Bidirectional Transport of IgE by CD23 in the Inner Ear of Patients with Meniere's Disease.

Meniere's disease (MD) is a disorder of the inner ear characterized by episodes of spontaneous vertigo, fluctuating hearing loss, and tinnitus. Recent studies have demonstrated that IgE may play a role in the pathogenesis of MD. Patients with MD (n = 103), acoustic neuroma (n = 5), and healthy subjects (n = 72) were recruited into the study. Serum from the participants was analyzed for IgE and type 2-related cytokines. IgE and CD23 expression levels in vestibular end organs of patients, C57BL/6 mice, or mouse HEI-OC1 cells were analyzed. Finally, the role of CD23 in IgE transcytosis was assessed using HEI-OC1 cells. Serum IgE was elevated in patients with MD and positively correlated with clinical symptoms. IL-4, IL-5, IL-10, IL-13, and CD23 levels were increased in patients with MD compared with the control group. In the transcytosis assay, mouse IgE was found to be bidirectionally transported across the HEI-OC1 cell monolayer. Additionally, CD23 downregulation using a small interfering RNA approach significantly reduced the efficiency of IgE transcytosis, suggesting that IgE is transported by CD23. Furthermore, exposure to IL-4 increased CD23 expression and enhanced IgE transcytosis in the HEI-OC1 cells and primary vestibular end organs. Our study indicated that IgE may play a role in the pathophysiology of MD. In addition, CD23-mediated IgE transcytosis in the hair cells may play a critical role in initiating inflammation in the inner ear. Thus, reducing the level of IgE may be a potentially effective approach for MD treatment.

Vestibular function and hearing preservation in children following a minimally invasive cochlear implantation.

PURPOSE: This retrospective cohort study aimed to investigate the effect of minimally invasive cochlear implantation (CI) on the vestibular function (VF) and residual hearing (RH) as well as their relationship in pediatric recipients before and after surgery. METHODS: Twenty-four pediatric patients with preoperative low frequency residual hearing (LFRH) (250 or 500 Hz ≤ 80 dB HL) who underwent minimally invasive CI were enrolled. Pure-tone thresholds, the cervical/ocular vestibular-evoked myogenic potential (cVEMP/oVEMP), and video head impulse test (vHIT) were all evaluated in the 24 pediatric patients with preoperative normal VF before and at 1 and 12 months after surgery. The relationship between changes in hearing and VF was analyzed preoperatively and at 1 and 12 months postoperatively. RESULTS: There were no significant differences on VF preservation and hearing preservation (HP) at both 1 and 12 months post-CI (p > 0.05). At 1 month post-CI, the correlations of the variations in vestibulo-ocular reflex (VOR) gains of horizontal semicircular canal (HSC) and posterior semicircular canal (PSC) and the shift in 250 Hz threshold were negatively correlated (r = - 0.41, p = 0.04 and r = - 0.43, p = 0.04, respectively). At 12 months post-CI, the shift in 250 Hz threshold negatively correlated to the variations in VOR gain of superior semicircular canal (SSC) (r = - 0.43, p = 0.04); the HP positively correlated to the variation in oVEMP-amplitude ratio (AR) (r = 0.41, p = 0.04). CONCLUSION: Our study confirmed that there were partial correlations between VF preservation and HP both in the short- and long-terms after atraumatic CI surgery, especially with the 250 Hz threshold. Regarding the variation of PSC function, the correlation with hearing status was variable with time after atraumatic CI surgery. Minimally invasive techniques for HP are successful and effective for the preservation of VF in pediatric patients both in the short- and long-terms.

Long-Term Auditory and Speech Outcomes of Cochlear Implantation in Children With Cochlear Nerve Aplasia.

OBJECTIVES: In this study, we aimed to (1) review the long-term outcomes of cochlear implantation in children with cochlear nerve aplasia and (2) compare the development of their auditory and speech abilities to children with normal-sized cochlear nerves. DESIGN: This is a retrospective case-control study. Patients who underwent unilateral cochlear implant (CI) surgery in a tertiary referral center from September 2012 to December 2018 were reviewed. The study group included 55 children with cochlear nerve aplasia diagnosed using preoperative images. The control group included 35 children with normal-sized cochlear nerves. The control group did not differ from the study group in terms of age at implantation, pre-implantation auditory and speech abilities, or the electrode array type. Cochlear implantation outcomes were assessed using a test battery, including the Categories of Auditory Performance (CAP) score, the Speech Intelligibility Rating (SIR) score, behavioral audiometry, and closed- or open-set speech recognition tests. The development of auditory and speech abilities was compared between the two groups using Generalized Linear Mixed-effect Models. RESULTS: The mean duration of CI usage was 4.5 years (SD = 1.5, range = 2.0 to 9.5) in the study group. The CAP scores, SIR scores, and aided hearing thresholds improved significantly post-implantation in the study group, but were significantly poorer than those in the control group. Generalized Linear Mixed-effect Models showed that the development of CAP and SIR scores was significantly slower in the study group than in the control group. Overall, 27 (49%) children with cochlear nerve aplasia had some degree of open-set speech perception skills, but the monosyllabic and bisyllabic word recognition rates were significantly lower than those in the control group. CONCLUSION: For children with cochlear nerve aplasia, auditory perception and speech intelligibility continued to improve in the long-term follow-up, but this progress was significantly slower than in children with normal-sized cochlear nerves. Most children with cochlear nerve aplasia could obtain the ability of common phrase perception and understanding simple spoken language with consistent CI usage and auditory rehabilitation.

