The effect of right versus left long-term single-sided deafness on sound source localization

Purpose: To analyze the effect of right versus left long-term single-sided deafness(SSD) on sound source localization(SSL), discuss the necessity of intervention and treatment for SSD patients, and analyze the therapeutic effect of long-term unilateral cochlear implantation(UCI) from the perspective of SSL.Methods: This study included 25 patients with SSD, 11 patients with UCI, and 30 participants with normal hearing(NH). Their SSL ability was tested by obtaining their average root mean square(RMS) error values of SSL test.Results: The results showed that the RMS error value of SSD, UCI and NH groups were 52.26 ± 20.25°, 69.84 ±12.14° and 4.27 ± 2.66°, respectively. The ability of SSL was better in the SSD-L group than that in the SSD-R group, and no significant difference existed in the SSD-R and the UCI group.Conclusion: When bilateral deafness patients select unilateral treatment, right-side cochlear implantation may be more beneficial in terms of SSL, which means that the central auditory cortex in long-term SSD patients is affected differently based on which side their deafness occurs.

Effects of Yigan Capsule on the expression of HMGB1,RAGE and NF-κB protein in rats with drug-induced liver injury

Objective:To study the effect of Yigan capsule on the expression of high mobility group protein B1(HMGB1),nuclear factor-B(NF-κB)and receptor for advanced glycation end products(RAGE)in anti-tuberculosis drug-induced liver injury(ATB-DILI),and to explore its protective effect and mechanism on ATB-DILI,so as to provide experimental basis for the clinical application of Yigan capsule.Methods:Twenty-four rats were divided into two groups.Except for the blank group(n=6),the other 18 rats were given isoniazid(INH)+rifampicin(RFP)(50 mg/kg.d)for 4 weeks.Then 18 rats were randomly divided into three groups(model group,low dose group of Yigan capsule and high dose group of Yigan capsule)according to 6 rats in each group.The blank group and the model group were given 0.9%sodium chloride solution by intragastric administration.The low dose group of Yigan capsule was 0.468 g/kg,and the high dose group of Yigan capsule was 1.872 g/kg[1].After 4 weeks,the pathological changes of liver were observed by HE staining.The contents of ALT,AST,ALP,γ-GT and TBIL were detected.The expression of HMGB1,NF-κBp65 and RAGE protein was detected by IHC.The expression levels of HMGB1,NF-κBp65,RAGE,TNF-αand IL-1βwere detected by WB.Result:HE staining showed that the structure of the liver in the model group was disordered,the liver cells showed swelling and fusion,the number of inflammatory cells increased and accompanied by punctate necrosis,while the above pathological changes in each treatment group of Yigan capsule were significantly improved.The contents of ALT,AST,ALP,γ-GT and TBIL in the model group were higher than those in the blank group(P<0.05).The contents of ALT,AST,ALP,γ-GT and TBIL in each treatment group were significantly lower than those in the model group(P<0.05).Compared with the blank group,the expression levels of TNF-αand IL-1βin the model group were increased(P<0.05),and the expression levels of HMGB1,NF-κBp65 and RAGE were increased(P<0.05).Compared with the model group,the expression levels of TNF-αand IL-1βin each treatment group of Yigan capsule decreased(P<0.05),and the expression of HMGB1,NF-κBp65 and RAGE decreased(P<0.05).Conclusion:Yigan capsule may inhibit the secretion of inflammatory factors through HMGB1/RAGE/NF-κBp65 signaling pathway,thus protecting ATB-DILI.

Association of Cytokines with Clinical Indicators in Patients with Drug-Induced Liver Injury

