A Case Study Of Auditory Brainstem Implant in a Patient with Neurofibromatosis Type Ⅱ / 听力学及言语疾病杂志

ABSTRACT

Objective To study the clinical characteristics and treatment of neurofibromatosis type Ⅱ (neuro-fibromatosis type 2 ,NF2 ,bilateral acoustic neuroma) ,and the effects of auditory brainstem implant for treating to-tal deafness after bilateral acoustic neuroma resection .Methods One case of bilateral acoustic neuroma and all clini-cal data in terms of diagnosis ,treatment and hearing -speech rehabilitation after surgery were retrospectively stud-ied .Results The patient was a thirteen years old boy .His clinical symptoms were hearing loss on the right ear ,tin-nitus ,hoarseness and gait instability three years .MRI showed space occupying lesion in the cerebellopontine angle . The postoperative pathological diagnosis was bilateral acoustic neuroma .The initial switch -on was peformed six weeks after the surgery ,and confirmed that all electrodes generated listening responses .As the extension of recov-ery time ,the correct recognition rate of patients on the natural environment sound ,vowel ,monosyllabic were on the rise and the pure tone hearing threshold gradually decreased .The vowel correct recognition rate of postoperative 6 , 9 ,12 ,24 ,and 36 months were 14% ,18% ,20% ,24% ,and 20% ,respectively .The recognition rate of monosyl-labic and open words at each postoperative rehabilitation stage were 0 .Conclusion The clinical characteristics and treatment of bilateral acoustic neuroma were different from the unilateral acoustic neuroma .The individualized treat-ment should be followed .Auditory brainstem implant could be performed in patients with post - bilateral acoustic neuroma resection .The accurate location of the cochlear complex during the surgery was the crucial point for the success of ABI .