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Introduction: Universal newborn hearing screening has been successfully implemented in many places around the world, and it is recommended that cases with risk factors for hearing loss be followed-up regardless of hearing screening results. However, there is a need for clarity regarding the recommended rate of follow-up and which tests should be performed. The aim of this study was to assess the audiologic follow-up program for the group with risk factors. Method: Our retrospective study involved children of various ages with a risk factor for hearing loss who passed the initial neonatal hearing test but were later diagnosed with hearing loss. Out of 113,708 children born at Hadassah University Medical Center during the years 2013–2021, 6763 were at risk of hearing loss, and their follow-up audiologic test results were studied. Results: Audiologic testing including ABR, OAE, tympanometry and behavioral audiometry was performed in 1534 of 6763 (23%) of the risk factor group that returned to the hospital. In total, 73 children (4.7%) were diagnosed with hearing loss, 54 of whom failed the initial screening and 19 who had passed it. Further examination of the children that passed the initial screening and were later diagnosed with a hearing loss revealed that four cases had been missed in screening (one familial mild hearing loss, one familial progressive loss, one premature infant with a high tone loss, and one NICU graduate with CNS involvement). Another nine cases had late-onset hearing loss (three meningitis, five CMV, and one with a mitochondrial disease). An additional six cases were diagnosed late, and the age of onset of the hearing loss was unknown (two intubated, two with hydrocephalus, one with Cerebral Palsy, and one with general developmental delay). Conclusions: These results reveal the importance of implementing a refined protocol for monitoring hearing in the high-risk group of children that pass neonatal hearing screening with respect to which hearing tests should be conducted, at what age, and the duration of follow-up. Also, barriers to follow-up must be dealt with, and parents should be more involved in the monitoring process. [ABSTRACT FROM AUTHOR]

Antibody affinity maturation occurs in secondary lymphoid organs within germinal centers (GCs). At these sites, B cells mutate their antibody-encoding genes in the dark zone, followed by preferential selection of the high-affinity variants in the light zone by T cells. The strength of the T cell–derived selection signals is proportional to the B cell receptor affinity and to the magnitude of subsequent Myc expression. However, because the lifetime of Myc mRNA and its corresponding protein is very short, it remains unclear how T cells induce sustained Myc levels in positively selected B cells. Here, by direct visualization of mRNA and active transcription sites in situ, we found that an increase in transcriptional bursts promotes Myc expression during B cell positive selection in GCs. Elevated T cell help signals predominantly enhance the percentage of cells expressing Myc in GCs as opposed to augmenting the quantity of Myc transcripts per individual cell. Visualization of transcription start sites in situ revealed that T cell help promotes an increase in the frequency of transcriptional bursts at the Myc locus in GC B cells located primarily in the LZ apical rim. Thus, the rise in Myc, which governs positive selection of B cells in GCs, reflects an integration of transcriptional activity over time rather than an accumulation of transcripts at a specific time point. Editor's summary: After positive selection by T cells in the germinal center (GC) light zone, GC B cells express MYC that activates downstream cell cycle and metabolic gene programs. The half-life of MYC is short, and it is unclear how Myc accumulates in GC B cells to determine their fate. Kagan Ben Tikva et al. used a single-molecule fluorescence in situ hybridization (smFISH) approach to visualize the dynamics of Myc mRNA transcription in murine GC B cells receiving varying degrees of T cell help. T cell–derived signals enhanced the frequency of Myc transcriptional bursts in GC B cells rather than the abundance of transcripts per cell at a single time point, indicating that Myc expression in positively selected B cells is integrated over time. —Hannah Isles [ABSTRACT FROM AUTHOR]

Background Inherited retinal diseases (IRDs) include a range of vision loss conditions caused by variants in different genes. The clinical and genetic heterogeneity make identification of the genetic cause challenging. Here, a cohort of 491 unsolved cases from our cohort of Israeli and Palestinian families with IRDs underwent whole exome sequencing (WES), including detection of CNVs as well as single nucleotide variants (SNVs). Methods All participants underwent clinical examinations. Following WES on DNA samples by 3 billion, initial SNV analysis was performed by 3 billion and SNV and CNV analysis by Franklin Genoox. The CNVs indicated by the programme were confirmed by PCR followed by gel electrophoresis. Results WES of 491 IRD cases revealed the genetic cause of disease in 51% of cases, of which 11% were due wholly or in part to CNVs. In two cases, we clarified previously incorrect or unclear clinical diagnoses. This analysis also identified ESRRB and DNM1 as potential novel genes. Conclusion This analysis is the most extensive one to include CNVs to examine IRD causing genes in the Israeli and Palestinian populations. It has allowed us to identify the causative variant of many patients with IRDs including ones with unclear diagnoses and potential novel genes. [ABSTRACT FROM AUTHOR]

Idiopathic sudden sensorineural hearing loss (SSNHL) represents a frequently encountered otological entity, of various types and severity, with an array of associated symptoms including vertigo. This is a devastating life-changing condition with a blurry prognosis. The objective of this study was to determine the clinical association of vestibular impairment by electronystagmography (ENG) and caloric tests, and their ability to predict prognosis. An observational, crossectional study was carried out amongst patients admitted with SSNHL. Each consenting patient had an audiometry test performed on admission as well as ENG and caloric tests. Treatment included oral steroids and carbogen with intratympanic steroids used only as salvage treatment. Follow-up was completed after 6 months when hearing gains were evaluated. Finally, an association was sought between the rate of recovery and ENG and caloric test results. Of 35 patients included, marked recovery was seen in patients without vertigo when compared to those with vertigo (p=0.003). A statistically significant association was found between the presence of vertigo and hearing deterioration (p=0.008). More so, normal electronystagmography results were associated with marked recovery (p=0.04). The vestibular end organs are both subjectively and objectively affected in SSNHL as demonstrated by the abnormal ENG and caloric tests in our study despite the small sample size. Concomitant vestibular involvement carries poorer prognosis and routine identification may help foresee the recovery of patients with SSNHL and as such, aid in patient counseling. ENG and caloric tests are easily available and may be recommended for all patients with SSNHL. [ABSTRACT FROM AUTHOR]

