Introduction: Temporal bone chondroblastoma is a rare, locally aggressive tumour originating from immature cartilage, which recurs to a high degree. Treatment is surgical. Radiotherapy is reserved for recurrence. We describe a case of a 15-year-old-boy choosing a conservative surgical approach with reconstruction of the posterior canal wall. This study aims to report a rare pediatric case.
Methods: A literature review was performed to better understand temporal bone chondroblastomas, to describe their histopathological and radiological characteristics and to establish the optimal surgical and non-surgical treatments. The research of previous published data was done using PubMed with keywords mentioned below.
Results: Authors present a case of a 15-year-old boy with hearing impairment and facial nerve palsy. Conservative surgery with reconstruction of the tympanic membrane and posterior wall of the external auditory canal, restoring the hearing has been performed. We did not administer any adjuvant therapies. No sign of recurrence was observed 1 year after primary surgery. Facial nerve function is normal, and hearing is satisfactory.
Conclusion: Chondroblastomas account for less than 1% of primary bone tumours. Temporal bone chondroblastoma is rare, locally aggressive, with a high prevalence of recurrence. This study describes specific histopathological and radiological findings, the chosen surgical approach and follow-up to improve the management and the prognosis of patients affected with this particular clinical entity.
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http://dx.doi.org/10.14712/18059694.2021.29 | DOI Listing |
Int J Pediatr Otorhinolaryngol
December 2024
Hacettepe University, Faculty of Medicine, Department of Opthalmology, Ankara, Turkey.
Aims And Objectives: This study aimed to investigate the presence, type, and severity of hearing losses in individuals with Duane Retraction Syndrome (DRS), and to ascertain if there are anomalies in the auditory pathways at the brainstem level in DRS, believed to arise from aberrant interaction between cranial nerves and brainstem nuclei.
Study Design: Cross-sectional observational study.
Setting: Tertiary referral centre.
Ear Hear
December 2024
Department of Communication Sciences and Disorders, James Madison University, Harrisonburg, Virginia, USA.
Objectives: Cervical vestibular evoked myogenic potentials (cVEMPs) reflect saccular stimulation that results in an inhibitory muscle reflex recorded over the sternocleidomastoid muscle. These responses are utilized to study basic vestibular functions and are also applied clinically. Traditionally, cVEMPs have utilized transient stimuli such as clicks and tonebursts to evoke onset responses.
View Article and Find Full Text PDFInt J Pediatr Otorhinolaryngol
December 2024
University of Utah, Department of Otolaryngology - Head & Neck Surgery, United States. Electronic address:
Introduction: Pediatric temporal bone fractures (TBFs) can result in adverse outcomes including meningitis, significant sensorineural hearing loss requiring cochlear implantation (CI), facial nerve weakness, cerebrospinal fluid (CSF) leak, and labyrinthitis. The objective of this study is to determine the risks of these outcomes for TBFs with hearing loss.
Methods: Using the multinational TriNetX database, a retrospective cohort study was performed of patients less than 18 years old with diagnostic codes for other fracture of base of skull and hearing loss to serve as an approximation of TBF.
Neurocase
December 2024
First Department of Neurology, Eginition University Hospital, School of Medicine, National and Kapodistrian University of Athens, NKUA, Athens, Greece.
Mutations in sequestosome 1 (SQSTM1) gene have been associated with frontotemporal dementia (FTD), amyotrophic lateral sclerosis (ALS), frontotemporal dementia - ALS (FTD-ALS), and very recently, progressive supranuclear palsy (PSP), paget disease of bone (PDB), distal myopathy with rimmed vacuoles (DMRV), and neurodegenerative disorders in childhood. We present a case of right temporal variant of FTD (rtvFTD) with heterozygous mutation (c.823_824del(p.
View Article and Find Full Text PDFAm J Surg Pathol
December 2024
Department of Pathology, Microbiology and Immunology, Vanderbilt University Medical Center.
DEK::AFF2 fusion nonkeratinizing squamous cell carcinoma (NKSCC) is an emerging entity in the sinonasal tract, temporal bone, and skull base. However, the clinical behavior of these tumors has not been well studied. Here, we report the largest cohort of DEK::AFF2 carcinomas to determine if morphology, mitotic rate, and/or Ki-67 IHC are associated with patient outcomes, including a comparison with high-risk human papillomavirus (HPV)-associated and independent patients.
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