Temporal Bone Adenoma: A Comprehensive Analysis of Clinical Aspects and Surgical Outcome on a Very Rare Entity

Conclusion Total surgical resection is the treatment of choice in MEAs to minimize the risk for recurrence; this can come with morbidity, mostly sensorineural deafness. Given the very limited literature, long-term follow-up is recommended. [...] Georg Thieme Verlag KG Stuttgart · New YorkArticle in Thieme eJournals: Table of contents  |  Abstract  |  Full text
Source: Journal of Neurological Surgery Part B: Skull Base - Category: Neurosurgery Authors: Tags: Original Article Source Type: research

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Abhinav C Bhagat, Jyoti Kumar, Anju Garg, Anjali Prakash, Ravi Meher, Shivali AryaIndian Journal of Radiology and Imaging 2020 30(2):139-148 Malformations of the inner ear are an important cause of congenital deaf-mutism. Arrest in embryologic development of inner ear during various stages gives rise to the variety of malformations encountered. Current treatment options include hearing aids, cochlear implants, and auditory brainstem implants (ABI). With the advent of cochlear implant surgery and ABI, decent functional outcomes can be obtained provided such cases are diagnosed correctly and timely. To that end, high-resolu...
Source: Indian Journal of Radiology and Imaging - Category: Radiology Authors: Source Type: research
CONCLUSION: Inner-ear malformations, especially cochlear aperture anomalies, are involved in the aetiology of single-sided deafness more than expected. The cause of single-sided deafness differs greatly between congenital and adult-onset cases. All children with single-sided deafness should undergo radiological evaluation, as the prognosis and management, as well as the aetiology, may be significantly influenced by inner-ear malformation type. PMID: 32508296 [PubMed - as supplied by publisher]
Source: Journal of Laryngology and Otology - Category: ENT & OMF Tags: J Laryngol Otol Source Type: research
Objective: The aim of the study was to analyze the long-term outcomes after cochlear implantation in deaf children with Down syndrome (DS) regarding age at the first implantation and refer the results to preoperative radiological findings as well as postoperative auditory and speech performance. Additionally, the influence of the age at implantation and duration of CI use on postoperative hearing and language skills were closely analyzed in children with DS. Study Design: Retrospective analysis. Setting: Referral center (Cochlear Implant Center). Materials and Methods: Nine children with Down syndrome were compa...
Source: Otology and Neurotology - Category: ENT & OMF Tags: COCHLEAR IMPLANTS Source Type: research
Shanshan Zhang1, Dongli Yuan2 and Ge Tan1* 1Department of Neurology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, China 2The Institute of Medical Information, Chongqing Medical University, Chongqing, China Primary systemic vasculitis can affect every structure in both the central and peripheral nervous system, causing varied neurological manifestations of neurological dysfunction. Early recognition of the underlying causes of the neurological symptoms can facilitate timely treatment and improve the prognosis. This review highlights the clinical manifestations of primary systemic vasc...
Source: Frontiers in Neurology - Category: Neurology Source Type: research
In conclusion, for the radiological assessment of TB fractures, the entire VA should be regarded as a part of the otic capsule, and delayed inner ear sequelae should be anticipated for retrolabyrinthine fracture lines that course into or through the VA. When considering treatment options in cases similar to the present, our findings suggest that surgical interventions targeting the eES (ES shunting/decompression procedures (31–33), which are used in MD with the intention to drain the hydropic endolymphatic fluid space, or to improve the fluid resorptive functions of the eES, respectively, most likely cannot work as p...
Source: Frontiers in Neurology - Category: Neurology Source Type: research
Conclusion: We identified a novel non-sense mutation in MTHFR gene in a single Egyptian family with severe MTHFR deficiency. The present investigation is clinically important, as it adds to the growing list of MTHFR mutations, which might help in genetic counseling of families of affected children and proper genotype-phenotype correlation. Background Severe 5,10-Methylenetetrahydrofolate reductase (MTHFR; OMIM 236250) deficiency is a rare inborn error of metabolism and inherited in an autosomal recessive fashion. It is a very common disorder of folate metabolism and is clinically characterized with low plasma methion...
Source: Frontiers in Neurology - Category: Neurology Source Type: research
ConclusionsWe suggest both imaging modalities in pediatric candidates. However, in adults, we think that superiority of either imaging modalities is still contradictive. We had only three adult patients and the decision of the side of surgery was made according to MRI in one of them and to both imaging modalities in the other two adults.
Source: European Archives of Oto-Rhino-Laryngology - Category: ENT & OMF Source Type: research
Most patients diagnosed with neurofibromatosis type 2 (NF2) have bilateral vestibular schwannomas (VS). Through reviewing surgical method and clinical outcomes, we tried to find out a strategy for treatments in NF2 patients with VS. We retrospectively reviewed patients diagnosed pathological NF2 and have had microsurgery (MS) for VS in the PLA Army General Hospital. Seventeen patients were included from January 2000 to December 2016. Fifteen patients had progressive hearing impairment, and 7 ears were totally deaf. Computed tomography and magnetic resonance imaging were used for preoperative and postoperative evaluation. ...
Source: Medicine - Category: Internal Medicine Tags: Research Article: Observational Study Source Type: research
Conclusion: We report a novel presentation and imaging findings of congenital bilateral profound sensorineural hearing loss in a patient with nodular internal auditory canal and labyrinthine enhancement and coexisting inner ear dysplasia. Despite the multiple radiologic abnormalities, the patient has demonstrated good benefit from cochlear implantation. Future study of rare variants of congenital deafness, such as this, is critical toward defining new disease processes and determining optimal treatment.
Source: Otology and Neurotology - Category: ENT & OMF Tags: Cochlear Implants Source Type: research
Objective: To investigate the prevalence and relative risk of semicircular canal dehiscence (SCD) in pediatric patients with CDH23 pathogenic variants (Usher syndrome or non-syndromic deafness) compared with age-matched controls. Study Design: Retrospective cohort study. Setting: Multi-institutional study. Patients: Pediatric patients (ages 0–5 years) were compared based on the presence of biallelic pathogenic variants in CDH23 with pediatric controls who underwent computed tomography (CT) temporal bone scan for alternative purposes. Interventions: Retrospective review of diagnostic high resolution CT temporal bo...
Source: Otology and Neurotology - Category: ENT & OMF Tags: Pediatric Otology Source Type: research
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