Otological IgG4-Related Disease With Inner Ear Involvement: A Case Report and Review of Literature.

Immunoglobulin G4-related disease (IgG4-RD) is a chronic inflammatory disease involving multiple organs. Some studies have reported otological manifestations of IgG4-RD, although most studies describe involvement of the middle ear, and reports on inner ear manifestations are limited. Here, we describe a case of a 30-year-old man with IgG4-RD involving the inner ear.

Neural Response Telemetry Thresholds in Patients with Cochlear Nerve Canal Stenosis.

Objective: To explore neural response telemetry (NRT) thresholds in patients with stenotic versus normal cochlear nerve canals.

Study Design: Case series with chart review.

Setting: Tertiary referral center.

[The relization of EABR test out of cochlea and the effects of different location of electrode on EABR wave].

Objective: To investigate the feasibility of the round window stimulation electrical evoked auditory brainstem response (EABR) test, and optimize the parameters of recording and stimulation electrodes positions.

Method: Ten healthy Hartley guinea pigs (20 ears) were used for the EABR test. The positive stimulation electrodes were placed into the round window niche, the animals were divided into three group according to the negative electrodes position, group A: the electric field was parallel with the projection of cochlear modiolus on the tympanic membrane, group B: the electric field was perpendicular to modiolus projection toward to the mastoid, group C: the electric field was perpendicular to modiolus projection toward to the zygomatic process.

Cochlear implantation in 21 patients with common cavity malformation.

Conclusion: The facial recess approach is preferred in common cavity (CC) malformation with an incomplete basal turn, and the transmastoid single-slit labyrinthotomy approach in classic CC malformation. Patients with CC benefit from cochlear implantation (CI) over time, but the audiological and speech development is poorer than in cases with normal cochleas.

Objectives: To discuss the surgical aspects and performance of CI in 21 patients with CC malformation.

[Development of electrically evoked auditory brainstem response modules of REZ-I domestic cochlear implant device].

Objective: To develop electrically evoked auditory brainstem response(EABR) modules of REZ-I domestic cochlear implant device, and testify the reliability and validity of the modules.

Methods: Postoperative EABR were recorded in guinea pigs by using the self-designed EABR module.

Results: EABR waves were recorded in all 15 ears of 9 guinea pigs with normal hearing.

[Morphologic feature and cochlear implant surgical approach for cochlear modiolus deficiency].

Objective: To review the classification of cochlear modiolus deficiency and decision on surgical approach for above case,in order to provide mastery for cochlear implant (CI) indication.

Method: Basing on temporal bone HRCT pre-operation, CI subjects with modiolus deficiency were defined as following groups: (1) deficiency caused by cochlear dysplasia (Mondini malformation); (2) deficiency caused by dysplasia of cochlear and vestibule (Common cavity malformation); (3) deficiency caused by absence of internal acoustic meatus fundus (IP-III malformation). Three types of surgical approach were utilized: type I, electrode array was introduced through facial recess, enlarged the round window, type II, opened the surface of chchlea, electrode array was introduced through facial recess, fenestration on posterior promontory and then inserted around lateral wall of inner-cochlear cavity.

[Surgery and electroneurophysiological evaluation for CI case with modiolus ossification].

Objective: The purpose of this study is to report surgical skills for CI cases with modiolus ossification and to investigate the relation between post-operational electroneurophysilogical test result and speech recognition result. Further more, we also attempt to confirm indications for CI in this specific population.

Method: Based on temporal bone HRCT, 7 subjects were identified as modiolus ossification from 101 cases with cochlear ossification.

Surgical methods and postoperative results of cochlear implantation in 79 cases of ossified cochlea.

Conclusion: A man-made bone tunnel of 1.5 turns around the modiolus can be created in cases of total cochlear ossification. Patients with ossified cochlea types I and II achieved satisfactory hearing results after cochlear implantation (CI).

[Diagnosis of cochlear nerve foramen stenosis and atresia of the cochlear implantation].

Objective: To summarize methods on diagnosis of congenital cochlear nerve canal (CNC) stenosis or atresia and to report results of post-operation such as auditory electrophysiological test and speech test.

Method: Based on temporal bone HRCT and internal acoustic canal MRI, 27 bilateral congenital CNC stenosis/bony atresia cases were distinguished from 3 700 CI cases. Unilateral cochlear implantations were conducted above 27 cases.

Electrically-evoked frequency-following response (EFFR) in the auditory brainstem of guinea pigs.

It is still a difficult clinical issue to decide whether a patient is a suitable candidate for a cochlear implant and to plan postoperative rehabilitation, especially for some special cases, such as auditory neuropathy. A partial solution to these problems is to preoperatively evaluate the functional integrity of the auditory neural pathways. For evaluating the strength of phase-locking of auditory neurons, which was not reflected in previous methods using electrically evoked auditory brainstem response (EABR), a new method for recording phase-locking related auditory responses to electrical stimulation, called the electrically evoked frequency-following response (EFFR), was developed and evaluated using guinea pigs.

