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1/46. Neurotologic follow-up after radiation of posterior fossa tumors.

    OBJECTIVE: Stereotactic radiation treatment, also known as gamma knife surgery or radiosurgery, has come into acceptance as a treatment alternative to surgical removal for posterior fossa tumors. The purpose of this article is to describe the role of the neurotologist in the optimal management of neurotologic complications after stereotactic radiation, as illustrated by five patients. STUDY DESIGN: Retrospective chart review. patients: Five patients who underwent stereotactic radiation of posterior fossa tumors. MAIN OUTCOME MEASURES: Presence or absence of neurotologic complications (tumor growth, hearing loss, imbalance/ataxia, vertigo, and facial paralysis) or neurosurgical complaints (facial numbness, motor weakness, headache, hydrocephalus, and subarachnoid cysts). RESULTS: Postradiation neurotologic complaints included vertigo, imbalance/ataxia, and progressive hearing loss in four of the five patients. Continued tumor growth occurred in two patients; two patients had no growth; in one patient the tumor became smaller. The complications of facial nerve paralysis, facial numbness, motor weakness, headache, hydrocephalus, cerebellar edema, and posterior fossa arachnoid cyst formation occurred less frequently. CONCLUSIONS: Stereotactic radiation of posterior fossa tumors can produce significant neurotologic problems. It is imperative that neurotologists remain involved in the follow-up care of patients with posterior fossa tumors to offer optimal treatment alternatives for the neurotologic disorders. ( info)

2/46. Otodental syndrome: a case report and genetic considerations.

    A 5-year-old boy presented with otodental syndrome. His maxillary and mandibular incisors were within normal limits. The premolar/molar areas in all quadrants were occupied by markedly macrodontic teeth showing globular shape. The canines had a similar rounded shape. Two canines and 1 of the other abnormal teeth demonstrated areas of yellow hypoplastic enamel. Radiographs revealed that some of the abnormal teeth had bifurcated pulp chambers and pulp stones. Premolar tooth germs were absent. audiometry demonstrated that the child had a marked bilateral sensorineural hearing loss for frequencies above 1000 Hz. No other members of the immediate family were clinically affected. The parents of the child were not aware of any similarly affected relatives. Suggestions with respect to possible genetic mechanisms and gene participation in the etiology of this syndrome are offered. ( info)

3/46. A case of sensorineural deafness following ingestion of Ecstasy.

    Ecstasy is a substance of abuse commonly associated with the dance scene and taken by many young people. A brief history of Ecstasy and its side-effects is given. A case of ototoxicity is presented, as an additional side-effect to the long list of complications caused by Ecstasy. ( info)

4/46. hearing loss after direct blunt neck trauma.

    OBJECTIVE: To report for the first time hearing impairment resulting from blunt neck trauma. STUDY DESIGN: Retrospective chart review of clinical, pure tone, and speech audiometric findings. The first obtained within 3 months and the follow-up ones between 6 and 12 months after injury. Three representative examples are given. patients: Eighty-three patients (166 ears) who reported hearing impairment after blunt neck trauma. RESULTS: Twenty of the 166 ears (12%) had normal hearing and 137 ears (81.3%) showed an acoustic trauma-like hearing impairment. Eight ears (4.8%) had a hearing loss of at least 30 dB in the speech frequencies (500-2,000 Hz) and two ears (1.2%) had additional impairment in the higher frequencies. Only one ear (0.8%) had a conductive hearing loss. No speech discrimination score was poorer than 80%. Forty-six subjects (55.4%) reported tinnitus. CONCLUSIONS: Blunt neck trauma, like whiplash injury, may cause objectively measurable hearing impairment. ( info)

5/46. erythromycin associated hearing loss in a patient with prior cis-platinum induced ototoxicity.

    A case is reported of reversible sensorineural hearing loss associated with intravenous erythromycin treatment. Cis-platinum induced high frequency hearing loss developed nine months previously during treatment for stage IV papillary cystadenocarcinoma. Renal and hepatic function were normal; however, serum erythromycin levels were elevated. Clinical recovery promptly followed discontinuation of erythromycin. ( info)

6/46. An absence of tinnitus.

