scholarly journals Chemopreventative celecoxib fails to prevent schwannoma formation or sensorineural hearing loss in genetically engineered murine model of neurofibromatosis type 2

Oncotarget ◽  
2017 ◽  
Vol 9 (1) ◽  
pp. 718-725 ◽  
Author(s):  
Benjamin M. Wahle ◽  
Eric T. Hawley ◽  
Yongzheng He ◽  
Abbi E. Smith ◽  
Jin Yuan ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Murine Model ◽  
Neurofibromatosis Type ◽  
Genetically Engineered ◽  
Neurofibromatosis Type 2 ◽  
Sensorineural Hearing

scholarly journals A murine model of neurofibromatosis type 2 that accurately phenocopies human schwannoma formation

2014 ◽  
Vol 24 (1) ◽  
pp. 1-8 ◽  
Author(s):  
Jeffrey R. Gehlhausen ◽  
Su-Jung Park ◽  
Ann E. Hickox ◽  
Matthew Shew ◽  
Karl Staser ◽  
...  
Keyword(s):  
Murine Model ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2

scholarly journals Characteristics of sensorineural hearing loss secondary to inner ear acoustic trauma

2008 ◽  
Vol 136 (5-6) ◽  
pp. 221-225
Author(s):  
Slobodan Spremo ◽  
Zdenko Stupar
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Hearing Threshold ◽  
Acoustic Trauma ◽  
Sensorineural Hearing ◽  
Cochlear Damage ◽  
Type 3 ◽  
Trauma Group

INTRODUCTION Cochlear damage secondary to exposure to acoustic trauma is the consequence of the acoustic energy effects on the hearing cells in Korti's organ. OBJECTIVE The objective was to assess the correlation between the degree of sensorineural hearing loss and the type of audiogram registered in acoustic trauma exposed patients. METHOD We analyzed 262 audiograms of patients exposed to acoustic trauma in correlation to 146 audiograms of patients with cochlear damage and hearing loss not related to acoustic trauma. "A" group consisted of acoustic trauma cases, while "B" group incorporated cases with hearing loss secondary to cochlear ischaemia or degeneration. All audiograms were subdivided with regard to the mean hearing loss into three groups: mild (21-40 dB HL), moderate (41-60 dB HL) and severe (over 60 dB HL) hearing loss. Based on audiogram configuration five types of audiogram were defined: type 1 flat; type 2 hearing threshold slope at 2 kHz, type 3 hearing threshold slope at 4 kHz; type 4 hearing threshold notch at 2 kHz; type 5 notch at 4 kHz. RESULTS Mild hearing loss was recorded in 163 (62.2%) ears in the acoustic trauma group, while in 78 (29.8%) ears we established moderate hearing loss with the maximum threshold shift at frequencies ranging from 4 kHz to 8 kHz. The least frequent was profound hearing loss, obtained in 21 (8%) audiograms in the acoustic trauma group. Characteristic audiogram configurations in the acoustic trauma patient group were: type 1 (N=66; 25.2%), type 2 (N=71; 27.1%), and type 3 (N=68; 25.9%). Audiogram configurations were significanly different in the acoustic trauma group in comparison to the cochlear ischaemia group of patients (p=0.0005). CONCLUSION Cochlear damage concomitant to acoustic trauma could be assessed by the audiogram configuration. Preserved hearing acuity at low and mild frequency range indicates the limited damage to the hearing cells in Korti's organ in the apical cochlear turn.

A novel gene for Usher syndrome type 2: mutations in the long isoform of whirlin are associated with retinitis pigmentosa and sensorineural hearing loss

2006 ◽  
Vol 121 (2) ◽  
pp. 203-211 ◽  
Author(s):  
Inga Ebermann ◽  
Hendrik P. N. Scholl ◽  
Peter Charbel Issa ◽  
Elvir Becirovic ◽  
Jürgen Lamprecht ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Retinitis Pigmentosa ◽  
Sensorineural Hearing Loss ◽  
Usher Syndrome ◽  
Sensorineural Hearing ◽  
Syndrome Type ◽  
Novel Gene ◽  
Usher Syndrome Type

scholarly journals Multicenter, Prospective, Phase II and Biomarker Study of High-Dose Bevacizumab as Induction Therapy in Patients With Neurofibromatosis Type 2 and Progressive Vestibular Schwannoma

2019 ◽  
Vol 37 (35) ◽  
pp. 3446-3454 ◽  
Author(s):  
Scott R. Plotkin ◽  
Dan G. Duda ◽  
Alona Muzikansky ◽  
Jeffrey Allen ◽  
Jaishri Blakeley ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Growth Factor ◽  
Phase Ii ◽  
Standard Dose ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
High Dose ◽  
Radiographic Response ◽  
Bevacizumab Treatment

