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Eccrine squamous syringometaplasia (ESS) has been associated with several conditions including morphea, linear scleroderma, and burns. It is yet to be reported in lichen sclerosus et atrophicus (LSA). We describe a bullous LSA plaque on the forearm of a woman with pre-existing genital LSA and vitiligo. Besides the histopathological findings of bullous LSA, numerous small irregular squamoid structures were present in the mid and upper dermis always above the normal eccrine glands. The histopathology, periodic acid-Schiff stain, and positive immunostains for P63, low molecular weight keratins 818, epithelial membrane antigen, and carcinoembryonic antigen supported the diagnosis of ESS. The pathogenesis of ESS in LSA may be related to ischemia, inflammation, and fibrosis.

To evaluate maternal and neonatal severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) immunoglobulin G (IgG) antibody levels at birth after a third (booster) dose of the Pfizer-BioNTech messenger RNA (Pfizer) coronavirus disease 2019 (COVID-19) vaccine during the second trimester of pregnancy, and compare them with those in women who received two vaccine doses during the second trimester.We conducted a prospective cohort study of women admitted to the delivery ward at a single center who received the third Pfizer COVID-19 vaccine dose (booster group) at 17-30 weeks of pregnancy and who did not have previous SARS-CoV-2 infection. Maternal and neonatal antibody levels were measured on admission for delivery and in the umbilical cord blood after birth. Antibody levels for the booster group were compared with those in a historical control group of pregnant women who received their second vaccine dose (two-dose group) within the same gestational age window.Between October 2021 and February 2022, antibody levels were measured in 121 women and 109 neonates at a mean±SD of 15.3±3.9 weeks after booster vaccination. Neonatal titers measured two times higher than maternal titers, with inverse correlation between maternal and neonatal titers at birth and time interval from third vaccination. The two-dose group included 121 women and 107 neonates, with antibody levels measured at a mean±SD of 14.6±2.6 weeks after the second dose. Median [interquartile range] maternal antibody titers were higher in the booster group (4,485 [2,569-9,702] AU/mL) compared with the two-dose group (1,122 [735-1,872] AU/mL) (P.001). Furthermore, neonatal antibody titers were higher in the booster group (8,773 [5,143-18,830] AU/mL) compared with the two-dose group (3,280 [2,087-5,754] AU/mL) (P.001).Maternal and neonatal SARS-CoV-2 IgG antibody titers after second-trimester maternal Pfizer COVID-19 vaccination were significantly higher after the booster dose compared with the two-dose vaccination series. Although there is uncertainty as to whether antibody levels correlate with protection, these data support the importance of booster vaccination during pregnancy to restore maternal and neonatal protection against COVID-19.

Background Definitive non-invasive detection of pediatric choledocholithiasis could allow more efficient identification of those patients who are most likely to benefit from therapeutic endoscopic retrograde cholangiopancreatography (ERCP) for stone extraction. Objective To craft a pediatric choledocholithiasis prediction model using a combination of commonly available serum laboratory values and ultrasound results. Methods A retrospective review of laboratory and imaging results from 316 pediatric patients who underwent intraoperative cholangiogram or ERCP due to suspicion of choledocholithiasis were collected and compared to presence of common bile duct stones on cholangiography. Multivariate logistic regression with supervised machine learning was used to create a predictive scoring model. Monte-Carlo cross-validation was used to validate the scoring model and a score threshold that would provide at least 90% specificity for choledocholithiasis was determined in an effort to minimize non-therapeutic ERCP. Results Alanine aminotransferase (ALT), total bilirubin, alkaline phosphatase, and common bile duct diameter via ultrasound were found to be the key clinical variables to determine the likelihood of choledocholithiasis. The dictated specificity threshold of 90.3% yielded a sensitivity of 40.8% and overall accuracy of 71.5% in detecting choledocholithiasis. Positive predictive value was 71.4% and negative predictive value was 72.1%. Conclusion Our novel pediatric choledocholithiasis predictive model is a highly specific tool to suggest ERCP in the setting of likely choledocholithiasis.

