Clinical features, diagnosis and treatment of intussusception secondary to inverted Meckel′s diverticulum in children / 中华实用儿科临床杂志

Objective:To explore the clinical characteristics, diagnosis, treatment and prognosis of inverted Meckel′s diverticulum in children, and to summarize the clinical experience.Methods:Ten children who received surgical treatment in Beijing Children′s Hospital, Capital Medical University from October 2018 to October 2021 were retrospectively analyzed.The children were diagnosed with Mecker′s diverticulum.Their data were complete and their fo-llow-up information was available.The clinical features, diagnosis, treatment and prognosis were summarized.There were 6 males and 4 females, whose age of onset ranged from 5 months to 12 years and 5 months.The clinical manifestations were abdominal pain in 8 cases, crying in 2 cases and bloody stool in 3 cases.B-ultrasound showed suspected diseases secondary to inverted Meckel′s diverticulum, including small intestinal intussusception in 6 cases and back junction type intussusception in 4 cases.One case of small intestinal intussusception was misdiagnosed after laparoscopic exploration in other hospitals and suffered from recurrent intussusception.All patients underwent segmental bowel resection.Results:Of the 10 children, 8 cases received laparoscopic exploration and 2 cases underwent open surgery for secondary intestinal necrosis.Postoperative pathology revealed Meckel′s diverticulum.Ectopic tissue was accessible in 8 cases, including ectopic gastric tissue in 7 cases, and ectopic pancreas in 1 case.During the postoperative follow-up period (1 month-3 years), the patients were generally in good condition with no complications such as intestinal obstruction.Conclusions:Intussusception secondary to inverted Meckel′s diverticulum is rare and difficult to diagnose before operation.The diagnosis should be confirmed by surgical exploration and postoperative pathology.However, careful surgical exploration is required since inverted Meckel′s diverticulum is prone to be misdiagnosed during exploration.

Reoperation for biliary-enteric strictures after hepaticojejunostomy in children with congenital biliary dilatation / 中华普通外科杂志

Objective:To evaluate clinical characteristics and treatment of postoperative anastomotic stricture in pediatric congenital biliary dilatation patients.Methods:The clinical data of 24 children with postoperative anastomotic stricture from Apr 2012 to Oct 2019 in Beijing Children's Hospital was retrospectively analyzed.Results:There were 6 males and 18 females. Patients were divided into bile- leak group (BL, n=6) and non bile-leak group (NBL, n=18) based on whether there was anastomotic leakage after primary surgery. The main symptoms in BL group was persistent obstructive jaundice, and recurrent cholangitis in NBL group. Postoperative symptoms were first shown in an average of 7.0 months in BL group, compared to 59.0 months in NBL group, P<0.05. In BL group, 4 underwent redoing hepaticojejunostomy, 2 underwent anastomosis plasty. In NBL group, 3 underwent redoing hepaticojejunostomy, 15 did anastomosis plasty with multiple biliary stones found necessitating extraction. After reoperation, one patient had bile leakage, 2 patients had recurrent cholangitis within one-month, 21 patients had uneventful recovery. Five were found to have biliary stones in long-term follow-up. Conclusions:Biliary-enteric anastomotic leakage can cause stricture in postoperative patients of congenital biliary dilatation ,reoperation is necessary in symptomatic patients.

Diagnosis and outcome analysis of diseases characterized by recurrent low-frequency sensorineural deafness / 临床耳鼻咽喉头颈外科杂志