Objective To explore characteristics of clinical parameters and cytokines in patients with drug-induced liver injury(DILI)caused by different drugs and their correlation with clinical indicators.Method The study was conducted on patients who were up to Review of Uncertainties in Confidence Assessment for Medical Tests(RUCAM)scoring criteria and clinically diagnosed with DILI.Based on Chinese herbal medicine,cardiovascular drugs,non-steroidal anti-inflammatory drugs(NSAIDs),antiinfective drugs,and other drugs,patients were divided into five groups.Cytokines were measured by Luminex technology.Baseline characteristics of clinical biochemical indicators and cytokines in DILI patients and their correlation were analyzed.Results 73 patients were enrolled.Age among five groups was statistically different(P=0.032).Alanine aminotransferase(ALT)(P=0.033)and aspartate aminotransferase(AST)(P=0.007)in NSAIDs group were higher than those in chinese herbal medicine group.Interleukin-6(IL-6)and tumor necrosis factor alpha(TNF-α)in patients with Chinese herbal medicine(IL-6:P<0.001;TNF-α:P<0.001)and cardiovascular medicine(IL-6:P=0.020;TNF-α:P=0.001)were lower than those in NSAIDs group.There was a positive correlation between ALT(r=0.697,P=0.025),AST(r=0.721,P=0.019),and IL-6 in NSAIDs group.Conclusion Older age may be more prone to DILI.Patients with NSAIDs have more severe liver damage in early stages of DILI,TNF-αand IL-6 may partake the inflammatory process of DILI.

APPLICATION OF GENETIC DEAFNESS GENE CHIP FOR DETECTION OF GENE MUTATION OF DEAFNESS IN PREGNANT WOMEN

Objective The study is to identify the carrier rate of common deafness mutation in Chinese pregnant women via detecting deafness gene mutations with gene chip. Methods The pregnant women in obstetric clinic without hearing impairment and hearing disorders family history were selected. The informed consent was signed. Peripheral blood was taken to extract genom- ic DNA. Application of genetic deafness gene chip for detecting 9 mutational hot spot of the most common 4 Chinese deafness genes, namely GJB2 （35delG, 176del16bp, 235delC, 299delAT）, GJB3 （C538T） ,SLC26A4 （ IVS72A〉G, A2168G） and mito- chondrial DNA 12S rRNA （A1555G, C1494T） . Further genetic testing were provided to the spouses and newborns of the screened carriers. Results Peripheral blood of 430 pregnant women were detected, detection of deafness gene mutation carri- ers in 24 cases（4.2%）, including 13 cases of the GJB2 heterozygous mutation, 3 cases of SLC26A4 heterozygous mutation, 1 cases of GJB3 heterozygous mutation, and 1 case of mitochondrial 12S rRNA mutation. 18 spouses and 17 newborns took further genetic tests, and 6 newborns inherited the mutation from their mother. Conclusion The common deafness genes muta- tion has a high carrier rate in pregnant women group, 235delC and IVS7-2A〉G heterozygous mutations are common.

HUMAN MITOCHONDRIAL tRNA MUTATIONS IN MATERNALLY INHERITED DEAFNESS

Mutations in mitochondrial tRNA genes have been shown to be associated with maternally inherited syn-dromic and non-syndromic deafness. Among those, mutations such as tRNALeu(UUR) 3243A>G associated with syndromic deafness are often present in heteroplasmy, and the non-syndromic deafness-associated tRNA mu-tations including tRNASer(UCN) 7445A>G are often in homoplasmy or in high levels of heteroplasmy. These tRNA mutations are the primary factors underlying the development of hearing loss. However, other tRNA mutations such as tRNAThr 15927G>A and tRNASer(UCN) 7444G>A are insufficient to produce a deafness phe-notype, but always act in synergy with the primary mitochondrial DNA mutations, and can modulate their phenotypic manifestation. These tRNA mutations may alter the structure and function of the corresponding mitochondrial tRNAs and cause failures in tRNAs metabolism. Thereby, the impairment of mitochondrial protein synthesis and subsequent defects in respiration caused by these tRNA mutations, results in mitochon-drial dysfunctions and eventually leads to the development of hearing loss. Here, we summarized the deaf-ness-associated mitochondrial tRNA mutations and discussed the pathophysiology of these mitochondrial tRNA mutations, and we hope these data will provide a foundation for the early diagnosis, management, and treatment of maternally inherited deafness.