Objective To describe the site of lesion responsible for the severe, bilateral, symmetrical, selective loss of vestibular function in Cerebellar Ataxia with Neuronopathy and Vestibular Areflexia Syndrome (CANVAS), an adult-onset recessively-inherited ataxia, characterized by progressive imbalance due to a combination of cerebellar, somatosensory, and selective vestibular impairment with normal hearing. Methods Histologic examination of five temporal bones and the brainstems from four CANVAS patients and the brainstem only from one more, each diagnosed and followed from diagnosis to death by one of the clinician authors. Results All five temporal bones showed severe loss of vestibular ganglion cells (cell counts 3-16% of normal), and atrophy of the vestibular nerves, whereas vestibular receptor hair cells and the vestibular nuclei were preserved. In contrast, auditory receptor hair cells, the auditory ganglia (cell counts 51-100% of normal), and the auditory nerves were relatively preserved. In addition, the cranial sensory ganglia (geniculate and trigeminal), present in two temporal bones, also showed severe degeneration. Conclusions In CANVAS there is a severe cranial sensory ganglionopathy neuronopathy (ganglionopathy) involving the vestibular, facial, and trigeminal ganglia but sparing the auditory ganglia. These observations, when coupled with the known spinal dorsal root ganglionopathy in CANVAS, indicate a shared pathogenesis of its somatosensory and cranial nerve manifestations. This is the first published account of both the otopathology and neuropathology of CANVAS, a disease that involves the central as well as the peripheral nervous system.

In the current proof-of-concept study, we aimed to examine the sensitivities and specificities of previously reported normal values for muscle ultrasound thickness in amyotrophic lateral sclerosis.Muscle ultrasound was performed in 65 healthy control subjects and 91 amyotrophic lateral sclerosis patients using a standardized assessment of eight relaxed muscles and four contracted muscles. Normal values for muscle thickness were determined as values above the 5th percentile stratified by age and gender using the weighted average method. Sensitivity for amyotrophic lateral sclerosis diagnosis was determined for muscles with and without the addition of muscle contraction.Amyotrophic lateral sclerosis patients showed reduced muscle sum thickness both in relaxed and in contracted states compared with control subjects. Muscle ultrasound of muscles with and without contraction showed excellent diagnostic accuracy for differentiating amyotrophic lateral sclerosis patients from control subjects (area under curve = 0.96, sensitivity: 93%-95%, specificity: 84-87). Muscle ultrasound sensitivity was lower within 6 months of symptom onset (83%) compared with longer disease duration (92%).Quantitative sonographic assessment of muscle thickness can be complementary in the diagnosis of amyotrophic lateral sclerosis with excellent accuracy for differentiating patients from healthy subjects, and might be useful in other neuromuscular disorders, although additional studies are required.

BACKGROUND Periodic acid-Schiff (PAS) staining of nail clippings is an adjunct diagnostic tool for onychomycosis. OBJECTIVE To detect histopathological findings as clues to the presence of PAS-positive (+) fungal elements in nail clippings. METHODS Four hundred sixteen consecutive nail clippings suspected of onychomycosis were stained with hematoxylin and eosin, and with PAS stains. All cases were studied histopathologically. The clinical files of the cases with neutrophils were reviewed. RESULTS PAS+ staining for fungi were demonstrated in 159 (38%) of the nail clippings. Neutrophils, parakeratosis, plasma globules, and bacteria were observed in 43 (27%), 108 (67%), 80 (50%), and 80 (50%) of the PAS+ cases, respectively, and in 17 (6%), 109 (41%), 84 (32%) and 140 (54%) of the PAS- cases, respectively (P < 0.01). Neutrophils showed by far the highest specificity (93%), although with low sensitivity (27%) for the presence of PAS+ fungi. Among the 43 PAS+ and 17 PAS- specimens with neutrophils, only 1 (2.3%) and 3 (17%) had overt psoriasis, respectively. CONCLUSION Neutrophils in nail clippings may serve as a clue for onychomycosis. PAS staining with neutrophils is not necessarily associated with psoriasis.