The aim of the study was to develop a sustained-release varnish (SRV) containing mometasone furoate (MMF) for sinonasal stents (SNS) to reduce mucosa inflammation in the sinonasal cavity. The SNS' segments coated with SRV-MMF or an SRV-placebo were incubated daily in a fresh DMEM at 37 °C for 20 days. The immunosuppressive activity of the collected DMEM supernatants was tested on the ability of mouse RAW 264.7 macrophages to secrete the cytokines' tumor necrosis factor α (TNFα) and interleukin (IL)-10 and IL-6 in response to lipopolysaccharide (LPS). The cytokine levels were determined by respective Enzyme-Linked Immunosorbent Assays (ELISAs). We found that the daily amount of MMF released from the coated SNS was sufficient to significantly inhibit LPS-induced IL-6 and IL-10 secretion from the macrophages up to days 14 and 17, respectively. SRV-MMF had, however, only a mild inhibitory effect on LPS-induced TNFα secretion as compared to the SRV-placebo-coated SNS. In conclusion, the coating of SNS with SRV-MMF provides a sustained delivery of MMF for at least 2 weeks, maintaining a level sufficient for inhibiting pro-inflammatory cytokine release. This technological platform is, therefore, expected to provide anti-inflammatory benefits during the postoperative healing period and may play a significant role in the future treatment of chronic rhinosinusitis. [ABSTRACT FROM AUTHOR]

Objective: Vertigo is a complex symptom which imposes diagnostic and treatment challenges. Laboratory evaluation of vertigo includes video-nystagmography (VNG) and computerized dynamic posturography (CDP) for the evaluation of different aspects of this complaint. There are vague indications for each test and potential disagreements between them. The aim of this study is to examine the association between the test results of the VNG and sensory organization test (SOT) of CDP in patients referred for both vestibular tests. Methods: Retrospective data regarding 56 patients age 17–82 years were collected. Patients suffered vestibular complaints and were referred for VNG and CDP evaluation on the same day. The level of agreement between VNG (including caloric test) and the vestibular input of the SOT for each patient was calculated. Results: Among the study group, 10 showed abnormal caloric test results, of which 3 (5.4%) had normal vestibular input in the SOT, and 7 (12.5%) had impaired input (p = 0.724). Spontaneous nystagmus was recorded in 13 patients by VNG, of which 2(3.6%) had normal vestibular input and 11(19.6%) had impaired vestibular input (p = 0.056). Conclusions: This study shows no statistically significant association between the VNG test and SOT test results. Our results emphasize the difference between the tested aspects in each laboratory test, and the need to define specific indications for each of them. There is a marginally significant association between impaired vestibular input and spontaneous nystagmus, demonstrating the non-localizing nature of this sign. [ABSTRACT FROM AUTHOR]

Objective: Hearing abnormalities frequently occur in Human Immunodeficiency Virus (HIV) infected individuals. Both conductive and uni- or bilateral sensorineural hearing loss (SNHL) have been described along with other audiological and vestibular symptoms such as tinnitus, vertigo and balance disturbances. While frequent middle ear infections may explain impairment of peripheral hearing abilities, the exact etiology of cochlear, and central auditory processing deficits still remains unclear. Direct effects of HIV, opportunistic infections, ototoxic side effects of antiretroviral therapy (ART), and immunologic responses to the central nervous system involving the auditory pathway have been proposed. We aim to review the audiological profile in HIV infected adults related to the effects of HIV and HAART on the inner ear structures. Methods: We present a review of the literature on cases of HIV related SNHL in adult patients and studies conducted to investigate audiometric changes in such patients. Data on presentation, diagnosis and pathophysiology were reviewed. Results: Sensorineural hearing loss in the higher frequencies is a common form of hearing loss in HIV infected individuals throughout disease progression, along with decreased otoacoustic emission (OAE) responses, increased PTA hearing thresholds and prolonged latencies for auditory brainstem responses (ABR). Conclusion: HIV affects all stages of auditory perception in a way similar to accelerated aging of the auditory system. And we postulate that synaptic loss may be the first step, followed by cochlear damage and central pathology as the virus remains present in all the structures of the auditory pathway causing local inflammation and degeneration. Evaluation of hearing function among all patients diagnosed with HIV infection seems to be an accepted approach; it should include OAE testing, pure tone and speech audiometry, speech-in-noise tests and ABR measurements. [ABSTRACT FROM AUTHOR]

