Objective:This study aimed to compare the effects of cochlear implantation（CI） on vestibular function in patients with large vestibular aqueduct syndrome（LVAS） and in patients with extremely severe deafness with normal inner ear structure. Methods:A total of 28 LVAS patients and 28 patients with normal inner ear structure who suffered from extremely severe deafness were selected. The parameters of caloric tests, bone conduction evoked cervical vestibular-evoked myogenic potentials（cVEMP）, bone conduction evoked ocular vestibular-evoked myogenic potentials（oVEMP） and video head impulse tests（v-HIT） were compared between the two groups before and after CI. The data were analyzed using SPSS 26.0 software. Results:There was no significant difference in the results of the preoperative caloric test, v-HIT, and oVEMP（P1, N1） between the LVAS group and the control group（P>0.05）. Compared to the control group, the LVAS group exhibited a shorter cVEMP P1[（13.41±0.71）ms vs （16.28±0.89）ms, P<0.000 1], shorter N1[（19.83±0.54）ms vs （28.18±1.56）ms, P<0.000 1], higher amplitude[（123.60±83.80）μV vs （73.92±79.85）μV, P=0.049 4] and higher oVEMP amplitude[（16.60±13.87）μV vs （9.96±10.47）μV, P=0.028 5] before CI. The abnormal rate of caloric test increased in both groups after CI（25.00% vs 57.14%, P=0.028 8, 32.14% vs 82.14%, P=0.000 3, respectively）. There was no significant difference in the v-HIT parameters in both groups before and after the operation. As for the LVAS group, there was no statistically significant difference in cVEMP and oVEMP induction rates before and after operation. In the control group, there was a decrease in cVEMP induction rate（96.42% vs 64.28%, P=0.005 2） and oVEMP induction rate（96.42% vs 57.14%, P=0.000 9） after CI. LVAS group showed a shorter cVEMP P1[（13.41±0.71）ms vs （10.30±0.60）ms, P<0.000 1]; shorter cVEMP N1[（19.86±0.53）ms vs （18.97±1.33）ms, P=0.004 7]; decreased amplitude[（124.50±84.86）μV vs （64.35±61.57）μV, P=0.001 0] and shorter oVEMP amplitude[（15.92±13.03）μV vs （9.16±9.20）μV, P=0.009 9] after CI. The oVEMP N1 in the control group was longer than that before operation[（11.73 ± 0.91）ms vs （13.35 ± 2.60）ms, P=0.019 6], whereas there was no significant difference in other VEMP parameters after CI. Conclusion:Before CI, there was no significant difference in the results of the caloric test and v-HIT between the LVAS group and the control group, but the LVAS group exhibited increased sensitivity to acoustic stimulation-induced myogenic potentials. After CI, the function of the semicircular canal was impaired in both groups in the low-frequency area, and remained largely unaffected in the high-frequency area. Additionally, the function of the otolith in the LVAS group was less affected than that in the control group after CI, which may be related to the fact that the enlarged vestibular aqueduct of the LVAS patients acted as the third window of the inner ear.
Humans ; Vestibular Aqueduct/physiopathology* ; Cochlear Implantation ; Male ; Female ; Vestibular Evoked Myogenic Potentials ; Deafness/physiopathology* ; Child ; Adolescent ; Adult ; Young Adult ; Hearing Loss, Sensorineural/physiopathology* ; Vestibular Function Tests

Objective:To explore the clinical characteristics and treatment methods of middle ear cholesteatoma with intracranial and extracranial complications as the first diagnosis. Methods:A total of 244 patients were initially diagnosed with intracranial and/or extracranial complications associated with middle ear cholesteatoma at the First Affiliated Hospital of Zhengzhou University from January 2015 to January 2022, and medical records were collected and retrospectively analyzed. Results:Among 244 patients with intracranial and extracranial complications of middle ear cholesteatoma, 203 cases had one complication, 34 cases had 2 complications, and 7 cases had 3 or more complications. One hundred and eighty-six cases presented labyrinthitis, 41 cases had peripheral facial paralysis, 27 cases had periauricular abscess, 12 cases had meningitis, 10 cases had brain abscess, 7 cases had sigmoid sinus lesions, 6 cases had epidural abscess, and 4 cases demonstrated hydrocephalus. Conclusion:The destructive nature of middle ear cholesteatoma can lead to intracranial and extracranial complications. The incidence rate of extracranial complications is highest for labyrinthitis. Patients with otitis media who complained dizziness should raise clinical suspicion for potential labyrinthitis. The second most prevalent extracranial complication is peripheral facial paralysis, and early facial nerve decompression surgery is critical for better recovery of facial paralysis symptoms. Brain abscess is the most common intracranial complications, which has the highest fatality rate. Clinicians should be alert to otogenic brain abscess. Otolaryngology and neurosurgery teams should cooperate and evaluate patients' middle ear lesions and brain abscess, and jointly develop personalized treatment plans.
Humans ; Cholesteatoma, Middle Ear/surgery* ; Retrospective Studies ; Facial Paralysis/etiology* ; Brain Abscess/diagnosis* ; Male ; Female ; Otitis Media/complications* ; Meningitis/etiology* ; Labyrinthitis/etiology* ; Adult ; Middle Aged ; Young Adult