Neonatal autoimmune subepidermal blistering disease is rare. Mucosal involvement is more common in neonatal linear immunoglobulin A (IgA) bullous dermatosis. We describe a neonate with subepidermal cutaneous blistering disease with severe laryngeal and esophageal involvement leading to acute respiratory distress. Histopathology demonstrated a subepidermal blister with neutrophils and eosinophils at the dermal base. Collagen IV was detected at the dermal floor, and direct immunofluorescence showed linear IgG, IgA, and C3 deposits at the basement membrane zone. The patient demonstrated markedly increased serum levels of anti-BP180 NC16A and anti-BP230 IgG antibodies (Abs) but failed to show anti-LAD-1 IgA Abs. His healthy mother showed serum anti-LAD-1 IgA Abs but did not show anti-BP180 and anti-BP230 Abs. The neonate responded promptly to systemic corticosteroid therapy. A review of the literature detected 11 cases of neonatal subepidermal blistering disease with linear IgA deposits. Nine of these cases demonstrated coexisting linear IgG deposits, often with C3. Respiratory compromise was present in most of the cases. Neutrophils and eosinophils were commonly present in the inflammatory cell infiltrates. Besides our case, 2 cases of neonatal IgG/IgA subepidermal blistering disease with esophageal involvement were previously described. IgA Abs were present in the sera of both cases. Anti-LAD-1 IgA Abs were detected in the mother's serum of our case alone, but IgA Abs do not cross the placenta. Our case was consistent with neonatal IgG/IgA pemphigoid. Neonatal IgG/IgA subepidermal blistering disease may be associated with severe laryngeal and esophageal involvement leading to respiratory compromise. Expedited diagnosis and prompt treatment are warranted.

INTRODUCTION This study was designed to evaluate outcomes in pediatric patients undergoing endoscopic retrograde cholangiopancreatography (ERCP) as compared with an American Society of Gastrointestinal Endoscopy (ASGE) complexity grade-matched adult cohort. METHOD In this retrospective case-control study, ERCPs performed in pediatric patients from January 2008 to December 2018 in 2 tertiary referral hospitals were compared with a complexity-matched adult control group with similar procedural indications. Primary outcomes included the clinical success rate, technical success rate, and complication rate. Secondary outcomes included postprocedural admission rates, mode of sedation, procedure time, fluoroscopy time, hospitalization length, and the number of repeat procedures. RESULTS Two hundred thirty-two ERCPs performed in 110 pediatric patients (average age 13.3) and 318 ERCPs performed in 160 ASGE grade-matched adult controls (average age 47.2 years) were analyzed. All procedures were therapeutic. There was no difference in the technical success rate (P = 0.2), clinical success rate (P = 0.5), complication rates (P = 0.1), and fluoroscopy time (P = 0.4), between the pediatric and adult cohorts. General anesthesia use and length of stay were significantly higher in the pediatric group (P = 0.0001). In subgroup analysis, technical (P = 0.2) and clinical success (P = 0.2) as well as complication rates (P = 0.6) were comparable between patients 10 years or less and patients 11 to 18 years within pediatric cohort. CONCLUSIONS ERCP in pediatric cohorts appears to be safe and effective with equivalent outcomes relative to an ASGE complexity-matched adult cohort. Pediatric patients are more likely to require general anesthesia and have a longer average length of stay relative to adult controls.

PURPOSE OF REVIEW The aim of this review is to understand why and how to improve the detection rate of the fetal anomalies in the first trimester. RECENT FINDINGS The role of the first trimester is evolving from aneuploid and basic anatomical screening to a necessary tool to identifying early fetal malformations. In the past 20 years, several studies were conducted to assess the detection rate of anomalies in the first trimester, and despite the improvement in the diagnoses of spina bifida and cleft lip, many anomalies detectable in the first trimester are still being diagnosed in the second trimester scan. Analyzing the factors that improve the detection rate can show the reason why, even in recent years, only 50% of the anomalies are diagnosed in the first trimester. SUMMARY Thanks to the improvement in the ultrasound technology, the fetus can be thoroughly studied even in early gestation and it is, therefore, indispensable to increase the detection rate of the anomalies diagnosable from the first trimester. The sonographer's utmost attention should be applied to all pregnancies, not only in high-risk patients, and new protocols based on the mid trimester scan are needed to spread the concept of first trimester anatomical scan.

