[Clinical analysis of Mondini @entity1141 with XXXX and preliminary genetic research of it].

@entity1 @entity2167 @entity693 @entity5173 @entity1141 @entity292 @entity6361
OBJECTIVE: To summarize and analyze the clinical characteristics of Mondini @entity1141 with @entity5173 , as well as preliminarily investigate the genetic mechanism of the disease. METHOD: The clinical data of 2 @entity1 diagnosed as Mondini @entity1141 with @entity5173 treated in our hospital were analyzed. Blood samples of these two @entity1 were obtained to extract DNA. We screened DNA samples for gene @entity6361 mutations by using polymerase chain reaction and direct sequencing. The sequencing results were analyzed in DNASTAR software. RESULT: Both @entity1 came to our hospital because of @entity693 , and the @entity292 were both located in vestibular window. @entity1 were cured one-time after surgical closure of the leakages with temporalis + temporalis fascia + temporalis through the mastoid approach. No pathogenic mutations of gene @entity6361 with exome sequencing were found. CONCLUSION: Mondini @entity1141 with @entity5173 should be considered in @entity1 with @entity693 and @entity2167 . Temporal bone HRCT is helpful to the diagnosis. Surgical closure is an effective therapeutic method and may prevent @entity693 . The molecular mechanism of simple Mondini @entity1141 needs further study.
