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Brain Sciences Mar 2024The middle cranial fossa (MCF) approach is a well-established procedure in surgery of the internal auditory canal, as well as with the retrosigmoid and translabyrinthine...
INTRODUCTION
The middle cranial fossa (MCF) approach is a well-established procedure in surgery of the internal auditory canal, as well as with the retrosigmoid and translabyrinthine approaches. It is commonly used in the hearing-preserving microsurgery of small vestibular schwannomas (VS). The debate about the "best" approach for the microsurgery of small VS without contact to the brainstem is controversial. It has been stated that the MCF approach leads to irreversible damage to the temporal lobe, which may be evident in follow-up magnet resonance imaging (MRI) as gliosis in up to 70% of patients.
MATERIALS AND METHODS
This study represents a retrospective chart analysis conducted at a tertiary university hospital. Here, 76 postoperative MRIs were re-evaluated by an experienced neuroradiologist and compared with the preoperative images. Temporal lobe gliosis was classified on an ordinal scale as absent, slight, moderate or severe. Occurrence of gliosis was matched to the clinical predictors (tumor stage, tumor volume, sex, age, and side).
RESULTS
No case of severe or moderate gliosis was found in the patient group. Slight gliosis of the temporal lobe was rare and was only detected in four patients (5%). There was no relation between clinical predictors and the incidence of gliosis.
CONCLUSIONS
In our cohort, postoperative MR imaging did not reveal relevant damage to the temporal lobe parenchyma. This confirms the safe concept of microsurgery of small tumors via the middle fossa approach. The severe glioses described in other studies may be caused by a forced insertion of the retractor or by more extended approaches. However, further prospective neurocognitive studies seem to be necessary in order to assess functional changes in the temporal lobe.
PubMed: 38539682
DOI: 10.3390/brainsci14030295 -
Auris, Nasus, Larynx Jun 2024To evaluate pre- and post-operative semicircular canal function in patients with vestibular schwannoma (VS) by the video Head Impulse Test (vHIT).
OBJECTIVES
To evaluate pre- and post-operative semicircular canal function in patients with vestibular schwannoma (VS) by the video Head Impulse Test (vHIT).
METHODS
Nineteen patients with VS who underwent surgery were enrolled in this study. The gain in vestibulo-ocular reflex (VOR) and the degree of scatter in catch-up saccades were examined pre- and post-operatively for the semicircular canals in VS patients.
RESULTS
Ten of 19 cases (52.6 %) with VS were defined as demonstrating both superior vestibular nerve (SVN) and inferior vestibular nerve (IVN) impairment from the results of pre-operative vHIT. Hearing level and subjective vestibular symptoms showed significant correlations with pre-operative semicircular canal function. Compared to pre-operative vHIT results, VOR gains within 1 month after surgery were significantly reduced in all three canals; however, significant differences had disappeared in the anterior and posterior semicircular canals at 6 months after surgery. Cases of unknown origin had a significantly greater reduction in posterior semicircular canal function after surgery compared with those with disease of IVN origin.
CONCLUSIONS
As vHIT could evaluate pre-operative vestibular nerve impairment, post-operative VOR gain reduction and the degree of vestibular compensation, semicircular canal function evaluated by vHIT provides a good deal of useful information regarding VS patients undergoing surgery compared to caloric testing, and vHIT should be performed pre- and post-operatively for patients with VS.
Topics: Humans; Neuroma, Acoustic; Head Impulse Test; Semicircular Canals; Female; Middle Aged; Male; Reflex, Vestibulo-Ocular; Adult; Aged; Video Recording; Saccades; Postoperative Period; Vestibular Nerve
PubMed: 38537557
DOI: 10.1016/j.anl.2024.03.001 -
American Journal of Clinical Pathology Mar 2024Distinguishing between sporadic and germline/mosaic NF2-related schwannomatosis is important to ensure that patients have appropriate long-term care. With this report,...
OBJECTIVES
Distinguishing between sporadic and germline/mosaic NF2-related schwannomatosis is important to ensure that patients have appropriate long-term care. With this report, we describe a unique case of a patient with 4 ipsilateral schwannomas and identify a combination of sequencing modalities that can accurately diagnose mosaic NF2-related schwannomatosis.