The development of T-cell lymphomas, granulomatous reactions, and autoimmunity has been observed in immunodeficiency due to milder forms of recombination activating gene (RAG) deficiency. A few cases of cutaneous clonal papulonodular CD8 lymphocytic infiltrates and cutaneous CD8 granulomatous T-cell lymphoma have been described in association with common variable immunodeficiency, and with X-linked agammaglobulinemia. We describe a 15-year-old girl with several autoimmune disorders and recurrent infections that presented with several nodules on her cheek. Histopathological studies demonstrate histological, immunohistochemical, and molecular findings compatible with a primary cutaneous clonal CD8 T-cell lymphoproliferative disorder. Vacuolar interface changes were also seen in the involved skin, reminiscent of cutaneous lupus erythematosus. Molecular genetic analysis revealed a germline novel homozygous missense mutation in RAG1 (T1003>C). The parents were heterozygous carriers. The facial cutaneous lesions recurred despite local radiation therapy. Because of recurrent life-threatening systemic infections, allogeneic bone marrow transplantation was performed. The pathogenesis of this primary cutaneous clonal CD8 T-cell lymphoproliferative disorder may have been related to a chronic stimulation of autoreactive T cells in the involved skin paired with reduced RAG1 activity.

Hypothesis This study evaluates the morphological changes of the tectorial membrane (TM) in conjunction with degeneration of hair cells, interdental cells, and presence of endolymphatic hydrops (EH) in sensorineural hearing loss (HL) in the human using histopathology techniques. Background The TM plays an important role in mechanical transduction of acoustic energy, and pathology of the TM may result in HL. Methods All temporal bone (TB) specimens from the Massachusetts Eye and Ear Otopathology Laboratory from patients with various causes of sensorineural HL and morphological abnormalities of the TM were evaluated. Cases with a history of cochlear trauma (other than acoustic trauma) and/or severe postmortem artifacts were excluded. The TBs were processed histologically, and the status of hair cells, supporting cells, interdental cells, presence of EH, and postmortem time were tabulated. Results Two thousand two hundred ninety TBs from 1340 individuals were evaluated, and 164 of 748 TBs from the otological disorders in which the TM were abnormal, met the inclusion criteria. The most common disorders were idiopathic sudden deafness (57.1%), genetic etiology (53.7%), and ototoxicity (40.0%), as compared with cases with presbycusis (2.9%). EH was found in 33.3% of all cases with an identified abnormality of the TM.Abnormalities of the TM were 1) deformed, 2) shrunken, 3) detached from the limbus, 4) encapsulated, or 5) missing. Encapsulated, shrunken and missing patterns (36, 35, 31%, respectively) were the most common. Conclusion A relative high prevalence of EH among disorders with TM abnormalities suggests a possible common pathophysiology in both. In addition, anatomic abnormalities of the TM may play a role in the pathophysiology of HL in these disorders.

Pigmented contact dermatitis (PCD) is a noneczematous variant of allergic contact dermatitis, and benzyl salicylate is one of its causes. This type of PCD shows nonlichenoid interface dermatitis with pigment incontinence. We aimed to characterize the earliest histopathological changes of this reaction. A 51-year-old man presented with persistent facial eruption composed of hyperpigmented and hypopigmented macules due to exposure to benzyl salicylate present in his aftershave. The biopsies obtained from hyperpigmented and hypopigmented macules, and from the positive patch test site to benzyl salicylate, showed a nonlichenoid focal vacuolar interface dermatitis with mononuclear cells in the papillary dermis and around the pilosebaceous units, along with melanophages. A MART-1 immunostain showed intact melanocytes in all 3 biopsies. A Fontana-Masson stain demonstrated intact melanin in the basal cell layer of a facial hyperpigmented macule and the patch test site, but melanin was reduced in the biopsy taken from a hypopigmented facial macule. There were more epidermal and dermal CD1a+ Langerhans cells in the patch test biopsy than in the other 2 biopsies. Most of the mononuclear cells were CD3+. The CD4+ to CD8+ ratio was approximately 1:1 in the facial macules; yet, CD4+ cells outnumbered CD8+ cells in the patch test biopsy. There were a few TIA-1+ cells in all 3 biopsies. In conclusion, the earliest histopathological and immunophenotypical events in PCD due to benzyl salicylate are similar to those of longer-standing lesions, i.e., a nonlichenoid focal interface dermatitis involving the epidermis and pilosebaceous unit, along with dermal melanophages.

