OBJECTIVE: To investigate the location, staging, clinical symptoms, imaging features, and surgical treatment of the congenital cholesteatoma of middle ear (CCME). METHOD: This was a retrospective review of 20 CCME cases. RESULT: Of 20 cases with CCME, 2 cases were classified as Postic stage I, 0 as stage II, 13 as stage III, 5 as stage IV. Conductive hearing loss was the most common clinical symptom. The mean preoperative PTA was 54.1 dB, and the mean ABG was 41.7 dB. One case underwent a modified mastoidectomy and a second-stage ossicular reconstruction; 2 cases experienced a radical mastoidectomy without ossicular reconsturction for extensive cholesteatoma; 5 cases underwent modified mastoidectomy and a one-stage tympanoplasty, with one case diagnosed as congenital malformation of ossicular chain (stapes hypoplasia); other 12 cases underwent a one-stage tympanoplasty. The cholesteatoma localized to the posterior-epitympanum or mesotympanum in all patients, mainly located in the incudostapedial joint. The mean postoperative PTA from 16 cases was 35.3 dB, and A-B gap was 20.2 dB. All patients were followed-up for at least 1 year after operation and recurrence was found in 2 cases. Three cases were accompanied with congenital malformation of ossicular chain. CONCLUSION CCME is a rare entity and diagnosis is usually delayed in clinical practice due to the silent nature of disease in its early stage. The prognosis of CCME mainly depended on the stage of the lesions.
Adolescent ; Adult ; Child ; Child, Preschool ; Cholesteatoma ; classification ; congenital ; pathology ; surgery ; Cholesteatoma, Middle Ear ; classification ; pathology ; surgery ; Female ; Follow-Up Studies ; Humans ; Male ; Retrospective Studies ; Young Adult

Aim To investigate the effect and mecha-nism of valproic acid on neuroglobin expression, and the neuroprotective role of valproic acid against H2 O2-induced neurotoxicity. Methods Western blot, RT-PCR and luciferase assay were used to detect the pro-tein levels, mRNA levels and promoter activity of mouse and human neuroglobin induced by valproic acid. Luciferase assay was used to investigate the role of transcription factor CREB in the up-regulation of neuroglobin by valproic acid. MTT assay was used to evaluate the effect of valproic acid against H2 O2-in-duced neurotoxicity. Results VPA treatment marked-ly increased the protein levels, mRNA levels and pro-moter activity of Ngb in mouse N2 a cells and human SKNSH cells. CREB specific inhibitor KG501 or CREB dominant negative mutant KCREB attenuated VPA-induced Ngb promoter activity. VPA could pro-tect N2a cells from H2 O2-induced neurotoxicity. Con-clusion CREB mediates VPA-induced Ngb up-regula-tion, which may contribute to the neuroprotective effects of VPA in oxidative stress in neurons.

Objective: To study the shift of oVEMP frequency tuning of unilateral Meniere′s disease, by analyzing oVEMP of air-conducted tone bursts in various frequencies. Methods: From May 2016 to October 2017, 33 patients with unilateral Meniere′s from Aerospace Center Hospital were tested for oVEMP in 500 Hz and 1 000 Hz air-conducted tone bursts respectively, and 20 healthy subjects(40 ears), matched for age and sex, were used as healthy control. The amplitudes of the N1-P1 wave and the frequency amplitude ratios of 500/1 000 Hz in affected ears, unaffected ears and normal ears were compared; and receiver operating characteristic curve was analyzed for frequency amplitude ratios of 500/1 000 Hz. Results: By the 500 Hz tone-burst stimulus, the provocation rates of the oVEMP were 84.9%(28/33), 93.9%(31/33) and 97.5%(39/40) in affected ears, contralateral ears and the ears of normal controls, respectively. By the stimulus of 1 000 Hz tone-burst, the provocation rates of the oVEMP were 81.8%(27/33), 87.9%(29/33) and 82.5% (33/40) in affected ears, contralateral ears and normal control ears, respectively. Amplitudes of N1-P1 waveforms in 500 Hz air-conducted tone bursts in affected ears were under normal control ears and contralateral ears. There was significant difference between affected ears and healthy control ears (P<0.05). Amplitudes of N1-P1 waveforms in contralateral ears were also significantly smaller than those in normal control ears (P<0.05). There were no significant differences in amplitudes of N1-P1 waveforms between affected ears, contralateral ears and normal control ears in 1 000 Hz tone bursts stimulus(P>0.05). Frequency amplitude ratios of 500/1 000 Hz in affected ears were significantly under contralateral and normal control ears (P<0.05). According to ROC, the frequency amplitude ratio critical value of frequency tuning was set as 1.17, the positive rate of frequency tuning shift in affected ears was 54.5%(18/33) and significantly higher than in contralateral ears(18.2%, 6/33, χ²=9.429, P=0.002) and normal control ears (7.5%, 3/40, χ²=19.530, P=0.000). In contrast, there was no significant difference in the incidence of frequency tuning shift between contralateral ears and normal ears(χ²=1.909, P=0.167). Conclusions Frequency tuning oVEMP with Meniere′s disease will be changed.Frequency tuning of oVEMP with Meniere′s disease shifts from low frequency region to high frequency region in comparison with healthy people. Frequency amplitude ratio is helpful for diagnosis of Meniere′s disease.