Characteristics and prognosis analysis of bilateral sudden sensorineural hearing loss: A retrospective case-control study.

OBJECTIVES: To evaluate the clinical characteristics and prognostic factors of simultaneous and sequential bilateral sudden sensorineural hearing loss (Si-BSSNHL and Se-BSSNHL, respectively). DESIGN: Retrospective case-control study. SETTING: A single tertiary referral centre. PARTICIPANTS: Patients diagnosed with unilateral sudden sensorineural hearing loss (USSNHL), Si-BSSNHL, or Se-BSSNHL between September 2018 and November 2019. MAIN OUTCOME MEASURES: Demographic and clinical characteristics, audiological features, laboratory results and hearing recovery were analysed for intergroup comparisons. Prognostic factors for BSSNHL were analysed using univariate and multivariate logistic analyses between the overall and no-recovery groups. RESULTS: Compared to the USSNHL group, a larger final pure-tone average (PTA) (H = 38.0 and 53.8, respectively, both adjusted p-value (p adj) <.05), lower hearing gain (H = -70.8 and - 74.6, respectively, both p adj <.001) and higher homocysteine levels (H = 46.8, 58.8, respectively, both p adj <.05) were observed in the Si-BSSNHL and Se-BSSNHL groups, while the rate of positive vestibular tests and proportion of tinnitus were lower in the Se-BSSNHL group (χ2  = 8.5 and 38.1, respectively, both p adj <0.05). The USSNHL group showed a significant difference in the degree of deafness and therapeutic outcome in the Se-BSSNHL and Si-BSSNHL groups, respectively (χ2  = 12.4, p adj <.05; χ2  = 13.6; p adj <.05). Hypertension (95% confidence interval, 1.014-28.623, p < .05) and onset days (95% confidence interval, 0.007-0.626, p < .05) were associated with the therapeutic effects of BSSNHL. CONCLUSIONS: Higher homocysteine levels in BSSNHL may implicate microvascular disorders as a causative factor of BSSNHL. Hypertension and onset days were associated with the prognosis of BSSNHL.

Planar cell polarity defects and hearing loss in sperm-associated antigen 6 (Spag6)-deficient mice.

Spag6 encodes an axoneme central apparatus protein that is required for normal flagellar and cilia motility. Recent findings suggest that Spag6 also plays a role in ciliogenesis, orientation of cilia basal feet, and planar polarity. Sensory cells of the inner ear display unique structural features that underlie their mechanosensitivity. They represent a distinctive form of cellular polarity, known as planar cell polarity (PCP). However, a role for Spag6 in the inner ear has not yet been explored. In the present study, the function of Spag6 in the inner ear was examined using Spag6-deficient mice. Our results demonstrate hearing loss in the Spag6 mutants, associated with abnormalities in cellular patterning, cell shape, stereocilia bundles, and basal bodies, as well as abnormally distributed Frizzled class receptor 6 (FZD6), suggesting that Spag6 participates in PCP regulation. Moreover, we found that the subapical microtubule meshwork was disrupted. Our observations suggest new functions for Spag6 in hearing and PCP in the inner ear.

Efficacy of surgical repair for the functional restoration of injured facial nerve.

BACKGROUND: Early surgical repair to restore nerve integrity has become the most commonly practiced method for managing facial nerve injury. However, the evidence for the efficacy of surgical repair for restoring the function of facial nerves remains deficient. This study evaluated the outcomes of surgical repair for facial nerve lesions. METHODS: This retrospective observational study recruited 28 patients with the diagnosis of facial nerve injury who consecutively underwent surgical repairs from September 2012 to May 2019. All related clinical data were retrospectively analyzed according to age, sex, location of the facial nerve lesion, size of the facial nerve defect, method of repair, facial electromyogram, and blink reflex. Facial function was then stratified with the House-Brackmann grading system pre-operation and 3, 9, 15, and 21 months after surgical repair. RESULTS: The 28 patients enrolled in this study included 17 male and 11 female patients with an average age of 34.3 ± 17.4 years. Three methods were applied for the repair of an injured facial nerve, including great auricular nerve transplantation in 15 patients, sural nerve grafting in 7 patients, and hypoglossal to facial nerve anastomosis in 6 patients. Facial nerve function was significantly improved at 21 months after surgery compared with pre-operative function (P = 0.008). Following surgical repair, a correlation was found between the amplitude of motor unit potential (MUP) and facial nerve function (r = -6.078, P = 0.02). Moreover, the extent of functional restoration of the facial nerve at 21 months after surgery depended on the location of the facial nerve lesion; lesions at either the horizontal or vertical segment showed significant improvement(P = 0.008 and 0.005), while no functional restoration was found for lesions at the labyrinthine segment (P = 0.26). CONCLUSIONS: For surgical repair of facial nerve lesions, the sural nerve, great auricular nerve, and hypoglossal-facial nerve can be grafted effectively to store the function of a facial nerve, and MUP may provide an effective indicator for monitoring the recovery of the injured nerve.

