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Background: Nosocomial rhino sinusitis causes major problems in all Intensive Care Units (ICUs). Objective: To describe incidence, epidemiologic, clinical manifestations, and microbiologic findings in ICUs admitted cases with nosocomial sinusitis. Materials and Methods: A prospective, cross sectional study done in Pediatric & Adult ICUs in Rasoul Akram Hospital; Tehran Iran (2007-2008). Para-nasal sinus computed tomography (CT) was performed in all adults with fever of unknown origin (FUO) within 48h of admission and repeated thereafter (4-7 days). Infectious sinusitis was diagnosed by microbiological analysis of sinus fluid aspirates. Results: Acute bacterial nosocomial sinusitis proved in 82% (51/ 63) of all cases. Head trauma was the most common cause (n = 22, 45%) of cases. The results of culture were positive for 45 cases (82%). Of 45 culture positives, 19 yielded Gram negative organisms (41%) and 9 (22%) gave Gram positives (S. aureous, Streptococus spp). The remainders (n = 17, 37%) consisted of mixed aerobic/anaerobic bacteria. Seven cases, were positive in gram staining of sinus drainage and these were positive in culture for S. pneumonia (n = 5), Hemophilus inluenza (n = 2). The type of organisms were not related to Glasgow Coma Scale in cases (P = 0.3). Conclusion: Nosocomial organisms isolated were quite different from community acquired rhino sinusitis cases. Investigation of CT scan and drainage of Para-nasal sinuses would be helpful in undiagnosed FUO cases, especially in traumatic patients. Optimal treatment usually consists of removal of the tubes, mobilizing the patient, and administration the broad-spectrum antibiotics. [ABSTRACT FROM AUTHOR]

Objectives:To investigate the prevalence of mutations in the coding exon of the GJB2 gene in Iranian children with cochlear implants, and to compare the outcomes of auditory perception and speech production in cochlear-implanted children with and without GJB2 mutation.Materials and methods:One hundred and sixty-six prelingually deaf children who had undergone cochlear implantation at the Iranian Cochlear Implant Center, Tehran, were selected from a pool of 428 implanted children. The prevalence of GJB2 gene mutations was assessed using nested polymerase chain reaction and direct sequencing. To enable comparisons, we also identified 36 implanted children with non-GJB2 deafness. Patients' speech perception and speech production were assessed using the Categorization of Auditory Performance and Speech Intelligibility Rating scales.Results:Thirty-three of 166 probands (19.9 per cent) were found to have GJB2 deafness-causing allele variants and were diagnosed with DFNB1 deafness. Results also indicated a significant improvement in speech perception and production scores in both GJB2 and non-GJB2 patients over time.Conclusion:Children with GJB2-related deafness benefit from cochlear implantation to the same extent as those with non-GJB2-related deafness. [ABSTRACT FROM PUBLISHER]

Objective: Sensorineural hearing loss (SNHL) can follow congenital toxoplasmosis. Treatment in the first year of life is associated with diminished occurrence of this sequel. In various parts of Iran, the prevalence of antibodies to Toxoplasma gondii ranges from 24% to 57.7%. We evaluate the possible role of Toxoplasma gondii infection on the occurrence of SNHL in children. Design and setting: This case-control study was performed in a tertiary care center in Tehran between 2002 and 2003. This study was carried out based on diagnostic parameters of the American Academy of Otolaryngology criteria for SNHL and a healthy control group. Main outcome measures: We compared the specific Toxoplasma gondii antibodies (IgM & IgG ) measured by ELISA in 95 blood samples of infants with SNHL and 63 healthy matched infants. Results: Acute (IgM) and previous (IgG) immunity to Toxoplasma gondii was found in 12 and 21.2% of SNHL children, respectively. Most cases with previous infections (IgG positive) were children aged less than 1 year old (i.e. maternal immunity), but acute infection (IgM positive) was higher in 3–5 year old age group. Acute infection (IgM) was significantly more frequent in the SNHL group, and previous immunity was higher in the controls (CI 95%, P-value = 0.01; 0.01). Conclusion: With respect to seropositive children, as we were unable to differentiate congenital from acquired cases, we recommend prevention of congenital toxoplasmosis by treatment of Toxoplasma infection in pregnant women and treatment of acquired Toxoplasma gondii infection after birth to minimise the risk of SNHL in children. [ABSTRACT FROM AUTHOR]

The prevalence of pressure ulcers of the foot is a major health care problem in frail elderly patients. A pressure sore dramatically increases the cost of medical and nursing care, and effective treatment has always been an essential nursing concern. Management options for pressure ulcers include local wound care; surgical repair and, more recently, topical application of growth factors. The main goal of this study was to examine the effects of intravenous treatment of Semelil (ANGIPARS™), a new herbal extract in patients with severe, noninfected pressure ulcers of the foot. As a randomized clinical trial, 18 patients with pressure ulcers were recruited from Vali-e- Asr hospital, Medical Sciences/ University of Tehran, Iran. Nine patients received intravenous Semelil (ANGIPARS™) besides to conventional therapy and nine received only conventional treatment. At the baseline, the treatment and control groups did not differ across demographic variables, clinical characteristics, and functional measures. The mean surface areas of the ulcers were reduced 43.2 ± 57.4 cm&sup 2; (80.3%) and 2.8 ± 6.2 cm&sup 2; (6.3%) in the treatment and control groups, respectively (p=0.000). The average reduction in pressure ulcer area at four weeks was statistically and clinically greater in the treatment group than in the control group So, intravenous Semelil (ANGIPARS™) can be recommended as an effective treatment for patients with severe pressure ulcers. [ABSTRACT FROM AUTHOR]

