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      Hearing loss in vestibular schwannoma Translated title: Hipoacusia en el schwannoma vestibular

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          Abstract

          SUMMARY: Purpose: The most common reason for consultation in patients suffering from Vestibular Schwannoma (VS) is progressive hearing loss. The main objective of this study is analyzing the hearing loss in patients with VS and determining the extent to which the tumor grade and the hearing loss are related. Methods: An observational retrospective study was conducted with a sample of 291 patients diagnosed with VS between 1995 and 2017 in the Department of Otorhinolaryngology and Head and Neck Surgery of the Tertiary Care Primary Center of Salamanca. We analyzed preoperative and postoperative data from patients with VS as well as radiological and surgical findings. Results: The most common reason for consultation at diagnosis was progressive unilateral hearing loss (31.6%). The relationship between the size of the VS and hearing loss in the patients who reported progressive unilateral hearing loss as a reason for consultation was not statistically significant (p=0.099). Conclusion: The most common reason for consultation in VS is progressive unilateral hearing loss. The hearing loss caused by VS does not have a statistically significant association with any tumor grade, although severe and profound hearing loss is more commonly associated with grade III-IV neurinomas, whereas mild hearing loss or normal hearing are more likely in grade I-II tumors.

          Translated abstract

          RESUMEN: Introducción y objetivo: La hipoacusia es uno de los síntomas más frecuentes en el debut de un schwannoma vestibular (SV). El objetivo de este estudio es analizar la pérdida auditiva en pacientes con SV y determinar en qué medida se relaciona con el tamaño tumoral. Método: Se lleva a cabo un estudio retrospectivo observacional en 291 pacientes diagnosticados de SV entre 1995 y 2017 en el servicio de Otorrinolaringología y Cirugía Cervicofacial del Hospital Clínico Universitario de Salamanca analizándose diferentes datos pre y postoperatorios de los pacientes, así como hallazgos radiológicos y quirúrgicos. Resultados: El síntoma más frecuente de consulta es la hipoacusia unilateral progresiva (31.6%). La asociación entre el tamaño del SV y la hipoacusia no es estadísticamente significativa (p=0.099). Conclusiones: La pérdida auditiva producida por un SV no es patrimonio de ningún estadio, aunque las pérdidas severas y profundas son más indicativas de neurinomas grados III-IV, y ante una pérdida auditiva leve o audición normal tendremos con más probabilidad un neurinoma grado I-II

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          Most cited references31

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          Neurofibromatosis type 2 (NF2): A clinical and molecular review

          D Evans (2009)
          Neurofibromatosis type 2 (NF2) is a tumour-prone disorder characterised by the development of multiple schwannomas and meningiomas. Prevalence (initially estimated at 1: 200,000) is around 1 in 60,000. Affected individuals inevitably develop schwannomas, typically affecting both vestibular nerves and leading to hearing loss and deafness. The majority of patients present with hearing loss, which is usually unilateral at onset and may be accompanied or preceded by tinnitus. Vestibular schwannomas may also cause dizziness or imbalance as a first symptom. Nausea, vomiting or true vertigo are rare symptoms, except in late-stage disease. The other main tumours are schwannomas of the other cranial, spinal and peripheral nerves; meningiomas both intracranial (including optic nerve meningiomas) and intraspinal, and some low-grade central nervous system malignancies (ependymomas). Ophthalmic features are also prominent and include reduced visual acuity and cataract. About 70% of NF2 patients have skin tumours (intracutaneous plaque-like lesions or more deep-seated subcutaneous nodular tumours). Neurofibromatosis type 2 is a dominantly inherited tumour predisposition syndrome caused by mutations in the NF2 gene on chromosome 22. More than 50% of patients represent new mutations and as many as one-third are mosaic for the underlying disease-causing mutation. Although truncating mutations (nonsense and frameshifts) are the most frequent germline event and cause the most severe disease, single and multiple exon deletions are common. A strategy for detection of the latter is vital for a sensitive analysis. Diagnosis is based on clinical and neuroimaging studies. Presymptomatic genetic testing is an integral part of the management of NF2 families. Prenatal diagnosis and pre-implantation genetic diagnosis is possible. The main differential diagnosis of NF2 is schwannomatosis. NF2 represents a difficult management problem with most patients facing substantial morbidity and reduced life expectancy. Surgery remains the focus of current management although watchful waiting with careful surveillance and occasionally radiation treatment have a role. Prognosis is adversely affected by early age at onset, a higher number of meningiomas and having a truncating mutation. In the future, the development of tailored drug therapies aimed at the genetic level are likely to provide huge improvements for this devastating condition.
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            Improved preservation of hearing and facial nerve function in vestibular schwannoma surgery via the retrosigmoid approach in a series of 200 patients.