Introduction Acute carotid blowout syndrome (aCBS) is a severe complication of head and neck cancer (HNC). It can be defined as a rupture of the extracranial carotid arteries, or one of their branches, that causes life-threatening hemorrhage, and which nowadays can be treated with urgent endovascular intervention. Objective We retrospectively evaluate the endovascular management of aCBS and its outcome in years of survival. Methods Retrospectively, we describe our experience with endovascular control of aCBS in patients treated for HNC. We review the characteristics, pathology, endovascular treatment and morbidity and assess the gain in life years. Results Nine individuals were included in this study. Four patients had been previously diagnosed with laryngeal squamous cell carcinoma (SCC), one with paranasal SCC, one with nasopharyngeal carcinoma and three with oral or maxillary adenocarcinoma. All subjects underwent radiotherapy and surgical excision to different extents. Twelve endovascular procedures were performed for injuries to the internal carotid artery (n = 3; 25%), external carotid artery (n = 1; 7%) or one of their branches (n = 8; 67%). Deconstructive methods were used in nine procedures, and three procedures were mainly reconstructive with deployment of covered stents. Total control of bleeding was achieved in all individuals with no intraprocedural complications. Conclusion Endovascular therapy is an effective alternative for the management of exsanguinating CBS. In our series, this palliative therapy increased the overall patient survival by an estimated 9 months. [ABSTRACT FROM AUTHOR]

Cogan's syndrome (CS) is a rare autoimmune disorder characterized by audiovestibular dysfunction and ocular inflammation. Currently, there is no specific serum autoantibody used in the diagnostic workup of CS. Treatment is based on immunosuppressive agents, mainly corticosteroids as first-line choice. Recently, novel therapeutic modalities in CS have emerged. These include tumor necrosis factor-α inhibitors and other biologicals. Despite medical treatment, hearing loss may progress to irreversible bilateral profound SNHL in approximately half of CS patients resulting in candidacy for cochlear implantation (CI). Due to the inflammatory nature of the disease that is causing endosteal reaction with partial obliteration or complete neoossification of the intracochlear ducts, early CI is recommended. CI provides excellent and stable hearing rehabilitation with high score of word and sentence recognition. In this review, we will discuss different aspects of CS including clinical presentation, diagnosis, treatment, and future directives. [ABSTRACT FROM AUTHOR]

The objective of this study was to describe the occurrence, clinical manifestations, audiometric findings, pathogenesis and approach to sensorineural hearing loss (SNHL) among patients diagnosed with vitiligo with a review of the literature. We present a systematic review of the literature on cases of SNHL in patients diagnosed with vitiligo and studies conducted to investigate audiometric changes in such patients. Data on presentation, diagnosis and medical approach were reviewed. A total of 21 studies and case reports revealed at least 102 cases of SNHL in patients diagnosed with vitiligo. Arguments for a common causative etiology related to melanocyte function were mentioned in most of the literature. Evaluation of hearing function among all patients diagnosed with vitiligo seems to be an accepted approach; it should include audiometry, otoacoustic emissions (OAE) and ABR measurements. Extra precaution to prevent ototoxic or noise-induced hearing loss is strongly recommended. Further research is needed to better understand its pathogenesis. [ABSTRACT FROM AUTHOR]

Objects: Dizziness makes up a diagnostic and treatment challenge. The diagnostic accuracy of the medical history and vestibular physical examination in cases of vestibular symptoms is not clear. The aim of this study is to determine the association between vestibular physical examination, vestibular questionnaires, and electronystagmography (ENG) test in patients with vestibular symptoms. Methods: This is a prospective study of 135 adults with vestibular symptoms. The subjects underwent targeted physical examination and filled vestibular questionnaires, including the Dizziness Handicap Inventory (DHI), before ENG testing. The results of the physical examination and questionnaires were compared with the final ENG findings. Results: Of patients who had normal ENG results, 32.1% (17/52) showed abnormal physical examination, and 48.8% (40/82) of the patient who had normal physical examination showed abnormal ENG results (P = .46). Among patients with severe disability by DHI, 46.4% (13/28) had an abnormal ENG, and 42.9% (12/28) had a normal ENG (P = .39). Conclusions: This study did not demonstrate association between vestibular physical examination, vestibular questionnaires, and ENG results. Although history (augmented by questionnaires) and physical examination are the initial steps in the evaluation of vertigo, the current study suggests that they should be complemented by objective testing for evaluation of inner ear origin of vertigo. [ABSTRACT FROM AUTHOR]

Background Complications associated with long-term tracheotomy are obstruction of the distal end of the tube by granulation tissue and tracheomalacia. These complications have traditionally been surgically treated. Methods Prospective study in a chronic ventilator-dependent division, including 234 consecutive patients with tracheotomy and mechanical ventilation. Endoscopic evaluation was performed in patients in whom there was respiratory distress with difficulty in passing a suction catheter through the tube, and/or increased inspiratory resistance and increased peak inspiratory pressure. Results Nineteen patients were diagnosed with granulation or tracheomalacia. Two patients were treated by surgical removal of the obstructing tissue. Nonsurgical patients were conservatively managed with symptoms' resolution by bypassing the pathology with a longer tube than the previous one or by an adjustable flange tube under endoscopic visualization, with a median symptom-free period of 433 days (range, 55-1230 days). Conclusion In nonsurgical candidates, insertion of a longer tube is a conservative and feasible long-term treatment. © 2014 Wiley Periodicals, Inc. Head Neck 37: 716-721, 2015 [ABSTRACT FROM AUTHOR]

