Hearing Loss, Sudden ; diagnosis ; Humans ; Practice Guidelines as Topic

Cerebellopontine Angle ; anatomy & histology ; Ear, Inner ; anatomy & histology ; Humans ; Microsurgery ; Neuroma, Acoustic ; surgery

Cerebellopontine Angle ; anatomy & histology ; Ear, Inner ; anatomy & histology ; Humans ; Microsurgery ; Neuroma, Acoustic ; surgery

OBJECTIVEThe aim of the study was to evaluate the effects of cochlear implantation on bilateral perceived tinnitus of postlingually deafened patients.

METHODTotal of 32 postlingually deafened cochlear implant recipients (13 males, 19 females; 31 unilateral,1 bilateral cochlear implantation) with bilateral tinnitus were assessed by Tinnitus Handicap Inventory (THI) before and after implantation. Furthermore, characteristics information of tinnitus were recorded to analyze the variety of tinnitus. Intensity of tinnitus in ispilateral and contralateral ears was investigated when cochlear implant was switched on and off. Statistical analysis was performed by SPSS 18.0.

RESULTSTwenty-two patients (71.0%) got a decreased THI score of more than 20 points after the operation. The average THI score of pre-operation was 56.4±18.1, and post-operation score was 24.7±22.7. A paired-samples t test analysis showed a significant difference of the THI total scores (t=8.037, P<0.05). Tinnitus in the ipsilateral ear improved in 81.9% (27/33) patients and 18.2% (6/33) unchanged. Tinnitus in the contralateral ear improved in 71.0% (22/31) patients, 16.1% (5/31) unchanged and 12.9% (4/31) worsen. Tinnitus of ipsilateral ears improved or abolished in 97.0% (32/33) patients, while 83.9% (26/31) of contralateral ear got reduction or absence of loudness when cochlear implant was switched on.

CONCLUSIONSAs an electrical stimulation treatment method, cochlear implant has the inhibitory effect on tinnitus. However, cases of tinnitus aggravation were found, further data is still necessary before considering CI as a treatment of tinnitus.

Cochlear Implantation ; Cochlear Implants ; Electric Stimulation ; Female ; Humans ; Male ; Perception ; Postoperative Period ; Tinnitus ; surgery

OBJECTIVEEndolymphatic sac tumors (ELSTs) are rare in the general population with much higher prevalence in von Hippel-Lindau(VHL) disease. The purpose of this study is to present two cases of endolymphatic sac tumor with VHL disease with analysis of VHL gene and to explore their association with VHL disease using molecular analysis.

METHODSClinical data of these two patients from different VHL families were studied. DNAs extracted from peripheral bloods were amplified by the polymerase chain reaction using oligonucleotide primers corresponding to the VHL gene, then compared the mutations with the Human Gene Mutation Database.

RESULTSIn case 1, 6 family members were enrolled in the study. Among them, three had been identified to have a germline missense point mutation at codon 194 of the VHL gene exon 1 (p.S65W). The little sister of the patient (case 1) underwent vitrectomy for retinal hemangioblastoma 5 years ago in another hospital. The mother of the patient (case 1) was further diagnosed to have a cerebellar hemangioblastoma and renal carcinoma in the following physical examination. Case 2 with her parents were also tested. Codon 499 of the VHL gene exon 3 (p.R167W) were detected in case 2 and her mother, but the mother refused further examination.

CONCLUSIONSThe genetic diagnosis plays an important role in early detection of symptomatic patients and suspected patients. Clinical screening for members of the VHL families, and close follow-up of carriers allow an early detection of tumors and the metastasis, which is the most common cause of death of these patients.

Adolescent ; Adult ; DNA Mutational Analysis ; Ear Neoplasms ; complications ; genetics ; Endolymphatic Sac ; Female ; Humans ; Von Hippel-Lindau Tumor Suppressor Protein ; genetics ; von Hippel-Lindau Disease ; complications ; genetics

OBJECTIVEThe purpose of the report was to investigate the causes of CSF leakage and discuss the methods for prevention and management of CSF leakage following translabyrinthine resection of acoustic neuromas.

METHODSA retrospective review of cerebrospinal fluid leakage following translabyrinthine approach for 152 acoustic neuromas patients, from January 1983 to December 2013, was performed. The cases were divided into two groups, traditional and modified closure techniques. The incidence of CSF leakage was compared between the two groups.

RESULTSThe incidence of postoperative CSF leakage by translabyrinthine approach was 5.9% (9/152), with four cases of rhinorrhea, two case of wound leakage, one case of rhinorrhea and otorrhea, one case of otorrhea, and one case of rhinorrhea and wound leakage. The CSF leakage incidence of traditional closure technique was 14.3% (5/35); the incidence of modified closure technique was 3.4% (4/117). After introducing a modified closure technique, the incidence of the CSF leakage significantly decreased.

CONCLUSIONSAs a common complication of translabyrinthine approach, the incidence of CSF leakage is closely related to the closure technique. The incidence of the CSF leakage should decrease dramatically when adopting the modified closure technique.

Adult ; Cerebrospinal Fluid Leak ; prevention & control ; Craniotomy ; Ear, Inner ; surgery ; Female ; Humans ; Male ; Middle Aged ; Neuroma, Acoustic ; surgery ; Otologic Surgical Procedures ; adverse effects ; methods ; Postoperative Complications ; prevention & control ; Retrospective Studies

OBJECTIVETo observe the long-term safety and performance of Nurotron cochlear implant system. To analyze the auditory and speech rehabilitation characteristic of pediatric deafened patients after cochlear implantation.

METHODSTo analyze 3 years results of pediatric deafened patients with Nurotron® Venus™ cochlear implants. From April to June 2011, sixty deaf children received domestic cochlear implant. All subjects were switched on after 3 to 4 weeks of surgery, and were assessed the auditory rehabilitation on 3 months, 12 months, 24 months and 36 months after switching on. To evaluate the rehabilitation results by Aided Hearing Thresholds, Auditory & Verbal Ability Test and IT-MAIS Questionnaire. Meanwhile, the safety and performance of cochlear implant system were followed-up. Before surgery, all the recipients in this study were 12 to 71 months of age with the average hearing threshold higher than 90 dBHL.

RESULTSSixty cases of deaf children were implanted successfully. All the recipients used their devices every day, and all of their devices worked well. No complication or side effect related to implantation were found. The sound field aided hearing thresholds, vowels, consonants, monosyllabic words and two-syllable words test, as well as Ling's test, and IT-MAIS Questionnaire of all recipients were improved significantly with prolonged use of CI device. The results of grammatical competence, understanding, communication and presentation skills tests also get significantly promotion in 3 years follow up.

CONCLUSIONSWith prolonged use of CI device, the hearing and language tests of children recipients could obtain significantly improvement. The Nurotron® Venus™ CI system works safely and effectively.

Child ; Cochlear Implantation ; Cochlear Implants ; Deafness ; rehabilitation ; Hearing Tests ; Humans ; Language ; Speech Perception ; Time Factors ; Treatment Outcome