Pressure ulcers are one of the major health care problems and results in a substantial amount of burden for both patients and health services. The aim of this study was to appraise effectiveness of topical Semelil (ANGIPARS™), a naive herbal extract, in pressure ulcers As a randomized controlled clinical trial, 18 patients with pressure ulcers were recruited from Vali-e-Asr hospital, Medical Sciences/ University of Tehran, Iran. Nine patients received topical Semelil (ANGIPARS™) during hospitalization and nine other patients received conventional treatment. Baseline characteristics of the topical and control groups did not differ across demographic, clinical and functional measures. The mean surface areas of the ulcers were reduced 48.2 a- 85.3 cm&sup 2; (78.3%) and 2.8± 6.2 cm&sup 2; (6.3%) in the treatment and control groups, respectively (p=0.000). From the results of this study it may be concluded that the use of topical Semelil (ANGIPARS™) with conventional treatment is more effective than those of only conventional treatment for patients with pressure ulcers. [ABSTRACT FROM AUTHOR]

Background: Burns is one of the most important causes of death, and the best way to reduce mortality due to burns is prevention., Aims: To investigate the factors responsible for mortality due to burns in south-western Islamic Republic of Iran., Methods: This was a cross-sectional study of 400 burns patients admitted to different wards of Ayatollah Taleghani Trauma and Burns Hospital, Ahvaz, Islamic Republic of Iran, from October 2020 to September 2021. Logistic regression was used to determine the factors responsible for mortality., Results: The mean age of the patients was 28.47 (19.09) years, and 252 (63.0%) patients were male. There were 257 survivors and 143 deaths (35.75% mortality rate). The multiple logistic regression model showed that age, sex, percentage of total body surface area, burn depth, length of hospital stay, and length of intensive care unit stay were significantly associated with deaths due to burns., Conclusion: The mortality rate due to burns was high at the Ayatollah Taleghani Trauma and Burns Hospital, Islamic Republic of Iran. Improving the quality of care provided to burns patients at health facilities can help reduce the current high mortality rate., (Copyright © Authors 2023; Licensee: World Health Organization. EMHJ is an open access journal. This paper is available under the Creative Commons Attribution Non-Commercial ShareAlike 3.0 IGO licence (CC BY-NC-SA 3.0 IGO; https://creativecommons.org/licenses/by-nc-sa/3.0/igo).)

This study aimed to evaluate bone mineral density (BMD), trabecular microarchitecture, and proximal hip geometry in diabetic postmenopausal women, where BMD alone cannot reflect bone strength adequately. We found significantly lower trabecular bone score and BMD at the distal radius and total forearm in diabetic subjects compared to controls., Purpose: The limitations resulting from the exclusive assessment of bone mineral density (BMD) in people with diabetes can lead to underestimation of microarchitectural and geometric changes, both of which play an essential role in the fracture risk. Therefore, we aimed to evaluate BMD, trabecular bone score (TBS), and hip structural analysis (HSA) in diabetic type-2 post-menopausal women and compare them with healthy postmenopausal subjects., Methods: BMD was assessed at the lumbar spine, femoral sites, distal radius, and total forearm using dual-energy X-ray absorptiometry (DXA); TBS was measured based on DXA images using the software at the same region of interest as the BMD measurements; geometric assessment at the proximal femur was performed by the HSA program., Results: A total of 348 ambulatory type-2 diabetic postmenopausal women and 539 healthy postmenopausal women were enrolled. TBS and BMD at the distal radius and total forearm were significantly (P value < 0.05) lower in cases compared to controls after age and body mass index (BMI) adjustment. In addition, degraded bone microarchitecture was significantly (P value < 0.05) more prevalent in diabetic subjects than in non-diabetic controls after adjusting for age and BMI. A number of geometric indices of the proximal hip were significantly lower in the controls than in those with diabetes (P-value < 0.05)., Conclusion: This study may highlight the utility of the TBS and BMD at the distal radius and total forearm in subjects with type-2 diabetes mellitus, where the BMD at central sites may not adequately predict fracture risk., (© 2023. International Osteoporosis Foundation and Bone Health and Osteoporosis Foundation.)