METHODS
We present a 32-year-old woman with a familial history of vestibular schwannoma in her father and right-sided schwannomas involving the apical and basal turns of cochlea, lateral semicircular canal, and internal auditory canal (IAC). Genetic analysis of blood and frozen tissue from 2 tumors (intralabyrinthine and IAC tumors) was performed using next-generation sequencing (NGS), multiplex ligation-dependent probe amplification (MLPA), and optical genome mapping (OGM).
RESULTS
Germline testing for NF2, LZTR1, and SMARCB1 was negative. Tumor genetic testing revealed a shared NF2 pathogenic variant between the 2 tumors ("first hit") but distinct "second hit" NF2 variants, including mosaic loss of chromosome 22 in the IAC tumor seen only with OGM, consistent with mosaic NF2-related schwannomatosis.
CONCLUSIONS
Multimodality sequencing, including NGS, MLPA, and OGM, was required to ensure appropriate diagnosis of mosaic NF2-related schwannomatosis in this patient. A similar approach can be used for other patients with multiple ipsilateral tumors and suspected tumor predisposition.
PubMed: 38527168
DOI: 10.1093/ajcp/aqae027 -
Scientific Reports Mar 2024NF2-related schwannomatosis (NF2-SWN) is a rare genetic disorder and is associated with progressive morbidities. This study aimed to investigate the relationship between...
NF2-related schwannomatosis (NF2-SWN) is a rare genetic disorder and is associated with progressive morbidities. This study aimed to investigate the relationship between NF2-SWN disease severity, health-related Quality of Life (QoL), and mental health aspects of patients. Standardised questionnaires assessing mental health problems (symptoms of depression, anxiety, and somatic burden), psychological factors (resilience, loneliness, and personality functioning), and health-related QoL were administered to 97 patients with NF2-SWN. The results of these questionnaires were compared with physician-rated disease severity. Questionnaires were completed by 77 patients. Physician-rated disease severity scores were available for 55 patients. NF2-SWN patients showed a high prevalence of clinically relevant symptoms of depression (30%), anxiety (16%), and somatic burden (32%). Almost all variables showed moderate to high correlations with NF2-SWN-related QoL. NF2-SWN-related QoL was associated with physician-reported disease severity (r = 0.614). In the stepwise hierarchical linear regression analysis, a significant model with four predictors (disease severity type, depression symptoms, personality functioning, and gender) explained 64% of the variance in NF2-SWN-related QoL. Our results showed a strong association between NF2-SWN-related QoL and depression symptoms. Moreover, personality functioning is an important influencing factor, representing a modifiable construct that can be targeted by prevention programs or psychotherapy.
Topics: Humans; Quality of Life; Mental Health; Neurofibromatoses; Neurofibromatosis 2; Neurilemmoma; Skin Neoplasms
PubMed: 38521834
DOI: 10.1038/s41598-024-57401-7 -
European Archives of... May 2024The expanded transpromontorial transcanal approach (ExpTTA) represents a recent addition to the surgical approaches available for the treatment of vestibular schwannoma....
BACKGROUND
The expanded transpromontorial transcanal approach (ExpTTA) represents a recent addition to the surgical approaches available for the treatment of vestibular schwannoma. An initial purely endoscopic version has been complemented by the use of the microscope and it is now one of the possible surgical options for small to medium-sized vestibular schwannomas with a predominantly intracanalar development.
METHODS
This is a series of 54 patients who underwent microsurgical resection of sporadic, unilateral vestibular schwannoma, mainly Koos I-II with non-serviceable hearing, between January 2016 and January 2023 using the expanded transcanal transpromontorial approach. We describe the surgical technique, focusing on anatomical landmarks, and analyzing its advantages and shortcomings. Retrospective analysis of clinical outcomes is presented, including early and late complications. The mean follow-up was 46.7 months.
RESULTS
We achieved gross total resection of the lesion in all cases, confirmed on the first follow-up MRI at least 6 months after each procedure. We did not record any intraoperative complication nor disease recurrence. We recorded two postoperative severe facial nerve palsies, one of which was permanent. No cases of disabling vertigo or imbalance were reported, and all patients reported full recovery of autonomy in daily activities. Three cases of otoliquorrhea were managed conservatively successfully.
