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Objective: To investigate the risk factors and clinical features of anacusis or severe deafness associated with chronic suppurative otitis media (CSOM ).
Method: A retrospective review of 62 ears consecutive cases of severe deafness (15 ears) and anacusis (47 ears) associated with CSOM were investigated . There were 5 ears severe deafness deteriorated to anacusis after procedures. This represented 6.42% of our total series of CSOM with or without cholesteatoma in adults and children (965 ears) in 1993-2000. The series with cholesteatoma or granuloma or tympanosclerosis were 46 (74.19%) ears 13 (20.97%) ears and 3 (4.84%) ears respectively. The symptoms and operative findings were analyzed. All patients underwent preoperative CT scans and preoperative audiometry. With respect to surgical technique, the canal-wall-down procedure was performed in(50/62) 80.6%, and classical modified mastoidectomy was performed in(12/62) 19.4%.
Result: The results demonstrated that chronic otorrhea with bloody, ossicular chain destructive and erosion of the supraplate structure or disappear of footplate were helpful predicting indicators for anacusis or severe deafness. There was a high significant association (Chi-square Fisher exact test P < 0. 001) with these factors compared with CSOM without anacusis or severe deafness. Labyrinthine fistula was also correlated to anacusis or severe deafness (P < 0.05). There were no significant with the courses and facial paralysis and revision mastoidectomy.
Conclusion: Anacusis or severe deafness intimately associated with chronic otorrhea with bloody, ossicular chain destructive, erosion of the supraplate structure or disappear of footplate and labyrinthine fistula. The surgical procedure should be performed earlier to eradicate the chronic or acute infection and to avoid anacusis or severe deafness.
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Pediatr Neurol
August 2025
Department of Neurology & Neurosurgery, McGill University, Montréal, Québec, Canada; Department of Pediatrics, McGill University, Montréal, Québec, Canada.
Background: Dyskinetic cerebral palsy (DCP) is a severe subtype of cerebral palsy in which children often present substantial functional impairment and multiple comorbidities. Our knowledge of the clinical picture of DCP is limited and our understanding of which markers best predict later impairment is scarce. This study aims to describe the presentation of DCP and examine the value of gestational age (GA) and magnetic resonance imaging (MRI) findings as early markers of eventual DCP prognosis.
View Article and Find Full Text PDFAm J Case Rep
September 2025
Oto-Rhino-Laryngology Surgery Clinic, Institute of Physiology and Pathology of Hearing - World Hearing Center, Kajetany, Poland.
BACKGROUND Duplicated internal auditory canal (dIAC) is a rare congenital temporal bone anomaly associated with ipsilateral sensorineural hearing loss (SNHL). The Bonebridge bone conduction implant has a magnet, an internal transducer, and an external audio processor. This report is of a 14-year-old girl with unilateral SNHL and vestibulocochlear nerve (VIII cranial nerve) aplasia due to dIAC who was treated with a Bonebridge bone conduction implant.
View Article and Find Full Text PDFHear Res
August 2025
Departments of Human Development & Quantitative Methodology and Hearing & Speech Sciences, University of Maryland, College Park, USA.
In the recent two decades it became possible to compensate severe-to-profound hearing loss using cochlear implants (CIs). The data from implanted children demonstrate that hearing and language acquisition is well-possible within an early critical period of 3 years, however, the earlier the access to sound is provided, the better outcomes can be expected. While the clinical priority is providing deaf and hard of hearing children with access to spoken language through hearing aids and CIs as early as possible, for most deaf children this access is currently in the second or third year of life.
View Article and Find Full Text PDFVestn Otorinolaringol
September 2025
St. Petersburg Research Institute of Ear, Throat, Nose and Speech, St. Petersburg, Russia.
An algorithm has been developed to accompany adolescents at the stages of determining indications and postoperative rehabilitation for sequential bilateral cochlear implantation with a long interval between operations, minimizing the risk of failure to use a cochlear implant (CI) in the second ear (CI2) and ensuring its effective use. The algorithm includes: 1) 2 groups of factors influencing the results of CI2 - unchangeable (medical and biological) and influenceable (psychological and pedagogical - teenager's motivation for CI2, adequate expectations of the results, regular auditory training with CI2); 2) 4 stages of adaptation to stimulation with CI2 and use of CI1 with CI2; 3) audiological, physiological, psychological, auditory training components of the adaptation process to CI2. The study involved 26 prelingually deaf adolescents aged 10-16 years (mean 13.
View Article and Find Full Text PDFProc Natl Acad Sci U S A
September 2025
The Solomon H. Snyder Department of Neuroscience, Johns Hopkins University School of Medicine, Baltimore, MD 21205.
The RNA-binding protein TRIM71 is essential for brain development, and recent genetic studies in humans have identified as a risk gene for congenital hydrocephal-us (CH). Here, we show that monoallelic missense mutations in are associated with hearing loss (HL) and inner ear aplasia in humans. Utilizing conditional knockout mice carrying a CH and HL-associated mutation, we demonstrate that loss of TRIM71 function during early otic development (embryonic day 9 to 10) causes severe HL.
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