A screening analysis of the GJB2 c.176 del 16 mutation responsible for hereditary deafness in a Chinese family

Objective:To determine whether a new-born child from a family carrying a deafness gene needs cochlear implantation to avoid dysphonia by screening and sequencing a deafness-related gene.Results:Both screening and sequencing results confirmed that the new born child had a normal GJB2 gene despite the fact that she has a brother suffering from hearing loss triggered by an allelic GJB2 c.176 del 16 mutation.We cloned the GJB2 genes derived from their respective blood genomic DNA into GFP fused plasmids and transfected those plasmids into the 293 T cell line to test for gene function.While the mutated GJB2gene(GJB2 c.176 del 16) of her deaf brother was found to be unable to form the gap junction structure between two adjacent cells,the baby girl’s GJB2 gene ran into no such problems.Conclusion:The screening and sequencing as well as the GJB2 gene function tests invariably showed results consistent with the ABR tested hearing phenotype,which means that the child,with a normal wild type GJB2 gene,does not need early intervention to prevent her from developing hearing loss and dysphonia at a later stage in life.

Efficacy and safety of rabeprazole in non-steroidal anti-inflammatory drug-induced ulcer in Japan

AIM: To investigate the effi cacy and safety of rabepra-zole under continuous non-steroidal anti-inflammatory drug (NSAID) administration for NSAID-induced ulcer in Japan. METHODS: Subjects comprised patients undergoing NSAID treatment in whom upper gastrointestinal en-doscopy revealed an ulcerous lesion (open ulcer) with diameter ≥ 3 mm, who required continuous NSAID treatment. Endoscopies were performed at the start of treatment, during the treatment period, and at the conclusion (or discontinuation) of treatment. Findings were evaluated as size (maximum diameter) and stage based on the Sakita-Miwa classifi cation. An ulcer was regarded as cured when the "white coating" was seen to have disappeared under endoscopy. As criteria for evaluating safety, all medically untoward symptoms and signs (adverse events, laboratory abnormalities, accidental symptoms, etc.) occurring after the start of rabeprazole treatment were handled as adverse events.RESULTS: Endoscopic cure rate in 38 patients in the efficacy analysis (endoscopic evaluation) was 71.1% (27/38). Among those 38 patients, 35 had gastric ulcer with a cure rate of 71.4% (25/35), and 3 had duodenal ulcer with a cure rate of 66.7% (2/3). Three adverse drug reactions were reported from 64 patients in the safety analysis (interstitial pneumonia, low white blood cell count and pruritus); thus, the incidence rate for adverse drug reactions was 4.7% (3/64).CONCLUSION: The treatment efficacy of rabeprazole for NSAID-induced ulcer under continuous NSAID ad-ministration was confi rmed.

Outcomes of treatment of sudden deafness using different Protocols:a retrospective analysis of 104 cases

Objective To compare different treatment protocols for sudden deafness(SD), for the purpose of identifying an appropriate approach to SD. Methods A total of 104 patients with diagnosis of sudden hearing loss treated from Jan 2006 to December 2008 were included in this study, of which 31 received the typical pharmaceutical treatment (groupⅠ), 40 received the typical pharmaceutical treatment plus polarized liquid (GroupⅡ) and 33 received the hyperbaric oxygen in addition to the treatment included in Group Ⅱ(Group Ⅲ). Results The total improvement rate (67.74%, 62.50% and 75.76% for Groups Ⅰ, Ⅱ and Ⅲ respectively) was not statistically different between the three groups (P > 0.05). Conclusion The three treatment protocols are similar when judged by the treatment outcomes in SD, neither being superior to the others. The two important factors that appear to influence treatment outcomes are the audiogram pattern and duration of hearing loss before seeking treatment. Patients with upsloping or peak-type audiograms and treated within 7 days from the onset have better prognosis than others.

