Stability of computed tomography densitometry in patients with otosclerosis:a two-year follow-up

Objectives:To quantify the progression of otosclerosis in the unoperated ear between two stapedotomy procedures for patients with bilateral otosclerosis which can help to determine whether a HRCT scan should be re-performed before the second surgery for patients who already received HRCT imaging before the initial surgery.Methods:35 patients who underwent bilateral stapedotomy were included.Two rounds of HRCT examination and audiometry were performed at the time of the first surgery and second surgery on the ear that was not operated on during the initial surgery.The relationship between the changes in HRCT densitometry and audiometry over time was analyzed.Results:The second round of HRCT did not add significant information about the changes to the otosclerosis lesions in either the imaging diagnosis or the HRCT density values except for small changes in the HRCT densitometry readings at the area anterior to the inner auditory(P=0.01).While the changes in HRCT manifestation are small,changes near the fissula ante fenestram(FAF)were still positively correlated with the air bone gap(ABG)of patients(p=0.031,r=0.388).Conclusions:The progression of lesions in otosclerosis is slow resulting in small and insignificant changes to the HRCT features.Therefore,a repeat HRCT evaluations prior to surgery is not necessary for patients who have had a previous HRCT evaluation within 2 years of the operation.The small changes in HRCT manifestation near the FAF were still correlated with negative effects on the ABG which could cause worsened hearing thresholds over this timeframe。

Novel heterozygous USH1C mutation impacts hair cell mechanotransduction and causes progressive hearing loss

Hearing loss is one of the most common sensory disorders worldwide,affecting approximately 466 million people,including 34 million children[1].Genetic mutations accounts for approximately 60%of inherited hearing loss cases[2,3].These genetic changes result in a wide variety of clinical manifestations,ranging from nonsyndromic hearing loss(NSHL)to over 400 syndromes involving hearing loss and from mild adult-onset hearing loss to profound congenital deafness,covering the entire spectrum of Mendelian inheritance.

Bioinspired structural color particles with multi-layer graphene oxide encapsulated nanoparticle components

Because of the unique features of spherical symmetry,angle-independency,good monodispersity,controllable components and morphologies,structural color particles(SCPs)have found great significances in various fields such as sensing,monitoring,biological assays,etc.Here,inspired by the melanosome-derived bright structural colors and the self-adhesivity of mussels,we present a kind of bioinspired SCPs assembled from polydopamine(PDA)-adhered multi-layer graphene oxide(GO)encapsulated silica nanoparticles(SNs).It is demonstrated that compared with traditional SCPs,the designed particles possess brighter and more vibrant structural colors,and no complicated modification is required during the functionalization process due to the abundant inherent functional groups of GO.The resultant SCPs are verified to be capable for direct hybridization chain reaction and multiplexed nucleic acid assays.These properties indicate the promising prospects of our designed SCPs.

Preventing autosomal-dominant hearing loss in Bth mice with CRISPR/CasRx-based RNA editing

CRISPR/RfxCas13d(CasRx)editing system can specifically and precisely cleave single-strand RNAs,which is a promising treatment for various disorders by downregulation of related gene expression.Here,we tested this RNA-editing approach on Beethoven(Bth)mice,an animal model for human DFNA36 due to a point mutation in Tmc1.We first screened 30 sgRNAs in cell cultures and found that CasRx with sgRNA3 reduced the Tmc1^(Bth)transcript by 90.8%,and the Tmc1 wild type transcript(Tmc1^(+))by 44.3%.We then injected a newly developed AAV vector(AAV-PHP.eB)based CasRx into the inner ears of neonatal Bth mice,and we found that Tmc1^(Bth)was reduced by 70.2%in 2 weeks with few off-target effects in the whole transcriptome.Consistently,we found improved hair cell survival,rescued hair bundle degeneration,and reduced mechanoelectrical transduction current.Importantly,the hearing performance,measured in both ABR and DPOAE thresholds,was improved significantly in all ages over 8 weeks.We,therefore,have validated the CRISPR/CasRx-based RNA editing strategy in treating autosomal-dominant hearing loss,paving way for its further application in many other hereditary diseases in hearing and beyond.

The p.(Pro170Leu) variant in NOG impairs noggin secretion and causes autosomal dominant congenital conductive hearing loss due to stapes ankylosis

Conductive hearing loss is the impairment in the mechanical transduction of sound wave through the external ear and the middle ear.Although most cases are sporadic due to acquired causes such as infections(otitis media and otitis externa),cerumen obstruction,and injuries,congenital structural defects are uncommon for significant etiologies to recognize.Stapes ankylosis is characterized by conductive hearing loss.It may be difficult to differentiate from otosclerosis,the most common cause of progressive conductive hearing loss,by audiologic evaluation,when the diagnosis is delayed.Skeletal anomalies may be subtle,such that the syndrome may not be recognized(Brown et al.,2002).

Atypical manifestations of acute coronary syndrome—throat discomfort:a multi-center observational study

To present the clinical characteristics and the misdiagnosis rate of acute coronary syndrome manifested primarily as throat discomfort,we conducted a multicentric and retrospective study in the cardiology and otorhinolaryngology departments.Records of patients with primary complaint of throat discomfort,absence of chest pain at onset,and an ultimate diagnosis of acute coronary syndrome,as well as patients with pharyngitis(as controls)were collected from May 2015 to April 2016.The patients’main manifestations were compared.Logistic regression results showed that chest tightness,dyspnea,perspiring,and exertional throat symptoms were significantly associated with acute coronary syndrome,with odds ratios of 8.3(95%CI 2.2–31.5),10.9(95%CI 1.8–66.9),25.4(95%CI 3.6–179.9),and 81.2(95%CI 13.0–506.7).A total of 25(56.82%)out of 44 acute coronary syndrome patients,who were first admitted to the otorhinolaryngology department,were misdiagnosed,with a 12%(3/25)mortality rate.Throat discomfort can be the principal manifestation of acute coronary syndrome.Such patients exhibit high misdiagnosis and mortality rates.Exertional throat symptoms,chest tightness,perspiring,and dyspnea were important indicators of acute coronary syndrome in patients whose main complaint was throat discomfort.The awareness of this condition will result in prompt diagnosis and reduce morbidity and mortality.

Structural insights into Cas9 mismatch:promising for development of high-fidelity Cas9 variants

The recent research published in Nature by Bravo and colleagues has deeply carifed the mechanisms of Cas9 recognizing the base-pair mismatches by kinetics-guided Cryo-EM analysis,which provides a perspective for the development of next-generation Cas9 variants with high fidelity.