Primary temporal bone angiosarcoma: a case report.

We present a rare case of temporal bone angiosarcoma diagnosed in a 26-year-old female patient at 36 week of pregnancy. The patient was referred with a 2 months history of left otalgia and tinnitus with a tender swelling above the mastoid. Cranial imaging studies showed a 7 x 5 x 4 cm hypervascularized mass located in the left middle fossa with lysis of the temporal bone and extension to the subcutis. After the baby was delivered by caesarean section, the patient entered the oncology protocol. Selective embolization of the feeding vessels was followed by gross total surgical resection using a combined supra- and infra-tentorial approach. Pathological findings were those of a poorly differentiated, highly malignant sarcoma with a large epitheloid component and immunohistochemical evidence of endothelial differentiation (CD31, Factor VIII related antigen, CD34), consistent with an angiosarcoma with epitheloid features. No extra-cranial tumor was found after extensive staging. The patient received adjuvant radiotherapy followed by a course of chemotherapy consisting of 6 cycles of paclitaxel. At 15 months follow-up, she developed multiple distant metastasis to a left postauricular lymph node and to the lungs and ribs. The patient was given a second line chemotherapy using doxorubicine and ifosfamide. Despite an initial good response, she died with metastatic disease 26 months after diagnosis. We present a rare case of primary temporal bone angiosarcoma and report our experience with a multimode therapeutic approach combining surgery, radiotherapy and chemotherapy.Peer reviewe

Characteristics and determinants of music appreciation in adult CI users

The main objective of this study was to assess the associations between self-reported listening habits and perception of music and speech perception outcomes in quiet and noise for both unilateral cochlear implant (CI) users and bimodal (CI in one ear, hearing aid in contra-lateral ear) users. Information concerning music appreciation was gathered by means of a newly developed questionnaire. Moreover, audiological data (pure-tone audiometry, speech tests in noise and quiet) were gathered and the relationship between speech perception and music appreciation is studied. Bimodal users enjoy listening to music more in comparison with unilateral CI users. Also, music training within rehabilitation is still uncommon, while CI recipients believe that music training might be helpful to maximize their potential with current CI technology. Music training should not be exclusively reserved for the good speech performers. Therefore, a music training program (MTP) that consists of different difficulty levels should be developed. Hopefully, early implementation of MTP in rehabilitation programs can enable adult CI users to enjoy and appreciate music and to maximize their potential with commercially available technology. Furthermore, because bimodal users consider the bimodal stimulation to be the most enjoyable way to listen to music, CI users with residual hearing in the contra-lateral ear should be encouraged to continue wearing their hearing aid in that ear