In the indoor environment of dental clinics, dental personnel and patients are exposed to a risk of infection because of the transmission of SARS-CoV-2 via particles or droplets. This study investigated the presence of SARS-CoV-2 RNA in indoor air of dental clinics in Tehran, Iran. Air sampling was done (n = 36) collecting particulate samples on PTFE filters at flow rates of 30 to 58 L/min. The samples were analyzed with novel coronavirus nucleic acid diagnostic real-time PCR kits. Only 13 out of 36 samples were positive for SARS-CoV-2 RNA. Logistic regression showed that sampling site's volume, PM 2.5 concentration, number of people, and number of active patient treatment units were significantly positively related with the presence of SARS-CoV-2 RNA. Thus, strategies to control the spread of COVID-19 should include reducing the number of infected people in dental clinics, adding filtration systems, and/or improving ventilation conditions., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Mutations in NARS2 (MIM: 612803) are associated with combined oxidative phosphorylation deficiency 24 (COXPD24; MIM: 616239) that is a rare mitochondrial and a multisystem autosomal recessive disorder., Aims: We aimed to detect the underlying genetic factors in two siblings with progressive ataxia, epilepsy, and severe-to-profound hearing impairment., Methods: After doing medical assessments and pertinent tests (i.e., auditory brainstem responses, pure tone otoacoustic emission test, cardiac examinations, computed tomography, and electroencephalogram), because of the clinical and probable genetic heterogeneity, whole-exome sequencing was performed, and co-segregation analysis was confirmed by Sanger sequencing. Biological impacts of the novel variant were evaluated using sequence-to-function bioinformatics tools., Results: A novel homozygous missense variant, NM&#95;024678.6:c.545 T > A; p.(Ile182Lys), in exon 5 of NARS2 was identified in both patients and verified by Sanger sequencing. In silico analyses introduced this variant as pathogenic. Mitral valve prolapses with mild regurgitation, brachymetatarsia, severe hallux valgus, and clubbed fingers were reported as novel manifestations in association with NARS2 gene. By doing a literature review, we also underscored the high heterogeneity of disease phenotype., Conclusions: Herein, we report some novel phenotype and genotype features of two female patients in an Iranian consanguineous family with COXPD24, caused by a variant in NARS2-NM&#95;024678.6: c.545 T > A; p.(Ile182Lys). Moreover, our data expanded the phenotype and genotype spectrum of NARS2-related disorder and confirmed an unpredictable nature of genotype-phenotype correlation in COXPD24., (© 2021. Royal Academy of Medicine in Ireland.)

Background: Transmembrane inner ear (TMIE) protein is an essential component of the mechanotransduction complex. In collaboration with other components, TMIE aids the maintenance and function of the sensory hair cells. Autosomal recessive deafness-6 (DFNB6) is caused by mutated TMIE, a gene in the high genetic heterogeneity spectrum of deafness. Hearing loss has a significant impact on the global economy and the quality of life of affected persons, their families, and society. Here, three unrelated families with TMIE variants are presented. All three cases were found while studying the genetic causes of an Iranian cohort of subjects with cochlear implants., Methods: Whole exome sequencing was performed to find possible genetic etiology in probands of families after a comprehensive medical evaluation for hearing loss. Co-segregation analysis in probands and other family members was performed by Sanger sequencing. The variants were interpreted per the American College of Medical Genetics and Genomics guidelines., Results: Three different variants associated with TMIE were confirmed as reasons for autosomal recessive non-syndromic deafness. The first novel ~ 10-kb deletion surrounding exon 1 of TMIE along with two previously reported variants co-segregated with families including a frameshift variant c.122&#95;125dup (p.Pro43fs) and a missense variant c.250 C > T; p.(Arg84Trp) in exons 2, and 3, respectively., Conclusion: This study increases the mutational spectrum of the TMIE gene and highlights the importance of the large deletion of this gene as a reason for hearing loss. Moreover, an efficient and simple multiplex PCR assay was developed to determine the exact breakpoints of the TMIE deletion., (© 2022. The Author(s).)

Post-traumatic olfactory dysfunction (PTOD) is associated with a significant decrease in quality of life. The present study aimed to explore whether PTOD is associated with depression and changes in sexuality. There were two groups in this case-control study. The patient group consisted of patients with PTOD (n = 55), and the control group comprised healthy individuals without the olfactory disorder (n = 115). Olfactory function, depression, partnership, and sexual satisfaction were assessed using the Iranian version of the Sniffin' Sticks test (Ir-SST), Beck Depression Inventory (BDI), Enrich Couple Scale (ECS) and Sexual Satisfaction Scale for Women (SSSW). The BDI scores were higher in the patient group than in the control group (p < 0.001). The SSSW score was lower in the patient group than in controls (p < 0.01), although the ECS score was not significantly different between patients and controls. Also, there was no significant difference in the severity of trauma between marital satisfaction and sexual satisfaction. However, the analysis showed a statistically significant difference in depression scores in connection with the head trauma severity. In the PTOD group, depression was increased and sexual satisfaction declined. Understanding the association of olfactory dysfunction with depression and sexuality allows patients and doctors to deal with less notable consequences of this disorder., (© 2021. The Author(s).)

Background: Klebsiella pneumoniae is one of the leading causes of hospital outbreaks worldwide. Also, antibiotic-resistant K. pneumoniae is progressively being involved in invasive infections with high morbidity and mortality. The aim of the current study was to determine antimicrobial susceptibility patterns and the incidence of resistance genes (integron types and β-lactamase-encoded genes) among clinical isolates of K. pneumoniae., Methods: In this cross-sectional study, a total of 100 clinical samples were obtained from hospitalized patients in three teaching hospitals in the north of Iran, from November 2018 and October 2019. Antimicrobial susceptibility testing was performed using disk agar diffusion test in line with CLSI recommendations. For colistin, minimum inhibitory concentration (MIC) was determined using broth microdilution. Based on antibiogram, multi-drug resistant (MDR) and extensive-drug resistant (XDR) strains were detected. Finally, integron types and β-lactamase resistance genes were identified using polymerase chain reaction technique., Results: The most and least clinical samples were related to the urine and bronchoalveolar lavage, respectively. Based on the antibiogram results, amikacin and gentamicin exhibited good activity against K. pneumoniae strains in vitro. The high resistance rate (93%) to ampicillin/sulbactam predicts the limited efficacy of this antibiotic, in the hospitals studied. Among all the 100 isolates, the frequency of MDR and XDR phenotypes were 58% and 13%, respectively, while no pan-drug resistant (PDR) strains were found. In the MDR K. pneumoniae strains, the prevalence of bla SHV , bla TEM , bla CTX-M-15 , bla KPC , bla OXA-48 , bla NDM β-lactamase genes were 91.4%, 82.7%, 79.3%, 29.3%, 36.2% and 6.9%, respectively, however 91.4% of the isolates were carrying intI gene. Class II and III integrons were not detected in any isolates., Conclusion: The MDR K. pneumoniae is becoming a serious problem in hospitals, with many strains developing resistance to most available antimicrobials. Our results indicate co-presence of a series of β-lactamase and integron types on the MDR strains recovered from hospitalized patients. The increasing rate of these isolates emphasizes the importance of choosing an appropriate antimicrobial regimen based on antibiotic susceptibility pattern., (© 2021. The Author(s).)

