[Efficacy evaluation of short-term personalized vestibular rehabilitation in the treatment of acute unilateral vestibulopathy].

Objective: To explore the efficacy and effective node of short-term personalized vestibular rehabilitation (ST-PVR) in treating acute unilateral vestibulopathy (AUVP). Methods: A randomized controlled trial was carried out. The AUVP patients who were admitted to the First Affiliated Hospital of Zhengzhou University from July 2022 to March 2023 were selected and randomized to the vestibular rehabilitation (VR) group and control group via computer-generated randomization. Standard care was the medical treatment with betahistine and prednisolone. Meanwhile, the VR group received ST-PVR. All the patients completed the baseline assessment and underwent follow-up assessments at 1 month and 3 months after the treatment. The assessments were consisted of spontaneous nystagmus (NYS), Romberg test (ROM), head thrust test (HTT), visual analogue scale (VAS) for vertigo, dizziness handicap inventory scale (DHI), activities-specific balance confidence scale (ABC), caloric test using video-electronystagmograph (VNG), and video-head impulse test (vHIT). The measurement data that did not conform to normal distribution were represented by M (Q1, Q3). Generalized estimating equation (GEE) was used to analyze the influence of the ST-PVR on the values of these clinical indicators and the VR grading score. The values of clinical indicators and the VR grading score were compared between the two groups at each follow-up point. Results: Seventy-one AUVP patients were included, with 35 cases in the VR group [14 males and 21 females, aged 51 (33, 55) years] and 36 cases in control group [17 males and 19 females, aged 46 (34, 59) years]. There were statistically significant differences in the impact of ST-PVR on the values of clinical indicators between the two groups (ABC: ß=10.89, P<0.001; VAS: ß=-1.64, P<0.001; DHI: ß=-8.70, P<0.001; NYS: ß=26.73, P<0.001; vHIT: ß=1.41, P=0.047; the VR grading score: ß=1.03, P=0.045). The assessments of the VR group in the positive rate of NYS [14.3% (5/35) vs 50.0% (18/36), P<0.001], ROM [48.6% (17/35) vs 55.6% (20/36), P<0.001], directional preponderance (DP) [34.3% (12/35) vs 75.0% (27/36), P<0.001] and DHI [26 (22, 32) vs 36 (30, 60), P=0.001] were significantly lower than that of the control group at 1 month after the treatment. The results showed a statistically significant difference in ABC [88 (80, 90) vs 76 (61, 88), P<0.001], VAS [2 (1, 3) vs 3 (2, 5), P<0.001] at 3-months after the treatment. The VR grading score of the VR group was improved significantly than those of the control group at 1 month after treatment [21 (17, 21) vs 16 (13, 20), P=0.001]. Conclusion: ST-PVR could improve the results of clinical indicators and VR grading score of the AUVP patients effectively after 1 month of the systematical treatment, and alleviate the symptoms and signs of dizziness in the acute phase as early as possible.

[Analysis of perrault syndrome caused by pathogenic variants in LARS2 and HARS2 genes].

Objective: To investigate the molecular etiology of Perrault syndrome by analyzing the clinical phenotype and pathogenic gene variants of 2 male patients with bilateral severe sensorineural deafness. Methods: Two male patients with Perrault syndrome characterized by severe sensonrineual deafness adimitted to the First Affiliated Hospital of Zhengzhou University between February 2021 and March 2022 were selected, and the clinical phenotype and pathogenic gene variants of them and their family members were summarized. The whole exome sequencing technology was used to screen the pathogenic variants of the probands, and the candidate variants were determined by combining with clinical phenotype. The probands and their family members were verified by the Sanger sequencing method. Results: The whole exome sequencing results showed that the proband of family 1 had a compound heterozygous variants of the LARS2 (NM_015340.4) gene c.1565C>A (p.Thr522Asn) and c.1079T>C (p.Ile360Thr). The reported pathogenic variant c.1565C>A came from the mother, and the novel variant c.1079T>C came from the father. The second proband harbored compound heterozygous variants of HARS2 gene (NM_012208.4) c.1273C>T (p.Arg425Trp) and c.1403G>C (p.Gly468Ala), with the former from the proband's mother, the latter from the father. The c.1273C>T was novel and c.1403G>C was the reported pathogenic variant. All above variants were respectively classified as pathogenic, uncertain significance, uncertain significance and likely pathogenic based on the ACMG guidelines. Conclusion: This study expands the mutational spectrum of LARS2 and HARS2 genes, which highlights that genetic testing plays an important role in the early diagnosis of syndromic deafness.