Concurrent symptoms and disease conditions in sudden deafness

Objective To study concomitant symptoms and disease conditions in sudden deafness. Methods Clinical data of 418 cases of sudden deafness treated in this department from 2000 to 2007 were reviewed. Results Of the 418 cases, 201 were males and 217 were females. Right ear was involved in 184 cases and left ear in 191 cases. Bilateral involvement was seen in 43 cases. The average age was 44.1 years. Tinnitus was reported in 369 cases (88.3%) either before or after hearing loss, of which 64.5% was of low pitch, 27.1% of high pitch and 8.4% of mixed tones. Constant tinnitus was reported in 83% of the cases, and muffled feelings in 33.3% of the cases. Hearing loss was the only complaint in 221 cases (52.9%). Dizziness was reported in 77 cases (18.4%) and vertigo attacks in 120 cases (28%). Hypertension, coronary artery disease and diabetes were found in 19.6% of 418 cases and hyperlipidemia in 54.5% of 211 cases. CT and / or MRI data were available in 147 cases, with positive findings in 18 cases (12.3%): 2 with acoustic neuroma (1.36%); 4 with emphraxis in the basal ganglia, cerebellum, temporal lobe or parietal lobe, and 12 with poor pneumatization of ipsior contralateral mastoid cells. Conclusion In this case series of sudden deafness, low-pitch constant tinnitus was a common complaint. Most of the studied cases presented with simple hearing loss. Vertigo attacks were more common than dizziness in this group of patients. The most common concomitant disorder was hyperlipidemia, especially high triglycerides. Imaging studies are important in managing sudden deafness in ruling out acoustic neuroma and other intracranial diseases.

Traditional Chinese Medicine Nursing Protocols for Sudden Deafness( Sudden Sensorineural Hearing Loss)

Sudden deafness is one of the commonest acute symptoms in otolaryngology.In recent years,the incidence of sudden deafness is on the rise all over the world,but its etiology is still unclear,and the prognosis is greatly different.Traditional Chinese medicine(TCM)believes that the onset of sudden deafness is related to the liver,gallbladder,heart and triple energizer meridians.This article mainly explored the key points of common syndromes,TCM nursing methods and health guidance of sudden deafness.

An efficient strategy for establishing a model of sensorineural deafness in rats

Ototoxic drugs can be used to produce a loss of cochlear hair cells to create animal models of deafness. However, to the best of our knowledge, there is no report on the establishment of a rat deafness model through the combined application of aminoglycosides and loop diuretics. The aim of this study was to use single or combined administration of furosemide and kanamycin sulfate to establish rat models of deafness. The rats received intravenous injections of different doses of furosemide and/or intramuscular injections of kanamycin sulfate. The auditory brainstem response was measured to determine the hearing threshold after drug application. Immunocytochemistry and confocal microscopy were performed to evaluate inner ear morphology. In the group receiving combined administration of furosemide and kanamycin, the auditory brainstem response threshold showed significant elevation 3 days after administration, higher than that produced by furosemide or kanamycin alone. The hair cells showed varying degrees of injury, from the apical turn to the basal turn of the cochlea and from the outer hair cells to the inner hair cells. The spiral ganglion cells maintained a normal morphology during the first week after the hair cells completely disappeared, and then gradually degenerated. After 2 months, the majority of spiral ganglion cells disappeared, but a few remained. These findings demonstrate that the combined administration of furosemide and kanamycin has a synergistic ototoxic effect, and that these drugs can produce hair cell loss and hearing loss in rats. These findings suggest that even in patients with severe deafness, electronic cochlear implants may partially restore hearing.

Sudden deafness as a prodrome of cerebellar artery infarction:Three case reports

BACKGROUND To summarize the clinical characteristics of acute cerebral infarction(ACI)in patients with sudden deafness(SD)as the first symptom,improve the awareness of the disease,and help diagnosis and treatment.CASE SUMMARY From 2019 to 2020,three patients with ACI with SD as the first symptom were admitted to our hospital.Pure tone audiometry,head magnetic resonance imaging(MRI),vertebral artery and carotid artery B-ultrasound,head and neck computed tomography angiography,and other examinations were performed.Following the treatment of SD,hearing and dizziness were not significantly improved.Then,the patients developed symptoms of related cranial nerve injury,and brain MRI showed cerebral infarction in the cerebellopontine angle area.All three cases were transferred to the neurology department for relevant conservative treatment.CONCLUSION Patients with ACI with SD as the first symptom usually attend the otolaryngology clinic.Here a diagnosis of SD,which is based on an audiological examination,is made and the corresponding treatment is administered.To reduce the misdiagnosis of this disease,close attention should be paid to the changes in the patient's clinical symptoms and related auxiliary examinations should be performed,such as brain MRI and cerebrovascular imaging.Otolaryngologists should pay attention to the type and severity of hearing loss,the accompanying symptoms,age,high-risk factors for cerebral infarction,and related cranial nerve symptoms in patients with SD.If the patient's early brain MRI does not show abnormalities,monitoring remains essential.The head MRI should be analyzed quickly based on the changes in the symptoms of the patient,to make an accurate diagnosis and provide the timely and correct treatment for the patients.

