Objective To explore the diagnosis and treatment strategy of Noonan syndrome(NS)patients with extremely severe sensorineural deafness.Methods The clinical phenotypic characteristics of NS were comprehensively understood,the patients were comprehensively evaluated before surgery,and the potential risks caused by systemic multi-system abnormalities in perioperative period were identified and effective preventive measures were taken.Gathered were comprehensive and detailed collection of ear related data,postoperative effect evaluation and follow-up.Results For the NS patients successfully implanted,the postoperative effect was promising,and 3 years after surgery,the sentence recognition rate in quiet environment was 100％.SIR Scores level 5 and CAP scores level 9.Conclusion Following a comprehensive pre-operative examination and preparation,The operation of implant can be successfully accomplished and the patient is capable of achieving excellent hearing improvement,which significantly improves the quality of life of patients.
关键词
努南综合征/人工耳蜗植入/感音神经性聋
Key words
Noonan syndrome/Cochlear implant/Sensorineural hearing loss
维普
万方数据