Background Usher syndrome (USH) is a heterogeneous group of inherited retinitis pigmentosa (RP) and sensorineural hearing loss (SNHL) caused by mutations in at least 12 genes. Our aim is to identify additional USH-related genes. Methods Clinical examination included visual acuity test, funduscopy and electroretinography. Genetic analysis included homozygosity mapping and whole exome sequencing (WES). Results A combination of homozygosity mapping and WES in a large consanguineous family of Iranian Jewish origin revealed nonsense mutations in two ciliary genes: c.3289C>T (p.Q1097*) in C2orf71 and c.3463C>T (p.R1155*) in centrosome-associated protein CEP250 (C-Nap1). The latter has not been associated with any inherited disease and the c.3463C>T mutation was absent in control chromosomes. Patients who were double homozygotes had SNHL accompanied by earlyonset and severe RP, while patients who were homozygous for the CEP250 mutation and carried a single mutant C2orf71 allele had SNHL with mild retinal degeneration. No ciliary structural abnormalities in the respiratory system were evident by electron microscopy analysis. CEP250 expression analysis of the mutant allele revealed the generation of a truncated protein lacking the NEK2-phosphorylation region. Conclusions A homozygous nonsense CEP250 mutation, in combination with a heterozygous C2orf71 nonsense mutation, causes an atypical form of USH, characterised by early-onset SNHL and a relatively mild RP. The severe retinal involvement in the double homozygotes indicates an additive effect caused by nonsense mutations in genes encoding ciliary proteins. [ABSTRACT FROM AUTHOR]

PMF of infancy is a recently described autosomal recessive disorder presenting with severe bone marrow failure, accelerated neutrophil apoptosis, and significant platelet dysfunction, caused by a mutation in the VPS45 gene. In this study, we update our group of patients with PMF, highlighting different aspects of this disease, and evaluating the effectiveness of HSCT for the treatment of this disorder. Update of clinical data, hematological features, molecular studies, treatment and final outcome of four children diagnosed with VPS 45-associated PMF of infancy. The patients described had clinical and hematological findings consistent with MF. Molecular studies showed that all patients were homozygous for the Thr224Asn mutation in the VPS 45 gene. HSCT was carried out in three patients and was successful in two. VPS 45-associated MF is a novel primary immune deficiency that can be successfully corrected by HSCT if applied early in the course of disease using appropriate conditioning. The diagnosis of VPS 45-associated PMF should be considered in all children presenting with SCN with subsequent development of pancytopenia. Long-term follow-up of these patients is necessary to identify extra-hematological manifestations of VPS45 deficiency. [ABSTRACT FROM AUTHOR]

We used a combined approach of homozygosity mapping and whole exome sequencing (WES) to search for the genetic cause of autosomal recessive retinitis pigmentosa (arRP) in families of Yemenite Jewish origin. Homozygosity mapping of two arRP Yemenite Jewish families revealed a few homozygous regions. A subsequent WES analysis of the two index cases revealed a shared homozygous novel nucleotide deletion (c.1220delG) leading to a frameshift (p.Gly407Glufs"56) in an alternative exon (#15) of USH1C. Screening of additional Yemenite Jewish patients revealed a total of 16 homozygous RP patients (with a carrier frequency of 0.008 in controls). Funduscopic and electroretinography findings were within the spectrum of typical RP. While other USH1C mutations usually cause Usher type I (including RP, vestibular dysfunction and congenital deafness), audiometric screening of 10 patients who are homozygous for c.1220delG revealed that patients under 40 years of age had normal hearing while older patients showed mild to severe high tone sensorineural hearing loss. This is the first report of a mutation in a known USH1 gene that causes late onset rather than congenital sensorineural hearing loss. The c.1220delG mutation of USH1C accounts for 23% of RP among Yemenite Jewish patients in our cohort. [ABSTRACT FROM AUTHOR]

Objectives/Hypothesis: Turban pin aspiration syndrome is a new clinical entity afflicting young Islamic girls wearing a turban.The goal of this study was to present our experience in diagnosis and treatment of this new entity, define its clinical and epidemiologic features, and shed a new light on the role of fashion in the increased incidence. Study Design: A retrospective study in a tertiary university hospital. Methods: Review of clinical parameters and epidemiologic features of 26 patients diagnosed with turban pin aspiration syndrome admitted to the Hadassah-Hebrew University Hospitals in Jerusalem from 1990 to 2010. Results: All patients were Muslim females with an average age of 16 years. In all cases, the history was positive for accidental aspiration. Most of the pins were located in the trachea (42%). In 20 cases, the pins were extracted by rigid bronchoscopy without major complications. Fluoroscopy-assisted rigid bronchoscopy was used successfully in three cases. In one case, the object was self-ejected by coughing before the bronchoscopy, and two patients were referred to the chest unit for thoracotomy. Conclusions: Clinicians should be aware of this distinct form of foreign body aspiration, its method of diagnosis, and extraction techniques. A cultural investigation showed a difference in the turban-fastening technique of young girls as compared with their mothers. Removal by rigid bronchoscopy is a safe method with a high success rate and should be considered as the preferred extraction method of choice. Laryngoscope, 2012 [ABSTRACT FROM AUTHOR]

Objectives and study design: Angiogenesis may be related to the pathogenesis and prognosis of chronic rhinosinusitis with nasal polyposis. This cross-sectional study, in a tertiary university hospital, evaluates angiogenesis parameters in nasal polyps, antrochoanal polyps and middle turbinates. Subjects: Nasal polyps obtained from 24 consecutive patients, were compared to 10 normal middle turbinates and to 11 antrochoanal polyps. Methods: Analyzing for the expression of the angiogenic marker CD34 by immunohistochemistry. Blood vessels count, vascular surface density and microvessel density were measured by manual and computerized methods. Results: Angiogenesis was significantly higher in nasal polyps compared to control turbinates and to antrochoanal polyps ( p = 0.0001 and p = 0.007, respectively). Antrochoanal polyps showed significantly more angiogenesis than normal middle turbinates ( p = 0.001). Angiogenesis was not elevated in sub-groups of nasal polyposis patients considered to have worse prognosis. Conclusions: Angiogenesis probably plays a role in the pathogenesis of both nasal polyposis and antrochoanal polyps. However, the significantly higher angiogenesis found in nasal polyps compared to antrochoanal polyps may support a different mechanism of growth. The lack of difference between angiogenesis and nasal polyposis patients sub-groups, may imply that angiogenesis is not associated with the prognosis of chronic rhinosinusitis with nasal polyposis. [ABSTRACT FROM AUTHOR]

