Characterization of otologic involvement in patients with X‐Linked Hypophosphatemia

xlomafota13 shared this article with you from Inoreader Message: DOI:10.1007/s00431-004-1504-zCorpus ID: 9983916 Hearing impairment in familial X-linked hypophosphatemic rickets G. Fishman, Denise R. Miller-Hansen, +2 authors U. Alon Published 2004 Medicine European Journal of Pediatrics Hearing impairment in patients with X-linked dominant hypophosphatemic rickets (XLH) is likely part of the natural history of the disease, developing during adulthood. Therefore, whereas close follow up and hearing monitoring is recommended in adults, serial audiograms in children with XLH are not justified. Furthermore, in a child with XLH and hearing impairment, other etiologies should be explored. Hearing impairment in XLH patients has been reported a few times in the 1970s and 1980s [1, 2, 4, 5]; however, it was our impression that our pediatric XLH patients did not manifest clinical hearing impairment. Therefore, in order to better clarify the association between XLH and hearing status, and consequently provide information regarding patients' management, we prospectively evaluated the hearing status in our XLH patients and their afflicted parents. Hearing evaluations including audiometry, tympanometry and stapedial reflex thresholds recording were performed in 16 children with XLH (age 1–18 years, median 9.0 years, six males) and all of their ten afflicted parents (age 22–55 years, three male). None had a history of treatment with ototoxic drugs, noise exposure, previous ear surgery or chronic ear disease. Of 16 children, 15 demonstrated normal hearing. One with bilateral profound hearing loss was found to have a Mondini congenital inner ear malformation, documented by a temporal bone CT scan. Two adult males and one female, all with severe XLH-related orthopedic manifestations, demonstrated sensorineural hearing loss. More than two decades passed since hearing impairment was first described in XLH [5]. Table 1 summarizes the main findings of previous studies that have reported on this association, showing evidence of hearing impairment in a significant percentage of adult XLH patients versus minimal incidence of hearing loss in children [1, 2, 4, 5]. We compared the hearing status in a relatively large group of XLH children in a wide range of ages w ith that of their afflicted parents. None of our children had a hearing impairment attributable to XLH. The only child with hearing impairment was the one with a Mondini inner ear malformation which is believed to result from a developmental arrest of the membranous labyrinth during the first trimester of pregnancy [3], and is not known to be associated with rickets which is a post-natal disease. In contrast to the normal hearingwe found in ourXLH children, three of their ten parents had sensorineural hearing loss. These patientswere under 52 years old; hence presbyacusis was ruled out. Since in all three etiologies other than the disease process were excluded and in the light of the fact that all had severe orthopedic manifestations of rickets, we suspect that their hearing impairment is part of the disease process. They were referred to adult otolaryngologists for further management. Even though two of the earlier studies [2, 5] found hearing impairment in a very few children, lat er studies which investigated much larger cohorts of children [1, 4] as well as ours, did not find evidence of XLH-related hearing impairment in childhood. Therefore, it cannot G. Fishman Department of Pediatric Otolaryngology, ''Dana'' Children's Hospital, Tel-Aviv Medical Center, ''Sackler'' School of Medicine, Tel-Aviv University, Tel-Aviv, Israel Characterization of otologic involvement in patients with X‐Linked Hypophosphatemia Via Latest Issue of Clinical Otolaryngology RSS Feed View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Radioresistance and genomic alterations in head and neck squamous cell cancer: A sub‐analysis of the ProfiLER protocol

