) |
9/73. Auditory disturbance as a prodrome of anterior inferior cerebellar artery infarction.OBJECTIVES: To investigate the clinical and radiological features of patients presenting with an acute auditory syndrome as a prodromal symptom of anterior inferior cerebellar artery (AICA) infarction. methods: 16 consecutive cases of AICA infarction diagnosed by brain magnetic resonance imaging completed a standardised audiovestibular questionnaire and underwent a neuro-otological evaluation by an experienced neuro-otologist. RESULTS: Five patients (31%) had an acute auditory syndrome as a prodrome of AICA infarction one to 10 days before onset of other brain stem or cerebellar symptoms. Two types of acute auditory syndrome were found: recurrent transient hearing loss with or without tinnitus (n = 3), and a single episode of prolonged hearing loss with or without tinnitus (n = 2). The episodic symptoms were brief, lasting only minutes. The tinnitus preceding the infarction was identical to the tinnitus experienced at the time of infarction. At the time of infarction, all patients developed hearing loss, tinnitus, vertigo, and ipsilateral hemiataxia. The most commonly affected site was the middle cerebellar peduncle (n = 5). Four of the five patients had incomplete hearing loss and all had absence of vestibular function to caloric stimulation on the affected side. CONCLUSIONS: Acute auditory syndrome may be a warning sign of impending pontocerebellar infarction in the distribution of the AICA. The acute auditory syndrome preceding an AICA infarct may result from ischaemia of the inner ear or the vestibulocochlear nerve.- - - - - - - - - - ranking = 51.839750499168keywords = brain stem, stem (Clic here for more details about this article) |
10/73. tinnitus in childhood.All of 1,420 children seen for clarification of a hearing disorder or to follow up for known difficulty in hearing were questioned as to whether they experienced tinnitus. The interview was carried out after a hearing test was conducted, which was based on play audiometry or normal pure-tone threshold audiometry, depending on the age of the child. When being interviewed, 102 children reported that tinnitus had appeared or was still present. Seventy-five children (73.5%) demonstrated difficulty in hearing in one or both ears, whereas 27 children (26.5%) had normal hearing in both ears. The most frequently obtained information (29.4%) was the progression of an existing hearing loss. meningitis is an important cause of hearing loss and of tinnitus and could be identified in 20% of our patients. We also considered as a cause of tinnitus skull or brain trauma, acute hearing loss, and stapes surgery. However, the mechanisms of tinnitus development were not immediately clear in a large proportion of the children studied: Problems included central sensory perception (14.7%) and emotional factors (11.8%). No additional information that might lead to an understanding of the hearing loss was available for 14.7% of the patients studied. tinnitus is a frequent symptom in childhood and, because children seldom complain about their tinnitus, such hearing problems that they report must always be taken seriously. The diagnosis should exclude metabolic disturbances, possible damage to the sensory level of the central nervous system, and circulatory disturbances. In addition, the physician should always consider emotional problems and disturbances of perception.- - - - - - - - - - ranking = 1keywords = stem (Clic here for more details about this article) |
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