[Prediction of round window visibility in cochlear implantation with temporal bone high resolution computed tomography].

Objective: To discuss the prediction of round window(RW) visibility in cochlear implantation(CI) with temporal bone high resolution computed tomography(HRCT). Methods: From January 2013 to January 2017, 130 cases underwent both HRCT and CI in our hospital were analyzed. The distance from facial nerve to posterior canal wall(FWD), the angle between facial nerve and inner margin of round window(FRA), and the angle between facial nerve and tympanic anulus to inner margin of round window(FRAA) were detected at the level of round window on axial temporal bone HRCT. A line parallel to the posterior wall of ear canal was drawn from the anterior wall of facial nerve at the level of round window on axial temporal bone HRCT and its relationship with round window was detected (facial-round window line, FRL): type0-posterior to the round window, type1-between the round window, type2-anterior to the round window. Their(FWD, FRA, FRAA, FRL) relationships with intra-operative round window visibility were analyzed by SPSS 17.0 software. Results: FWD(F=18.76, P=0.00), FRA(F=34.57, P=0.00), FRAA (F=14.24, P=0.00) could affect the intra-operative RW visibility significantly. RW could be exposed completely during CI when preoperative HRCT showing type0 FRL. RW might be partly exposed and not exposed when preoperative HRCT showing type1 and type2 FRL respectively. Conclusion: FWD, FRA, FRAA and FRL of temporal bone HRCT can predict intra-operative round window visibility effectively in CI surgery.

[Possible reasons for cerebrospinal fluid gusher in cochlear implantation with inner ear abnormality].

Objective: To discuss the possible reasons for cerebrospinal fluid (CSF) gusher in cochlear implantation (CI) with inner ear abnormality. Method: A retrospective analysis was performed on 340 cases who underwent CI from January 2013 to December 2016 in Division of Otology, Otorhinolaryngology Hospital, the First Affiliated Hospital of Zhengzhou University. Among them, 96 cases had inner ear abnormalities. Imaging examinations were performed on these patients, and classification of inner ear malformation was done according to the results. Results: Among the cases with inner ear abnormality, 9.4% (9/96) suffered from CSF gusher during CI. The inner ear abnormalities were found to be as follows: 3 cases had incomplete partition type Ⅰ; 1 case had incomplete partition type Ⅰ with semicircular canal dysplasia; 1 case had common cavity deformity; 1 case had enlarged vestibular aqueducts and common cavity deformity; 2 cases had Mondini deformity. All of these cases had bony defect in the fundus of the internal acoustic meatus observed on CT scans. Another case was type 1 cochlear aqueduct with round window aplasia. Conclusions: Defects in the modiolus or fundus of the internal acoustic meatus is the main reason for CSF gusher during CI. A patent cochlear aqueduct is another possible reason.

[Cortical auditory evoked potentials in congenital hearing impaired children with cochlear implants].

OBJECTIVE: By investigating the auditory cortical evoked potential in congenital hearing impaired children with cochlear implants, the association between central auditory development and the age of implantation was studied. METHODS: P1-N1-P2 were recorded in 110 profound hearing impaired children, aged from 12 to 80 months old and being implanted with cochlear implants before the age of 5 years. Their implant using time ranged from just at the switch-on to 48 months. The stimuli were /m/, /t/, /g/, presented at 65 dB SPL in sound field. The presence rate of each wave was obtained and the relationship between P1 latency and implant age, the time of speech processor switch-on were analyzed. RESULTS: The presence rate of P1, N1 and P2 was 66.4%, 15.5% and 12.7%, respectively. The presence of P1 was significantly higher than that of N1(χ(2)=228.542, P=0.00)and P2(χ(2)=257.438, P=0.00). There was no significant difference of P1 presence rate elicited by /m/, /t/ and /g/(64.1%, 66.9% and 68.3%, χ(2)=0.589, P=0.75). There existed no significant difference either among P1 latency(P=0.22)or amplitude(P=0.09) elicited by /m/, /t/ and /g/. There was significant difference between the implant age before and after 42-month-old regarding the proportion that entered the age-appropriate normal P1 latency range(P=0.02). No significant difference was found among groups of implant using time of 1, 2, 3 and 4 years in aspect of the proportion that entered the age-appropriate normal P1 latency range(P=1.00). CONCLUSIONS: Compared with implanted after the age of 42-month-old children with prelingual hearing impairment younger than 5 years old, the ones implanted before 42-month-old have more chance for normal development for central auditory system. Once implanted before 42-month-old, the cortical auditory system restored its normal development as early as 1 year after implantation.