xlomafota13 shared this article with you from Inoreader Radioresistance and genomic alterations in head and neck squamous cell cancer: A sub‐analysis of the ProfiLER protocol Via Head & Neck Abstract Background Genome analysis could provide tools to assess predictive molecular biomarkers of radioresistance. Methods Head and neck squamous cell carcinoma patients included in ProfiLER study and who underwent a curative radiotherapy were screened. Univariate and Cox multivariate analyses were performed to explore the relationships between molecular abnormalities, infield relapse and complete tumor response after radiation. Results One hundred and forty-three patients were analyzed. PIK3CA mutation and genomic instability of MAP kinases pathway were found to be prognostic factors of loco-regional relapse in multivariate analysis with respectively HR 0.33, 95% CI 0.13–0.83, p = 0.005 and HR 0.61, 95% CI 0.38–0.96, p = 0.025. Instability of apoptosis pathway was found to be a prognostic factor of complete response after radiotherapy with HR 0.24, 95% CI 0.07–0.88, p = 0.04. Conclusion This sub analysis suggests that PIK3CA mutation, variation of copy number of MAP kinases and apoptosis pathways play a significant role in the radioresistance phenomenon. View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Transcriptome Analysis Identified 2 New lncRNAs Associated with the Metastasis of Papillary Thyroid Carcinoma

xlomafota13 shared this article with you from Inoreader Transcriptome Analysis Identified 2 New lncRNAs Associated with the Metastasis of Papillary Thyroid Carcinoma Via O.R.L. Journal for oto-rhino-laryngology and its borderlands Introduction: Papillary thyroid microcarcinoma (PTMC) is a specific subgroup of papillary thyroid carcinoma and defined with the dimension ≤1 cm by the WHO. Although it shows a relatively high 10-year livability, the metastasis of PTMC into other tissues and organs seriously affects the daily life of patients with relatively high mortality. Therefore, the genetic basis for the metastasis of PTMC needs to be explored for effective therapeutic targets. Here, we conducted a series of comparati ve analysis of the transcriptional expression profile between PTMC patients with and without lymph node metastasis. Methods: Gene expression profile and gene function were analyzed using RNA extracted from pathological tissues of 12 patients with PTMC, and the core biomarkers closely related to its metastasis were identified. Results: Our results showed that 7,507 genes and 42 RNAs showed remarkably different expression patterns. More sophisticated analysis showed that the high expression of 2 lncRNAs (T077499 and T004533) resulted in the metastasis of PTMC, which suggests that the expression pattern of the 2 lncRNAs may act as a potential biomarker for pathogenesis and prognosis of PTMC metastasis. Conclusion: Our findings preliminarily reveal the molecular mechanisms for PTMC metastasis, which will provide vital reference for subsequent studies about the genetic basis and molecular targeted therapy for PTMC metastasis. ORL View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Sentinel lymph node biopsy in high-risk cutaneous squamous cell carcinoma of the head and neck

xlomafota13 shared this article with you from Inoreader Sentinel lymph node biopsy in high-risk cutaneous squamous cell carcinoma of the head and neck Via Journal of plastic, reconstructive & aesthetic surgery : JPRAS J Plast Reconstr Aesthet Surg. 2021 Sep 17:S1748-6815(21)00431-9. doi: 10.1016/j.bjps.2021.08.041. Online ahead of print. ABSTRACT INTRODUCTION: Cutaneous squamous cell carcinoma (cSCC) shows malignant behaviour in 3-4% of patients with locoregional metastases and a poor prognosis, metastases that are difficult to predict clinically. Therefore, sentinel lymph node biopsy (SLNB) has been assessed, with contradictory findings thus far. We aimed to clarify the prognostic value of SLNB in high-risk cSCC patients. PATIENTS AND METHODS: We completed a retrospective clinical study amongst 63 patients, preoperatively classified as N0 with a high-risk primary cSCC of the head and neck who underwent SLNB between 2001 and 2014 at Helsinki University Hospital (Finland). Considered high risk, the inclusion criteria comprised at least two of the following characteristics: tumour diameter ≥10 mm and/or thickness ≥4 mm and a specific tumour location, such as the lips, ear, scalp and central face. Patients were followed-up postoperatively for a median of 4.1 years (0.2-13.8 years). RESULTS: Only four (6.3%) patients had positive sentinel nodes. One of these patients died of cSCC, while the other three ultimately survived their disease. Five (7.9%) patients showed a negative SLNB, but developed recurrence within one year postoperatively. Recurrence appeared in the neck lymph nodes concurrently with locoregional soft-tissue invasion in all patients. Amongst these patients, three died for cSCC and the remaining two from other causes. Comparing the SLNB-positive and SLNB-negative groups with recurrence, we identified no significant differences in terms of patient or tumour characteristics. CONCLUSIONS: SLNB appears to carry no prognostic value for identifying recurrent disease amongst high-risk cSCC in the head and neck area. PMID:34645585 | DOI:10.1016/j.bjps.2021.08.041 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Factors associated with success following transcervical innominate artery suspension

