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 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Chiari-I malformation is a deformity of the structures of the posterior fossa in which there is inferior herniation of the cerebellar tonsils through the foramen magnum without significant caudal dislocation of the brainstem. Patients are usually asymptomatic until adulthood, when they commonly present with recurrent headaches, weakness, vertigo and/or imbalance, nystagmus and hearing loss. A review of 226 consecutive patients evaluated for asymmetric sensorineural hearing loss revealed 32 patients with retrocochlear pathology. Three of these patients were discovered to have a Chiari-I malformation by magnetic resonance imaging as their only pathology. We suggest a possible association between a Chiari-I malformation and isolated asymmetric sensorineural hearing loss secondary to long-standing traction on the eighth cranial nerve.
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PMID:Chiari-I malformation associated with asymmetric sensorineural hearing loss. 158 2

We have described the important clinical features and aspects of the Chiari-I malformations, with particular emphasis on Chiari-I malformation. Previously thought to be a rare finding with only minor significance, Chiari-I malformation is an important cause of a variety of symptoms, and will be diagnosed even more frequently as the use of MRI increases. The clinician must consider Chiari-I malformation in any patient with unexplained sensorineural hearing loss, headache, vertigo, ataxia, dysequilibrium, dysphagia or other cranial nerve symptom, especially if accompanied by more classic symptoms of this disorder, such as cervical pain or weakness.
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PMID:The Chiari-I malformation. 187 53

We report temporal bone pathology in a 25-year-old man with bilateral temporal bone adenocarcinoma which was caused by metastasis from a primary lesion in the pancreas. The initial symptoms began with vertigo and headache and the patient noticed left hearing loss in the left ear on the following day. A few days later, he noticed hearing loss in the right ear, and bilateral hearing was totally lost within two weeks of the onset. In addition to severe bilateral sensorineural hearing loss, left IInd, bilateral Vth and VIIIth cranial nerve paralysis occurred. Brain CT showed multiple metastatic lesions in the brain. The patient's general condition rapidly deteriorated, and he died of acute pneumonia on the 42nd day after onset. At autopsy it was revealed adenocarcinoma of the tail and body of the pancreas and its metastasis to the brain and meninges. Pathological study of the temporal bone showed infiltration of carcinomatous cells along the VIIth and VIIIth nerves in the bilateral internal auditory canals.
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PMID:[A case of bilateral sudden hearing loss and vertigo caused by bilateral temporal bone metastasis from pancreatic carcinoma--comparison of clinical findings and temporal bone pathological findings]. 201 18

An epidemic of group A meningococcal disease began in Auckland in May 1985. There were 122 paediatric cases of meningococcal disease in the next 25 months including 98 cases due to group A. The commonest clinical symptoms were vomiting, headache and photophobia, while frequent signs included fever, seizures, petechial rash and meningism or a bulging fontanelle. Complications were uncommon and included sterile arthritis and prolonged fever. The majority had disease confirmed by positive blood or cerebrospinal fluid culture. Significantly fewer positive cultures were seen in those treated with antibiotics prior to admission. The overall mortality was 7%. If the acute illness was survived, the only detected long term sequela was sensorineural hearing loss seen in 6%. A vaccine programme has been undertaken to control this epidemic.
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PMID:The clinical features of paediatric meningococcal disease Auckland, 1985-87. 249 88

A patient with a large, right-sided basilar artery aneurysm was evaluated. Major symptoms included progressive hearing loss, facial numbness, occipital headaches, dizziness, and diplopia of less than a year's duration. Audiologic results indicated a low-frequency sensorineural hearing loss with marked discrepancies between ascending and descending pure-tone thresholds for the ear ipsilateral to the lesion. Auditory brain-stem response demonstrated bilateral abnormalities, but the early waves were normal. Contralateral acoustic reflexes were absent on the right and elevated on the left. Electronystagmography results showed bilaterally absent caloric responses as well as gaze nystagmus and abnormal pursuit movements. Additional audiologic results as well as radiologic and medical findings are also presented.
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PMID:Audiologic and other clinical findings in a case of basilar artery aneurysm. 358 Jan 63