Objective:X-linked non-syndromic hearing loss is an extremely rare type of hearing impairment. This study conducted a phenotypic and genetic analysis of a family with X-linked dominant inheritance to explore the causes of hearing loss. Methods:Clinical data were collected from a patient with non-syndromic hearing loss who visited the Otorhinolaryngology Department of the First Affiliated Hospital of Zhengzhou University in June 2023. Phenotypic and genetic analyses were performed on family members, including audiometric tests, whole-exome sequencing, and PCR-Sanger sequencing verification. Audiological assessments comprised pure-tone audiometry, impedance audiometry, auditory brainstem response, and otoacoustic emission tests. Results:The affected individuals in this pedigree have X-linked dominant non-syndromic deafness caused by mutations in the SMPX gene. The proband, along with their mother and maternal grandmother, exhibit varying degrees of sensorineural hearing loss. Whole-exome sequencing revealed a novel pathogenic variant, NM_014332.3: c. 133-2A>C, in the SMPX gene in the proband. Sanger sequencing confirmed that the proband, proband's mother, and grandmother all carried this pathogenic variant. Conclusion:This study reports a novel pathogenic variant in the SMPX gene, providing additional medical evidence for the diagnosis and treatment of X-linked dominant inherited non-syndromic hearing loss. It enriches the mutation spectrum of the SMPX gene.
Humans ; Pedigree ; Mutation ; Phenotype ; Male ; Hearing Loss, Sensorineural/genetics* ; Exome Sequencing ; Female ; Adult ; Hearing Loss/genetics* ; Evoked Potentials, Auditory, Brain Stem ; Muscle Proteins

Objective To study a distinct type of cochlear malformation,differentiated by its radiological feature,from the current Sennaroglu's classification criteria along with its clinical manifestations.Methods By analyzing the radio-logical findings from high-resolution CT(HRCT)of the temporal bone and 3D-dimensional magnetic resonance imaging(3D-MRI)of the inner ear in 11 cases(13 ears)with special cochlear malformations,we summarized the characteristics of this type of cochlear deformity.Results ① The radiological features of this type of cochlear malformation showed that only a part of the cochlear basal turn originated ventrally from the vestibule,with no or only partial inward turning.There were no upper,middle,or apical turns(cochlea with less than 0.5 turns),and the modiolus and interscalar septa were absent.On HRCT axial images,there was no connection observed between the cochlea and the internal auditory canal(IAC).MRI results consistently showed a cochlear nerve deficiency(CND),with audiometric findings indicating complete hearing loss.Most cases were accompanied by abnormally developed vestibules and malformed semicircular canals,and the cochlea on the opposite side often had severe malformations as well.② Based on the imaging characteristics,this type of cochlear malfor-mation,with only partial development of the basal turn(cochlea with less than 0.5 turns),was categorized as cochlear hy-poplasia(CH).We provisionally designated it as cochlear hypoplasia type X(CH-X).Conclusion Cochlear hypoplasia(CH-X),characterized by cochlea with less than 0.5 turns,represents a severe type of inner ear malformation,accounting for approximately 2％of such deformities.Due to the malformed cochlea lacking a distinct bony cochlear nerve canal(BC-NC)and cochlear nerve(CN),the potential benefits of cochlear implant surgery may be relatively limited.

