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Plastic and Reconstructive Surgery Feb 2024Facial paralysis secondary to neurofibromatosis type 2 (NF2) presents the reconstructive surgeon with unique challenges because of its pathognomonic feature of bilateral... (Review)
Review
BACKGROUND
Facial paralysis secondary to neurofibromatosis type 2 (NF2) presents the reconstructive surgeon with unique challenges because of its pathognomonic feature of bilateral acoustic neuromas, involvement of multiple cranial nerves, use of antineoplastic agents, and management. Facial reanimation literature on managing this patient population is scant.
METHODS
A comprehensive literature review was performed. All patients with NF2-related facial paralysis who presented in the past 13 years were reviewed retrospectively for type and degree of paralysis, NF2 sequelae, number of cranial nerves involved, interventional modalities, and surgical notes.
RESULTS
Twelve patients with NF2-related facial paralysis were identified. All patients presented after resection of vestibular schwannoma. Mean duration of weakness before surgical intervention was 8 months. On presentation, one patient had bilateral facial weakness, 11 had multiple cranial nerve involvement, and seven were treated with antineoplastic agents. Two patients underwent gracilis free functional muscle transfer, five underwent masseteric-to-facial nerve transfer (of whom two were dually innervated with a crossfacial nerve graft), and one patient underwent depressor anguli oris myectomy. Trigeminal schwannomas did not affect reconstructive outcomes if trigeminal nerve motor function on clinical examination was normal. In addition, antineoplastic agents such as bevacizumab and temsirolimus did not affect outcomes if stopped in the perioperative period.
CONCLUSIONS
Effectively managing patients with NF2-related facial paralysis necessitates understanding the progressive and systemic nature of the disease, bilateral facial nerve and multiple cranial nerve involvement, and common antineoplastic treatments. Neither antineoplastic agents nor trigeminal nerve schwannomas associated with normal examination affected outcomes.
CLINICAL QUESTION/LEVEL OF EVIDENCE
Therapeutic, V.
Topics: Humans; Neurofibromatosis 2; Facial Paralysis; Retrospective Studies; Facial Nerve; Neurilemmoma; Antineoplastic Agents
PubMed: 37075282
DOI: 10.1097/PRS.0000000000010561 -
Science Advances Nov 2023Vestibular schwannoma (VS) is an intracranial tumor arising from neoplastic Schwann cells and typically presenting with hearing loss. The traditional belief that hearing...
Vestibular schwannoma (VS) is an intracranial tumor arising from neoplastic Schwann cells and typically presenting with hearing loss. The traditional belief that hearing deficit is caused by physical expansion of the VS, compressing the auditory nerve, does not explain the common clinical finding that patients with small tumors can have profound hearing loss, suggesting that tumor-secreted factors could influence hearing ability in VS patients. We conducted profiling of patients' plasma for 66 immune-related factors in patients with sporadic VS ( > 170) and identified and validated candidate biomarkers associated with tumor size (S100B) and hearing (MCP-3). We further identified a nine-biomarker panel (TNR-R2, MIF, CD30, MCP-3, IL-2R, BLC, TWEAK, eotaxin, and S100B) with outstanding discriminatory ability for VS. These findings revealed possible therapeutic targets for VS, providing a unique diagnostic tool that may predict hearing change and tumor growth in VS patients, and may inform the timing of tumor resection to preserve hearing.
Topics: Humans; Neuroma, Acoustic; Hearing Loss; Hearing; Biomarkers; Deafness
PubMed: 37948527
DOI: 10.1126/sciadv.adf7295 -
Cellular and Molecular Neurobiology Nov 2023To study key proteins associated with changes in synaptic transmission in the spiral ganglion in tinnitus, we build three gene lists from the GeneCard database: 1....
To study key proteins associated with changes in synaptic transmission in the spiral ganglion in tinnitus, we build three gene lists from the GeneCard database: 1. Perception of sound (PoS), 2. Acoustic stimulation (AcouStim), and 3. Tinnitus (Tin). Enrichment analysis by the DAVID database resulted in similar Gene Ontology (GO) terms for cellular components in all gene lists, reflecting synaptic structures known to be involved in auditory processing. The STRING protein-protein interaction (PPI) network and the Cytoscape data analyzer were used to identify the top two high-degree proteins (HDPs) and their high-score interaction proteins (HSIPs) identified by the combined score (CS) of the corresponding edges. The top two protein pairs (key proteins) for the PoS are BDNF-GDNF and OTOF-CACNA1D and for the AcouStim process BDNF-NTRK2 and TH-CALB1. The Tin process showed BDNF and NGF as HDPs, with high-score interactions with NTRK1 and NGFR at a comparable level. Compared to the PoS and AcouStim process, the number of HSIPs of key proteins (CS > 90. percentile) increases strongly in Tin. In the PoS and AcouStim networks, BDNF receptor signaling is the dominant pathway, and in the Tin network, the NGF-signaling pathway is of similar importance. Key proteins and their HSIPs are good indicators of biological processes and of signaling pathways characteristic for the normal hearing on the one hand and tinnitus on the other.