xlomafota13 shared this article with you from Inoreader Message: Innominate artery compression of the trachea: diagnosis and treatment by anterior suspension. A 25-year experience S C Adler 1, G Isaacson, R K Balsara Affiliations collapse Affiliation 1Department of Otolaryngology-Head and Neck Surgery, Temple University School of Medicine, Philadelphia, Pennsylvania, USA. PMID: 7492062 DOI: 10.1177/000348949510401202 Abstract Suspension of the innominate artery to the sternum has been a widely accepted therapy for the relief of tracheal compression. Recently, reimplantation of the innominate artery has been advocated as a superior operative procedure. While generally successful, arterial transfer carries the risk of early bleeding and stroke, and the potential for late stenosis at the anastomotic site. Between 1969 and 1994, 25 infants and children at our institution received diagnoses of innominate artery compression and were treated by anterior suspension. All presented with stridor and one third had a history of suspected or proven apnea. Twenty-four children had excellent results, while 1 required resuspension after stridor returned. There were no major complications. Our series strongly supports the belief that anterior suspension of the innominate artery is a successful and reliable operation with minimal morbidity and mortality. More complex procedures are rarely indicated. Factors associated with success following transcervical innominate artery suspension Via International Journal of Pediatric Otorhinolaryngology Publication date: Available online 13 October 2021 Source: International Journal of Pediatric Otorhinolaryngology Author(s): Douglas Clayton von Allmen, Cherie Torres-Silva, Michael J. Rutter View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

The PRO-ACTIVE trial protocol: a randomized study comparing the effectiveness of PROphylACTic swallow InterVEntion for patients receiving radiotherapy for head and neck cancer