Profound deafness and the acquisition of spoken language in children

Profound congenital sensorineural hearing loss(SNHL) is not so infrequent,affecting 1 to 2 of every 1000 newborns in western countries.Nevertheless,universal hearing screening programs have not been widely applied,although such programs are already established for metabolic diseases.The acquisition of spoken language is a time-dependent process,and some form linguistic input should be present before the first 6 mo of life for a child to become linguistically competent.Therefore,profoundly deaf children should be detected early,and referred timely for the process of auditory rehabilitation to be initiated.Hearing assessment methods should reflect the behavioural audiogram in an accurate manner.Additional disabilities also need to be taken into account.Profound congenital SNHL is managed by a multidisciplinary team.Affected infants should be bilaterally fitted with hearing aids,no later than 3 mo after birth.They should be monitored until the first year of age.If they are not progressing linguistically,cochlear implantation can be considered after thorough preoperative assessment.Prelingually deaf children develop significant speech perception and production abilities,and speech intelligibility over time,following cochlear implantation.Age at intervention and oral communication,are the most important determi-nants of outcomes.Realistic parental expectations are also essential.Cochlear implant programs deserve the strong support of community members,professional bodies,and political authorities in order to be successful,and maximize the future earnings of pediatric cochlear implantation for human societies.

The Effect of a High-Frequency-Hearing-Threshold Weighted Value on the Diagnosis of Occupational-Noise-Induced Deafness

Objective: In order to provide a theoretical basis for the revision of the current diagnostic criteria for occupational noise-induced deafness (ONID), we evaluated the degree of ONID by analyzing different high-frequency-hearing- threshold-weighted values (HFTWVs). Methods: A retrospective study was conducted to evaluate the diagnosis of patients with ONID from January 2016 to January 2017 in Guangdong province, China. Based on 3 hearing tests (each interval between the tests was greater than 3 days), the minimum threshold value of each frequency was obtained using the 2007 edition’s diagnostic criteria for ONID. The speech frequency and the HFTWVs were analyzed based on age, noise exposure, and diagnostic classi-fication using SPSS21.0. Results: 168 patients in total were involved in this study, 154 males and 14 females, and the average age was 41.18 ± 6.07. The diagnosis rate was increased by the weighted value of the high frequencies and was more than the mean value of the pure speech frequency (MVPSF). The diagnosis rate for the weighted 4 kHz frequency level increased by 13.69% (χ2 = 9.880, P = 0.002), the weighted 6 kHz level increased by 15.47% (χ2 = 9.985, P = 0.002), and the weighted 4 kHz + 6 kHz level increased by 15.47% (χ2 = 9.985, P = 0.002). The differences were all statistically significant. The diagnostic rate of the different thresholds showed no obvious difference between the genders. The age groups were divided into less than or equal to 40 years old (group A) and 40 - 50 years old (group B). There were several groups with a high frequency: high frequency weighted 4 kHz ( group A χ2 = 3.380, P = 0.050;group B χ2 = 4.054, P = 0.032), high frequency weighted 6 kHz (group A χ2 = 6.362, P = 0.012;group B χ2 = 4.054, P = 0.032), weighted 4 kHz + 6 kHz (group A χ2 = 6.362 P = 0.012;B χ2 = 4.054, P = 0.032) than those of MVPSF in the same group on ONID diagnosis rate. The differences between the groups were statistically significant. There was no significant difference between the age groups (χ2 = 2.265, P = 0.944). The better ear’s (the smaller hearing threshold weighted value) MVPSF and the weighted values for the different high frequencies were examined in light of the number of working years;the group that was exposed to noise for more than 10 years had significantly higher values than those of the average thresholds of each frequency band in the groups with 3 - 5 years of exposure (F = 2.271, P = 0.001) and 6 - 10 years of exposure (F = 1.563, P = 0.046). The differences were statistically significant. The different HFTWVs were higher than those of the MVPSF values, and the high frequency weighted 4 kHz + 6 kHz level showed the greatest difference, with an average increase of 2.83 dB. The diagnostic rate that included the weighted high frequency values was higher for the mild, moderate, and severe cases than those patients who were only screened with the pure frequency tests. The results of the comparisons of the diagnosis rates for mild ONID were as follows: the weighted 3 kHz high frequency level (χ2 = 3.117, P = 0.077) had no significant difference, but the weighted 4 kHz level (χ2 = 10.835, P = 0.001), 6 kHz level (χ2 = 9.985, P = 0.002), 3 kHz + 4 kHz level (χ2 = 6.315, P = 0.012), 3 kHz + 6 kHz level (χ2 = 6.315, P = 0.012), 4 kHz + 6 kHz level (χ2 = 9.985, P = 0.002), and 3 kHz + 4 kHz + 6 kHz level (χ2 = 7.667, P = 0.002) were significantly higher than the diagnosis rate of the mean value of the PSF. There was no significant difference between the 2 groups for the moderate and severe grades (P > 0.05). Conclusion: Different HFTWVs increase the diagnostic rate of ONID. The weighted 4 kHz, 6 kHz, and 4 kHz + 6 kHz high frequency values greatly affected the diagnostic results, and the weighted 4 kHz + 6 kHz high frequency hearing threshold value has the maximum the effect on the ONID diagnosis results.

