Correlation of semi-quantitative findings of endolymphatic hydrops in MRI with the audiometric findings in patients with Meniere's disease

Purpose:To investigate the correlation between vestibular hydrops(VH),cochlearhydrops(CH),vestibular aqueduct non-visibility(VANV),and visually increased perilymphatic enhancement(VIPE)with the findings of pure-tone audiometry(PTA)in Meniere’s disease(MD)patients.Methods:In this cross-sectional study,53 ears belonging to 48 patients were divided into two groups and evaluated.In group“MD patients,”there were 24 ears of 19 patients diagnosed with the definite MD(14 patients with unilateral and 5 patients withbilateral involvements).The“control group”consisted of 29 non-symptomatic ears belonging to patients diagnosed with unilateral sudden sensory-neural hearing loss or unilateral schwannoma.All the patients underwent 2 sessions of temporal bone MRI using the same 3T system:an unenhanced axial T1,T2,and 3D-FLAIR MRI,an intravenous gadoliniumenhanced axial T1 fat-sat,and 4 h after the injection,an axial 3D-T2 cube and 3D-FLAIR session.VH,CH,VANV,and VIPE were assessed.Subsequently,the correlation between EH indices and PTA findings(in three frequency domains of low,middle,and high)were evaluated,and the predictive value of MRI was calculated.Results:VH was significantly correlated with the hearing threshold in the low,middle,and highfrequency domains.CH was also correlated with the hearing threshold in the low and middle domains.Contrarily,VIPE was not associated with hearing thresholds,and VANV was only correlated with the hearing threshold in low frequencies.Conclusion:The grade of VH,CH,and VANV were significantly correlated with the hearing thresholds in PTA.

A case report of prenatally detected achondrogenesis typeⅡ with an occipital cephalocele

Achondrogenesis is a very rare lethal skeletal disorder. Here we describe a case of prenatally diagnosed achondrogenesis typeⅡ in a 28 year-old woman at (17+4) wk. She had history of 5 first trimester missed abortions. The couple is consanguineous. Ultrasonography showed extreme micromelia, short neck and trunk, large head and prominent abdomen. Delayed ossification in sacral bones was detected and ossification of pubic rami was poor. There were associated large cystic hygroma, anasarca and also high occipital cephalocele. Posterior fossa was normal. Mild hypothelorism, depressed nasal bridge, low set ear and mild retrognathia were identified too. Amniocentesis result was compatible with a normal female fetus. Post mortem whole body radiography confirmed the diagnosis. To our knowledge, this is the second case report with association of cephalocele and achondrogenesis typeⅡ.

Congenital Disseminated Pyogenic Granuloma:A Case With Numerous Mucocutaneous and Visceral Lesions

Congenital disseminated pyogenic granuloma(CDPG)is characterized by eruptive disseminated or localized lesions,which may arise spontaneously or secondary to predisposing factors.Even rarer is the occurrence of CDPG with numerous lesions affecting variable organs,which develop during the fetal period.This report describes the case of a 32-week-old fetus presenting with severe hydrocephalus and vascular intracranial and right lung masses on magnetic resonance imaging.Preterm labor occurred at the 32^(nd)week due to preterm premature rupture of membranes,and the newborn died due to cardiac dysfunction within 2 hours postpartum.The subsequent autopsy revealed multiple violaceous to dark red papules,nodules,pedunculated and un-pedunculated mucocutaneous masses,as well as two brain lesions,a lung lesion,a thoracic wall intramuscular mass,and a pyloric mass.Microscopic examination and immunohistochemical evaluation for glucose transporter 1(GLUT1)confirmed the diagnosis of CDPG.CDPG represents a rare condition with an elusive etiology and limited reports in the literature.Differential diagnosis from multifocal infantile hemangioma,based on GLUT1 negativity of CDPG,is imperative due to differing clinical course and treatment modalities.This report underscores a severe case of CDPG characterized by preterm labor and demise shortly after delivery,notable for its extensive involvement across multiple organs,including the brain,lung,intestine,musculoskeletal system,mucosal,and numerous cutaneous sites.