The aim of this study was to explore the clinical features, hearing prognosis and differential diagnosis of recurrent low frequency sensorineural hearing loss （RLFD） . The clinical characteristics, clinical manifestations, audiological examination and auxiliary examination of RLFD patients were retrospectively analyzed. We summarized clinical features, draw the pure tone audiometry curves, and analyze the diagnosis of RLFD. Forty-seven patients （53 ears） with RLFD had a hearing review time of 1-124 months. The course of disease ranged from 3 to 320 months with an average course of 29 months. ①The incidence of tinnitus in the accompanying symptoms was 93.6%（44 cases）, and the ear suffocation was 83.0%（39 cases）, 38.3% （18 cases） of the patients developed vestibular symptoms during the course of the disease. ②During the observation period, 27 cases（57.4%） were diagnosed with related diseases: 7 cases（14.9%） Meniere's disease, 6 cases（12.8%） vestibular migraine, 2 cases（4.3%） with Meniere's disease and migraine, and 1 case（2.1%） with idiopathic intracranial hypotension 11 cases（23.4%） were possible cochlear migraine; ③Migraine-related RLFD had a younger onset age, more common in women; ④83.0%（44 ears）of the affected ears had stable or improved low-frequency hearing during the observation period, 17.0%（9 ears）of the affected ears experienced low-frequency hearing; ⑤18.9%（10 ears）of the affected ears had high-frequency hearing loss; ⑥RLFD had 6 types of audiological outcomes: low-frequency improvement combined with high-frequency stability; low-frequency stability combined with high-frequency stability; low frequency progress combined with high frequency stability type; low frequency improvement combined with high frequency progress type; low frequency stability combined with high frequency progress type; low frequency progress combined with high frequency progress type; ⑦Rising type hearing curve low frequency hearing prognosis is good, mountain type and descending low frequency hearing prognosis is poor. Tinnitus and ear stuffiness are the early symptoms and the most disturbing symptoms in patients with RLFD. The mechanism of Migraine may play an important role in the pathogenesis of RLFD. The rare causes such as intracranial hypotension syndrome should not be ignored. Most of the patients with RLFD had stable or improved hearing after long-term fluctuation, but some patients with low or high frequency hearing might decline. The type of initial hearing curve was a prognostic factor. Long-term hearing follow-up is helpful for prognosis evaluation.

Diagnosis and outcome analysis of diseases characterized by recurrent low-frequency sensorineural deafness / 临床耳鼻咽喉头颈外科杂志

Objective@#The aim of this study was to explore the clinical features, hearing prognosis and differential diagnosis of recurrent low frequency sensorineural hearing loss （RLFD） . @*Method@#The clinical characteristics, clinical manifestations, audiological examination and auxiliary examination of RLFD patients were retrospectively analyzed. We summarized clinical features, draw the pure tone audiometry curves, and analyze the diagnosis of RLFD. @*Result@#Forty-seven patients （53 ears） with RLFD had a hearing review time of 1-124 months. The course of disease ranged from 3 to 320 months with an average course of 29 months. ①The incidence of tinnitus in the accompanying symptoms was 93.6%（44 cases）, and the ear suffocation was 83.0%（39 cases）, 38.3% （18 cases） of the patients developed vestibular symptoms during the course of the disease. ②During the observation period, 27 cases（57.4%） were diagnosed with related diseases: 7 cases（14.9%） Meniere's disease, 6 cases（12.8%） vestibular migraine, 2 cases（4.3%） with Meniere's disease and migraine, and 1 case（2.1%） with idiopathic intracranial hypotension 11 cases（23.4%） were possible cochlear migraine; ③Migraine-related RLFD had a younger onset age, more common in women; ④83.0%（44 ears）of the affected ears had stable or improved low-frequency hearing during the observation period, 17.0%（9 ears）of the affected ears experienced low-frequency hearing; ⑤18.9%（10 ears）of the affected ears had high-frequency hearing loss; ⑥RLFD had 6 types of audiological outcomes: low-frequency improvement combined with high-frequency stability; low-frequency stability combined with high-frequency stability; low frequency progress combined with high frequency stability type; low frequency improvement combined with high frequency progress type; low frequency stability combined with high frequency progress type; low frequency progress combined with high frequency progress type; ⑦Rising type hearing curve low frequency hearing prognosis is good, mountain type and descending low frequency hearing prognosis is poor. @*Conclusion@#Tinnitus and ear stuffiness are the early symptoms and the most disturbing symptoms in patients with RLFD. The mechanism of Migraine may play an important role in the pathogenesis of RLFD. The rare causes such as intracranial hypotension syndrome should not be ignored. Most of the patients with RLFD had stable or improved hearing after long-term fluctuation, but some patients with low or high frequency hearing might decline. The type of initial hearing curve was a prognostic factor. Long-term hearing follow-up is helpful for prognosis evaluation.