Objective: This study aimed to measure the duration and recovery rate of olfactory loss in patients complaining of recent smell loss as their prominent symptom during the coronavirus disease 2019 outbreak., Method: This was a prospective telephone follow-up observational study of 243 participants who completed an online survey that started on 12 March 2020., Results: After a mean of 5.5 months from the loss of smell onset, 98.3 per cent of participants reported improvement with a 71.2 per cent complete recovery rate after a median of 21 days. The chance of complete recovery significantly decreased after 131 days from the onset of loss of smell (100 per cent sensitive and 97.7 per cent specific). Younger age and isolated smell loss were associated with a rapid recovery, whereas accompanying rhinological and gastrointestinal symptoms were associated with longer loss of smell duration., Conclusion: Smell loss, occurring as a prominent symptom during the coronavirus disease 2019 pandemic, showed a favourable outcome. However, after 5.5 months from the onset, around 10 per cent of participants still complained of moderate or severe hyposmia.

Background: A reliable and valid tool to assess hearing health literacy in Iranian young people is lacking., Aims: This study aimed to develop a tool to assess the hearing health literacy of young people in the Islamic Republic of Iran and to use the tool to determine the hearing health literacy of a sample of Iranians aged 12-25 years., Methods: A questionnaire was designed with three skill sections: obtaining health information, evaluating this information and applying it to benefit health. The validity and reliability of the tool were determined. Cluster sampling was used to select 50 urban clusters across the country from which 5000 Iranians aged between 12-25 years old were selected to complete the questionnaire., Results: The final questionnaire had 22 items with scores ranging from 22 to 44. The Cronbach alpha was 0.65, and content validity ratio and index were 0.92 and 0.82, respectively. Of the 5000 questionnaires completed, 4890 were included in the analysis. The mean (standard deviation (SD)) age of the participants was 17 (SD 3) years and 45.9% were males. The mean score on the tool was 30.81 (SD 3.75), indicating inadequate health literacy (score 22-36). Only 137 (2.8%) participants had adequate health literacy (score 37-44). Statistically significant differences in mean hearing health literacy were seen for sex, age, years of education, occupation, marital status and income (P < 0.05)., Conclusions: Given the low level of hearing health literacy in Iranian young people, programmes to improve the ear and hearing health literacy are urgently needed., (Copyright © World Health Organization (WHO) 2021. Open Access. Some rights reserved. This work is available under the CC BY-NC-SA 3.0 IGO license (https://creativecommons.org/licenses/by-nc-sa/3.0/igo).)

Background: Ménière's disease (MD) is a common inner ear disorder which is characterized by recurrent attacks of vertigo, fluctuating sensorineural hearing loss (SNHL), tinnitus, and a sense of fullness in the affected ear. MD is a complex disorder; although six genes have been linked to familial autosomal dominant form of the disease, in many cases, the exact genetic etiology remains elusive., Methods: To elucidate the genetic causes of MD in an Iranian family, we performed exome sequencing on all members of the family: consanguineous parents and four children (two affected and two unaffected). Variant filtering was completed using a customized workflow keeping variants based on segregation with MD in autosomal recessive (AR) inheritance pattern, minor allele frequency (MAF), and in-silico prediction of pathogenicity., Results: Analysis revealed that in this family, 970 variants co-segregated with MD in AR pattern, out of which eight variants (one intergenic, four intronic, and three exonic) were extremely rare. The exonic variants included a synonymous substitution in USP3 gene, an in-frame deletion in ZBED2 gene, and a rare, highly conserved deleterious missense alteration in LSAMP gene., Conclusion: The phenotype observed in the proband described here, i.e. vertigo, poor sense of smell, tinnitus, and borderline hearing ability, may originate from aberrant changes in the cerebellum and limbic system due to a deleterious mutation in the LSAMP gene; hence, LSAMP mutation is a possible candidate for the etiology of MD in this family., (© 2020 The Author(s). This is an open-access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.)

Dust storm in Khuzestan region is strongly influenced by transportation and influx of large amount of particulate matter from internal sources (Hawizeh Marshes and East Ahwaz) and external sources (the Arabian Desert in Saudi Arabia, Jordan, Syria and the Sahara Desert). Particulate matter is one of the main components of indoor and outdoor air quality that can be very dangerous for human. The principal objective of this study was the pinpoint of the source of airborne particulate matter by the NOAA HYSPLIT model in Ahvaz City, southwest of Iran. The investigation of dust storm and their origin was performed by the GFSG Meteorological Data (backward trajectories ending analysis of the NOAA HYSPLIT model) and collecting particulate samples with high-volume air samplers during the fall and winter seasons. The results showed the average ambient particulate matter concentration in the cold and warm seasons was 158 and 161 µg/m 3 , respectively. Moreover, the average particulate matter concentration in the cold season was significantly higher than the standard level as presented in the National Ambient Air Quality Standard. Consequently, it seems essential to develop green space, decrease particulate emission from source and make determined efforts to control dust at governmental and international scales.