Objectives/Hypothesis: To determine the effect of mouth breathing during childhood on craniofacial and dentofacial development compared to nasal breathing in malocclusion patients treated in the orthodontic clinic. Study Design: Retrospective study in a tertiary medical center. Methods: Clinical variables and cephalometric parameters of 116 pediatric patients who had undergone orthodontic treatment were reviewed. The study group included 55 pediatric patients who suffered from symptoms and signs of nasal obstruction, and the control group included 61 patients who were normal nasal breathers. Results: Mouth breathers demonstrated considerable backward and downward rotation of the mandible, increased overjet, increase in the mandible plane angle, a higher palatal plane, and narrowing of both upper and lower arches at the level of canines and first molars compared to the nasal breathers group. The prevalence of a posterior cross bite was significantly more frequent in the mouth breathers group (49%) than nose breathers (26%), (P = .006). Abnormal lip-to-tongue anterior oral seal was significantly more frequent in the mouth breathers group (56%) than in the nose breathers group (30%) (P = .05). Conclusions: Naso-respiratory obstruction with mouth breathing during critical growth periods in children has a higher tendency for clockwise rotation of the growing mandible, with a disproportionate increase in anterior lower vertical face height and decreased posterior facial height. Laryngoscope, 2010 [ABSTRACT FROM AUTHOR]

Objectives: The purpose of this study was to define the clinicopathologic features and outcome of the oncocytic variant of papillary thyroid carcinoma (OVPTC) with a review of the literature. Methods: Twenty-three patients with OVPTC over a 10-year period were studied. Demographic, clinical, and histopathcilogic features and outcome data were analyzed retrospectively, Results: Seventeen women and 6 men, ages ranging from 20 to 76 years (95% confidence interval, 43.0 to 54.48), were studied. Cervical lymph node involvement was found in 43.4% of the patients. Most of the recurrences were associated with thyroid masses greater than 2 cm in diameter. Evaluation of the overall survival data by the Kaplan-Meier method revealed that most recurrences took place earlier than 30 months, and the majority of patients (74%) were well, with no evidence of disease, up to 78 months after the last treatment. All of the OVPTC cases presented as nonencapsulated tumors, and 78.2% demonstrated extrathyroid strumal invasion. Conclusions: OVPTC is a unique variant of papillary thyroid carcinoma that has distinctive clinicopathologic features. Since OVPTC is often associated with local invasion and may involve cervical lymph nodes, it may require more extensive surgery than classic papillary thyroid carcinoma. [ABSTRACT FROM AUTHOR]

It is commonly accepted that aging is associated with a decline in the antioxidant defense of the cell; accordingly, certain redox enzymes are used as markers of biological senescence. To further test and specify this general concept, we studied age-related changes in the enzymes of the methionine-centered redox cycle (MCRC) in four aero-digestive organs of rats. The levels of cytosolic thioredoxin (Trx), thioredoxin reductase (TrxR), and methionine sulfoxide reductase (Msr), all tended to decline with age. The enzymatic activities of MsrA and MsrB were significantly lower in the organs of aged animals. In general, the magnitude of this decline increased in the order: tongue < sternohyoid muscle < larynx < esophagus. The relative stability of MCRC in the old tongues might be part of the well-preserved oxidative metabolism as confirmed by the age-related increase in mitochondrial marker and muscle tissue in these tongues. In total, the results suggest that age-associated oxidative damage is organ-specific and could reflect differences in morphological composition of these tissues, and among them, relative content of striated muscles. [ABSTRACT FROM AUTHOR]

