Shusheng Gong

Clinical characteristics of pulsatile tinnitus caused by sigmoid sinus diverticulum and wall dehiscence: a study of 54 patients

Abstract Conclusions: CT angiography (CTA) and digital subtraction angiography (DSA) are valuable tools in imaging work-ups for the diagnosis of sigmoid sinus diverticulum (SSD) and sigmoid sinus wall dehiscence (SSWD). The development of pulsatile tinnitus (PT) resulting from SSD and SSWD may be associated with the dominance of venous systems. Objective: Our goal was to evaluate the clinical characteristics of PT caused by SSD and SSWD. Methods: This was a retrospective chart review undertaken in a tertiary academic referral center. Fifty-four patients with PT due to SSD and SSWD were recruited. Hospital files of these patients were assessed. Data included medical history, physical examinations, auxiliary examinations, and radiographic findings of CTA and DSA. Results: The study population comprised 51 females and 3 males. Most patients with PT caused by SSD and SSWD were middle-aged women. All had normal otoscopy results. Anomalies occurred in or adjacent to the region of the transverse–sigmoid sinus junction in 52 patients. Half of the patients (27/54) presented abnormal results of examination of blood lipids. There were 57.41% (31/54) cases with ipsilateral dominance of the venous system, 9.26% (5/54) cases with contralateral dominance, and 33.33% (18/54) cases with co-dominance of the venous system.

Facial nerve course in congenital aural atresia – identified by preoperative CT scanning and surgical findings

Conclusions. The facial nerve courses shown by preoperative CT imaging coincide with the surgical findings in most congenital aural atresia cases. CT scanning is critical in evaluation of a patients candidacy for atresia surgery. Objectives. To evaluate the diagnostic value of preoperative CT scanning of the facial nerve course in congenital aural atresia. Subjects and methods. All inpatients (135 cases) with congenital aural atresia had routine preoperative CT examination. The facial nerve courses were observed in the operation and compared with the preoperative CT imaging. Results. CT imaging showed that the mastoid portions of the facial nerves in 57% of cases (77/135) were located at the level of the round window, and 17% (23/135) were located at the levels of the oval window and the cochlea. Differential overlapping of the oval window by the facial nerve could also be shown in most of the cases. In the majority of cases (75%, 61/81) the tympanic portions of the facial nerve on CT imaging could be confirmed by surgical findings. In severe overlapping of the oval window or cases with anterior displacement, stapedectomy or tympanoplasty, respectively, could not be performed as usual.

Surgical treatment of pulsatile tinnitus caused by the sigmoid sinus diverticulum: a preliminary study.

AbstractThe sigmoid sinus diverticulum (SSD) is an increasingly recognized cause of pulsatile tinnitus (PT). Surgical reconstruction of the sigmoid sinus wall has been found to be highly effective for SSD; however, surgical techniques still need to be refined to reduce the incidence of serious postoperative complications. Moreover, scrutinizing more cases of SSD is desirable for establishing the diagnostic criteria and standardizing the surgical technique. This study was retrospectively undertaken in 28 patients who were diagnosed with SSD upon computed tomography angiography (CTA) and digital subtraction angiography findings at Beijing Tongren Hospital in China. A majority of patients (20/28) presented with SSD and accompanying sigmoid sinus wall dehiscence (SSWD). Twenty-five patients opted to undergo sigmoid sinus wall reconstruction, and 3 patients refused surgery. Following surgery, 17 patients experienced complete resolution of PT, 3 patients experienced partial resolution, and 5 patients experienced no change in PT. No serious complications were found postoperatively. A comparative analysis of the preoperative and postoperative CTA findings suggested that completely resolving SSD and the accompanying SSWD resulted in the elimination of PT. In conclusion, SSD is generally accompanied by SSWD. Sigmoid sinus wall reconstruction is a safe and effective approach for the treatment of SSD. During surgery, completely resolving both SSD and SSWD is advisable, and maintaining the normal diameter of the sigmoid sinus is imperative.

Integrated protocol of auricle reconstruction combined with hearing reconstruction

Abstract Conclusions: Auricle reconstruction combined with hearing reconstruction can help patients with microtia to obtain practical hearing improvement as well as cosmetic improvement. The integrated procedures can facilitate the incision design and obtain a natural-looking three-dimensional shape of the reconstruction auricle. Objectives: To discuss the advantages of an integrated protocol for auricle reconstruction combined with hearing reconstruction. Methods: We retrospectively analyzed the clinical information and follow-up results of 1300 cases (1460 ears) of congenital aural atresia with microtia who underwent auricle reconstruction combined with hearing reconstruction from January 1988 to December 2007 in Tongren Hospital. In the first stage, rib cartilage was harvested to sculpt the framework then implanted into the subcutaneous pockets in the desired position. In addition, hearing reconstruction was carried out by the antrum approach. In the second stage, the auricle framework was elevated to accomplish the auricle reconstruction. Results: In all, 1445 of 1460 ears were closed by primary healing. The other 15 ears were successfully reconstructed with revision surgeries without graft failure complications. The three-dimensional profiles were well defined in 1248 ears. Total satisfaction rate was 85.5%. During the long-term follow-up, no cartilage resorption was found and all auricles retained good cosmetic effects.