Topics: Humans; Tinnitus; Spiral Ganglion; Brain-Derived Neurotrophic Factor; Synaptic Transmission; Neurons
PubMed: 37736859
DOI: 10.1007/s10571-023-01405-w -
European Archives of... Jul 2024The purpose of this retrospective study is to compare the results of electrically evoked compound action potential (ECAP) measurements using automatic auditory response... (Comparative Study)
Comparative Study
PURPOSE
The purpose of this retrospective study is to compare the results of electrically evoked compound action potential (ECAP) measurements using automatic auditory response telemetry (AutoART) with those obtained by ART in adults. The study also aimed to evaluate the predictive value of intraoperative ART and AutoART ECAPs for speech intelligibility (SI) and hearing success (HS), and to determine if cochlear nerve (CN) cross-sectional area (CSA) obtained preoperatively by magnetic resonance imaging (MRI) scans could predict ART and AutoART ECAPs and SI and HS outcome.
METHODS
The study analyzed and correlated ART and AutoART ECAP thresholds at electrodes E2, E6, and E10, as well as averaged ECAP thresholds over electrodes E1-E12, using data from 32 implants. Correlations were also examined for ART and AutoART ECAP slopes. In addition, averaged ART and AutoART ECAP thresholds and slopes over all 12 electrodes for each participant were correlated with CN CSA measured from MRI sequences. SI of the monosyllabic Freiburg Speech Test at 65 dB sound pressure level was examined along with averaged ART and AutoART thresholds and slopes over all 12 electrodes. A parallel analysis was performed for HS, derived from the difference between baseline and 6-month SI. Finally, correlations between CN CSA and SI, as well as CN CSA and HS were examined.
RESULTS
The results of the study showed a significant positive correlation between ART and AutoART ECAP thresholds and as well as slopes for E2, E6, E10 and averaged thresholds and slopes of E1-E12. However, no significant correlation was observed between ART and AutoART averaged ECAP thresholds and slopes and either SI and HS or CN CSA. Furthermore, no significant correlation was found between CN CSA and SI and HS.
CONCLUSION
While AutoART is a reliable and safe program for measuring ECAPs in adults, the study found no preoperative prognostic information on intraoperative ECAP results using parameters extracted from current MRI sequences or pre-/intraoperative information on subsequent hearing outcome using ECAP and CN CSA.
Topics: Humans; Cochlear Nerve; Retrospective Studies; Male; Middle Aged; Female; Adult; Cochlear Implants; Aged; Magnetic Resonance Imaging; Evoked Potentials, Auditory; Cochlear Implantation; Telemetry; Speech Intelligibility; Young Adult; Predictive Value of Tests; Auditory Threshold; Action Potentials
PubMed: 38219245
DOI: 10.1007/s00405-023-08444-5 -
The Eurasian Journal of Medicine Oct 2023The aim of this study is to evaluate the relationship between the cochlear nerve and the anatomical structures of the cochlea and internal acoustic canal in patients...
OBJECTIVE
The aim of this study is to evaluate the relationship between the cochlear nerve and the anatomical structures of the cochlea and internal acoustic canal in patients with congenital hearing loss.
MATERIALS AND METHODS
Temporal tomography and magnetic resonance images of 44 patients (88 ears) with non-syndromic congenital hearing loss were retrospectively analyzed between 2018 and 2021. Patients were divided into 2 groups according to cochlear nerve hypoplasia. Cochlear nerve canal width, cochlear basal/ middle turn widths, and internal auditory canal widths were examined.
RESULTS
Cochlear nerve hypoplasia was detected in 18.2% (n=16) of the patients and all of the patients with cochlear nerve hypoplasia had severe hearing loss. A statistically significant difference was found between the structures' widths in patients with and without cochlear nerve hypoplasia, in cochlear nerve canal and coronal width of the internal auditory canal. When stenosis is accepted as <1.4 mm for cochlear nerve canal and <3.80 mm for coronal width of the internal auditory canal, cochlear nerve hypoplasia differs statistically between the groups in measurements (respectively; P < .001, P=.018).