Cutaneous T-cell lymphomas are very rare in children. Although mycosis fungoides is the most common of these rare cutaneous T-cell lymphomas in children, transformation to an aggressive malignancy remains extremely uncommon, and there are no clear guidelines for clinical management in the pediatric population. In addition, the increased usage of next-generation sequencing for pediatric patients with unusual malignancies may result in the discovery of pathogenic germline mutations, though the association between these mutations and the patient's cancer is not always clear. We present here a unique pediatric case of transformed mycosis fungoides in a patient with BRCA2 mutation.

Objective Audiometric outcomes following cochlear implantation (CI) in patients with temporal bone fracture (TBF) are variable and the pathophysiology is not well understood. Herein, we describe otopathologic findings in two such cases to provide insight into pathophysiology and outcomes. Patients Two patients had a history of head trauma resulting in bilateral TBF and sudden, profound, sensorineural hearing loss. Intervention Both cases underwent unilateral CI following head trauma. Main outcome measures Both TBs from each patient were harvested postmortem and histologically analyzed to determine fracture lines, intralabyrinthine fibroosseous changes, and spiral ganglion neuron (SGN) counts. Clinical histories and audiometric outcomes were also reviewed. Results In both cases, the implanted TB showed extensive intracochlear ossification, which was greater when fracture involved the cochlea. In contrast, the nonimplanted cochleae were patent with small osseous deposits at the round window membrane. Interaural SGN counts were lower on the implanted side and hearing results were moderate to poor. Conclusions Otopathologic analyses of patients with a CI after bilateral TBF are rare. Based on otopathology of two patients with bilateral TBF and subsequent CI, implanted TBs show extensive intracochlear fibroosseous changes greater than the nonimplanted side. Findings have implications for auditory rehabilitation after TBF.

Hypocomplementemia has been reported in patients with rheumatoid arthritis treated with tocilizumab (TCZ), but its long-term consequences are unknown. We assessed the long-term outcome of patients treated with TCZ who developed hypocomplementemia regarding serious bacterial infections or autoimmune diseases (AID).The charts of patients treated with TCZ at two rheumatology centers were reviewed retrospectively. Data regarding patients' age, gender, disease duration, autoantibodies status, previous or concomitant treatments, blood counts, liver enzymes, C3 and C4 levels at baseline and during TCZ treatment, episodes of infections, allergic reactions, and AID were analyzed. Univariate analysis was used to compare patients with low C3, C4 levels versus patients with normal C3, C4 levels. Variables that were statistically significant associated or tended to be associated with low C3 or C4 were included in multiple variable logistic regression.Of 132 patients treated with TCZ, 108 had serial measurements of serum complement concentration. Thirty-three (30%) patients developed low C4 levels and 23 (21%) had also low C3. Mean TCZ treatment period was 4.9 years (range, 1-14 years). All patients had normal complement levels at baseline. Leukopenia occurred in 18 (16.7%) patients, 14 of whom (77%) had low complement. Persistent leukopenia was observed in 8% and 5.3% of patients with normal C3 and C4 levels, respectively, as opposed to 47% and 42% of patients with low C3 or low C4, respectively. Low C3, C4 levels correlated with prolonged TCZ treatment retention time and effectiveness. There were no serious bacterial infections or new onset AID.Hypocomplementemia during TCZ treatment was accompanied by leukopenia that correlated with treatment duration. Hypocomplementemia was not associated with serious bacterial infections or new onset AID. Decreased complement levels were associated with treatment longevity. The role of monitoring complement level in predicting treatment response or assessing disease activity deserves further investigation.