Transcription Factors Expressed in Mouse Cochlear Inner and Outer Hair Cells

Regulation of gene expression is essential to determining the functional complexity and morphological diversity seen among different cells. Transcriptional regulation is a crucial step in gene expression regulation because the genetic information is directly read from DNA by sequence-specific transcription factors (TFs). Although several mouse TF databases created from genome sequences and transcriptomes are available, a cell type-specific TF database from any normal cell populations is still lacking. We identify cell type-specific TF genes expressed in cochlear inner hair cells (IHCs) and outer hair cells (OHCs) using hair cell-specific transcriptomes from adult mice. IHCs and OHCs are the two types of sensory receptor cells in the mammalian cochlea. We show that 1,563 and 1,616 TF genes are respectively expressed in IHCs and OHCs among 2,230 putative mouse TF genes. While 1,536 are commonly expressed in both populations, 73 genes are differentially expressed (with at least a twofold difference) in IHCs and 13 are differentially expressed in OHCs. Our datasets represent the first cell type-specific TF databases for two populations of sensory receptor cells and are key informational resources for understanding the molecular mechanism underlying the biological properties and phenotypical differences of these cells.

Tumor Necrosis Factor-α-Induced Ototoxicity in Mouse Cochlear Organotypic Culture.

Tumor necrosis factor (TNF)-α is a cytokine involved in acute inflammatory phase reactions, and is the primary upstream mediator in the cochlear inflammatory response. Treatment of the organ of Corti with TNF-α can induce hair cell damage. However, the resulting morphological changes have not been systematically examined. In the present study, cochlear organotypic cultures from neonatal mice were treated with various concentrations and durations of TNF-α to induce inflammatory responses. Confocal microscopy was used to evaluate the condition of hair cells and supporting cells following immunohistochemical staining. In addition, the ultrastructure of the stereocilia bundle, hair cells, and supporting cells were examined by scanning and transmission electron microscopy. TNF-α treatment resulted in a fusion and loss of stereocilia bundles in hair cells, swelling of mitochondria, and vacuolation and degranulation of the endoplasmic reticulum. Disruption of tight junctions between hair cells and supporting cells was also observed at high concentrations. Hair cell loss was preceded by apoptosis of Deiters’ and pillar cells. Taken together, these findings detail the morphological changes in the organ of Corti after TNF-α treatment, and provide an in vitro model of inflammatory-induced ototoxicity.

Sigmoid Sinus Wall Reconstruction for Pulsatile Tinnitus Caused by Sigmoid Sinus Wall Dehiscence: A Single-Center Experience

Objective To evaluate clinical characteristics and present surgical outcomes of PT caused by sigmoid sinus wall dehiscence (SSWD) Methods This study retrospectively reviewed 34 patients with PT who were diagnosed with SSWD in our institution between December 2008 and July 2013. Among them, 27 patients underwent sigmoid sinus wall reconstruction (surgery group) and 7 patients refused surgery (non-surgery group). Preoperative data were obtained from the patients’ medical records. All patients were followed up regularly for at least 25 months. Preoperative and postoperative computed tomography angiography (CTA) images were compared. Student’s t-tests were used to compare age, body mass index (BMI) and preoperative Tinnitus Handicap Inventory (THI) scores between the surgery and the non-surgery groups and to compare pre- and follow-up THI scores. Results There was no significant difference in age, body mass index, or preoperative THI scores between groups. Following surgery, 14 patients had complete resolution, 5 had partial resolution, 7 experienced no change and PT was aggravated in 1 patient. The difference between preoperative and postoperative THI scores was significant. No severe complications were found postoperatively. Comparison of the preoperative and postoperative CTA images revealed that remnant unrepaired dehiscences were the cause of unsatisfactory outcomes following surgery. In the non-surgery group, PT remained largely unchanged. Conclusions Sigmoid sinus wall reconstruction is an effective and safe treatment for PT caused by SSWD. It is imperative that all regions of the dehiscence are sufficiently exposed and resurfaced during surgery.