Experimental study of transient cochlear ischemia as a cause of sudden deafness

The etiology of sudden deafness or idiopathic sudden sensorineural hearing loss(ISSHL) remains unclear. Over the past 15 years, we have investigated the mechanisms of ischemic-induced hearing loss using a gerbil model of transient cochlear ischemia. In the gerbil, cochlear ischemia can be induced by occluding the bilateral vertebral arteries simultaneously at the neck, because the posterior communicating arteries of the Circle of Willis close spontaneously around 1 mo after birth. When 15 min ischemia was loaded on this animal, permanent hearing loss of about 25 d B and the death of hair cells, especially inner hair cells were induced. These pathological changes were mainly due to lack of an energy source, glutamate excitotoxicity, and the production of free radicals, especially superoxide and nitrous oxide species. Ischemic damage could be prevented by various procedures, such as cooling the cochlea, intratympanic administration of insulin-like growth factor 1 or AM-111(an anti-apoptotic agent), and systemic administration of prednisolone(steroid), edarabone(free radical scavenger), ginsenoside Rb1(Kanpo), hematopoietic stem cells, glia-cell derived neurotrophic factor, and liposome-encapsulated hemoglobin(artificial red blood cells). We also found that the cochlea was protected by the ischemic tolerance, indi-cating that minor cochlear ischemia alleviates or prevents inner ear damage in subsequent severe cochlear ischemia. As ISSHL usually occurs suddenly, with no preceding sign or symptom, we suggest that most ISSHL cases are caused by circulatory disturbance, probably at the stria vascularis.

Child Deafness in Sub-Saharan Africa: Experience of Two ENT Services in Casamance, South of Senegal

Introduction: Hearing impairment is the most common sensory deficit at birth. It is a public health problem because of the repercussions on the communication development, on the education and subsequent social integration of the child. The objective of this study was to determine the epidemiological, clinical, audiometric and etiological profiles of child deafness in Casamance, South of Senegal. Materials and Methods: This was a retrospective multicenter study, which extended a period of 7 years from January 1st, 2012 to December 31st, 2019. All children aged between 1 to 18 years old and received during their first ENT consultations at the regional and PEACE hospital in Ziguinchor were included. Results: One hundred and seventy-eight records of children were collected during this period, that is a prevalence of 1.30%. The average age was 9 years old. Conductive hearing loss was found in 68% of patients, followed by reception hearing deafness in 24%. The deafness was acquired in the majority of cases (93%) and the predominant etiology was infectious. However, the cause was unknown in 7.51% of cases. Conclusion: Child deafness is common in Casamance and is most often underdiagnosed. The acquired forms are the most common, hence the importance of early detection after a rigorous family investigation.