Sudden sensorineural hearing loss (SSNHL) is frequently classified as ‘idiopathic’ since the causative factor responsible for its onset is not identified in most cases. In the present study, we determined whether SSNHL is clinically associated with serum anti-heat shock protein-70 (anti-HSP70) and antiphospholipids (anti-PLs) autoantibodies and whether these autoantibodies have an impact on the prognosis of SSNHL. Sera from 63 patients with SSNHL were screened prospectively for the presence of anti-HSP70 and anti-PLs autoantibodies by an enzyme-linked immunosorbent assay test. Anti-PLs antibodies in this study consisted of anticardiolipin, and anti-β2-glycoprotein-1 antibodies. Serum was assayed for anti-HSP70 IgG antibodies using recombinant human HSP70. Demographic, clinical, and audiometric variables were analyzed to find the possible role of serum autoantibodies in SSNHL patients. Sixteen patients (25.4%) had demonstrable anti-HSP70 antibodies in serum. Twenty-one patients (33.3%) showed a positive result for at least one isotype (IgM or IgG) of anti-PLs. In 19% of the patients, anti-HSP70 and anti-PLs antibodies were positive in two combinations. A statistically significant association was found between anti-HSP70 antibodies and the Siegel recovery grade subgroup. SSNHL patients who were positive for anti-HSP70 antibodies showed a significantly higher rate of complete recovery and incomplete but partial recovery than SSNHL patients without anti-HSP70 antibodies (p = 0.0496). Statistically significant association was found between total anticardiolipin, total anti-β2-glycoprotein-1, total anti-PLs, and anti-PLs in combination with anti-HSP70 antibodies and age (p = 0.0229). The detection of autoantibodies to HSP70 and PLs offers a pliable explanation for the immune-mediated mechanism of SSNHL. The present study confirms and supports previous studies regarding the association between anti-HSP70 and anti-PLs antibodies with SSNHL, and is the first to identify a positive association between anti-HSP70 antibodies and a positive outcome of SSNHL. Further studies are necessary in order to identify and further clarify the immunologic role of the presence of autoantibodies and their impact on the prognosis of SSNHL. Copyright © 2008 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Sudden sensorineural hearing loss (SSNHL) is frequently classified as ‘idiopathic’ since the causative factor is not identified in most cases. In the present study we determined whether SSNHL is associated with common viral infections, namely enterovirus, cytomegalovirus (CMV) and Epstein-Barr virus (EBV). Between April 2004 and March 2005, we conducted a prospective cohort study on 48 unselected patients with unilateral idiopathic SSNHL. Plasma samples obtained at presentation were analyzed for the presence of enterovirus RNA by reverse-transcription polymerase chain reaction (RT-PCR). Recent occurrence of CMV and EBV infection was determined by serological tests. Among 23 men and 25 women aged 18–84 years (mean age 51.67 years), none presented with clinical symptoms of a viral infection compatible with preceding or concurrent viral infection. One patient (2.08%) had evidence of enterovirus infection by RT-PCR, 3 patients (6.25%) had EBV IgM, and none were positive for CMV IgM. The majority of SSNHL patients (91.67%) did not have evidence of any of the three common viral infections upon presentation. The present study failed to identify recent or concurrent systemic viral infections among SSNHL patients, except for a small proportion who demonstrated recent viral infection. Therefore the theory that viral infection is a factor in SSNHL pathogenesis is not supported by this study. Further studies in larger patient populations and different epidemiological settings are required in order to verify the true role of viral infections in SSNHL. Copyright © 2007 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Idiopathic sudden sensorineural hearing loss (SSNHL) represents a frequently encountered otological disease of unknown etiology. In recent years, several inherited risk factors have been found in the pathogenesis of vascular diseases. In the present study, we determined whether specific polymorphism or the combination of polymorphisms in folate-dependent homocysteine metabolism genes can act as predisposing inherited vascular risk factors in the development of SSNHL. We conducted a prospective case-control study using DNA samples extracted from 81 patients diagnosed as suffering from SSNHL and 264 healthy control subjects. Three functional polymorphisms were analyzed by polymerase chain reaction amplification, restriction enzyme digestion, and DNA fragment separation by electrophoresis: methylenetetrahydrofolate reductase (MTHFR) C677T, MTHFR A1298C, and methionine synthase (MTR) A2756G polymorphisms. The prevalence of the homozygous genotype of MTR 2756GG in the SSNHL patients (9%) was significantly higher than in the control group (4%) (p = 0.011). The allelic frequency of the G allele of the MTR A2756G polymorphism among SSNHL patients (12.5%) was also significantly higher than in the control group (5%) (p = 0.033). The prevalence of patients possessing two polymorphisms (31%) and three polymorphisms (17%) in the SSNHL group was significantly higher than in the control group (23 and 9%, respectively; p = 0.019). The frequency of patients with a very high rank risk (double homozygous) was significantly higher in the SSNHL group, MTHFR 677TT/MTR 2675GG – 7%, than the frequency of patients in the control group, MTHFR 677TT/MTR 2675GG – 3% (p = 0.030). Certain polymorphisms in genes encoding enzymes in the folate-dependent homocysteine metabolism are associated with SSNHL. In our case-control study, a significant association between MTR 2756GG genotype and SSNHL was found which may represent an inherited vascular risk factor in the pathogenesis of SSNHL. Copyright © 2006 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Objective: The aim of this study was to evaluate the routine use of packing or local hemostatic agents in endoscopic sinus surgery (ESS). Methods: Packing and/or hemostatic agents were used only when necessary in 100 consecutive adult ESS patients in a tertiary academic hospital. Necessity for packing the nose after excessive bleeding was analyzed in relation to demographic characteristics, medical history, previous surgeries, current surgical procedure, type of anesthesia, and amount of intraoperative bleeding. Results: Three patients who required packing because of other reasons (such as bolstering of mucosa) were excluded from the study. The remaining 97 patients included 61 males and 36 females between the ages of 16 to 86 (mean 44). Forty-nine patients underwent only ESS, 40 ESSs associated with nasal polypectomy, and 8 underwent other endoscopic procedures. Fifty-four underwent the operation under general anesthesia and 43 under local anesthesia. Intraoperative blood loss was less than 30 mL in 82 patients (85%), 30 to 50 mL in 11 (11%), and more than 50 mL in 4 (4%). In 89 patients (92%), packing or a hemostatic agent was not used. No patient had bleeding complications postoperatively. A comparison between patients who required packing to those who did not showed that the only statistically significant associations related to general anesthesia (P = 0.0082) and to the amount of intraoperative bleeding (P < 0.001). Conclusions: Most ESS procedures can be managed without packing or any other hemostatic measures. Local anesthesia, use of local vasoconstrictors, and careful operative technique minimize the need for nose packing, thus reducing patient’s discomfort, postoperative complications, and cost of surgery. EBM rating: C-4 [Copyright &y& Elsevier]

This manuscript reports on our experience with purulent chondritis of the laryngeal cartilages (PCLC), an entity that has not yet been described. Three patients bad a diagnosis of PCLC. The probable causes were relapsing polychondritis, a previous prolonged intubation, and an idiopathic cause. The patients suffered from hoarseness and inspiratory stridor for 1 to 3 months before diagnosis. None complained of pain in the neck. Laryngoscopy showed supraglottic edema. A computed tomography scan revealed abscess formation between the intact inner and outer perichondria of the thyroid cartilage. The treatment included rigid endoscopy, external incision and drainage, and prolonged medical therapy. The culture results were Staphylococcus aureus in the first 2 cases and Aspergillus fumigatus in the third. The second patient (in whom the cricoid cartilage was also affected) required emergency tracheotomy. The other 2 patients did not require airway intervention. The rarity of PCLC and the relatively mild symptoms require a high index of suspicion for its diagnosis. [ABSTRACT FROM AUTHOR]