CONCLUSIONS
The transcanal transpromontorial approach combines the advantages of endoscopy with the possibilities provided by microsurgery. Our experience confirms its safety in terms of surgical complications and facial nerve outcome. This approach is amongst the treatment options for small-medium schwannomas in patients with impaired hearing, especially in young patients, ensuring radical resection, disease control, and minimal morbidity.
Topics: Humans; Neuroma, Acoustic; Retrospective Studies; Neoplasm Recurrence, Local; Neurilemmoma; Endoscopy; Treatment Outcome
PubMed: 38519593
DOI: 10.1007/s00405-024-08565-5 -
The Annals of Otology, Rhinology, and... Jun 2024Treatment of vestibular schwannoma (VS) has been extensively studied, but a gap in knowledge exists demonstrating how racial and socioeconomic status influence VS...
Disparities in Sporadic Vestibular Schwannoma Initial Presentation Between a Public Safety Net Hospital and Tertiary Academic Medical Center at the Same Zip Code 2010 to 2020.
INTRODUCTION
Treatment of vestibular schwannoma (VS) has been extensively studied, but a gap in knowledge exists demonstrating how racial and socioeconomic status influence VS presentation. Our institution has a unique setting with a public safety net hospital (PSNH) and tertiary academic medical center (TAMC) in the same zip code, which we study to evaluate initial VS presentation disparities in patient populations presenting to these hospital settings.
METHODS
Retrospective chart review was performed of all adult patients (n = 531) presenting 2010 to 2020 for initial VS evaluation at TAMC (n = 462) and PSNH (n = 69). Ethnicity, insurance, maximum tumor size, audiometry, initial treatment recommendation, treatment received, and follow up were recorded and statistical analysis performed to determine differences.
RESULTS
Average age at diagnosis (51.7 ± 13.6 TAMC vs 52.3 ± 12.4 PSNH) and gender (58.4% TAMC vs 52.2% PSNH female) were similar. Patients' insurance (TAMC 75.9% privately insured vs PSNH 82% Medicaid) and racial/ethnic profiles (TAMC 67.7% White and 10.0% Hispanic/Latinx, vs PSNH 4.8% White but 59.7% Hispanic/Latinx) were significantly different. Tumor size was larger at PSNH (20.2 ± 13.3 mm) than TAMC (16.6 ± 10.0 mm). Hearing was more impaired at PSNH than TAMC (mean pure tone average 58.3 dB vs 43.9 dB, word recognition scores 52.3% vs 68.2%, respectively). Initial treatment recommendations and treatment received may include more than 1 modality. TAMC patients were offered 66.7% surgery, 31.2% observation, and 5.2% radiation, while PSNH patients offered 50.7% observation, 49.3% surgery, and 8.7% radiation. TAMC patients received 62.9% surgery, 32.5% observation, and 5.3% radiation, while PSNH patients received 36.2% surgery, 59.4% observation, and 14.5% radiation. Follow up and treatment at the same facility was not significantly different between hospitals.
CONCLUSIONS
Hearing was worse and tumor size larger in patients presenting to PSNH. Despite worse hearing status and larger tumor size, the majority of PSNH patients were initially offered observation, compared to TAMC where most patients were initially offered surgery.
Topics: Humans; Male; Female; Neuroma, Acoustic; Middle Aged; Safety-net Providers; Academic Medical Centers; Retrospective Studies; Healthcare Disparities; Tertiary Care Centers; Adult; United States; Aged
PubMed: 38517145
DOI: 10.1177/00034894241241201 -
Journal of Clinical Neuroscience :... May 2024People diagnosed with Vestibular Schwannoma (VS) can experience several symptoms both pre and post-treatment. These, alongside the diagnosis experience, can...