Migraine-type photophobia, most commonly described as exacerbation of headache by light, affects nearly 90% of the patients. It is classified as one of the most disabling symptoms that accompany an attack. Using subjective psychophysical assessments, we showed recently that migraine patients are more sensitive to all colors of light during the ictal than during the interictal phase and that control subjects do not experience pain when exposed to different colors of light. Based on these findings, we suggested that color preference is unique to migraineurs (as it was not found in control subjects) rather than migraine phase (as it was found in the ictal and interictal phases). To identify the origin of this photophobia in migraineurs, we compared the electrical waveforms that were generated in the retina and visual cortex of 46 interictal migraineurs to those generated in 42 healthy controls using color-based electroretinography and visual evoked potential paradigms. Unexpectedly, it was the amplitude of the retinal rod-driven b-wave, which was consistently larger (by 14-19% in the light-adapted and 18-34% in the dark-adapted flash ERG) in the migraineurs than in the controls, rather than the retinal cone-driven a-wave or the visual evoked potentials that differ most strikingly between the two groups. Mechanistically, these findings suggest that the inherent hypersensitivity to light among migraine patients may originate in the retinal rods rather than retinal cones or the visual cortex. Clinically, the findings may explain why migraineurs complain that the light is too bright even when it is dim to the extent that non-migraineurs feel as if they live in a cave.

INTRODUCTION AND OBJECTIVE:Patients with secondary extrinsic malignant ureteral obstruction (EMUO) seek for durable, simple and low-cost solutions of drainage. We report on long-term outcomes with ...

Background Several cases of pityriasis lichenoides (PL) have been reported to evolve into mycosis fungoides (MF). Objective To elucidate clues to this progression. Methods Fifty-eight patients with PL between 2000 and 2013 (follow-up: 3-16 years, average: 8.3). Results A total of 3 (5.2%) of the 58 patients with PL developed MF after 3-11 years of prolonged clinical course. Papules and small plaques characterized PLs, and patches and larger plaques subsequent MFs. A total of 35 of 41 (85%) followed up non-MF associated patients with PL reported lasting complete remissions. Histopathologically, apoptotic keratinocytes disappeared mostly or completely in subsequent MFs. The presence of epidermotropism, folliculotropism, and epidermal lymphocytic nuclear atypia in PLs was not predictive of MF. CD8 cells were the dominant intraepidermal lymphocytes in the 3 PLs but remained so in only 1 subsequent MF. CD7 lymphocytes decreased substantially in 2 MFs, and lymphocytic nuclear atypia increased markedly in 1. T-cell receptor gene rearrangement studies demonstrated clonal populations in 1 of 2 studied PLs and in all 3 subsequent MFs. Conclusions A few PLs may evolve into MF. Prolonged clinical course, appearance of patches and larger plaques, markedly increased lymphocytic nuclear atypia, marked diminution of apoptotic keratinocytes and CD7 and CD8 lymphocytes, and clonal T-cell receptor gene rearrangement may serve as clues.

Pseudolymphomatous reactions have been described to occur in tattoos. Most cases have exhibited T-cell predominance and polyclonal T-cell receptor gene rearrangements. One case with monoclonal IgH gene rearrangements progressed into B-cell lymphoma. Lichenoid infiltrates are commonly described but lymphoid follicles much less frequently. We report a case with mixed lichenoid and follicular T- and B-cell reaction to red tattoos. The histopathology and the immunohistochemical studies were constant with a mixed T- and B-cell pseudolymphoma, the IgH gene rearrangement study was polyclonal, but the T-cell receptor gene rearrangement study was monoclonal. The patient who responded to intralesional corticosteroid injections remains under close scrutiny.