    We present and discuss a case of lifelong tinnitus in an otolaryngologist (L.D.L.) followed by complete elimination of the tinnitus as a result of a cerebral vascular accident located in the left corona radiata. Pre- and post-CVA audiograms showed no change in hearing. A magnetic resonance imaging study of the brain documents the size and location of the lesion. Discussion looks at recent studies of the central nervous system showing evidence of increased activity related to tinnitus. Our assessment of the lesion and the resulting loss of tinnitus can be explained by the neurophysiological model of tinnitus, which includes plasticity of the central nervous system. ( info)

7/46. Autosomal dominant palmoplantar hyperkeratosis and sensorineural deafness in three generations.

    A family is presented with autosomal dominant progressive palmoplantar hyperkeratosis, which is invariably associated with a slowly progressive, bilateral, high frequency, sensorineural hearing loss. The family show no other ectodermal abnormality. The differential diagnosis and possible mechanisms are discussed. This family appears to represent a unique variant in the hyperkeratosis-deafness association. ( info)

8/46. Audiovestibular evolution in a patient with multiple sclerosis.

    multiple sclerosis is characterized by the presence of multiple plaques within the central nervous system, manifesting as remission and exacerbation of neurologic dysfunction over variable time courses. We present the case of a 20-year-old woman. Before treatment, her auditory brain stem response (ABR) test revealed bilateral prolongation. A caloric test showed canal paresis of the right ear and a normal response on the left. A vestibular evoked myogenic potential (VEMP) test displayed an absent response in the right ear and a delayed response in the left. A magnetic resonance imaging (MRI) scan demonstrated multiple diffuse high signal lesions in the hemispheres, brain stem, and cerebellum. Six months after treatment, the demyelinating plaques were shown to have resolved spontaneously on MRI. Recovery of caloric responses was anticipated. Bilateral prolongation of ABRs remained, but the VEMP test disclosed a normal response in the right ear and a delayed response in the left. Accordingly, in addition to MRI, caloric tests and ABR and VEMP tests are useful in monitoring the evolution of audiovestibular function in patients with multiple sclerosis. ( info)

9/46. Symptomatic high frequency/acceleration vestibular loss: consideration of a new clinical syndrome of vestibular dysfunction.

    CONCLUSION: Symptomatic high frequency/acceleration vestibular loss is a distinct clinical entity that can be missed on conventional ENG with caloric testing. Under certain circumstances, symptomatic patients with a high frequency/acceleration vestibular loss should undergo an MSSC study for confirmation, if required. OBJECTIVE: To document that normal electronystagmography (ENG) with conventional bithermal caloric testing is inadequate for diagnosing clinically significant high frequency/acceleration vestibular loss. MATERIAL AND methods: patients with clinical symptoms and signs of persistent peripheral vestibular dysfunction despite normal conventional bithermal caloric testing on ENG underwent high frequency/acceleration horizontal magnetic scleral search coil (MSSC) eye movement studies. The clinical findings and results from audiometric tests, conventional ENG with bithermal caloric tests and MSSC tests were reviewed. RESULTS: Eleven patients were identified as having an abnormal MSSC study, indicating a high frequency/acceleration vestibular loss consistent with their clinical history despite normal or equivocal bithermal caloric responses on conventional ENG. Although valuable, ENG caloric testing evaluates lateral semicircular canal function and should be considered a non-physiological test primarily of low frequency vestibular function. High frequency/acceleration head thrust testing clinically detected a "high frequency/acceleration vestibular loss" in 8/11(72.7%) cases. ( info)

10/46. Epstein syndrome: oral lesions in a patient with nephropathy, deafness and thrombocytopenia.

    Epstein syndrome is a rare dominantly inherited disorder in which the main features are nephritis, deafness and macrothrombocytopathic thrombocytopenia. A case with oral haemorrhage and purpuric lesions is reported. This appears to be the first report of this syndrome in the dental literature. ( info)
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