CONCLUSIONS
In patients with sensorineural hearing loss, cochlear nerve hypoplasia may accompany. Anatomical structures are important in predicting cochlear nerve hypoplasia from temporal computed tomography. Cochlear nerve hypoplasia should be suspected if the cochlear nerve canal and coronal width of the internal auditory canal are less than 1.4 mm and 3.8 mm, respectively, on temporal computed tomography.
PubMed: 37909185
DOI: 10.5152/eurasianjmed.2023.22196 -
Journal of Voice : Official Journal of... Aug 2023Voice changes are a common complication after a thyroidectomy, which is a surgical procedure involving partial or total removal of the thyroid gland. The main objective...
OBJECTIVES
Voice changes are a common complication after a thyroidectomy, which is a surgical procedure involving partial or total removal of the thyroid gland. The main objective of this work is to examine the possible voice disorders after thyroid surgery. More precisely, it is an investigation of partial and total thyroidectomy, as well as the effects that cancerous and noncancerous thyroid glands can have regarding postsurgical vocal and their association with age and gender.
METHODS
Patients were evaluated using acoustic voice parameters, including harmonics-to-noise ratio (HNR), fundamental frequency (F0), jitter, speaker phonation frequency (SPF) range, cepstral peak prominence (CPP), maximum phonational frequency range (MPFR), and shimmer at the preoperative stage and postoperatively at the 1 day, and first-month stages.
RESULTS
Results demonstrated a significant change in F0 parameters, SPF range, and CPP feature 1 month after surgery, depending on the type of thyroidectomy and thyroid pathology. No significant changes were observed in the HNR, shimmer, and jitter features. Age was associated with the CPP parameter in the entire sample. In contrast, the MPFR parameter was also related to the type of thyroidectomy in the entire sample. However, maximum F0 was significantly associated with the type of thyroidectomy, specifically in the female sample.
CONCLUSIONS
Results indicated that a thyroidectomy can have a negative impact on voice quality. The age and type of thyroidectomy performed are not responsible for this change. Potentially this change can be due to factors such as nerve damage or the subjects' experience, such as job, anxiety, and their physical condition, as well as treatments they may have undergone before thyroidectomy. Further efforts are needed to fully understand the background of voice changes after thyroidectomy.
PubMed: 37612171
DOI: 10.1016/j.jvoice.2023.06.027 -
Neurobiology of Disease Oct 2023Age-related hearing loss (ARHL) is a prevalent condition affecting millions of individuals globally. This study investigated the role of the cell survival regulator Bcl2...
Age-related hearing loss (ARHL) is a prevalent condition affecting millions of individuals globally. This study investigated the role of the cell survival regulator Bcl2 in ARHL through in vitro and in vivo experiments and metabolomics analysis. The results showed that the lack of Bcl2 in the auditory cortex affects lipid metabolism, resulting in reduced synaptic function and neurodegeneration. Immunohistochemical analysis demonstrated enrichment of Bcl2 in specific areas of the auditory cortex, including the secondary auditory cortex, dorsal and ventral areas, and primary somatosensory cortex. In ARHL rats, a significant decrease in Bcl2 expression was observed in these areas. RNAseq analysis showed that the downregulation of Bcl2 altered lipid metabolism pathways within the auditory pathway, which was further confirmed by metabolomics analysis. These results suggest that Bcl2 plays a crucial role in regulating lipid metabolism, synaptic function, and neurodegeneration in ARHL; thereby, it could be a potential therapeutic target. We also revealed that Bcl2 probably has a close connection with lipid peroxidation and reactive oxygen species (ROS) production occurring in cochlear hair cells and cortical neurons in ARHL. The study also identified changes in hair cells, spiral ganglion cells, and nerve fiber density as consequences of Bcl2 deficiency, which could potentially contribute to the inner ear nerve blockage and subsequent hearing loss. Therefore, targeting Bcl2 may be a promising potential therapeutic intervention for ARHL. These findings provide valuable insights into the molecular mechanisms underlying ARHL and may pave the way for novel treatment approaches for this prevalent age-related disorder.
Topics: Animals; Rats; Aging; Lipid Metabolism; Neurons; Presbycusis; Spiral Ganglion
PubMed: 37813166
DOI: 10.1016/j.nbd.2023.106320 -
EMBO Molecular Medicine Apr 2024Approximately half a billion people—5% of the world’s population—suffer from disabling hearing impairment (HI) according to the WHO...