Objective --Cochlear involvement manifested by sensorineural or mixed-type hearing loss (HL) has been reported to occur in 30-67% of patients suffering from myringitis bullosa (MB). The goal of this study was to investigate the incidence and nature of vestibular involvement in MB. Material and Methods --All adult patients presenting to Hadassah University Hospital with MB between 2000 and 2002 were evaluated for inner ear involvement. Audiometry, tympanometry and electronystagmography (ENG) were performed within the first 48 h after presentation. The affected ears were examined on a regular basis and audiometric studies were repeated every other day. ENG was not repeated but a detailed questionnaire was administered and a thorough physical examination performed to rule out persistent vestibular dysfunction. Results --Thirteen patients (17 ears) were diagnosed as suffering from MB. All 13 patients reported HL in the affected ears and 7 (54%) reported a sensation of vertigo at presentation. Audiometric tests revealed HL in all 17 affected ears: sensorineural HL in 2, mixed-type HL in 12 and conductive HL in 3. ENG was normal in only two cases, both of whom did not suffer from vertigo. Four patients with no vestibular symptoms whatsoever had an abnormal electronystagmogram. All seven patients who complained of vertigo had an abnormal electronystagmogram and recovered fully after treatment. All but two patients recovered their auditory function. Conclusions --Not only is the cochlear part of the inner ear affected in patients suffering from MB, but the vestibular part as well. Sensorineural HL and vestibular abnormalities should both be considered as manifestations of MB. [ABSTRACT FROM AUTHOR]

The aim of the study was to develop a sustained-release varnish (SRV) containing chlorhexidine (CHX) for sinonasal stents (SNS) to reduce bacterial growth and biofilm formation in the sinonasal cavity. Segments of SNS were coated with SRV-CHX or SRV-placebo and exposed daily to bacterial cultures of Staphylococcus aureus subsp. aureus ATCC 25923 or Pseudomonas aeruginosa ATCC HER-1018 (PAO1). Anti-bacterial effects were assessed by disc diffusion assay and planktonic-based activity assay. Biofilm formation on the coated stents was visualized by confocal laser scanning microscopy (CLSM) and high-resolution scanning electron microscopy (HR-SEM). The metabolic activity of the biofilms was determined using the 3-(4,5-dimethyl-2-thiazolyl)-2,5-diphenyl-2H-tetrazolium bromide (MTT) method. Disc diffusion assay showed that SRV-CHX-coated SNS segments inhibited bacterial growth of S. aureussubsp. aureus ATCC 25923 for 26 days and P. aeruginosa ATCC HER-1018 for 19 days. CHX was released from coated SNS segments in a pH 6 medium up to 30 days, resulting in growth inhibition of S. aureussubsp. aureus ATCC 25923 for 22 days and P. aeruginosa ATCC HER-1018 for 24 days. The MTT assay showed a reduction of biofilm growth on the coated SNS by 69% for S. aureussubsp. aureus ATCC 25923 and 40% for P. aeruginosa ATCC HER-1018 compared to the placebo stent after repeated exposure to planktonic growing bacteria. CLSM and HR-SEM showed a significant reduction of biofilm formation on the SRV-CHX-coated SNS segments. Coating of SNS with SRV-CHX maintains a sustained delivery of CHX, providing an inhibitory effect on the bacterial growth of S. aureussubsp. aureus ATCC 25923 and P. aeruginosa ATCC HER-1018 for approximately 3 weeks. [ABSTRACT FROM AUTHOR]

Fungal biofilm formation on voice prosthesis (VP) is a major health problem that requires repeated replacement of the prosthesis. Candida albicans is one of the pathogens that frequently inhabits the VP. We proposed that coating VPs with sustained-release varnish (SRV) containing clotrimazole (CTZ) might prevent fungal biofilm formation. The long-term antifungal activities of SRV-CTZ- versus SRV-placebo-coated VPs was tested daily by measuring the inhibition zone of C. albicans seeded on agar plates or by measuring the fungal viability of C. albicans in suspension. The extent of biofilm formation on coated VPs was analyzed by confocal microscopy and scanning electron microscopy. We observed that SRV-CTZ-coated VPs formed a significant bacterial inhibition zone around the VPs and prevented the growth of C. albicans in suspension during the entire testing period of 60 days. Fungal biofilms were formed on placebo-coated VPs, while no significant biofilms were observed on SRV-CTZ-coated VPs. HPLC analysis shows that CTZ is continuously released during the whole test period of 60 days at a concentration above the minimal fungistatic concentration. In conclusion, coating VPs with an SRV-CTZ film is a potential effective method for prevention of fungal infections and biofilm formation on VPs. [ABSTRACT FROM AUTHOR]

We report the case of an eighteen-month-old child who suffered a tympanic membrane perforation caused by a digital thermometer. This injury led to a CSF leak. The patient was followed conservatively with no surgical intervention or prophylactic antibiotic therapy and developed pneumococcal meningitis 7 days later. He was then treated with Ceftriaxone for 12 days with full recovery, from a short and long term perspective. Issues concerning the management of CSF leak will be discussed along with review of the literature. This is the first report of post-traumatic meningitis as a result of mild trauma not involving maxillofacial or basilar fractures. The aim of our report is to raise awareness to this cause of meningitis and to stress the importance of immunizing against Streptococcal pneumoniae, a measure which may have prevented the sequelae in our case. [ABSTRACT FROM AUTHOR]