Reports of sudden hearing loss as the first sign of an intracranial aneurysm are sparse and published primarily in the neurologic literature. A case report is presented in which the initial signs and symptoms of a lobular aneurysm in the posterior communicating artery were the sudden onset of bilateral sensorineural hearing loss, tinnitus, and headache. Following evaluation and identification of the aneurysm, this patient underwent a craniotomy with clipping of the aneurysm; hearing sensitivity improved dramatically following surgery. The world literature is reviewed for cases in which aneurysms have initially occurred as hearing loss, tinnitus, or both. Intracranial aneurysm is discussed as a rare, potential source of a sudden sensorineural hearing loss.
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PMID:Intracranial aneurysm occurring as sensorineural hearing loss. 678 27

Mitochondrial myopathy, encephalopathy with lactic acidosis and stroke-like episodes (MELAS) syndrome is one of the mitochondrial encephalomyopathies that has distinct clinical features including stroke-like episodes with migraine-like headache, nausea, vomiting, encephalopathy and lactic acidosis. We report a 27-year-old woman who presented with partial seizure, stroke-like episodes including hemiparesis, hemianopia and hemihypethesia, sensorineural hearing loss, migraine-like headache, and lactic acidosis. Brain computed tomographic scan showed encephalomalacia in the right parieto-occipital area and recent hypodensity in the left temporoparieto-occipital area with cortical atrophy. Muscle biopsy revealed ragged-red fibers and paracrystaline inclusions in the mitochondria. Genetic study revealed an A to G point mutation at nucleotide position (np) 3243 of mitochondrial DNA. External ophthalmoplegia and ptosis were also found during two exaggerated episodes in this patient. Therefore, the overlapping syndrome of chronic progressive external ophthalmoplegia in the MELAS syndrome is considered in this case. Furthermore, we also found carnitine deficiency in this patient and she was responsive well to steroid therapy. Muscle biopsy also revealed excessive lipid droplets deposits. Therefore, the carnitine deficiency may occur in MELAS syndrome with the A to G point mutation at np 3243. We recommend the steroid or carnitine supplement therapy be applied to the MELAS syndrome with carnitine deficiency.
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PMID:CPEO and carnitine deficiency overlapping in MELAS syndrome. 748 81

We have had 30 patients with Harada's disease at our clinic from 1980 to 1993 and their neurotological findings were retrospectively reviewed. There were 12 males and 18 females, whose ages ranged from 15-years to 67-years. Preceding symptoms such as headache or common cold were observed in 50% of the patients. Pleocytosis of the cerebrospinal fluid was found in 89% of the patients. Subjective cochlear and/or vestibular symptoms were noted in 60% of the patients. In 43 of the 50 ears, sensorineural hearing loss (< 40 dB) was observed. In 77% of the patients, vestibular function tests gave abnormal results. On the basis of our findings, it was suggested that Harada's disease with vertiginous and cochlear symptoms could be classified as follows: cochlear type, peripheral vestibular type, cochleovestibular type, central type, and 'unclassified', based on the neurotological results.
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PMID:Neurotological evaluation of Harada's disease. 761 Aug 43

A 36-year-old man was admitted to our university hospital, complaining of acute hearing loss of the right ear, which was his only hearing ear. He had developed complete deafness due to mumps in the left ear at the age of 26. Before admission, he had been diagnosed as having rubella infection. He noticed hearing loss in the right ear two days after disappearance of macular rash. Headache, nausea and vertigo were not present. Three weeks after the onset of hearing loss, he was admitted to our university hospital. Pure tone audiometry revealed sensorineural hearing loss; a mild ascending audiometric curve with a marked loss at 4,000 Hz and 8,000 Hz. SISI test was positive. Caloric stimulation (ice water 10 ml/10 s) to the right ear demonstrated a normal response. Rubella hemagglutination antibody titer was 512. Specific IgG antibody was over 3,200. Specific IgM antibody was positive (7.01 Enzyme Immunoassay). The data indicated recent infection with rubella. He was given betamethasone for 14 days. Pure tone audiometry showed hearing improvement.
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PMID:Unilateral hearing loss following rubella infection in an adult. 807 85

The type I Chiari malformation consists of a caudal displacement of the cerebellar tonsils through the foramen magnum into the cervical spinal canal. The most common presenting symptoms, such as pain, weakness and headache, are frequently preceded by otoneurological symptoms. Sensorineural hearing loss, vertigo, nystagmus, dysequilibrium, tinnitus and other cranial nerve involvement have been reported in Chiari-I malformation. A case report is presented and the clinical features of the disease are discussed with emphasis on the otoneurological aspects.
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PMID:Otoneurological manifestations in Chiari-I malformation. 832 27


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