Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0011849 (diabetes)
277,896 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

One hundred persons were selected from our audiology records who showed at least 20 dB bilateral sensorineural hearing loss. The number 100 was picked for an adequate statistical analysis. The patients were taken from the chart files which are filed in chronological order. Letters were mailed with instructions for fasting 14 hours prior to testing except for the consumption of water. On presentation, a history was taken for diabetes, height and weight, and the blood pressure was recorded. The blood samples were drawn in clot tubes and taken to a commercial laboratory for overnight refrigeration and testing of the serum. Testing included observation of serum for massive chylomicronemia, triglycerides, cholesterol and lipoprotein electrophoresis (by the cellulose acetate method). Lipoprotein testing results showed 12 patients with Type II A or II B and 8 patients with Type IV abnormalities. No Types I, III or V were found. These 20 patients represent a lower number of hyperlipoproteinemic patients than would be expected in the general population.
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PMID:Study of 100 patients with bilateral sensorineural hearing loss for lipid abnormalities. 20 13

This study is base on 438 patients (age 45-99), 250 women and 188 men, divided into decades from 45 to 95 years. Mean hearing levels were measured at 63 up to 12000 Hz using the atlas pure-tone audiometer. Statistical analyses helped estimating the influence of various anamnestic datas on sensorineural hearing loss. 65 patients with diabetes had no hearing loss related to their disease. In 115 noise-exposed patients 'correction for noise' was 5 to 7 dB at 2000 and 4000 Hz. There was no difference in men and women at any frequency and any age group. 'Average presbycusis' in the literature today is too positive. Starting age 20, a linear progressive hearing loss for all frequencies ranging from 6 to 18 dB per decade can be detected, being less for the lower than for the higher frequencies.
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PMID:[Presbycusis (author's transl)]. 65 82

This is a prospective in-depth study of patients with sudden idiopathic sensorineural hearing loss. We found that 65% recover completely to functional hearing levels spontaneously and independent of any type of medical treatment. The majority do so within 14 days and many within the first few days. Prognosis can be predicted according to the slope of the initial audiogram (low-frequency losses do better than high-frequency losses), hearing at 8 kHz, erythrocyte sedimentation rates, in some select instances spatial disorientation symptoms, and speech discrimination scores. There was a very poor correlation between hearing and vestibular test abnormalities, except hypoactive calories. There were no correlations with age (excepting the very elderly), with antecedent respiratory infections, hypertension, diabetes, or other chronic diseases. We conclude that there is a fundamental difference in the behavior of apical and basal cochlea losses, that hearing recovery is always better at low than at high frequencies, that because of the high spontaneous recovery rates, tympanotomies seeking peri-lymph fistulas should be delayed ten days unless there is a progressive hearing loss, and that none of the current recommended treatments, especially histamine, have any effect on the outcome.
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PMID:Natural history of sudden sensorineural hearing loss. 88 23

Hearing was studied audiometrically in 20 diabetic patients with peripheral neuropathy, and the results were compared with those from a group of normal, age-matched subjects. Although the patients gave no history of hearing loss or ear disease, 11 (55%) had symmetrical sensorineural hearing loss involving at least one frequency. Regression analysis demonstrated significantly higher hearing thresholds for the diabetic group at nine of 11 frequencies tested. Decreased hearing acuity in diabetes mellitus may be related to neuropathy of the auditory nerve.
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PMID:Hearing and diabetic neuropathy. 113 72

Non-insulin-dependent (type II) diabetes mellitus (NIDDM) is characterized by hyperglycaemia and insulin resistance, and affects nearly 5% of the general population. Inherited factors are important for its development, but the genes involved are unknown. We have identified a large pedigree in which NIDDM, in combination with a sensorineural hearing loss, is maternally inherited. The maternal inheritance and the observed decrease in mitochondrial enzyme activities of the respiratory chain indicate a genetic defect in the mitochondrial DNA. An A to G transition was identified at nucleotide 3,243, a conserved position in the mitochondrial gene for tRNA(Leu)(UUR). This mutation cosegregates with the disease in this family and is absent in controls, and indicates that a point mutation in mitochondrial DNA is a pathogenetic factor for NIDDM.
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PMID:Mutation in mitochondrial tRNA(Leu)(UUR) gene in a large pedigree with maternally transmitted type II diabetes mellitus and deafness. 128 50

We have recently identified a point mutation in the mitochondrially encoded tRNA(Leu(UUR)) gene which associates with a combination of type II diabetes mellitus and sensorineural hearing loss in a large pedigree. To extend this finding to other syndromes which exhibit a combination of diabetes mellitus and hearing loss we have sequenced all mitochondrial tRNA genes from two patients with the Wolfram syndrome, a rare congenital disease characterized by diabetes mellitus, deafness, diabetes insipidus and optic atrophy. In each patient, a single different mutation was identified. One is an A to G transition mutation at np 12,308 in tRNA(Leu(CUN)) gene in a region which is highly conserved between species during evolution. This mutation has been described by Lauber et al. (1) as associating with chronic progressive external ophthalmoplegia (CPEO). The other is a C to T transition mutation at np 15,904 in tRNA(Thr) gene. Both mutations are also present in the general population (frequency tRNA(Leu(CUN)) mutation 0.16, tRNA(Thr) mutation 0.015). These findings suggest that evolutionarily conserved regions in mitochondrial tRNA genes can exhibit a significant polymorphism in humans, and that the mutation at np 12,308 in the tRNA(Leu(CUN)) gene is unlikely to be associated with CPEO and Wolfram syndrome.
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PMID:Mutations in mitochondrial tRNA genes: non-linkage with syndromes of Wolfram and chronic progressive external ophthalmoplegia. 154 64