A patient with severe Behcet's disease (BD) was referred to our clinic because of severe pharyngolaryngeal involvement. This complication developed under treatment of prednisone and etanercept (Enbrel) 25 mg twice a week. A surgical approach was considered, but following three daily doses of Solu-Medrol (500 mg each) and 300 mg infliximab on the fourth day, the patient improved significantly. This is the first case reporting treatment with infliximab in laryngeal involvement of BD. Although some cases may still require surgical intervention, the therapeutic option of anti-TNF agents, specifically anti-TNF antibodies such as infliximab, may decrease the need for the surgical option. [ABSTRACT FROM AUTHOR]

Background Foreign bodies traversing the aerodigestive tract lining into the surrounding soft tissues is a well-known entity. A 14-year-old girl was referred to our hospital after swallowing a pin that was not visualized by laryngoscopy, despite a neck film that localized it to the level of the larynx. Methods A CT scan demonstrated a bent pin piercing the left vertebral artery 'through and through,' and this was confirmed by angiography. The pin was extracted via an external approach to the neck without preoperative intravascular manipulation. Results Angiography immediately after the surgery demonstrated a stable thrombotic sleeve in the vertebral artery without further clot propagation or bleeding from the vessel wall. This is the first report of a 'through and through' thrombotic sleeve in an artery. Conclusions Simple surgical removal of a sharp foreign body from the vertebral artery without intravascular management may be a reasonable option. © 2010 Wiley Periodicals, Inc. Head Neck, 2012 [ABSTRACT FROM AUTHOR]

Thyroid carcinoma usually presents as an asymptomatic thyroid nodule. Thyroid cancer may show microscopic vascular invasion: however, internal jugular vein tumor thrombus is a rare complication of thyroid cancer. A unique case of internal jugular vein tumor thrombus associated with thyroid carcinoma is presented and discussed. [ABSTRACT FROM AUTHOR]

Objective Parotid gland enlargement due to diffuse pathologic processes represents a diagnostic challenge for the surgeon. In such cases, fine-needle aspiration is inconclusive and superficial parotidectomy is usually performed. Material and Methods The authors treated eight patients who underwent open incisional biopsy of the parotid gland with continuous monitoring of the facial nerve. Results In all cases, fine-needle aspiration cytology did not provide a definitive diagnosis while open incisional biopsy provided an accurate diagnosis of a lymphoproliferative disorder, with no operative complications. Conclusion In certain infiltrative parotid diseases such as lymphoepithelial or lymphoproliferative diseases, when fine-needle aspiration biopsy is insufficient as tissue architecture is an important component of the pathological diagnosis, an open incisional biopsy can be considered. This procedure can provide an accurate histological diagnosis while avoiding superficial parotidectomy. [ABSTRACT FROM AUTHOR]

The laryngeal maculae flavae (MF) have been previously described as cellular condensations at the extremities of the vocal cords, but the exact nature and role of the MF are unknown. The purpose of this study is a histomorphological analysis of the nature and development of the MF in human vocal cords, from the beginning of fetal life to adulthood. Eighty-six larynges, from the fetus at 12 weeks of amenorrhea (WA) to adulthood, were collected from autopsies and studied by light microscopy with hematoxylin and eosin, orcein, and Alcian blue staining and pS 100 immunostaining. Our observations allowed us to separate the fetal development of the MF into 4 periods. The first corresponds to the appearance of the posterior MF between 13 and 15 WA. In the second period, the anterior MF appeared between 16 and 18 WA, allowing definition of the intermacular space. Fibrous structures, composed of collagen and elastic fibers, of the MF and intermacular space developed between 20 and 33 WA. The last period, from 33 WA to birth, corresponded to a maturation of these structures. In adults, the histologic structure of the MF presented a concentric peripheral fibrous layer associated with a central cellular component. Structural analysis of the MF concludes in favor of the chondroid nature of the macular cell. These observations bring into question the current knowledge on the MF. From these observations, the role of the MF in the developing vocal cord is discussed. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 27-year-old male with penetrating trauma due to blast injury. He was taken to the hospital in conscious condition and doctor revealed severe swelling to right eye due to periorbital hematoma. His head and neck are scanned under computer tomography (CT) angiography scan and revealed radio-opaque or penetrating metallic foreign body in the parotid gland. He developed facial nerve paresis, undergo surgery and treated with antibiotics and steroids.

Acute upper airway obstruction is a potentially life-threatening event. Hemophilia-A is a coagulopathy with high risk for spontaneous bleeding. Here we describe for the first time a spontaneous epiglottic internal hemorrhage leading to upper airway narrowing in a hemophilia-A patient. The patient was admitted to the intensive care unit for airway observation and treatment with factor VIII supplementation. In the first 48 hours there was rapid respiratory improvement. The epiglottic swelling resolved on the 5th day. In this patient no oral intubation or surgical management was needed. Laryngoscope, 2010 [ABSTRACT FROM AUTHOR]

Osteomas are benign, slow growing tumors of bone that occur almost exclusively in the craniofacial region. They are uncommon in the temporal bone. Surgical excision is indicated in symptomatic cases. An osteoma arising from the lateral semicircular canal growing into the mastoid cavity is extremely rare and usually discovered on routine radiography. We present a case of osteoma arising from the lateral semicircular canal growing into the mastoid cavity, followed by a discussion of the etiology, presentation, and management of osteomas. [ABSTRACT FROM AUTHOR]