A significant contribution to the causes of hereditary hearing impairment comes from genetic factors. More than 120 genes and 160 loci have been identified to be involved in hearing impairment. Given that consanguine populations are more vulnerable to most inherited diseases, such as hereditary hearing loss (HHL), the genetic picture of HHL among the Iranian population, which consists of at least eight ethnic subgroups with a high rate of intermarriage, is expected to be highly heterogeneous. Using an electronic literature review through various databases such as PubMed, MEDLINE, and Scopus, we review the current picture of HHL in Iran. In this review, we present more than 39 deafness genes reported to cause non-syndromic HHL in Iran, of which the most prevalent causative genes include GJB2, SLC26A4, MYO15A, and MYO7A. In addition, we highlight some of the more common genetic causes of syndromic HHL in Iran. These results are of importance for further investigation and elucidation of the molecular basis of HHL in Iran and also for developing a national diagnostic tool tailored to the Iranian context enabling early and efficient diagnosis of hereditary hearing impairment.

Background: Countries with culturally accepted consanguinity provide a unique resource for the study of rare recessively inherited genetic diseases. Although hereditary hearing loss (HHL) is not uncommon, it is genetically heterogeneous, with over 85 genes causally implicated in non-syndromic hearing loss (NSHL). This heterogeneity makes many gene-specific types of NSHL exceedingly rare. We sought to define the spectrum of autosomal recessive HHL in Iran by investigating both common and rarely diagnosed deafness-causing genes., Design: Using a custom targeted genomic enrichment (TGE) panel, we simultaneously interrogated all known genetic causes of NSHL in a cohort of 302 GJB2-negative Iranian families., Results: We established a genetic diagnosis for 67% of probands and their families, with over half of all diagnoses attributable to variants in five genes: SLC26A4, MYO15A, MYO7A, CDH23 and PCDH15. As a reflection of the power of consanguinity mapping, 26 genes were identified as causative for NSHL in the Iranian population for the first time. In total, 179 deafness-causing variants were identified in 40 genes in 201 probands, including 110 novel single nucleotide or small insertion-deletion variants and three novel CNV. Several variants represent founder mutations., Conclusion: This study attests to the power of TGE and massively parallel sequencing as a diagnostic tool for the evaluation of hearing loss in Iran, and expands on our understanding of the genetics of HHL in this country. Families negative for variants in the genes represented on this panel represent an excellent cohort for novel gene discovery., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/)

Background: Aspirin-exacerbated respiratory disease (AERD) is a syndrome frequently seen in patients with chronic rhinosinusitis and nasal polyposis (CRSwNP). However, there are few studies on evaluating the prevalence of aspirin (acetylsalicylic acid [ASA]) hypersensitivity in patients with CRSwNP using the oral aspirin challenge (OAC) test. This cross-sectional study was designed to determine the frequency of ASA hypersensitivity and factors associated with it in patients with CRSwNP in Tehran, Iran., Methods: Adult patients with CRSwNP who were presented to the asthma and allergy clinic were recruited for the study. After confirming CRS and NP, OAC was performed to evaluate/confirm the diagnosis of ASA hypersensitivity. Atopic evaluation was performed using skin-prick test, nasal smear, blood eosinophil count, and serum total IgE., Results: Eighty Iranian patients (43 women and 37 men) with CRSwNP were enrolled (mean age, 38.9 ± 10.7 years). OAC was performed in all of the patients and 39 patients (48.8%) had a positive reaction; among them, 14 (35.8%) had a self-reported history of ASA hypersensitivity. Concomitant asthma, previous polyp surgery, high polyp score, and ASA hypersensitivity history were all associated with positive OAC (p < 0.05). Presence of AERD was not associated with age, stage of asthma, blood eosinophilia, nasal smear eosinophilia, and atopy., Conclusion: ASA hypersensitivity is common in patients with CRSwNP in Tehran, Iran. Patients at risk for AERD should be evaluated for the presence of ASA hypersensitivity with ASA provocation challenge test to confirm the diagnosis.

Objectives: We here present the results of the first eight years of the newborn hearing screening programme in Iran, with a view to establishing the prevalence of hearing impairment among infants, and the efficacy of the programme., Methods: A total of 3,350,995 infants were screened using the series method of transient evoked otoacoustic emissions (TEOAEs)/automated auditory brainstem responses (AABRs), between 2005 and 2012. The infants were first tested for TEOAEs (three times). Based on the results of this test, the positive cases were referred to the next stage, where they were tested for AABRs. If they also tested positive on AABRs, they were referred to the diagnostic and rehabilitation stages., Results: Results of this study indicated an infant hearing impairment prevalence of 3 per 1000. Although this rate was as high as 5 per 1000 in the early years of the programme, it decreased to 2.6 per 1000 in the last year. The absolute referral rate was 14.5% in the first stage, which decreased to 0.9% and 0.2% in the second and the third stages, respectively. The follow-up rate was 70% in the first stage, which increased up to 73% and 85% in the second and the third stages, respectively., Conclusion: The study results suggest that the prevalence of hearing impairment in infants in Iran is comparable with that in developed and developing countries, and that the series TEOAEs/AABRs method used in the screening programme in Iran is efficient.

