Recurrent Bacterial Meningitis in a Child with Mondini Dysplasia [PDF]
Case Reports in Pediatrics, 2014 Mondini dysplasia, also known as Mondini malformation, is a developmental abnormality of the inner and middle ears that can cause hearing loss, cerebrospinal fluid (CSF) leakage, and recurrent bacterial meningitis (RBM), which is defined as two or more
Eda Kepenekli-Kadayifci +7 more
doaj +3 more sources
The Development of Auditory Skills in Young Children with Mondini Dysplasia after Cochlear Implantation [PDF]
PLoS ONE, 2014 The aim of this study is to survey and compare the development of auditory skills in young children with Mondini dysplasia and profoundly-deaf young children with radiologically normal inner ears over a period of 3 years after cochlear implantation.
Yongxin Li, Shusheng Gong, Guoqiang Zhao
exaly +3 more sources
Mondini Dysplasia Presenting as Otorrhea without Meningitis
Pediatrics and Neonatology, 2012 Mondini dysplasia is a rare inner ear malformation that is usually only diagnosed after recurrent meningitis. Surgical intervention is mandatory. This report highlights the case of a patient with Mondini dysplasia who presented with hearing impairment ...
Hung-Ching Lin, Nan-Chang Chiu
exaly +3 more sources
Concomitant Mutations in the Thyroglobulin and SLC26A4 Genes Leading to Fetal Goiter and Congenital Hypothyroidism in a Patient With Pendred Syndrome. [PDF]
Case Rep EndocrinolWe described new forms of thyroglobulin gene (TG) mutation resulting in fetal goiter and congenital hypothyroidism in a pendred syndrome (PS) patient. Fetal hypothyroidism was diagnosed, based on ultrasonographic evidence of goiter alongside with fetal blood measurement of TSH (>100 mIU/L); levothyroxine intrauterine treatment was performed.
Calcaterra V +9 more
europepmc +2 more sources
Abnormalities of the Facial Nerve in Temporal Bones With Inner Ear Malformations. [PDF]
Laryngoscope Investig OtolaryngolThis study investigates the abnormalities of the facial nerve in temporal bones with inner ear malformations (IEMFs), using histopathological evaluations from 28 human temporal bone specimens. It reveals a high prevalence of facial nerve hypoplasia, narrow facial recess, and aberrant nerve positioning, highlighting the surgical challenges these ...
Shimura T +7 more
europepmc +2 more sources
CSF gushers in cochlear implantation: surgical planning and management [PDF]
BMC SurgeryBackground Cochlear implants (CIs) have made it possible to significantly improve hearing in people with profound hearing loss. Although, cochlear implants are considered a safe procedure, this minimally invasive surgery has an overall complication rate ...
Amal Hajjij +8 more
doaj +2 more sources
Fully automated segmentation in temporal bone CT with neural network: a preliminary assessment study [PDF]
BMC Medical Imaging, 2021 Background Segmentation of important structures in temporal bone CT is the basis of image-guided otologic surgery. Manual segmentation of temporal bone CT is time- consuming and laborious.
Jiang Wang +7 more
doaj +2 more sources
The role of SLC26A4 in bony labyrinth development and otoconial mineralization in mouse models [PDF]
Frontiers in Molecular NeuroscienceInner ear malformations are predominantly attributed to developmental arrest during the embryonic stage of membranous labyrinth development. Due to the inherent difficulty in clinically assessing the status of the membranous labyrinth, these ...
Taku Ito +5 more
doaj +2 more sources
Hemifacial microsomia and bilateral mondini dysplasia: A rare clinical presentation
MGM Journal of Medical Sciences, 2021 We present a rare association of bilateral Mondini dysplasia with hemifacial microsomia (HFM). HFM is a clinical spectrum of malformations involving the orbit, mandible, ears, facial nerve, and soft tissues of the face.
Padma Ramesh +2 more
doaj +1 more source
The development of early numeracy in deaf and hard of hearing children acquiring spoken language
Child Development, Volume 93, Issue 5, Page e468-e483, September/October 2022., 2022 Abstract Most deaf and hard‐of‐hearing (DHH) children are born to hearing parents and steered toward spoken rather than signed language, introducing a delay in language access. This study investigated the effects of this delay on number acquisition. DHH children (N = 44, meanage = 58 months, 21F, >50% White) and typically‐hearing (TH) children (N = 79,
Anna Shusterman +3 more
wiley +1 more source