Comparison of clinical features between very earl-onset inflammatory bowel diseases and late-onset inflammatory bowel diseases / 中华实用儿科临床杂志

Objective To summarize the clinical data of the children with inflammatory bowel diseases (IBD),including Crohn's disease (CD) and ulcerative colitis (UC),and to analyze and compare the clinical features of very early-onset IBD (VEO-IBD) and late-onset IBD (LO-IBD).Methods A retrospective analysis of the clinical data of 184 cases of IBD hospitalized children diagnosed at Beijing Children's Hospital,Capital Medical University from January 2000 to December 2014.According to their ages of onset,the patients were divided into VEO-IBD group (＜6 years old) and LO-IBD group (6-16 years old);the patients with CD were divided into VEO-CD group (＜ 6 years old) and LO-CD group (6-16 years old);UC were divided into VEO-UC group (＜ 6 years old) and LO-UC group (6-16 years old).The clinical features among each group were analyzed and compared.Results A total of 184 IBD patients were included in the study,77 cases(41.8％) were VEO-IBD and 107 cases(58.2％) were LO-IBD.Comparison between VEO-CD group and LO-CD group indicated that abdominal pain was more common in LO-CD group (P ＜ 0.05),while diarrhea and hematochezia were more common in VEO-CD group (all P ＜ 0.05).In addition,comparison between VEO-UC group and LO-UC group indicated that abdominal pain was more common in LO-UC group(P ＜ 0.001),while diarrhea,fever,and oral ulcers were more common in VEO-UC group (all P ＜0.05).Both VEO-CD and LO-CD group were mainly ileocolonic[15/27 cases (55.6％),20/47 cases (42.6％)],non-narrow,non-penetrating [20/27 cases (74.1％),30/47 cases (63.8％)] and moderate-to-severe activity[23/27 cases(85.2％),37/47 cases (78.7％)].The incidence of perianal lesions in the VEO-CD group was as high as 51.9％ (14/27 cases),which was significantly higher than that in the LO-CD group (9/47 cases,19.1％) (P ＜ 0.05).Left-sided UC and severe UC were more common in VEO-UC group(all P ＜ 0.05),while pancolitis and mild UC were more common in LO-UC group (all P ＜ 0.05).The incidence of intestinal perforation in the VEO-UC group was significantly higher than that in the LO-UC group (P ＜0.05).The incidence of surgical rate,intestinal obstruction,and intestinal perforation in the LO-CD group were significantly higher than those in the LO-UC group (all P ＜ 0.05).Conclusions Compared between VEO-IBD and LO-IBD,VEO-IBD patients are more severe,with perianal lesions more common,and the incidence of intestinal perforation is higher.

Single center retrospective study of 184 children with inflammatory bowel disease seen from 2000-2014 / 中华儿科杂志

Objective@#To investigate the clinical data of children with inflammatory bowel disease (IBD) retrospectively, including Crohn′s disease (CD) and ulcerative colitis (UC) and identify the clinical characteristics and trends of change.@*Method@#Clinical data of hospitalized patients diagnosed as IBD in Beijing Children′s Hospital from January 2000 to December 2014 were collected and retrospectively analyzed. Patients were divided into six groups based on type of disease and year of admission: Group A1(CD, 2000-2004) included 12 patients, Group B1(CD, 2005-2009) included 11, Group C1(CD, 2010-2014) included 51; Group A2(UC, 2000-2004) included 17, Group B2(UC, 2005-2009) included 25, Group C2(UC, 2010-2014) included 68.@*Result@#A total of 184 IBD patients were included in the study, 74 had CD and 110 had UC. The hospitalization constituent ratio of CD increased from 0.6/10 000 in Year 2000 to 2.9/10 000 in Year 2014. The hospitalization constituent ratio of UC increased from 0.5/10 000 in Year 2001 to 3.9/10 000 in Year 2014. The hospitalization constituent ratios of CD and UC both increased gradually(P<0.05). Up to 61.4%(113/184) of IBD patients belong to early onset IBD, furthermore the very early onset IBD and infantile IBD accounted for 41.8%(77/184) and 26.6%(49/184) respectively. For CD, ileocolonic type(47.3%, 35/74) and non-structuring, non-penetrating type (67.6%, 50/74) were more common. Perianal disease occured in 31.1%(23/74) of CD patients; 81.1%(60/74) of CD patients had moderate/severe activity. For UC, pancolitis type(59.1%, 65/110) was more common. There were no significant changes for location of pathological change, disease behavior, activity degree of CD, extent of UC lesion and incidence of surgery, intestinal perforation and hemorrhage of gastrointestinal tract for IBD in the past 15 years(P>0.05). Severe UC(S3) was more common in Group A2(64.7%, 11/17), but moderate UC(S2) was more common in Group C2(48.5%, 33/68), the difference was statistically significant (P=0.001 7).@*Conclusion@#During the past 15 years, the hospitalization constituent ratio for IBD in our hospital showed a growing trend. The ratio of infantile IBD and very early onset IBD was high. For CD, perianal disease was commonly seen and most patients had moderate/severe activity. The surgery rate and incidence of intestinal obstruction and perforation were higher in the CD patients than UC patients. For UC, the lesions were more extensively combined with higher disease activity.