People diagnosed with Vestibular Schwannoma (VS) can experience several symptoms both pre and post-treatment. These, alongside the diagnosis experience, can significantly impact their daily life. The present research is a continuation of a larger study aiming to explore the impacts of symptomology and body image/fear of negative evaluation (FNAE) on the quality of life (QOL) for people with VS. The research design was exploratory and involved a nationwide survey with a total of 52 participants. FNAE was assessed using a measurement of the same name, and QOL was assessed using the Penn Acoustic Neuroma Quality of Life scale (PANQOL). Comparing management groups revealed a significant difference in FNAE with higher scores for surgery compared to radiation treatment. Regression analyses revealed that FNAE significantly accounted for 10.9% of the variance in QOL. However, no symptom was significantly predictive of FNAE. In conclusion, VS is associated with several symptoms that can persist post-treatment. Body satisfaction contributes to QOL and may differ between management types. However, due to inconclusive findings on the predictability of symptoms on FNAE, other moderator factors could influence these direct relationships. Future studies should evaluate the variables that could mitigate or protect from the impacts of FNAE for this population.
Topics: Humans; Neuroma, Acoustic; Quality of Life; Female; Male; Middle Aged; Adult; Fear; Body Image; Aged; Surveys and Questionnaires
PubMed: 38508019
DOI: 10.1016/j.jocn.2024.03.011 -
Vestnik Otorinolaringologii 2024Neurofibromatosis type 2 (NF2) is a rare autosomal dominant disease (frequency 1 in 25-90 000) characterized by the formation of tumors of the central nervous system due...
Neurofibromatosis type 2 (NF2) is a rare autosomal dominant disease (frequency 1 in 25-90 000) characterized by the formation of tumors of the central nervous system due to a mutation in the gene on chromosome 22q12. Bilateral vestibular schwannomas are recognized as absolute diagnostic criteria of NF2 and occur in 95% of patients, are accompanied by hearing impairment, manifest at the age of 18-24 years. Skin manifestations can precede vestibular schwannomas for several years and predict the course of the disease: neurofibromas, cafe-au-lait macules, hypopigmented spots, recently described mesh capillary malformations. Despite the benign nature of schwannomas, they can lead to hearing loss, vestibular dysfunction, facial nerve paralysis, gait disorders, pain and convulsions, there is a risk of early death from compression of the brain stem. The probability of progressive hearing loss is partly determined by the type of mutation. We described a clinical case of NF2 in a 21-year-old patient with bilateral vestibular schwannomas without hearing loss, whose skin examination by ENT specialist revealed this disease. The importance of the presented observation is that the doctor should assume neurofibromatosis type 2 in a young patient with bilateral vestibular schwannomas. It is necessary to undertake a further examination of this patient, including: skin examination for the identification of characteristic neurofibromas and cafe-au-lait macules, consultation with an ophthalmologist, neurologist, MRI of the brain and spinal cord with contrast, genetic analysis - for timely initiation of therapy that prevents hearing loss and vestibular disorders.
Topics: Humans; Adolescent; Young Adult; Adult; Neurofibromatosis 2; Neuroma, Acoustic; Mutation; Hearing Loss
PubMed: 38506024
DOI: 10.17116/otorino20248901137 -
Cureus Feb 2024Acoustic neuroma excision in patients with cerebellopontine angle (CPA) tumours offers particular rehabilitation problems due to the complicated architecture of the...
Acoustic neuroma excision in patients with cerebellopontine angle (CPA) tumours offers particular rehabilitation problems due to the complicated architecture of the cerebellum and brainstem tissues involved. CPA tumours (acoustic neuromas) are slow-growing tumours that arise from the vestibulocochlear nerve. Surgical excision of these tumours can cause neurological abnormalities that compromise motor coordination, balance, and facial nerve function. The case study emphasises the importance of a comprehensive physiotherapeutic approach in rehabilitating a patient following acoustic neuroma excision, with a focus on particular CPA tumour deficits. The rehabilitation programme focuses on improving functional outcomes through balance, proprioception, and vestibular rehabilitation that is customised to the demands and deficiencies of the patient. Our comprehensive approach seeks to improve patients' quality of life, promote neurological healing, and support easy reintegration into normal activities following CPA tumour surgery.
PubMed: 38496073
DOI: 10.7759/cureus.54208 -
Journal of Neurosurgery Mar 2024
Topics: Humans; Treatment Outcome; Neuroma, Acoustic; Hearing
PubMed: 38489806
DOI: 10.3171/2023.12.JNS232876