xlomafota13 shared this article with you from Inoreader The PRO-ACTIVE trial protocol: a randomized study comparing the effectiveness of PROphylACTic swallow InterVEntion for patients receiving radiotherapy for head and neck cancer Via swallowing exercises BMC Cancer. 2021 Oct 13;21(1):1100. doi: 10.1186/s12885-021-08826-0. ABSTRACT BACKGROUND: Swallowing therapy is commonly provided as a treatment to lessen the risk or severity of dysphagia secondary to radiotherapy (RT) for head and neck cancer (HNC); however, best practice is not yet established. This trial will compare the effectiveness of prophylactic (high and low intensity) versus reactive interventions for swallowing in patients with HNC undergoing RT. METHODS: This multi-site, inter national randomized clinical trial (RCT) will include 952 adult patients receiving radiotherapy for HNC and who are at high risk for post-RT dysphagia. Participants will be randomized to receive one of three interventions for swallowing during RT: RE-ACTIVE, started promptly if/when dysphagia is identified; PRO-ACTIVE EAT, low intensity prophylactic intervention started before RT commences; or, PRO-ACTIVE EAT+EXERCISE, high intensity prophylactic intervention also started before RT commences. We hypothesize that the PRO-ACTIVE therapies are more effective than late RE-ACTIVE therapy; and, that the more intensive PRO-ACTIVE (EAT + EXERCISE) is superior to the low intensive PRO-ACTIVE (EAT). The primary endpoint of effectiveness is duration of feeding tube dependency one year post radiation therapy, selected as a pragmatic outcome valued equally by diverse stakeholders (e.g., patients, caregivers and clinicians). Secondary outcomes will include objective measures of swallow physiology and function, pneumonia and weight loss, along with various patient-reported swallowing-related outcomes, such as quality of life, symptom burden, and self-efficacy. DISCUSSION: Dysphagia is a common and potentially life-threatening chronic toxicity of radiotherapy, and a priority issue for HNC survivors. Yet, the optimal timing and intensity of swallowing therapy provided by a speech-language pathologist is not known. With no clearly preferred strategy, current practice is fraught with substantial variation. The pragmatic PRO-ACTIVE trial aims to specifically address the decisional dilemma of when swallowing therapy should begin (i.e., before or after a swallowing problem develops). The critical impact of this dilemma is heightened by the growing number of young HNC patients in healthcare systems that need to allocate resources most effectively. The results of the PRO-ACTIVE trial will address the global uncertainty regarding best practice for dysphagia management in HNC pat ients receiving radiotherapy. TRIAL REGISTRATION: The protocol is registered with the US Patient Centered Outcomes Research Institute, and the PRO-ACTIVE trial was prospectively registered at ClinicalTrials.gov , under the identifier NCT03455608 ; First posted: Mar 6, 2018; Last verified: Jun 17, 2021. Protocol Version: 1.3 (January 27, 2020). PMID:34645411 | DOI:10.1186/s12885-021-08826-0 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Association of Performance on Dichotic Auditory Tests With Risk for Incident Dementia and Alzheimer Dementia

xlomafota13 shared this article with you from Inoreader Message: Dichotic Dysaudia occurs when both ears are weak at transferring auditory information to the correct brain hemisphere. A child with either of these deficits will struggle to hear in a noisy classroom and will generally have poorer attention than their peers. Auditory processing is a term used to describe what happens when your brain recognizes and interprets the sounds around you. Humans hear when energy that we recognize as sound travels through the ear and is changed into electrical information that can be interpreted by the brain. Dichotic listening is the auditory process that involves listening with both ears. ... Binaural separation is the ability to perceive an acoustic message in one ear while ignoring a different acoustic message in the other ear. Dichotic listening (DL) tests are among the most frequently included in batteries for the diagnosis of auditory processing disorders (APD) in children. A finding of atypical left ear advantage (LEA) for speech-related stimuli is often taken by clinical audiologists as an indicator for APD. Dichotic listening is a psychological test commonly used to investigate selective attention and the lateralization of brain function within the auditory system. It is used within the fields of cognitive psychology and neuroscience. Key Points Question Is the association between poor hearing and dementia associated with signal sensitivity, central auditory processing, or both? Findings In this prospective cohort study of 280 adults without dementia, measures of dichotic auditory processing were associated with increased dementia and Alzheimer dementia risk independently of signal sensitivity. Measures of signal audibility had a weak and insignificant association with dementia risk, and that association disappeared when controlling for dichotic auditory processing. Meaning The associations between poor hearing and dementia risk may be more specific to dichotic processing than signal audibility or monaural speech tests. Abstract Importance Age-related hearing difficulties can include problems with signal audibility and central auditory processing. Studies have demonstrated associations between audibility and dementia risk. To our knowledge, limited data exist to determine whether audibility, central processing, or both drive these associations. Objective To determine the associations between signal sensitivity, central auditory processing, and dementia and Alzheimer dementia (AD) risk. Design, Setting, and Participants This follow-up observational study of a sample from the prospective Adult Changes in Thought study of dementia risk was conducted at Kaiser Permanente Washington, a western Washington health care delivery system, and included 280 volunteer participants without dementia who were evaluated from October 2003 to February 2006 with follow-up through September 2018. Analyses began in 2019 and continued through 2021. Exposures Hearing tests included pure tone signal audibility, a monaural word recognition test, and 2 dichotic tests: the Dichotic Sentence Identification (DSI) test and the Dichotic Digits test (DDT). Main Outcomes and Measures Cognition was assessed biennially with the Cognitive Abilities Screening Instrument (range, 1-100; higher scores are better), and scores of less than 86 prompted clinical and neuropsychological evaluations. All data were reviewed at multidisciplinary consensus conferences, and standardized criteria were used to define incident cases of dementia and probable or possible AD. Cox proportional hazard models were used to determine associations with hearing test performance. Results A total of 280 participants (177 women [63%]; mean [SD] age, 79.5 [5.2] years). As of September 2018, there were 2196 person-years of follow-up (mean, 7.8 years) and 89 incident cases of dementia (66 not previously analyzed), of which 84 (94.4%) were AD (63 not previously analyzed). Compared with people with DSI scores of more than 80, the dementia adjusted hazard ratio (aHR) for DSI scores of less than 50 was 4.18 (95% CI, 2.37-7.38; P < .001); for a DSI score of 50 to 80, it was 1.82 (95% CI, 1.10-3.04; P = .02). Compared with people with DDT scores of more than 80, the dementia aHR for DDT scores of less than 50 was 2.66 (95% CI, 1.31-5.42; P = .01); for a DDT score of 50 to 80, it was 2.40 (95% CI, 1.45-3.98; P = .001). The AD results were similar. Pure tone averages were weakly and insignificantly associated with dementia and AD, and associations were null when controlling for DSI scores. Conclusions and Relevance In this cohort study, abnormal central auditory processing as measured by dichotic tests was independently associated with dementia and AD risk. Association of Performance on Dichotic Auditory Tests With Risk for Incident Dementia and Alzheimer Dementia Via JAMA Otolaryngology–Head & Neck Surgery Online First This cohort study examines associations between signal sensitivity, central auditory processing, and dementia and Alzheimer dementia risk. View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Association of Bone Conduction Devices for Single-Sided Sensorineural Deafness With QOL