Prognostic factors of sudden sensorineural hearing loss in children / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#The aim of this retrospective study was to analyze the recovery rate of sudden sensorineural hearing loss in children, and explore the prognostic factors in order to guide the clinical diagnosis and treatment.@*METHOD@#A retrospective review was conducted for the prognosis of children with sudden sensorineural hearing loss during the past 5 years (from November 2010 to May 2015) in Chinese PLA General Hospital. This paper have a complete clinical data of 101 patients (113 ears)with sudden hearing loss, ranging from 0 to 18 years old Patients were divided into four groups according to hearing recovery and eight putative prognostic factors were analyzed.@*RESULT@#Among 101 patients (113 ears), the ratio of male and female was 60:53. Treatment was initiated from 1 to 183 days after disease onset, with an average of (18.5 ± 22.1) d. Bilateral and unilateral hearing loss were 24 ears and 89 ears, respectively. The proportion of mild hearing loss, moderate hearing loss, severe hearing loss and profound hearing loss were 7.1%, 6.2%, 23.9% and 62.8%, respectively. Vertigo and tinnitus occurred in 54.9% and 77.9% of the patients, respectively. After the treatment, the complete recovery rate was 9.7% and the overall recovery rate was 36.3%. The degree of hearing loss, earlier treatment onset, sex and bilateral involvement were significantly associated with hearing recovery (P < 0.05).@*CONCLUSION@#Sudden sensorineural hearing loss in children was generally identified as severe and profound hearing loss, but after positive and timely treatment, it can be improved or even cured. The mild hearing loss, earlier treatment onset, unilateral hearing loss and female were positive prognostic factors. The concurrence of tinnitus or vertigo, the results of ABR and DPOAE had no significant influence on prognosis.

The study of clinical characteristics of sudden sensorineural hearing loss patients with tinnitus / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To analysis the characteristics of sudden sensorineural hearing loss (SSHL) patients with tinnitus, and explore the relationship of characteristics of tinnitus and audiology.@*METHOD@#Patients diagnosed as SSHL with tinnitus were studied in the research. All patients' clinical features were analyzed, such as tinnitus frequency, pure tone audiometry, tinnitus, hearing loss degree, results of residual inhibition test.@*RESULT@#Thirty cases were identified as mild degree hearing loss, 13 cases as moderate degree, 28 cases as severe degree and 34 cases as profound degree. And hearing impaired frequency of 13 cases was ascertained at low-frequency, 39 cases at middle-high-frequency, and 53 cases at full-range-frequency. The incidence of patients with low-frequency was about 41. 9% (44/105), and it was about 21. 9% (23/105) in those with middle-frequency. And it was 36. 2% (38/105) in cases of high-frequency tinnitus. The chi-square test show statistically significant differences between patients with the low-frequency, middle-frequency and high-frequency of the hearing loss (P<0. 05). In tinnitus residual inhibition test, positive rate of convergence type masking curve was about 72.0%, tinnitus separated type masking curve 20.0%, overlapping type was 57.9%, and the spacing type was 43.5%. There was a statistically significant difference among cases with different type masking curve (P<0. 05)with the spacing residual inhibition test positive rate.@*CONCLUSION@#There are individual differences of clinical characteristics among SSHL patients with tinnitus. Tinnitus frequency is consistent with the frequency of hearing loss. Patients had the more serious the degree of hearing loss, who had more serious tinnitus grading. Cases with the converged type curve will be fit for tinnitus masking. Therefore, combining the tinnitus detection with the audiological tests, we could obtain the clinical characteristics of SSHL patients with tinnitus.