Objective To investigate the application value of multiple audiological testing methods in the audi-ological diagnosis of children with no response at maximum output intensity of auditory brainstem response(ABR).Methods We retrospectively studied the clinical data of 69 cases(138 ears)of children with no ABR response with maximum intensity,aged 42 days to 5 years old,with an average of 1 year and 6 months.The tympanogram showed type-A or positive single peak,and acoustic reflex was absent.Imaging examination showed no malformation of the inner ear.All 69 children underwent ABR,cochlear microphonic potential(CM),distortion product otoacoustic e-missions(DPOAE),and auditory steady-state response(ASSR)tests.Results Among 69 cases(138 ears),8 ca-ses(11.59％)recorded CM in 16 ears,of which 10 ears(7.25％)recorded DPOAE.The ASSR response thresh-olds at 0.5,1,2,and 4 kHz were 83.2±13.1,82.9±13.0,75.3±12.4,and 63.1±9.1 dB nHL,respectively.Combined with other examination results,these subjects were diagnosed with auditory neuropathy.The CM and DPOAE responses of the remaining 61 cases(122 ears)were absent,and the extraction rates of ASSR at 0.5,1,2,and 4 kHz were 82.3％,81.9％,76.9％,and 60.2％,respectively.Among them,20 ears of ASSR were absent at all frequencies,and 102 ears had responses in at least one frequency.The response thresholds of ASSR at 0.5,1,2,and 4 kHz were 93.2±6.1,99.8±7.0,105.4±5.4,108.2±9.8 dB nHL,respectively.These subjects were diagnosed with profound sensorineural hearing loss.Conclusion For children with no ABR response at maximum output intensity,while CM and/or DPOAE responses are present and ASSR frequency response thresholds are low-er than those with sensorineural hearing loss are helpful in the diagnosis of auditory neuropathy.Neither CM nor DPOAE response is helpful in the diagnosis of profound sensorineural hearing loss,and ASSR testing is helpful in e-valuating residual hearing.

Objective To study the changes of tinnitus after cochlear implantation in post-lingual adault recip-ients and analyze the factors that affect tinnitus.Methods A total of 47 postilingually-deafened adult subjects with tinnitus who underwent cochlear implantation at the Department of Otology,the first affiliated hospital of Zheng-zhou University,from January 2017 to December 2021.The subjects were evaluated using tinnitus handicap invento-ry(THI)and visual analogue scale(VAS)before cochlear implantation and 6 months after cochlear implant surger-y.Results Among 47 subjects who were eligible for this study,the THI scores were 36.94±13.337,14.48± 12.726,respectively,before CI and 6 months after cochlear implantation.The VAS scores were 5.13±1.676 be-fore and 2.34±1.903 after cochlear tmplantation.Statistical analysis showed significant differences in THI and VAS scores before and after cochlear implantation(P＜0.05).A total of 18 patients experienced complete tinnitus suppression,14 patients experienced alleviation of tinnitus,tinnitus remained unchanged in 13 patients,tinnitus worsened in 2 patients,and the overall efficiency was 66.0％(31/47).The tinnitus alleviation rate was signifant higher in the patients with tinnitus history of ≤5 years than the patients with tinnitus history of＞5 years(P＜0.05).There was a statistically significant difference in tinnitus alleviation between the patients with mild tinnitus and the patients with more than mild tinnitus before surgery(P＜0.001).Conclusion Cochlear implantation has an inhibitory effect on tinnitus in adults.Patients with shorter duration of the tinnitus and higher tinnitus handicap are more likely to experience tinnitus improvement after cochlear implantation.

Objective To induce human umbilical cord mesenchymal stem cells (hUC-MSCs) to hair-cell like cells in the inner ear, using a two-step neural differentiation method.Methods The hUC-MSCs were obtained from human umbilical cords by tissue adherence culture,whose surface antigen CD29, CD34, CD44, CD45, CD90, HLA-ABC, and HLA-DR could be identified by flow cytometry.In the neural stem cells induced phase, the NSE positive cells were analyzed by microscope and immunohistochemistry.In the second stage, the expression of hair-cell like cells markers (Math1, MyosinⅦa, Brn3c) were tested by qRT-PCR and immunofluorescence method.Results The control group and the protocol group had little NSE after differentiation while the protocol B group presented a neurobiological structure and demonstrated a higher NSE positive ratio after 5 days' neural stem cells induction (P<0.05).Compared to the control group, the mRNA and protein level of Math1, MyosinⅦa, and Brn3c exhibited a significant increase in the differential group,which induced for 4 weeks in the hair-cell like cells in the inner ear's induced phase(P<0.05).Conclusion The two-stage induction (hUC-MSCs-neural stem cells-hair-cell like cells) could produce more MyosinⅦa,Brn3c and Math1,which may provide an appropriate way to treat sensorineural deafness.