The authors report on one case of Wolfram syndrome, a rare condition, which is characterized by juvenile onset diabetes mellitus, diabetes insipidus, optic atrophy and sensorineural deafness. The findings of this 13-year follow-up show that this patient developed typical neurological complications of long-standing diabetes mellitus as in the common type 1 variant. Moreover, some peculiar signs occurred such as anosmia, ophthalmoplegia interna, and central nystagmus. Since Wolfram syndrome is probably part of a more generalized neurodegenerative disorder, long-term prognosis will depend both upon the severity of chronic diabetic complications and upon the rapidity, by which degeneration of cerebellar, pontine and brain stem structures appear. Prognosis of the cardinal clinical signs is such that optic atrophy, though usually quite rapid in the beginning, generally does not lead to complete blindness. Sensorineural hearing loss progresses very slowly so that deafness might be expected exceptionally only. The hearing deficit in classical diabetics, however, is of retrocochlear origin. Therefore, in Wolfram syndrome, a combined inner-ear and retrocochlear hearing loss may occur.
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PMID:Juvenile onset diabetes mellitus, central diabetes insipidus and optic atrophy (Wolfram syndrome)--neurological findings and prognostic implications. 185 94

A 9-year-old girl and an 11-year-old boy had ptosis, progressive external ophthalmoplegia, pigmentary retinopathy, and sensorineural hearing loss. The girl had diabetes mellitus and the boy had hypoparathyroidism. Both children also developed recurrent vomiting and cerebral infarcts with lactic acidosis. Muscle biopsy specimens showed ragged-red fibers and Southern analysis demonstrated a distinct heteroplasmic deletion of muscle mitochondrial DNA in each patient but no evidence of the point mutation in the transfer RNALeu(UUR) gene recently identified in mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS). These 2 children had combined features of Kearns-Sayre syndrome and MELAS, suggesting that mitochondrial DNA deletions occasionally can have pleomorphic clinical expression.
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PMID:Deletion of mitochondrial DNA in patients with combined features of Kearns-Sayre and MELAS syndromes. 189 71

The most frequent hormonal diseases attracting audiological interest are hypothyroidism (myxodema) and diabetes mellitus. For many years these diseases have been considered to cause hearing disorders with the lesion located predominantly in the inner ear and central auditory pathways, resulting in sensorineural hearing loss. However, the causal relationship between hearing loss and hypothyroidism has been questioned, and thus a study was undertaken to elucidate this problem. A sample of hypothyroid patients (n = 15) with a median age of 76 years (range 61-92) underwent audiological examination before and after treatment with L-thyroxine (average 5 months; range 2-12), and re-examination (n = 13) after an observation period of average 40 months (range 32-46). No significant improvement in their hearing thresholds, speech reception thresholds or discrimination scores could be demonstrated, and the hearing ability in the hypothyroid patients did not differ significantly from that found in an age- and sex-matched population. In addition, histological investigation of the temporal bones from an 83-year-old woman with myxoedema showed no morphological changes or deposition of glycosaminoglycans. Audiological examinations in patients with insulin-dependent diabetes mellitus also show contradictory results. Therefore, the cochlear and retrocochlear hearing functions were evaluated in 20 patients with diabetic microangiopathy with a median age of 41 years (range 25-66), and in 19 patients without microangiopathy with a median age of 27 years (range 17-42). No significant differences in hearing thresholds or discrimination scores were present between the two diabetic groups, nor was any difference found between the diabetic patients and an age- and sex-matched population. In the patients with long-term insulin-dependent diabetes mellitus, brainstem audiometry revealed abnormal responses in 40%, indicating the presence of diabetic encephalopathy.
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PMID:Hearing problems and hormonal disturbances in the elderly. 208 79

A case of mitochondrial encephalomyopathy (Kearns-Sayre syndrome) with corneal endothelial abnormality is reported. A 22-year-old woman had retinitis pigmentosa, external ophthalmoplegia, complete heart block, ataxia, muscle weakness, dementia, sensorineural hearing loss, and was of short stature. Renal dysfunction, diabetes mellitus, and amenorrhea were also observed. Biopsy revealed decreased cytochrome c oxidase (complex IV) activity in muscle mitochondria. The corneal endothelium examined by specular microscope showed decreased cell density, severe polymegathism, and pleomorphism in both eyes. To our knowledge, this is the first report concerning primary corneal endothelial abnormality in a case with mitochondrial encephalomyopathy. The corneal endothelium is one of the tissues that could be affected by the enzyme deficiency present in this disease.
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PMID:Corneal endothelium in a case of mitochondrial encephalomyopathy (Kearns-Sayre syndrome). 274 82


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