The clinical effects of atlantoaxial pedicle screw combined with unilateral axial spinous process and lamina screws for atlantoaxial instability / 中华骨科杂志

Objective To investigate the clinical effects of the posterior atlantoaxial pedicle screw fixation combined with unilateral axial spinous process and lamina screws and allogeneic bone graft for atlantoaxial instability.Methods From March 2010 to April 2014,data of 10 patients with atlantoaxial instability who had undergone atlanto-axial vertebral pedicle fixation and interbody fusion combined with posterior atlanto-axial spines lamina nails were retrospectively analyzed.There were 6 males and 4 females with an average age of 39 (range,16-62) years old.The inclusion criteria was atlantoaxial instability together with unilateral vertebral artery segment high cross deformity (7 patients) or atlantoaxial joint break bad involving unilateral pedicle (7 patients).3 patients have spinal cord disease symptoms and physical signs,both suffered limited neck mobility and pain.The VAS scores were 1-8,with an average of 3.70±2.11.Preoperative X-ray,CT three-dimensional reconstruction and MRI were collected.X-ray and CT examination were performed 7 d and 1,3,6,12 months after operation to evaluate the internal fixation and bone grafting fusion.Results There's no occurrence of cervical spinal cord and vertebral artery injury.Screws were successfully implanted.1 case's incision occurred infiltration liquid 3 days after operation.For allograft rejection consideration,we changed the plan,and the infiltrating stopped 5 days later.Other incisions were all primary healing.VAS scores were 1.01±0.89 after operation,which was significantly reduced.X-ray showed good recovery of cervical sequence after the operation,and CT revealed 1 patient with atlas pedicle screw in the medial cortex damage.Spinal canal was without infringing,and the other screw positions were normal.6 months after operation,X-ray or CT examination both showed bony fusion.Conclusion Atlantoaxial pedicle screw fixation combined with unilateral axial spinous process and lamina screws and allograft bone graft fusion for the treatment of atlantoaxial instability can observe good clinical effects.

Study of clinical characteristics and curative effects of sudden hearing loss patients with vertigo / 中华耳鼻咽喉头颈外科杂志

<p><b>OBJECTIVE</b>To analyze the clinical characteristics, prognosis and therapeutic effects of sudden sensorineural hearing loss (SSHL) patients associated with vertigo, and to investigate the strategy of diagnosis and treatment.</p><p><b>METHODS</b>We retrospectively analyzed the clinical characteristics of 240 patients diagnosed as SSHL with vertigo, who were treated in the Chinese PLA General Hospital from July 2008 to August 2012. Various factors affecting the therapeutic effects were analyzed, such as audiological features, vestibular function tests, genders, audiograms, lasting before seeing a doctor, courses of vertigo and vascular factors.</p><p><b>RESULT</b>Among the contemporaneous SSHL patients (873 cases), the cases with vertigo accounted for 27.49% (240/873). Among the 240 patients with vertigo, the cases with different hearing impaired degree of mild, moderate, severe and profound were 30, 13, 28 and 34, respectively, primarily by the profound cases. Detailed vestibular function tests were performed in 97 patients, with 54 cases having unilateral vestibular disfunction and 43 patients having normal vestibular function, among which 23 cases were diagnosed as benign paroxymal positional vertigo (BBPV). The relationship between vestibular function and different hearing impaired degrees or various audiogram types had no statistically significant difference. 219 cases had detailed records of the onset time of cochlear and vestibular symptoms, including 122 patients with cochlear symptoms and dizziness occurring simultaneously. After standardized drug treatment, the total effective rate was 46.67%, with recovery in 17 cases, excellent in 34 cases, better in 61 cases and poor in 128 cases, respectively. Statistical analysis showed that different genders, audiogram types, vertigo courses of time, the results of vestibular function and neck vascular ultrasounds were not related to the curative effects, while, the treatment time after onset was significantly associated with treatment effects.</p><p><b>CONCLUSIONS</b>SSHL with vertigo has a high incidence, primarily single side affected, with relatively severe hearing impairment, and total deafness and downslope hearing curve mainly. Vestibular function can be normal or low in SSHL patients with vertigo, with a higher incidence of BPPV. Vestibular and cochlear symptoms occur simultaneously in more than half of the patients. The detection rate of vestibular dysfunction gradually increased, as the degree of hearing loss increased, without statistical significance although. The therapeutic effects of sudden hearing loss with vertigo cases have no relationship with dizziness duration or vestibular function, while the disease course plays an important role in treatment.</p>

Multi-center comparative study of methylprednisolone and dexamethasone in treatment of all-frequency sudden hearing loss / 中华耳鼻咽喉头颈外科杂志