Objective To investigate the influencing factors of the indwelling catheter time in patients with prostate hyperplasia and malignant tumor after prostate surgery.Methods A total of 447 prostate surgery patients with prostate hyperplasia and malignant tumor were selected for medical record review using convenience sampling method. The demographic data,diagnose,complications,surgical method,medical advice and nursing care items were collected. The indwelling catheter time in or outward of the hospital and its influence factors were analyzed.ResultsA number of 300(44.74%) patients had urination disorders before operation.A number of 258(57.72%) patients had complications. Little information about rehabilitated exercise of urination function was recorded.There were statistically significant differences in indwelling catheter time in the hospital among benign prostatic hyperplasia patients with different education levels, health payments and complications (t/F=5.048, -2.433, 2.086;P<0.05). Significant differences in indwelling catheter time outward of the hospital were also observed in patents with different preoperative urination disorders (t=0.551, P<0.05). Prostate malignancy patients with different resident places and bad habits had statistically significant differences in indwelling catheter time outward of the hospital (t=2.062, -1.974;P<0.05). For patients with benign prostatic hyperplasia, education levels, health payment and complications could explain 60.8% of changes in indwelling catheter time in the hospital. For patients with prostate malignancy, resident place and bad habits could explain 68.4% of changes in indwelling catheter time outward of the hospital. According to medical records, only 42.5% patients had urinary flow rate, urodynamic and residual urine volume examinations. Only 17.4% of medical records had urinary function related admission, which were found in 26.4% of medical records when patients were discharged.ConclusionsMedical workers are lack of attentions to the rehabilitated exercise of urination function for patients with prostate hyperplasia and malignant tumor after prostate surgery. It is urgent to build a scheme of rehabilitated exercise of urination function for postoperative patients of prostate, which based on the fast-track surgery theory and used the time as axis.

Objective The aim of the present study was to investigate the ability to perceive syllables phrases ,mono-syllabic words ,disyllabic words ,when using a CI and an HA in non -implanted ears (bimodal fitting) ,and the relationship be-tween aided thresholds in the HA ear and bimodal effectiveness .Methods Eighteen children who consistently used a bimodal fitting participated in the study .The loudness of speech in the HA ear matched that with implanted ear ,and the aided thresh-olds in the HA ear were obtained .The recognition rate of syllables phrases ,monosyllabic words ,and disyllabic words was tested under the aforementioned two modes in both the quiet and noisy backgrounds .Results The speech performance of children for monosyllabic words were 82 .67% ± 12 .23% ,83 .61% ± 12 .22% ,for disyllabic words 76 .00% ± 16 .13% ,78 .11% ± 14 .84% , for syllables phrases 60 .11% ± 17 .18% ,65 .43% ± 16 .76% ,with a CI alone or with bimodal fitting in a quiet environment ,re-spectively .In a noisy environment ,monosyllabic words were 75 .50% ± 14 .12% ,76 .83% ± 14 .15% ,and disyllabic words were 68 .22% ± 17 .15% ,77 .18% ± 16 .83% ,and syllables phrases were 49 .39% ± 19 .26% ,56 .33% ± 19 .55% .The results sug-gested that speech performance in a quiet or a noisy environment was significantly better with bimodal hearing than with a CI a-lone .All were statistically significant except the recognition rate of monosyllabic words in quiet background (P<0 .05) .Signifi-cant negative correlations were found between aided thresholds at 250 and 500 Hz ,and the bimodal advantages were noticeable for the some speeching perception .Conclusion Bimodal hearing enhances speech performance for deaf patients .The low -fre-quency residual hearing in the HA ear may play a major role in enhancing auditory and speech performance .

Objective To study the expression of signal transducer and activator of transcription 3 (STAT3) and Suppressor of cytokine signaling 3(SOCS3) in the middle ear cholesteatoma epithelium ,and the possible roles of STAT3 and SOCS3 in middle ear cholesteatoma .Methods The immunohistochemical assay was used to detect ex-pression of STAT3 and SOCS3 protein in 30 cases of middle ear cholesteatoma epithelial tissue and 20 cases of nor-mal external auditory canal skin tissues as the control group .Results STAT3 immunoreactivity was detected in the nuclei and cytoplasm of epithelial cells .The expression rates of STAT3 in middle ear cholesteatoma epithelial tissue were 76 .7% and higher than in the normal epithelium (25 .0% ) .The differences between the two groups were sta-tistically significant (P<0 .05) .SOCS3 immunoreactivity was detected in the cytoplasm of epithelial cells .The ex-pression rates of SOCS3 in middle ear cholesteatoma epithelial tissue were 33 .3% and lower than in the normal epi-thelium (65 .0% ) .The differences were statistically significant (P<0 .05) .The expression of STAT3 and SOCS3 in the middle ear cholesteatoma had negative correlation (r= - 0 .476 ,P<0 .05) .Conclusion The abnormal ex-pression of STAT3 and SOCS3 in the middle ear cholesteatoma may be involved in hyper proliferation and anti -ap-optosis of cholesteatoma cell ,and play an important role in the formation and development of middle ear cholesteatoma .