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The use of hearing aids may not be sufficiently helpful for elderly people with advanced bilateral hearing loss when conversing with others. The patient in this case was an 82-year-old man whose main complaint was difficulty in verbal communication with his family despite using hearing aids. He was diagnosed with advanced bilateral sensorineural hearing loss using Western medicine techniques. He was first prescribed the Kampo formulation, ryokeijutsukanto, followed by goshajinkigan. Later, he took a combination of both of these Kampo formulations, and his hearing ability improved. Pure tone audiometry and speech audiometry demonstrated hearing loss ;however, speech audiometry better reflected his improvement in hearing speech sounds after he began taking the Kampo formulations. Thus, in this case, the patient's hearing and communication abilities improved with Kampo formulations combined with the use of hearing aids. In the future, speech audiometry (maximum discrimination score) can be applied to evaluate the efficacy of Kampo treatment for hearing loss.
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Objective To study the distribution of cochlear dead regions in the cochlea with sensorineural hearing loss (SNHL),and to investigate the effects of cochlear dead regions on speech recognition.Methods A total of 41 SNHL patients (81 ears) were divided into the cochlear dead region group (35 ears) and the group without cochlear dead regions (46 ears) by using threshold equalizing noise test (TEN test).Then we used speech recognition threshold (SRT) and speech discrimination score (SDS) tests to study the distribution of cochlear dead regions and to investigate the effects of cochlear dead regions on speech recognition.Results There were 41 cases (81 ears) sensorineural hearing loss patients and 43.21％ (35/81) were found to have the cochlear dead regions.The cochlear dead region detection rate for patients with mild SNHL was 0(0/11);in patients with moderate SNHL,the cochlear dead region detection rate was 24.1％ (7/29);in patients with severe SNHL the cochlear dead region detection rate was 66.7％ (24/36);the cochlear dead regions of profound SNHL patients were 80.0％ (4/5) respestively.The existence of the cochlear dead regions was significantly correlated with the degree of hearing loss (P＜0.05).The proportion of high frequency cochlear dead regions (16 ears)was much higher than that of the low frequency cochlear dead regions(8 ears).There was no significant reduction of SRT and SDS between high and low cochlear dead regions groups(P＞0.05).The SRT and SDS of the patients with cochlear dead regions were 61.63± 16.76 dB,86.35％±12.03％.The SRT and SDS of the patient with no cochlear dead regions were 75.54 ± 9.56 dB and 64.97％±20.84％.Theresults showed a significant (P＜0.05) reduction of SRT and SDS between the patient with cochlear dead regions and the patient with no cochlear dead regions.Conclusion The greater the degree of hearing loss is,the higher possibility of the existence of cochlear dead regions there is.Cochlear dead regions are common in high frequencies than in low frequencies.The speech recognition ability can be affected.
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Objective To investigate the clinical features,etiology,diagnosis and treatment of acute auditory agnosia.Methods We studied the clinical manifestation,diagnosis and treatment of acute auditory agnosia in a patient in our hospital.Results A 28 year oldyoung woman visited our department because she suffered from the tinnitus for 7 days and she could not distinguish the semantics for 1 day.There were no other abnormal symptoms in the central and peripheral nervous system on admission.Audiological testing showed normal,language testing showed that the speech discrimination score was zero.MRI showed extensive damage to temporal lope.MR spectroscopy revealed increased lactate and reduced N-acetyl aspartate.Acute auditory agnosia resulted from mitochondrial myopathy was considered.After symptomatic treatment,the symptoms were significantly improved.Molecular genetics examination showed the A3243G mtDNA mutation,further confirmed the diagnosis of mitochondrial encephalomyopathy with lactic acidosis and stroke like episodes (MELAS) syndrome.Conclusion Acute auditory agnosia and acute tinnitus can be the first symptoms in MELAS,thus,MELAS should be suspected in patients with acute auditory agnosia,acute tinnitus,sudden hearing loss in children and youth.Imaging examination plays an important role in the etiological diagnosis of acute auditory agnosia.
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Background & objectives: Wilson’s disease (WD) is an autosomal recessive disorder caused by mutations in ATP7B, a copper transporter gene, leading to hepatic and neuropsychiatric manifestations due to copper accumulation. If diagnosed early, WD patients can be managed by medicines reducing morbidity and mortality. Diagnosis of this disease requires a combination of tests and at times is inconclusive due to overlap of the symptoms with other disorders. Genetic testing is the preferred alternative in such cases particularly for individuals with a family history. Use of DNA microarray for detecting mutations in ATP7B gene is gaining popularity because of the advantages it offers in terms of throughput and sensitivity. This study attempts to establish the quality analysis procedures for microarray based diagnosis of Wilson’s disease. Methods: A home-made microarrayer was used to print oligonucleotide based low-density microarrays for addressing 62 mutations causing Wilson’s disease reported from Indian population. Inter- and intra- array comparisons were used to study quality of the arrays. The arrays were validated by using mutant samples generated by site directed mutagenesis. Results: The hybridization reaction were found to be consistent across the surface of a given microarray. Our results have shown that 52 °C post-hybridization wash yields better reproducibility across experiments compared to 42 °C. Our arrays have shown > 80 per cent sensitivity in detecting these 62 mutations. Interpretation & conclusions: The present results demonstrate the design and evaluation of a low-density microarray for the detection of 62 mutations in ATP7B gene, and show that a microarray based approach can be cost-effective for detecting a large number of mutations simultaneously. This study also provides information on some of the important parameters required for microarray based diagnosis of genetic disorders.