<p><b>OBJECTIVE</b>Through a prospective cohort study, to assess the clinical efficacy of methylprednisolone (MP) and dexamethasone (DXM) in treatment of all-frequency sudden hearing loss.</p><p><b>METHODS</b>A total of 76 cases of all-frequency sudden hearing loss were included in this study and divided into two groups. The MP group (n = 40) was treated with MP 40 mg qd, for 5 days, combined with conventional treatment. The DXM group (n = 36) was treated with DXM 10 mg qd, for 5 days, combined with conventional treatment. The total period of treatment was 14 days.</p><p><b>RESULTS</b>After the treatment for 14 days, in the MP group,17 cases were cured (42.5%), 7 cases were markedly improved (17.5%), 9 cases were effective (22.5%), and 7 cases were invalid (17.5%), the total effective rate was 82.5%. As for the patients in the DXM group, 13 cases were cured (36.1%), 6 cases were markedly improved (16.7%), 8 cases were effective (24.2%), and 9 cases were invalid (25%), the total effective rate was 75.0%. The pure tone audiometry in all-frequency was improved (31.5 ± 17.8) dB in the MP group, and (33.1 ± 24.2) dB in the DXM group. The speech recognition rate was improved (41.7 ± 29.8) %, and (42.0 ± 39.1) % in the DXM group. There were no significant differences between two groups.</p><p><b>CONCLUSION</b>There is no significant difference of therapeutic efficacy between the low-dose MP group and High-dose DXM group.</p>

Cinical and genetic characteristics of familial Meniere's disease: three families report / 中华耳鼻咽喉头颈外科杂志

<p><b>OBJECTIVE</b>To investigate the clinical and genetic characteristics of three Chinese Meniere's disease (MD) families and decipher the mechanism of MD further.</p><p><b>METHODS</b>Personal and family medical evidence of hearing loss, vestibular symptoms, and other clinical abnormalities of the participants were identified, clinical and genetic features were analyzed. Targeted 307 genes capture and high-throughput sequencing were performed on the two ascertained members of family 1007184.</p><p><b>RESULTS</b>Eight patients from these three families showed post-lingual sensorineural hearing loss, six women and two men were involved. Age of onset in these affected members concentrated in the middle age, with the average age of 39.3 years old. Among them, patients from 1407278 were accompanied by migraine. All of the three probands presented as recurrent vertigo firstly, and then fluctuated hearing loss showed up, accompanying by tinnitus and ear fullness feeling. The hearing loss manifested as late-onset, low frequency-involved pattern, with subsequent gradual progression from moderate to severe level. Some of the patients progressed to severe level involving all frequencies at higher ages. In addition, most of the cases showed revitalization. Four cases received vestibular function tests, three of which had varying dysfunction of vestibular function, while the other one had normal vestibular function. Patients who had abnormal vestibular function showed much more severe hearing impairment. The three-generation family 1007193 had an autosomal recessive genetic characteristics, family 1007184 showed autosomal dominant inheritance of characteristics, family 1407278 were either autosomal dominant or X-linked dominant pattern. Through target genes capture high-throughput sequencing technology, we identified two candidate variants in the two members of family 1007184, named c. 2057G>A in EGFLAM and c. 1961C>T in ITGA8.</p><p><b>CONCLUSION</b>Meniere's disease has some genetic and familial aggregation in Chinese population, but its complex genetic pathogenic mechanisms need further study.</p>

The curative effect analysis of combined medical therapy for auditory neuropathy spectrum disorder / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To investigate the efficacy of combined medical treatment on auditory neuropathy spectrum disorder and the effect of related factors on the prognosis.@*METHOD@#Eleven cases (22 ears) diagnosed as auditory neuropathy spectrum disorder using multiple criteria including pure tone auditory threshold, impedance audiometry, acoustic reflexes, distortion products otoacoustic emission (DPOAE) and auditory brainstem response (ABR) were subjected to combined medical treatment . Eleven auditory neuropathy spectrum disorder patients diagnosed during the corresponding period but refused treatment were selected as control group. The change of pure tone auditory threshold and speech discrimination score after treatment or follow-up were evaluated for both 2 groups, and the relationship between the patients' gender, age, accompanying symptoms and curative effect were also analyzed. Data were analyzed by SPSS 19.0 statistical software using pared-sample t-test, independent-sample test and Pearson's chi-square test.@*RESULT@#The effective rate of combined medical therapy was 59.09% (13/22) in the therapy group. PTA levels before and after-treatment were (53.92 +/- 18.86) dB HL and (47.44 +/- 14.98) dB HL respectively in 22 ears with the combined medical therapy, the improvement of which showed statistically significance (t = 5.20, P 0.05). Speech discrimination score before and after-treatment were (29.20 +/- 25.80)% and (41.60 +/- 22.90)% respectively for the treatment group. The average improvement of speech discrimination score was (12.40 +/- 13.80)% with statistically significant difference (t = 4.02, P 0.05).@*CONCLUSION@#The combined medical therapy with glucocorticoids helps improving the pure tone auditory threshold and speech discrimination score of auditory neuropathy spectrum disorder. In light of our findings we support the combined medical therapy as an option for patients with auditory neuropathy spectrum disorder.