[Surgical approach of cochlear implantation in patients with common cavity].

Objective: To discuss the surgical approach of cochlear implantation in patients with common cavity.

Method: Seventeen patients with common cavity underwent cochlear implantations through facial recess approach or transmastoid lateral semicircular canal approach,according to the preoperative imaging and audiological evaluation.

Result: Common cavity was opened and electrodes were inserted smoothly in all cases.

Cochlear implantation surgery in patients with narrow facial recess.

Conclusion: The new method of facial recess enlargement through suspending, antedisplacing, and adhering the chorda tympani nerve to the posterior wall of the auditory canal can expose the round window, make electrode insertion easier, and preserve the function of the facial nerve and chorda tympani nerve.

Objective: To describe and report cochlear implantation surgery in patients with narrow facial recess, including surgical technique and postoperative outcomes.

Methods: Cochlear implantation surgery was performed in our hospital in 39 cases with narrow facial recess by enlarging the facial recess.

[Canal-wall-down mastoidectomy and tympanoplasty surgery preserving chorda tympani nerve integrality].

Objective: To report the way for searching the chorda tympani nerve and the significance for preserving the chorda tympani nerve during canal-wall-down mastoidectomy and tympanoplasty surgery.

Method: Sixty-six cases with chronic suppurative otitis media underwent canal-wall-down mastoidectomy and tympanoplasty surgery. According to the marker of the short crus of incus, the posterior wall of auditory canal was lowered and crista of the chorda tympani nerve was found through tracing the facial nerve contour.

The prediction of CSF gusher in cochlear implants with inner ear abnormality.

Conclusion: The incidence rate of the cerebrospinal fluid (CSF) gusher in cochlear implant with inner ear abnormality is 30%. Bony defect in the fundus of the internal acoustic meatus is most often responsible for the occurrence of CSF gusher. This feature of the CT scan can be used to predict the occurrence of a CSF gusher.

[Perioperative complications of 1396 patients with cochlear implantation].

Objective: To discuss the perioperative complications of 1396 cases (1402 ears) with cochlear implantation (CI), and to supply clinical experience for risk reduction in the perioperative period.

Method: All patients were profound sensorineural deafness, with 1379 prelingual cases and 17 postlingual cases. (1) Preoperative examinations: audiology,imaging studies, evaluation of physical and intellectual development.

[Analysis of imaging and clinical feature of leukoencephalopathy with hearing loss first complaint].

Objective: To investigate the imaging and clinical feature of leukoencephalopathy with hearing loss first complaint.

Method: The head MRI and clinical feature of 7 cases leukoencephalopathy with hearing loss first complaint were retrospectively analysed, including their place, shape, range and blood plasma very-long-chain fatty acid (VLCFA).

Result: The head MRI of 2 cases shows symmetry distributed long T, and T2 signal like butterfly aliform in white matter beside both cornu posterior ventriculi lateralis.

Cochlear implantation in China: review of 1,237 cases with an emphasis on complications.

Objective: The purpose of this study is to retrospectively review the complications of patients who underwent cochlear implantation at 51 hospitals in mainland China over a decade.

Methods: A retrospective analysis of 1,237 patients who underwent cochlear implantation from February 1998 to December 2008. The patients were reviewed for demographic information, type of hearing loss, abnormal findings on temporal CT scans, cochlear implant device, procedure time, and complications that included meningitis, hematoma (intracranial and extracranial), wound infection and implant extrusion, cerebrospinal fluid leak and facial palsy.

[Cochlear implantation in patients with secretory otitis media].

Objective: To evaluate the indications and surgery time of cochlear implantation in patients with otitis media with effusion (OME).

Methods: Cochlear implantation was performed in 26 patients with bilateral profound sensorineural hearing loss and OME. Non-Surgical Treatment was conducted for OME before the following implantation.

[Cochlear implant approach in children patients with sigmoid sinus antedisplacement].

Objective: To summarize the operation approach and management during cochlear implant operation in children with sigmoid sinus antedisplacement.

Method: Five hundred and thirty-eight profound hearing loss children were performed auditory and imagiological examinations before cochlear implant. We analyzed the location of the sigmoid sinus from the high resolution CT scan and then performed cochlear implant to all these patients.

[Indication and approach of cochlear implantation with common cavity].

Objective: To discuss the indication and approach of cochlear implantation with common cavity inner ear malformation.

Method: Through imaging examination preoperatively for profound sensorineural hearing loss patients, we found 6 cases with common cavity inner ear malformation, in which cochlear, vestibule and lateral semicircular canal formed a big cavity. Among these cases, five had residual hearing, in the others, residual hearing was not detected.