PURPOSE Bevacizumab treatment at 7.5 mg/kg every 3 weeks results in improved hearing in approximately 35%-40% of patients with neurofibromatosis type 2 (NF2) and progressive vestibular schwannomas (VSs). However, the optimal dose is unknown. In this multicenter phase II and biomarker study, we evaluated the efficacy and safety of high-dose bevacizumab in pediatric and adult patients with NF2 with progressive VS. PATIENTS AND METHODS Bevacizumab was given for 6 months at 10 mg/kg every 2 weeks, followed by 18 months at 5 mg/kg every 3 weeks. The primary end point was hearing response defined by word recognition score (WRS) at 6 months. Secondary end points included toxicity, radiographic response, quality of life (QOL), and plasma biomarkers. RESULTS Twenty-two participants with NF2 (median age, 23 years) with progressive hearing loss in the target ear (median baseline WRS, 53%) were enrolled. Nine (41%) of 22 participants achieved a hearing response at 6 months (1 of 7 children and 8 of 15 adults; P = .08). Radiographic response was seen in 7 (32%) of 22 patients with VS at 6 months (7 of 15 adults and 0 of 7 children; P = .05). Common mild to moderate adverse events included hypertension, fatigue, headache, and irregular menstruation. Improvement in NF2-related QOL and reduction in tinnitus-related distress were reported in 30% and 60% of participants, respectively. Paradoxically, high-dose bevacizumab treatment was not associated with a significant decrease in free vascular endothelial growth factor but was associated with increased carbonic anhydrase IX, hepatocyte growth factor, placental growth factor, stromal cell-derived factor 1α, and basic fibroblast growth factor concentrations in plasma. CONCLUSION High-dose bevacizumab seems to be no more effective than standard-dose bevacizumab for treatment of patients with NF2 with hearing loss. In contrast to adults, pediatric participants did not experience tumor shrinkage. However, adult and pediatric participants reported similar improvement in QOL during induction. Novel approaches using bevacizumab should be considered for children with NF2.

The link between Brain and Hearing system

2020 ◽  
Vol 12 (4) ◽  
pp. 114-117
Author(s):  
Alireza Bina
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Auditory System ◽  
Auditory Nerve ◽  
Neurofibromatosis Type ◽  
Auditory Brainstem ◽  
Auditory Neuropathy ◽  
Sensorineural Hearing ◽  
Brain Disorder ◽  
Hearing System

There are some studies which confirmed that dysfunction in Central Nervous System(CNS) may cause a malfunction in the Peripheral Auditory system (Cochlea_ Auditory Nerve, Auditory Neuropathy), but the question is could Brain Disorder without any lesion in the Cochlea and/or Auditory nerve cause Sensorineural Hearing Loss? It means that the Audiogram shows that the patient suffers from sensorineural hearing loss but the site of the lesion is neither Sensory nor Neural while Brain may be involved in charge of this. And if the answer is yes then could we hear with our Brain and without Cochlea and /or Auditory nerve? We deal with this subject in this paper by: Otosclerosis and Meniere’s disease and The Brain Involvement. Hearing Loss following dysfunction in the Central Auditory and/or central non auditory system. Auditory Brainstem Implant in Patients who suffer from Neurofibromatosis Type two compare to Non Tumor cases, Mondini Syndrome, Michel aplasia. Possible role of Utricle and Saccule in Auditory (Hearing) System We propose a new Hypothesis that the External Ear Canal is not the only input of Auditory Signals, Sounds could transfer by our eyes and skin to the Cerebral Cortex and approach to the Cochlea (Backward Auditory input pathway of Sounds).

scholarly journals Age at Onset and Presenting Symptoms of Neurofibromatosis Type 2 as Prognostic Factors for Clinical Course of Vestibular Schwannomas

Cancers ◽  
2020 ◽  
Vol 12 (9) ◽  
pp. 2355
Author(s):  
Isabel Gugel ◽  
Florian Grimm ◽  
Julian Zipfel ◽  
Christian Teuber ◽  
Ulrike Ernemann ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Clinical Course ◽  
Age At Onset ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Vestibular Schwannomas ◽  
Speech Discrimination ◽  
Predictive Values ◽  
Presenting Symptoms

The presenting symptoms of the tumor suppressor gene syndrome neurofibromatosis type 2 (NF2) are often non-specific and unrelated to the disease hallmark bilateral vestibular schwannomas (VS). However, age at onset and presenting symptoms may have predictive values for the clinical course of VS. In this retrospective single-center study, we addressed this issue by reviewing 106 patients with 194 VS. Presenting symptoms attributable to VS commonly occur in 87% of adults and 31% of children. Age at onset significantly correlates with tumor volumes at presentation (p = 0.034). In addition, age at onset significantly correlates with pure-tone average (p = 0.0001), speech discrimination scores (p = 0.001), age at beginning of hearing loss (p = 0.0001), age at deafness (p = 0.0001), and age at first surgery (p = 0.0001). Patients presenting with VS related symptoms had significantly (p < 0.05) worse hearing values at presentation and after surgery. These patients also exhibited higher growth rates and tumor volumes compared to patients with non-VS related presenting symptoms, but this difference did not reach the significance level of p < 0.05. Due to the late appearance of these symptoms, the time of beginning hearing loss, surgery and deafness is significantly delayed (p < 0.05) compared to patients not presenting with VS. In summary, age at onset and type of presenting symptom provide excellent prognostic parameters for predicting VS- and hearing-related clinical course.

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