Analysis of characteristics of tinnitus in patients with auditory neuropathy spectrum disorder / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To investigate the characteristics of tinnitus in patients with auditory neuropathy spec- trum disorder (ANSD).@*METHOD@#This study recruited 14 ANSD patients with tinnitus. All the ANSD patients un- derwent detailed history taking, audiological examinations and assessments of tinnitus. This study analyzed the correlation of tinnitus status and hearing loss, and discussed the effects of sex, age, and the course of disease on tinnitus in ANSD patients.@*RESULT@#(1) In the ANSD patients, tinnitus often occurred in 3 years after the onset of hearing loss; (2) Tinnitus was highly prevalent in ANSD patients, and the severity of tinnitus was mostly from mild to moderate; (3) There was no obvious correlation between the subjective grading of tinnitus and hearing loss de- gree, and the impact of curve patterns of hearing loss on the level of tinnitus need much more evidence-based proof; (4) Along with the course extension, the impact of tinnitus on the quality of life was much more obvious; (5) Some risk factors such as noise exposure could be the reasons of aggravating the degree of tinnitus.@*CONCLUSION@#Tinnitus in ANSD patients has its unique clinical features. The study of Tinnitus in ANSD patients can provide clinical basis for further research in ANSD.

The study of the pure tone audiometry characteristics and curative effect in sudden hearing loss patients with hypertension / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To investigate the pure tone audiometry characteristics and curative effect in sudden hearing loss patients with hypertension.@*METHOD@#One hundred and fifty-seven inpatients (168 ears) with hypertension suffered from sudden hearing loss were included in this study. We retrospectively analyzed the audiological index of these patients by comparing the pure tone audiometry (PTA) among patients in the aspects of gender, age, affected side, duration of hypertension, with or without inducement, concomitant symptoms and other combined diseases. The hearing threshold at different frequency was also compared, as well as the curative effect among patients with diverse audiological characteristics.@*RESULT@#Of the contemporaneous sudden hearing loss patients (874 cases), the prevalence of hypertension was 17.96%, where the male ones accounted for. 28.69% (103/359) and the female ones accounted for 19.42% (54/278) respectively with statistically significant difference between genders (P 0.05). The hearing threshold means of each frequency of unilateral hearing loss patients was significantly higher than that of bilateral hearing loss patients (P < 0.05). The hearing threshold means at 125 Hz, 250 Hz, 500 Hz and 1 kHz showed significant difference among patients with different duration of hypertension (P < 0.05). The total effective rate of sudden hearing loss in patients with hypertension was significantly lower than that in the sudden hearing loss patients without hypertension (19.64%, 61.57% respectively, P < 0.01). The total effective rate presented significant difference among patients with different duration of hypertension and different Cardiovascular Risk Stratification (P < 0.05).@*CONCLUSION@#The prevalence of sudden hearing loss in hypertension patients was higher in male than in female, which rose with age and combined disease. The hearing threshold means at mid-frequency and high-frequency were higher than that at low-frequency. The total effective rate of sudden hearing loss was relatively low in patients with hypertension. The longer the duration of hypertension and the higher the Cardiovascular Risk Stratification, the lower the total effective rate. Comprehensive understanding of audiological characteristics and hypertension condition plays a crucial role in type-specific treatment of sudden hearing loss.