xlomafota13 shared this article with you from Inoreader Association of Bone Conduction Devices for Single-Sided Sensorineural Deafness With QOL Via JAMA Otolaryngology–Head & Neck Surgery Online First This systematic review and meta-analysis assesses quality of life measures among patients with single-sided sensorineural deafness who received a unilateral bone conduction device. View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Prophylactic Steroids for Postthyroidectomy Hypocalcemia and Voice Dysfunction

xlomafota13 shared this article with you from Inoreader Prophylactic Steroids for Postthyroidectomy Hypocalcemia and Voice Dysfunction Via JAMA Otolaryngology–Head & Neck Surgery Current Issue This randomized clinical trial investigates the use of dexamethasone to prevent postoperative hypocalcemia and voice dysfunction in patients undergoing thyroidectomy. View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

The Substantial Omission of Postoperative Radiotherapy in Patients With Advanced-Stage Oral Cancer

xlomafota13 shared this article with you from Inoreader The Substantial Omission of Postoperative Radiotherapy in Patients With Advanced-Stage Oral Cancer Via JAMA Otolaryngology–Head & Neck Surgery Current Issue This cohort study aims to identify factors associated with omission of adjuvant postoperative radiotherapy in patients with advanced-stage oral squamous cell cancers in the US. View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Acute Vision Loss From IgG4-Related and Bacterial Rhinosinusitis After COVID-19

xlomafota13 shared this article with you from Inoreader Acute Vision Loss From IgG4-Related and Bacterial Rhinosinusitis After COVID-19 Via JAMA Otolaryngology–Head & Neck Surgery Current Issue This case report describes a man in his 70s who presented to the emergency department with a headache for 2 weeks and vision loss in the right eye for 2 days and was subsequently diagnosed with IgG4-related and Streptococcus constellatus rhinosinusitis. View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.