            The aim of this study was to evaluate and present the results of current surgical treatment of vestibular schwannomas (VSs) and to report the refinements in the operative technique. The authors performed a retrospective study of 200 consecutive patients who had undergone VS surgery over a 3-year period. Patient records, operative reports, follow-up data, and neuroradiological findings were analyzed. The main outcome measures were magnetic resonance imaging, neurological status, patient complaints, and surgical complications. Complete tumor removal was achieved in 98% of patients. Anatomical preservation of the facial nerve was possible in 98.5% of patients. In patients treated for tumors with extension Classes T1, T2, and T3, the rate of facial nerve preservation was 100%. By the last follow-up examination, excellent or good facial nerve function had been achieved in 81% of the cases. By at least 1 year postsurgery, no patients had total facial palsy. In the patients with preserved hearing, the rate of anatomical preservation of the cochlear nerve was 84%. The overall rate of functional hearing preservation was 51%. There was no surgery-related permanent morbidity in this series of patients. Cerebrospinal fluid leakage was diagnosed in 2% of the patients. The mortality rate was 0%. The goal of VS treatment should be total removal in one stage and preservation of neurological function, as they determine a patient's quality of life. This goal can be safely and successfully achieved using the retrosigmoid approach.
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              Growth rate of vestibular schwannoma

              Vestibular schwannoma (VS) is the most common tumor in the extra-axial posterior fossa compartment in adults. Growth rate is paramount to decision making regarding treatment and follow up of these tumors. We conducted a comprehensive review of the literature to answer four questions: What percentage of newly diagnosed VS will grow on follow-up? What factors correlate to tumor growth? What is the "normal" growth rate for sporadic VS? What factors characterize VS with rapid growth? Thirty-seven reports, with more than 4000 patients, fit our review criteria. One third of newly diagnosed VS will grow on follow-up of 1-3years. However, after 5years, up to one half will grow. Patient age and sex do not influence growth of VS. Hearing loss and vertigo at presentation do not predict tumor growth. It is unclear whether balance disturbance or tinnitus predict tumor growth. Tumor size and location do not predict tumor growth. Growth in the first year of observation is a strong predictor of tumor growth. The average growth rate of a VS is 0.99-1.11mm/year. However, the expected growth rate for VS that have been shown to grow at first follow-up is 3mm/year. Factors that may predict tumor growth of above 4mm/year are cystic and hemorrhagic features in the tumor, and hormonal treatment. VS grow at an average 1mm/year. VS that have been shown to grow at first follow-up should be considered for treatment, unless contraindicated. Long term follow-up is recommended for VS.
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                Author and article information

                Journal
                orl
                Revista ORL
                Rev. ORL
                Ediciones Universidad de Salamanca (Salamanca, Salamanca, Spain )
                2444-7986
                March 2022
                : 13
                : 1
                : 31-39
                Affiliations
                [1] Salamanca orgnameUniversity Hospital of Salamanca orgdiv1Department of Otorhinolaryngology -Head & Neck Surgery Spain
                Article
                S2444-79862022000100004 S2444-7986(22)01300100004
                10.14201/orl.27266
                48579e9c-6fe6-4c92-a9c3-eae3f2abd855

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

                History
                : 16 September 2021
                : 07 January 2022
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 33, Pages: 9
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                SciELO Spain

                Categories
                Original

                cirugía del schwannoma vestibular,vestibular schwannoma,acoustic neurinoma,hearing loss,vestibular schwannoma surgery,schwannoma vestibular,neurinoma del acústico,hipoacusia

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