Frequency-Dependent Neural Activity in Patients with Unilateral Vascular Pulsatile Tinnitus

Previous resting-state functional magnetic resonance imaging (rs-fMRI) studies have shown that neurological changes are important findings in vascular pulsatile tinnitus (PT) patients. Here, we utilized rs-fMRI to measure the amplitude of low-frequency fluctuations (ALFF) in forty patients with unilateral PT and forty age-, gender-, and education-matched normal control subjects. Two different frequency bands (slow-4, 0.027–0.073 Hz, and slow-5, 0.010–0.027 Hz, which are more sensitive to subcortical and cortical neurological signal changes, resp.) were analyzed to examine the intrinsic brain activity in detail. Compared to controls, PT patients had increased ALFF values mainly in the PCu, bilateral IPL (inferior parietal lobule), left IFG (inferior frontal gyrus), and right IFG/anterior insula and decreased ALFF values in the multiple occipital areas including bilateral middle-inferior occipital lobe. For the differences of the two frequency bands, widespread ALFF differences were observed. The ALFF abnormalities in aMPFC/ACC, PCu, right IPL, and some regions of occipital and parietal cortices were greater in the slow-5 band compared to the slow-4 band. Additionally, the THI score of PT patients was positively correlated with changes in slow-5 and slow-4 band in PCu. Pulsatile tinnitus is a disease affecting the neurological activities of multiple brain regions. Slow-5 band is more sensitive in detecting the alternations. Our results also indicated the importance of pathophysiological investigations in patients with pulsatile tinnitus in the future.

Abnormal resting-state functional connectivity study in unilateral pulsatile tinnitus patients with single etiology: A seed-based functional connectivity study

OBJECTIVE Previous studies demonstrated altered regional neural activations in several brain areas in patients with pulsatile tinnitus (PT), especially indicating an important role of posterior cingulate cortex (PCC). However, few studies focused on the degree of functional connectivity (FC) of this area in PT patients. In this study, we will compare the FC of PCC in patients affected with this condition and normal controls by using resting-state functional magnetic resonance imaging (fMRI). METHODS Structural and functional MRI data were obtained from 36 unilateral PT patients with single etiology and 36 matched healthy controls. FC feature of the region of interest (PCC) were characterized using a seed-based correlation method with the voxels in the whole-brain. RESULTS Compared with healthy controls, patients showed significant decreased FC to the right middle temporal gyrus (MTG), right thalamus and bilateral insula. By contrast, PCC demonstrated increased functional connectivity between the precuneus, bilateral inferior parietal lobule and middle occipital gyrus. We also found correlations between the disease duration of PT and FC of PCC-right MTG (r=-0.616, p<0.001). CONCLUSIONS Unilateral PT patients could have abnormal FC to the PCC bilaterally in the brain. PCC, as a highly integrated brain area, is an example of nucleus that was involved in mediation between different neural networks. It might be a modulation core between visual network and auditory network. The decreased FC of MTG to PCC may indicate a down regulation of activity between PCC and auditory associated brain cortex. Decreased FC between limbic system (bilateral AI) and PCC may reflect the emotional message control in patient group. This study facilitated understanding of the underlying neuropathological process in patients with pulsatile tinnitus.

The development of auditory skills in young children with Mondini dysplasia after cochlear implantation.

The aim of this study is to survey and compare the development of auditory skills in young children with Mondini dysplasia and profoundly-deaf young children with radiologically normal inner ears over a period of 3 years after cochlear implantation. A total of 545 young children (age 7 to 36 months) with prelingual, severe to profound hearing loss participated in this study. All children received cochlear implantation. Based on whether or not there was a Mondini dysplasia as diagnosed with CT scanning, the subjects were divided into 2 groups: (A) 514 young children with radiologically normal inner ears and (B) 31 young children with Mondini dysplasia. The Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) was used to assess the childrens auditory skills that include vocalization changes, spontaneous alerting to sounds in everyday living environments, and the ability to derive meaning from sounds. The assessment was performed prior to surgery and at 1, 3, 6, 9, 12, 24, and 36 months after implant device switch-on. The mean scores for overall auditory skills were not significantly different between groups A and B at pre-surgery, 1, 12, 24, and 36 months post-surgery, but were significantly different at 3, 6, and 9 months post-surgery. The mean scores for all auditory skills in children with Mondini dysplasia showed significant improvement over time. The mean scores for the three subcategories of auditory skills in children with Mondini dysplasia also showed significant differences at pre-surgery, 1, 3, 6, and 9 months, however, there were no significant differences at 12, 24, and 36 months. Overall, the auditory skills of young children with Mondini dysplasia developed rapidly after cochlear implantation, in a similar manner to that of young children with radiologically normal inner ears. Cochlear implantation is an effective intervention for young children with Mondini dysplasia.