Acquired immune deficiency syndrome is one of the world's serious health problems. Immune-based therapy is a new approach in the treatment of HIV infected patients. IMOD™ with the ability to correct immune deficiencies has been introduced for the management of HIV infection. In the phase IV trial study the main objectives were to assess the possible side effects, evaluate its effect on CD4⁺ T lymphocyte count and patients' and physicians' satisfactions for 600 HIV infected patients in 13 centers during 2007. The observed adverse events in patients included: headache and vertigo (1.2%), nausea (1.2%), gastritis (1.2%), phlebitis (1%) and mild rash (1%); serious adverse events were not observed in any of IMOD™ recipients. Therefore it was not needed to terminate the treatment in any of patient. The results of this study demonstrated that daily prescription of IMOD™ significantly increases T lymphocyte CD4⁺ and total lymphocyte count in HIV-positive patients. In addition, nearly 90% of the patients and 70% physicians are satisfied by IMOD™ treatment.

A wide range of population, 4% to 30%, suffers from tinnitus that is defined as perception of sound without apparent acoustic stimulus. We conducted the present study to determine the prevalence of tinnitus in Iranian population; Tehran province. This cross-sectional study was conducted from January 2009 to December 2009, recruiting 3207 individuals (age range, 7-98) who were residing in Tehran province, Iran. Participants were asked to fill two questionnaires; the validated Persian version of Tinnitus Questionnaire (TQ) and another one that was specifically designed for this study. Prevalence of tinnitus and its association factors were evaluated. 3207 participants enrolled into our study comprising 1429 (44.7%) male and 1765 (55.3%) female with mean age of 55.01±17.85. Of total of 3207 participants, 146 (4.6%) had tinnitus consisting of 80 male (54.8%) and 66 (45.2%) female participants. It showed a rising trend with increasing age that was especially significant after the sixth decade of life (P=0.001). The analysis showed mean TQ global score of 35.96±25.52 that was significantly different between male and female participants (P=0.051) and had no significant correlation with increasing age (Spearman's r=0.1, P=0.10). The tinnitus intensity was moderate to very severe in 95 (56.1%) of the participants. Its severity level was not significantly different between men and women (P=0.09). Tinnitus intensity had no significant association with increasing age (Spearman's r=0.1, P=0.31). Patients with higher TQ global score had higher tinnitus intensities (P=0.001). The annoyance level was significantly different between men and women (P=0.04) and its impact on the participants daily routine functions were significantly higher in men (P=0.003). Given the results of the study, demonstrating that prevalence of tinnitus in Iranian population (Tehran province) was lower than the other countries and had a direct correlation with increasing age only after the sixth decade. TQ global score had significant correlation with tinnitus intensity, annoyance and impact on the participants' daily routine functions. However, none of the above had correlation with increasing age. Tinnitus TQ global score and intensity were not different between men and women; however annoyance of tinnitus and its impact on participants' daily routine functions were significantly higher in men.

Obstructive sleep apnea (OSA) is a common sleep disorder which can result in mood problems. The aim of this study was to evaluate the severity of depression and anxiety symptoms as the most prevalent psychological disturbances present in different severity of OSA. We performed a cross-sectional study of 685 recently diagnosed sleep-disordered patients, over the age of 18, referred to Noor Sleep Lab from August 2008 to November 2010. The participants filled the Beck depression inventory-II (BDI-II) and the Beck anxiety inventory (BAI) to assess the depression and anxiety symptoms. We collected other characteristics of subjects such as age, sex, body mass index (BMI) and Epworth sleepiness scale (ESS). Apnea hypopnea index (AHI) was determined by an overnight polysomnography. Mean age of the participants was 47.63 years (SD 11.73). More than half of patient had some degrees of depression and anxiety. AHI showed no significant correlation with BDI (p = 0.105, r = -0.070) or BAI (p = 0.712, r = -0.016). Obesity was not either correlated with depression or anxiety (p = 0.18, r = 0.05). Nonetheless, ESS was weakly correlated with depression (p = 0.001, r = 0.148) and anxiety scores (p = 0.006, r = 0.120). BMI and ESS means were significantly higher in patients with severe OSA (p = 0.000). In comparison with men, the severity of depressive and anxiety symptoms was significantly higher in women (p = 0.000). In this cross-sectional study of patients with sleep problems, OSA was not associated with severity of depression and anxiety symptoms.

Lipoid proteinosis (LP) is a rare autosomal recessive disorder. Classical clinical features include warty skin infiltration, papules on the eyelids, skin scarring, as well as extracutaneous abnormalities such as hoarseness of the voice, epilepsy, and neuropsychiatric abnormalities. A defect in the ECM1 gene is responsible for this disease. A 21-year-old female patient from consanguineous parents (first cousins) was referred to our clinic with many symptoms of LP, such as hoarse voice from infancy, diffuse acneiform scars on her face, and hyperkeratosis on her knees and elbows. The entire ECM1 gene was screened using PCR and sequencing. A novel missense mutation was found in exon 7 of this patient. We report a novel missense mutation in exon 7 of the ECM1 gene found in an Iranian LP patient that causes a C269Y amino acid exchange.