Comparative study of mutation spectrums of MT-RNR1 m.1555A>G, GJB2, and SLC26A4 between familial and sporadic patients with nonsyndromic sensorineural hearing loss in Chinese Han / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>The mutation frequencies of three common deafness genes (MT-RNR1 m.1555A>G, GJB2, and SLC26A4) among patients with nonsyndromic sensorineural hearing loss (NSHL) were different in previous studies. Inconsistent selection criteria for recruiting patients could have led to differences in estimating the frequencies of genetic mutations thus resulting in different mutation frequencies among these studies. The aim of this study was to reveal the differences in the mutation spectrums of the three common genes between familial and sporadic Chinese Han patients.</p><p><b>METHODS</b>Totally, 301 familial probands and 703 sporadic patients with NSHL were enrolled in this study. Three genes, MT-RNR1 m.1555A>G, GJB2, and SLC26A4, were screened for mutation in our study cohort. A χ(2) test was performed to compare the mutation frequencies between the two groups.</p><p><b>RESULTS</b>The study showed that the disease-causing mutation frequencies of MT-RNR1 m.1555A>G, GJB2, and SLC26A4 were 12.29%, 14.62%, and 18.27% in familial probands and 3.56%, 18.63%, and 18.92% in sporadic patients, respectively. The mutation frequency of MT-RNR1 m.1555A>G in familial probands was significantly higher than in sporadic patients (χ(2) test, P = 0.000), while there were no significant differences in the mutation frequencies of GJB2 and SLC26A4 between the familial and sporadic groups (χ(2) test, P > 0.05).</p><p><b>CONCLUSIONS</b>It is necessary to reveal the differences in gene mutation frequencies between patients of different sources or characteristics by comparative studies in order to avoid selection bias. The mutations of GJB2, SLC26A4, and MT-RNR1 m.1555A>G are the most important etiological factors in Chinese Han patients, among which SLC26A4 might be the most frequent.</p>

Advances in genetics of congenital malformation of external and middle ear / 临床耳鼻咽喉头颈外科杂志

Congenital malformation of external and middle ear is a common disease in ENT department, and the incidence of this disease is second only to cleft lip and palate in the whole congenital malformations of the head and face. The external and middle ear malformations may occur separately, or as an important ear symptom of the systemic syndrome. We systematically review and analysis the genetic research progress of congenital malformation of external and middle ear, which would be helpful to understand the mechanism of external and middle ear development, and to provide clues for the further discovery of new virulence genes.

Clinical analysis of in-patients with large vestibular aqueduct syndrome / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#This study is to investigate the clinical materials of in-patients with the large vestibular aqueduct syndrome (LVAS), and explore the feature, diagnosis and treatment measures of the disease.@*METHOD@#A retrospective review was conducted including the medical history, audiological examinations, vestibular function examinations, imaging examinations and treatment methods of 44 in patients (87 ears) suffering LVAS admitted to our hospital in the past 4 years(from 2008 to 2012).@*RESULT@#ln the 44 in patients, there were 24 male cases and 20 female cases, and the male-female ratio was 1.2 :1. The average of the onset age was 3.39 years. Five cases (11. 36%) had related familial history. The profound hearing loss was found in 67 ears (77.01%), and the severe hearing loss was found in 20 ears (22.99%). After systemic treatment,the hearing of 38 ears improved effectively,but that of 49 ears did not improve obviously. The analysis found that patients suffering sudden hearing loss got better curative effect than those with progressive hearing loss. Patients received combined drug therapy improving arterial circulation as well as venous reflux got better therapeutic effect. There was a significant difference on effect between the patients with course of treatment more than 7 days and those less than 7 days. There was no significant correlation between therapeutic effect and other factors.@*CONCLUSION@#In part of LVAS patients,the hearing level can be effectively improved through a standard internal medicine treatment. We can improve the personalized and standardized treatment strategy for this disease through analysis of diagnosis and treatment of in-patients with complete clinical data.

The Significance and Structure Analysis on Function of an Information System for Medical Center of Clinical Audiology (MCCAIS301)Based on Hospital Information System / 听力学及言语疾病杂志

Objective To design and develop a Information System for Medical Center of Clinical Audiology , (MCCAIS301 ) .Methods The system framework was established by developing software ,constructing user platform and creating database .An implication procedure was also established for clinical use for the MCCA IS301 .The MC-CAIS301 was connected with the Hospital Information System (HIS) in order to connect the equipment in the auditory clinical center and other clinical database system .Results The MCCAIS301 was a new database system for hospital in-formation management specifically designed for audiological tests .It provided an extra functions of the existing HIS system .The MCCAIS301 could store the testing results from more than ten different hearing instruments made from five different companies .The data from the MCCAIS301 could be transferred to the HIS system .The results of the MCCAIS301 could be retrieved and analyzed using the HIS system .MCCAIS301 system had nine sets of standardized hearing testing results ,five output formats and three statistical analyzing functions .Conclusion The MCCAIS301 is an effective information management system which has a strong practical use to improve the efficiency of daily audiology data analysis .The MCCAIS301 using digital technology moves the audiology data analysis from a manual low efficient stage to an effective and intelligent level .