Audiometric Profile of Deafness at the University Hospital Center Gabriel Toure of Bamako

Objective: To determine the audiometric profile of deafness in our practice. Materials and Methods: Longitudinal prospective study was conducted out in the ENT department and cervicofacial surgery of Gabriel Toure University Hospital in Bamako, we made an exhaustive sampling of all the patients who consulted for hearing loss, tinnitus, vertigo, hiring report, medical expertise and whose deafness was confirmed to tonal audiometry with an age greater than or equal to 15 years. It was spread over 10 months (June 2016 to March 2017). A total of 200 patients were collected. Exclusion criteria were all patients under 15 years of age as well as deafness related to earwax or foreign bodies, and refusal to participate in the study Results: During our study period, 6055 outpatients were admitted out of which 734 patients underwent audiometric testing and 200 patients (3.30%) met our criteria. The male sex was the most represented with a rate of 60%. The sex ratio was 1.5 or 3 men for 2 women. The 15 to 25 age group was the most represented at 37.5%. The average age was 37.18 years old with extremes ranging from 15 to 83 years old. Pupils/students were the most represented with a rate of 29.5%, followed by housewives in 23%, farmers in 10.5% and military in 10%. As antecedent 26% of our patients had a chronic otitis media (OMC), against 23% who had no antecedent otological and 17% had a traumatic antecedent. Bilateral deafness was the most common with a rate of 64.5%. The mode of progressive appearance was the most frequent in 74.5%. As functional signs 46.22% of our patients had a hearing loss associated with tinnitus. Otoscopic examination was pathological in 34.5% of our patients. Mixed deafness was the most common in 43.35%, followed by perception deafness in 32.19% and transmission deafness 24.46%. Mean deafness was the most common with a rate of 48.91%, was severe in 20.22%, mild in 18.31%, deep in 11.47% and cophotic in 01.09%. Asymmetrical curves were the most found in 65.89% of our patients. Conclusion: Deafness is a sensory disability responsible for communication disorder, sometimes disabling. Audiometry, although subjective, remains essential in the diagnosis of deafness.

Acute liver failure caused by drug-induced hypersensitivity syndrome associated with hyperferritinemia

Drug-induced hypersensitivity syndrome(DIHS) is a severe reaction usually characterized by fever,rash,and multiorgan failure,occurring 2-6 wk after drug introduction.It is an immune-mediated reaction involving macrophage and T-lymphocyte activation and cytokine release.A 54-year-old woman was diagnosed with rheumatic arthritis and initiated salazosulfapyridine by mouth.About 10 d later,she had a high fever,skin rash and liver dysfunction.She was admitted to hospital and diagnosed with a drug eruption.She was treated with oral prednisolone 30 mg/d;however,she developed high fever again and her blood tests showed acute liver failure and cytopenia associated with hyperferritinemia.She was diagnosed with acute liver failure and hemophagocytosis caused by DIHS.She was transferred to the Department of Medicine and Bioregulatory Science,Kyushu University,where she was treated with arterial steroid injection therapy.Following this treatment,her liver function improved and serum ferritin immediately decreased.We hypothesized that an immune-mediated reaction in DIHS may have generated over-activation of macrophages and T-lymphocytes,followed by a cytokine storm that affected various organs.The measurement of serum ferritin might be a useful marker of the severity of DIHS.

Fine Mapping of a Deafness Mutation hml on Mouse Chromosome 10

Objective To map a mouse deafness gene, identify the underlying mutation and develop a mouse model for human deafness. Methods Genetic linkage cross and genome scan were used to map a novel mutation named hypoplasia of the membranous labyrinth (hml), which causes hearing loss in mutant mice. Results ① hml was mapped on mouse Chr 10 (～43 cM from the centromere) suggests that the homologous human gene is on 12q22-q24, which was defined on the basis of known mouse-human homologies (OMIM, 2004). ② This study has generated 25 polymorphic microsatellite markers, placed 3 known human genes in the correct order in a high-resolution mouse map and narrowed the hml candidate gene region to a 500 kb area.