Background Histopathology plays an important role in the diagnosis of cutaneous leishmaniasis (CL) but Leishman-Donovan (LD) bodies may not always be discernible. Recently, anti-CD1a antibody (Ab), clone MTB1, was found to decorate LD bodies immunohistochemically. Objective Can histopathology without discernible LD bodies be used to diagnose CL, and can immunohistochemistry using anti-CD1a Ab, clone MTB1, detect LD bodies in these cases. Methods Suspected CL lesions were studied histopathologically and immunohistochemically, and the patients' clinical files were reviewed. Results Of the 196 patients with suspected CL, direct smear demonstrated LD bodies in 50 (25.5%). Of the remaining 146 patients, 118 underwent biopsy. In 56 (47.5%) patients, the hematoxylin-eosin-stained sections revealed LD bodies. In 47 (39.8%) patients, LD bodies were not discerned but the histopathology demonstrated histiocytic infiltrates with varying numbers of plasma cells along with other inflammatory cells, and negative Ziehl-Neelsen and periodic acid-Schiff stains. This pattern was termed "histopathology consistent with leishmaniasis." The history, clinical findings, and response to anti-leishmania therapy supported the diagnosis of CL in all of them, and immunostains for CD1a, clone MTB1, detected LD bodies in 11 (23.4%) of these 47 patients. Conclusions "Histopathology consistent with CL" along with appropriate clinical findings supports the diagnosis of CL in an endemic area, and immunostains with CD1a Ab, clone MTB1, may help in the minority of the cases.

Objectives 1) To evaluate the long-term incidence and degree of the sensorineural component of hearing loss (SNHL) in patients with otosclerosis after accounting for expected age-related hearing loss. 2) To identify variables that might predict development of sensorineural hearing loss due to otosclerosis. Study design Retrospective audiometric database and chart review. Setting Tertiary referral center. Patients Consecutive patients with otosclerosis observed between 1994 and 2004, with ≥10 years follow-up, excluding patients with postoperative hearing loss or surgery before the initial audiogram. Intervention Bone conduction (BC) thresholds at 0.5, 1, 2, and 4 kHz and Word Recognition. Main outcome measure BC threshold change (BCTC) over ≥10 years minus estimated age-related threshold change (ARTC) specific to age and sex for each patient (based on ISO 7029 reference population). Results Three-hundred fifty-seven ears (290 patients) met study criteria, including 217 ears that had undergone stapedectomy during the study period. Mean follow-up was 14.0 years. The average BCTC after subtracting estimated ARTC was 4.6, 2.6, 3, and 2.7 dB for 0.5, 1, 2, and 4 kHz frequencies, respectively. However, 34% of ears (122 ears) had clinically significant progression of SNHL during the study period (>10 dB BCTC beyond expected ARTC at ≥2 frequencies). Multivariate analysis demonstrated that the probability of developing clinically significant SNHL was higher for women (odds ratio 1.86, p = 0.018) and lower for operated patients (odds ratio 0.46, p = 0.002). Conclusion The average long-term sensorineural hearing loss due to otosclerosis was statistically significantly more than for age alone at each frequency, but these average values (from 2.6 to 4.6 dB for tested frequencies) were clinically insignificant. Approximately one-third of patients with otosclerosis demonstrated a clinically significant progression of the sensorineural component of hearing loss, with the average BCTC above expected age-related changes ranging from 10.2 to 14.6 dB for tested frequencies among this subgroup.

INTRODUCTION AND OBJECTIVES:To the best of our knowledge the physical effort of a resident during a working shift has not been systematically assessed yet. Today, smartphones are widely used for be...

Demodex is a saprophytic mite in humans commonly present in the pilosebaceous units, which has been implicated as a pathogen in several skin conditions. The clinical presentation and histopathology of Demodex folliculitis of the scalp have been described in only a few case reports. This study was performed to further elucidate the clinicopathological features of this entity. We have studied 333 consecutively submitted scalp biopsies performed for hair loss and alopecia. All specimens were completely step-sectioned. Biopsies with Demodex mites were further studied histopathologically, and the patients' clinical files were reviewed. There were 17 biopsies (5.1%) with Demodex in at least 1 pilosebaceous unit. Based on the clinical presentation, histopathology, and response to therapy, Demodex was considered to be nonpathogenic in 13 cases. The remaining 4 cases were characterized by hair loss, scalp erythema, scales, and pustules. There were 2 or more pilosebaceous units with Demodex along mononuclear and/or neutrophilic infiltrates around and in the involved follicles and occasionally granulomas. All 4 cases responded completely to metronidazole therapy. In conclusion, Demodex is infrequently found in scalp biopsies for hair loss and alopecia, and, in most cases, it does not seem to be pathogenic. Occasionally, however, it is associated with folliculitis characterized by hair loss, erythema, scales, and pustules clinically; neutrophilic and/or mononuclear-cell folliculitis with occasional granulomas histopathologically; and a prompt response to anti-Demodex therapy.

