BACKGROUND: As new treatment modalities develop for the management of vestibular schwannomas (VS) in patients with neurofibromatosis type 2, it remains crucial to ascertain the natural history of the disease. OBJECTIVE: To determine the relationship between hearing and tumor growth in patients undergoing conservative VS management. METHODS: Patients harboring bilateral VS with at least 1 year of radiological follow-up were selected. Conservative management was proposed based on the small tumor size and/or serviceable hearing at presentation. Tumor size was calculated by using the 2-component box model and reported as mean tumor diameter. Hearing was evaluated by using pure-tone average and the American Academy of Otololaryngologists and Head and Neck Surgery classification. RESULTS: Forty-six patients harboring 92 VS were included. The mean clinical and radiological follow-up times were 6.0 and 4.2 years, respectively. The mean tumor diameter was 13 mm at presentation and 20 mm at the end of follow-up. Mean tumor growth rate was 1.8 mm/year. During follow-up, 17 patients (37%) underwent surgery for VS. Surgery-free rate for VS was 88% at 5 years. The number of patients with at least 1 serviceable ear was 39 (85%) at presentation and 34 (74%) at the end of follow-up, including 22 (66%) with binaural serviceable hearing maintained. There was no statistical correlation between tumor growth rate and preservation of serviceable hearing. Tumor growth rates and age at presentation were inversely correlated. CONCLUSION: This study illustrates the high variability among neurofibromatosis type 2 patients regarding hearing status and VS growth rate and justifies the choice of initial conservative management in selected cases. ABBREVIATIONS: : AAO-HNS, American Academy of Otololaryngologists and Head and Neck Surgery classificationMTD, mean tumor diameterNF2, neurofibromatosis type 2PTA, pure-tone averageSDS, speech discrimination scoreVS, vestibular schwannomas.
BACKGROUND: As new treatment modalities develop for the management of vestibular schwannomas (VS) in patients with neurofibromatosis type 2, it remains crucial to ascertain the natural history of the disease. OBJECTIVE: To determine the relationship between hearing and tumor growth in patients undergoing conservative VS management. METHODS:Patients harboring bilateral VS with at least 1 year of radiological follow-up were selected. Conservative management was proposed based on the small tumor size and/or serviceable hearing at presentation. Tumor size was calculated by using the 2-component box model and reported as mean tumor diameter. Hearing was evaluated by using pure-tone average and the American Academy of Otololaryngologists and Head and Neck Surgery classification. RESULTS: Forty-six patients harboring 92 VS were included. The mean clinical and radiological follow-up times were 6.0 and 4.2 years, respectively. The mean tumor diameter was 13 mm at presentation and 20 mm at the end of follow-up. Mean tumor growth rate was 1.8 mm/year. During follow-up, 17 patients (37%) underwent surgery for VS. Surgery-free rate for VS was 88% at 5 years. The number of patients with at least 1 serviceable ear was 39 (85%) at presentation and 34 (74%) at the end of follow-up, including 22 (66%) with binaural serviceable hearing maintained. There was no statistical correlation between tumor growth rate and preservation of serviceable hearing. Tumor growth rates and age at presentation were inversely correlated. CONCLUSION: This study illustrates the high variability among neurofibromatosis type 2patients regarding hearing status and VS growth rate and justifies the choice of initial conservative management in selected cases. ABBREVIATIONS: : AAO-HNS, American Academy of Otololaryngologists and Head and Neck Surgery classificationMTD, mean tumor diameterNF2, neurofibromatosis type 2PTA, pure-tone averageSDS, speech discrimination scoreVS, vestibular schwannomas.
Authors: Georgios Kontorinis; Jaya Nichani; Simon R Freeman; Scott A Rutherford; Samantha Mills; Andrew T King; Deborah Mawman; Sue Huson; Martin O'Driscoll; D Gareth Evans; Simon K W Lloyd Journal: Eur Arch Otorhinolaryngol Date: 2014-10-08 Impact factor: 2.503
Authors: Alvin T deTorres; Carmen C Brewer; Chris K Zalewski; Kelly A King; Robert Walker; Gretchen C Scott; Ashok R Asthagiri; Prashant Chittiboina; Hung Jeffrey Kim Journal: Otol Neurotol Date: 2018-03 Impact factor: 2.311
Authors: Eva Dombi; Simone L Ardern-Holmes; Dusica Babovic-Vuksanovic; Fred G Barker; Steve Connor; D Gareth Evans; Michael J Fisher; Stephane Goutagny; Gordon J Harris; Diego Jaramillo; Matthias A Karajannis; Bruce R Korf; Victor Mautner; Scott R Plotkin; Tina Y Poussaint; Kent Robertson; Chie-Schin Shih; Brigitte C Widemann Journal: Neurology Date: 2013-11-19 Impact factor: 9.910
Authors: Katrina A Morris; John F Golding; Patrick R Axon; Shazia Afridi; Claire Blesing; Rosalie E Ferner; Dorothy Halliday; Raj Jena; Pieter M Pretorius; D Gareth Evans; Martin G McCabe; Allyson Parry Journal: Neurooncol Pract Date: 2016-01-07