Approximately half a billion people—5% of the world’s population—suffer from disabling hearing impairment (HI) according to the WHO (http://www.who.int/features/factfiles/deafness/en/). HI commonly hampers speech acquisition, leads to social isolation and increases risk for depression and cognitive decline. One to two per thousand children are born with disabling HI and over 50% of sensorineural HI is caused by defects in individual genes (deafness genes) of which roughly 150 have been identified so far (http://hereditaryhearingloss.org/). In case of profound hearing impairment or deafness, cochlear implants that bypass the dysfunctional or lost sensory hair cells and electrically stimulate the auditory nerve partially restore hearing. However, hearing with cochlear implants has shortcomings such as limited understanding of speech in background noise. So, there remains a major unmet medical need for improved hearing restoration (Wolf et al, 2022). Yet, despite major research efforts, a causal treatment based on pharmacology, gene therapy, or stem cells had, so far, not been clinically available. Now, this is finally changing at least for some patients: first in human trials prove the concept for inner ear gene therapy of otoferlin-related synaptic deafness.
Topics: Humans; Deafness; Genetic Therapy
PubMed: 38528140
DOI: 10.1038/s44321-024-00058-6 -
NeuroImage Jan 2024Multimodal stimulation can reverse pathological neural activity and improve symptoms in neuropsychiatric diseases. Recent research shows that multimodal...
Multimodal stimulation can reverse pathological neural activity and improve symptoms in neuropsychiatric diseases. Recent research shows that multimodal acoustic-electric trigeminal-nerve stimulation (TNS) (i.e., musical stimulation synchronized to electrical stimulation of the trigeminal nerve) can improve consciousness in patients with disorders of consciousness. However, the reliability and mechanism of this novel approach remain largely unknown. We explored the effects of multimodal acoustic-electric TNS in healthy human participants by assessing conscious perception before and after stimulation using behavioral and neural measures in tactile and auditory target-detection tasks. To explore the mechanisms underlying the putative effects of acoustic-electric stimulation, we fitted a biologically plausible neural network model to the neural data using dynamic causal modeling. We observed that (1) acoustic-electric stimulation improves conscious tactile perception without a concomitant change in auditory perception, (2) this improvement is caused by the interplay of the acoustic and electric stimulation rather than any of the unimodal stimulation alone, and (3) the effect of acoustic-electric stimulation on conscious perception correlates with inter-regional connection changes in a recurrent neural processing model. These results provide evidence that acoustic-electric TNS can promote conscious perception. Alterations in inter-regional cortical connections might be the mechanism by which acoustic-electric TNS achieves its consciousness benefits.
Topics: Humans; Consciousness; Reproducibility of Results; Electric Stimulation; Auditory Perception; Acoustic Stimulation; Acoustics; Trigeminal Nerve
PubMed: 38030051
DOI: 10.1016/j.neuroimage.2023.120476 -
Biomedicine & Pharmacotherapy =... Sep 2023When activated by unconjugated bilirubin (UCB), inflammatory mediators such as IL - 18 and TNF contribute to the neurotoxicity and ototoxicity observed in severe...
When activated by unconjugated bilirubin (UCB), inflammatory mediators such as IL - 18 and TNF contribute to the neurotoxicity and ototoxicity observed in severe neonatal hyperbilirubinemia. However, in cell and molecular level, the regulation and mechanism of UCB-induced ototoxicity are remained unclear. In this study, 7-day-old mammary rats were exposed to various concentrations of UCB to imitate the infant auditory damage. The auditory brainstem response result (ABR) indicated severe hearing loss, which occurred with increasing concentration. Morphological analysis of organotypic cochlear cultures treated with different concentrations of UCB indicated that auditory nerve fibers (ANF) were demyelinated and the density of spiral ganglion neurons (SGN) were decreased. In addition, HEI-OC1 cells treated with different concentrations of UCB showed severe necrosis by Flow Cytometry. The morphologic feature of pyroptosis has been observed by scanning electronic microscope. Cleaved Caspase-1, GSDMD and NLRP3 expression were significantly increased in cochlear explants with UCB-induced. To further clarify the molecular mechanism of UCB-induced inner ear cell pyroptosis, specific inhibitors of pyroptosis were applied, the protein associated with pyrotosis such as Cleaved Caspase-1, GSDMD, ASC, IL-18 and NLRP3 were significantly lower than the group with UCB alone. All the data above indicated that ERK /NLRP3/GSDMD signaling pathway involved in UCB-induced ototoxicity.
Topics: Animals; Rats; Bilirubin; Caspase 1; Hyperbilirubinemia, Neonatal; NLR Family, Pyrin Domain-Containing 3 Protein; Ototoxicity; Pyroptosis; Animals, Newborn; Disease Models, Animal
PubMed: 37467648
DOI: 10.1016/j.biopha.2023.115162