Background: Sleep disturbances are common among adult populations and can have a significant effect on daytime activities. The aim of this study is to determine the prevalence of sleep problems and subjective sleep quality in the adult population of Tehran, Iran., Methods: From an urban community of Tehran, a random sample of 3400 adult men and women were selected by a cross-sectional design. Using the Persian version of the Pittsburgh Sleep Quality Index (PSQI), subjects were interviewed face-to-face. There were 3114 completed questionnaires returned and analyzed., Results: The mean age of the subjects was 43.57 (± SD 17.5) years. Overall 37% (95% CI: 35-39) of the population were categorized as poor sleepers. The PSQI > 5 showed 27% were males versus 35% among females. The global PSQI scores ranged from 4.20 ± 2.67 to 5.60 ± 3.74 for males and 5.03 ± 3.00 to 7.97 ± 4.31 for females by age groups. The difference across age groups for global PSQI score was significant in females (P < 0.01)., Conclusion: The prevalence rate of sleep complaints in this population-based study was high. Females, older adults, widows and separated couple were the most important risk factors for sleep disturbances.

Mutations in GJB2, encoding connexin 26 (Cx26), cause both autosomal dominant and autosomal recessive nonsyndromic hearing loss (ARNSHL) at the DFNA3 and DFNB1 loci, respectively. Most of the over 100 described GJB2 mutations cause ARNSHL. Only a minority has been associated with autosomal dominant hearing loss. In this study, we present two families with autosomal dominant nonsyndromic hearing loss caused by a novel mutation in GJB2 (p.Asp46Asn). Both families were ascertained from the same village in northern Iran consistent with a founder effect. This finding implicates the D46N missense mutation in Cx26 as a common cause of deafness in this part of Iran mandating mutation screening of GJB2 for D46N in all persons with hearing loss who originate from this geographic region., (Copyright © 2011 Wiley-Liss, Inc.)

Unlabelled: Tinnitus is a debilitating condition that is widespread yet difficult to successfully diagnose and treat. This symptom can seriously affect the individual's life quality., Objectives: The aim of current study was to compose and validate a Persian version of the Tinnitus Handicap Inventory (THI-P)., Materials and Methods: The linguistic validation of the original version of THI into Persian version (THI-P) included translation, back translation and data gathering. The THI-P was administered to 112 tinnitus subjects. Age, gender, medical history and tinnitus characteristics were recorded as baseline information. All participants complained of chronic unilateral or bilateral subjective idiopathic tinnitus lasting for at least 6 months before consulting about their tinnitus., Results: There was no significant difference between gender, age, hearing impairment and total score and subscales of THI-P. Pearson product-moment correlations revealed adequate test-retest reliability for the THI-P (r = 0.96). Cronbach's-alpha coefficient indicated adequate internal stability of the THI-P (r= 0.943), with a total item correction varying between r=0.939 and r=0.944, indicating its reproducibility., Conclusion: The present study proved the internal consistency/ coherency of the Persian version of THI (THI-P). This provides satisfactory application in clinical/research environments.

Objective: To evaluate the possible role of infectious agents on the occurrence of idiopathic sensorineural hearing loss (SNHL) in children., Methods: This case control study was carried out at Rasul Hospital in Tehran, Iran from 2002-2003. We compared specific serum antibodies (IgG, IgM) measured by enzyme linked immunosorbent assay in 95 sensory hearing loss cases and 63 controls., Results: Acute infections (IgM) detected in cases included: cytomegalovirus (CMV) 34.6%, toxoplasma 11.5%, mumps 8.7%, rubella 5.3%, and herpes simplex 5.3%. Previous infections (IgG) detected in cases included CMV 72%, herpes 6.6%, toxoplasma 26%, mumps 23.3%, and rubella 17.2%. Acute CMV and toxoplasma infections were more frequent in cases. Previous CMV, toxoplasma, rubella, and herpes infections were higher in controls. There was no significant difference for acute mumps, rubella, and herpes infections between cases and controls., Conclusion: These data are compatible with infectious agents having a significant role in the studied idiopathic SNHL cases, but association does not prove causation. We recommend specific drugs for confirmed active infections (CMV, toxoplasma, herpes) in idiopathic SNHL infants diagnosed before their first birthday. Mumps and rubella induced SNHL are preventable with routine vaccination.

Assessment of nasal airway is helpful in understanding nasal breathing function. Acoustic rhinometry is one of the most commonly-used objective measurements of nasal airway. This test has the ability to measure the volume and the cross-sectional area of the nose in different distances. It also determines the site of minimal cross-sectional area. These variables are different in various races. Defining these normal values in adult Iranian population, as a criterion for diagnosis and treatment of nasal diseases can be very helpful.One hundred eighty adult Iranians (18 - 60 years old) without nasal breathing complaints underwent acoustic rhinometry. The mean volume and minimal cross-sectional area of the nose and also correlation of these variables with age, sex, height, weight, smoking habit, and topical nasal decongestion were assessed. In this sample, total nasal volume was 8.12+/-0.94 cm 3 and total minimal cross-sectional area was 0.9+/-0.17 cm 2 before decongestion. There was no correlation between these values and age, sex, height, weight, and smoking habit. There was a significant increase in volume and cross-sectional area after decongestion. Because of the differences in volume and minimal cross-sectional area in various races, measurement of these values in Iranian population is a proper way for diagnosis of rhinologic diseases and planning the appropriate treatment (medical or surgical). It is also helpful for comparison of preoperative and postoperative results after nasal surgeries.