OBJECTIVES 1) To evaluate the long-term (≥10 year) clinical incidence of endolymphatic hydrops (EH) after stapedectomy for otosclerosis, using low-frequency sensorineural hearing loss (LFSNHL) as a marker for EH. 2) To determine the histologic incidence of EH in human temporal bone specimens (TBS) with a history of stapedectomy for otosclerosis. 3) To determine the histologic incidence of EH in a control group of human TBS. STUDY DESIGN Retrospective review and temporal bone study. SETTING Tertiary medical center and temporal bone pathology laboratory. PATIENTS Patients with otosclerosis, human TBS with otosclerosis, and human TBS with presbycusis as the control group. INTERVENTION Pure-tone audiometry, temporal bone pathology. MAIN OUTCOME MEASURES 1) LFSNHL, defined as >10 decibel elevation of bone conduction thresholds at 250 and 500 Hz, after correcting for age-related hearing loss (per ISO 7029). 2) Histologic assessment of EH. RESULTS In patients with otosclerosis, 8 of 110 (7.3%) operated patients versus 3 of 123 (2.4%) nonoperated patients developed LFSNHL (p = 0.08). No patients with LFSNHL had other symptoms of EH. In TBS with otosclerosis, 11 of 93 (11.8%) operated TBS versus 3 of 156 (1.9%) nonoperated TBS had evidence of EH (p

Bicycling is gaining popularity in the United States, and laws and safety recommendations are being established to keep bicyclists safer. To improve road safety for bicyclists, there is a need to characterize their compliance with road laws and safety behaviors. Adult bicyclists were observed at three high-traffic intersections in Boston, MA, with state recommendations of wearing a helmet and riding in a bike lane. State law compliance for displaying reflectors during the day and of a front light and a rear light/reflector at night, obeying traffic signals, and giving pedestrians the right of way was also observed. Variables were compared between personal and shared/rented bicyclists and analyzed by time of day. A total of 1,685 bicyclists were observed. Because of the speed of the bicyclists and obstructed views, only a sampling of 802 bicyclists was observed for reflectors/front light. Overall, 74% wore a helmet, 49% had reflectors/front lights, 95% rode in bike lanes, 87% obeyed traffic signals, and 99% gave the right of way to pedestrians. Compared with shared bicyclists (n = 122), personal bicyclists (n = 1563) had a higher helmet-wearing behaviors (77% vs. 39%, p = .0001). Shared bicyclists had a higher (p = .0001) compliance with reflectors/lights (100%) than personal bicyclists (39%, n = 265). Boston bicyclists ride in bike lanes, obey traffic signals, give pedestrians the right of way, and wear helmets while having suboptimal compliance with light/reflector use. Educational programs and stricter law enforcement aimed at these safety behaviors should be part of the effort to improve safety for all road users.

Herpes simplex virus (HSV) is rarely the cause of pneumonia in immunocompetent patients. We describe a previously healthy child, with no evidence of an immunodeficiency, who presented to the emergency department with severe pneumonia, wheezing, and pleural effusions with a history of orolabial HSV infection. On admission, he was started on antibiotics and systemic corticosteroids but continued to deteriorate. Oral lesions, blood, and pleural fluid tested positive for HSV, and improvement was achieved only after the addition of acyclovir and discontinuation of steroids. We suggest that steroids should be used with caution in patients presenting with lower respiratory tract symptoms and herpetic oral lesions.