Microtia, a congenital deformity manifesting as an abnormally shaped or absent external ear, occurs in one out of 8,000-10,000 births. We ascertained a consanguineous Iranian family segregating with autosomal-recessive bilateral microtia, mixed symmetrical severe to profound hearing impairment, and partial cleft palate. Genome-wide linkage analysis localized the responsible gene to chromosome 7p14.3-p15.3 with a maximum multi-point LOD score of 4.17. In this region, homeobox genes from the HOXA cluster were the most interesting candidates. Subsequent DNA sequence analysis of the HOXA1 and HOXA2 homeobox genes from the candidate region identified an interesting HOXA2 homeodomain variant: a change in a highly conserved amino acid (p.Q186K). The variant was not found in 231 Iranian and 109 Belgian control samples. The critical contribution of HoxA2 for auditory-system development has already been shown in mouse models. We built a homology model to predict the effect of this mutation on the structure and DNA-binding activity of the homeodomain by using the program Modeler 8v2. In the model of the mutant homeodomain, the position of the mutant lysine side chain is consistently farther away from a nearby phosphate group; this altered position results in the loss of a hydrogen bond and affects the DNA-binding activity.

Hereditary hearing loss (HHL) is a very common disorder. When inherited in an autosomal recessive manner, it typically presents as an isolated finding. Interestingly and unexpectedly, in spite of extreme heterogeneity, mutations in one gene, GJB2, are the most common cause of congenital severe-to-profound deafness in many different populations. In this study, we assessed the contributions made by GJB2 mutations and chromosome 13 g.1777179&#95;2085947del (the deletion more commonly known as del (GJB6-D13S1830) that includes a portion of GJB6 and is hereafter called Delta(GJB6-D13S1830)) to the autosomal recessive non-syndromic deafness (ARNSD) genetic load in Iran. Probands from 664 different nuclear families were investigated. GJB2-related deafness was found in 111 families (16.7%). The carrier frequency of the 35delG mutation showed a geographic variation that is supported by studies in neighboring countries. Delta(GJB6-D13S1830) was not found. Our prevalence data for GJB2-related deafness reveal a geographic pattern that mirrors the south-to-north European gradient and supports a founder effect in southeastern Europe., ((c) 2005 Wiley-Liss, Inc.)

Aim: To investigate the relation of human papillomavirus (HPV) and esophageal squamous cell carcinoma (ESCC) in Iranian patients as compared to normal controls., Methods: Using MY09/MY11 consensus primers, we compared the prevalence of a HPV L1 gene in tumor tissues from 38 ESCC cases and biopsied tissues from 38 endoscopically normal Iranian individuals. We also compared the presence of HPV16 and HPV18 in the same samples using type-specific E6/E7 primers., Results: Fourteen (36.8%) of the 38 ESCC samples but only 5 (13.2%) of the 38 control samples were positive for the HPV L1 gene (P = 0.02). Five (13.2%) of the ESCC samples but none of the control samples were positive for the HPV16 E6/E7 gene (P = 0.05). Three (7.9%) of the ESCC samples and 5 (13.2%) of the control samples were positive for the HPV18 E6/E7 gene (P = 0.71)., Conclusion: Our data are consistent with HPV DNA studies conducted in other high-risk areas for ESCC. HPV should be considered as a potential factor contributing to the high incidence of ESCC in Iran and other high-incidence areas of the world.

Background/objective: Studies about the prevalence of coeliac disease in countries in western Asia are scarce and there is no study on the prevalence of coeliac disease in Iran. The aim of this study was to determine the prevalence of coeliac in healthy, Iranian, blood donors., Study Design and Methods: Blood samples were obtained from 2000 apparently healthy blood donors (1580 males, 420 females; mean age 35.5 years, range 18-65 years) at the Tehran Blood Donation Centre during a 4 month period from November 1998 through February 1999. Total serum IgA was measured in all donors, and IgA deficient cases were excluded. All cases were analysed for IgA anti-gliadin (AGA) by an ELISA test and those with positive results were tested for IgA anti-endomysium antibody (EMA) using immunofluorescence. All donors who had a positive serology for both AGA and EMA underwent small intestinal biopsy. The biopsy samples were classified according to revised Marsh criteria (UEGW 2001)., Results: Forty-nine cases showed positive IgA AGA (38 males and 11 females, mean age 38.6 years). Of the 49 AGA positive cases 12 were EMA positive. All subjects with positive serology (both AGA and EMA) were found to have small bowel biopsies compatible with gluten sensitive enteropathy. Three of 12 had Marsh I, 4/12 Marsh II and 5/12 showed a Marsh IIIa lesion., Conclusion: The minimum prevalence of gluten sensitivity among apparently healthy urban Iranian blood donors is 1/166. Further epidemiological studies in adults from the general population and in high risk groups seems indicated.

Hereditary hearing loss (HHL) is an extremely common disorder. About 70% of HHL is non-syndromic, with autosomal recessive forms accounting for approximately 85% of the genetic load. Although very heterogeneous, the most common cause of HHL in many different world populations is mutations of GJB2, a gene that encodes the gap junction protein connexin 26 (Cx26). This study investigates the contribution of GJB2 to the autosomal recessive non-syndromic deafness (ARNSD) load in the Iranian population. One hundred sixty eight persons from 83 families were studied. GJB2-related deafness was diagnosed in 9 families (4, 35delG homozygotes; 3, 35delG compound heterozygotes; 1, W24X homozygote; 1, non-35delG compound heterozygote). The carrier frequency of the 35delG allele in this population was approximately 1% (1/83). Because the relative frequency of Cx26 mutations is much less than in the other populations, it is possible that mutations in other genes play a major role in ARNSD in Iran., (Copyright 2002Wiley-Liss, Inc)