A novel MYH14 mutation in a Chinese family with autosomal dominant nonsyndromic hearing loss.

BACKGROUND: MYH14 gene mutations have been suggested to be associated with nonsyndromic/syndromic sensorineural hearing loss. It has been reported that mutations in MYH14 can result in autosomal dominant nonsyndromic deafness-4A (DFNA4). METHODS: In this study, we examined a four-generation Han Chinese family with nonsyndromic hearing loss. Targeted next-generation sequencing of deafness genes was employed to identify the pathogenic variant. Sanger sequencing and PCR-RFLP analysis were performed in affected members of this family and 200 normal controls to further confirm the mutation. RESULTS: Four members of this family were diagnosed as nonsyndromic bilateral sensorineural hearing loss with postlingual onset and progressive impairment. A novel missense variant, c.5417C > A (p.A1806D), in MYH14 in the tail domain of NMH II C was successfully identified as the pathogenic cause in three affected individuals. The family member II-5 was suggested to have noise-induced deafness. CONCLUSION: In this study, a novel missense mutation, c.5417C > A (p.A1806D), in MYH14 that led to postlingual nonsyndromic autosomal dominant SNHL were identified. The findings broadened the phenotype spectrum of MYH14 and highlighted the combined application of gene capture and Sanger sequencing is an efficient approach to screen pathogenic variants associated with genetic diseases.

The Effects of GJB2 or SLC26A4 Gene Mutations on Neural Response of the Electrically Stimulated Auditory Nerve in Children.

OBJECTIVES: This study aimed to (1) investigate the effect of GJB2 and SLC26A4 gene mutations on auditory nerve function in pediatric cochlear implant users and (2) compare their results with those measured in implanted children with idiopathic hearing loss. DESIGN: Participants included 20 children with biallelic GJB2 mutations, 16 children with biallelic SLC26A4 mutations, and 19 children with idiopathic hearing loss. All subjects except for two in the SLC26A4 group had concurrent Mondini malformation and enlarged vestibular aqueduct. All subjects used Cochlear Nucleus devices in their test ears. For each subject, electrophysiological measures of the electrically evoked compound action potential (eCAP) were recorded using both anodic- and cathodic-leading biphasic pulses. Dependent variables (DVs) of interest included slope of eCAP input/output (I/O) function, the eCAP threshold, and eCAP amplitude measured at the maximum comfortable level (C level) of the anodic-leading stimulus (i.e., the anodic C level). Slopes of eCAP I/O functions were estimated using statistical modeling with a linear regression function. These DVs were measured at three electrode locations across the electrode array. Generalized linear mixed effect models were used to evaluate the effects of study group, stimulus polarity, and electrode location on each DV. RESULTS: Steeper slopes of eCAP I/O function, lower eCAP thresholds, and larger eCAP amplitude at the anodic C level were measured for the anodic-leading stimulus compared with the cathodic-leading stimulus in all subject groups. Children with GJB2 mutations showed steeper slopes of eCAP I/O function and larger eCAP amplitudes at the anodic C level than children with SLC26A4 mutations and children with idiopathic hearing loss for both the anodic- and cathodic-leading stimuli. In addition, children with GJB2 mutations showed a smaller increase in eCAP amplitude when the stimulus changed from the cathodic-leading pulse to the anodic-leading pulse (i.e., smaller polarity effect) than children with idiopathic hearing loss. There was no statistically significant difference in slope of eCAP I/O function, eCAP amplitude at the anodic C level, or the size of polarity effect on all three DVs between children with SLC26A4 mutations and children with idiopathic hearing loss. These results suggested that better auditory nerve function was associated with GJB2 but not with SLC26A4 mutations when compared with idiopathic hearing loss. In addition, significant effects of electrode location were observed for slope of eCAP I/O function and the eCAP threshold. CONCLUSIONS: GJB2 and SLC26A4 gene mutations did not alter polarity sensitivity of auditory nerve fibers to electrical stimulation. The anodic-leading stimulus was generally more effective in activating auditory nerve fibers than the cathodic-leading stimulus, despite the presence of GJB2 or SLC26A4 mutations. Patients with GJB2 mutations appeared to have better functional status of the auditory nerve than patients with SLC26A4 mutations who had concurrent Mondini malformation and enlarged vestibular aqueduct and patients with idiopathic hearing loss.

Prevalence of Mutations in Deafness-Causing Genes in Cochlear Implanted Patients with Profound Nonsyndromic Sensorineural Hearing Loss in Shandong Province, China.

The mutations of GJB2, SLC26A4, and mtDNA12SrRNA are the most common inherited causes of nonsyndromic sensorineural hearing loss (NSHL) in China, yet previous genetic screenings were mainly carried on patients with moderate-to-profound impairment. We aimed to detect the mutation frequencies in NSHL population within a more specified range of severity. Patients with profound NSHL who had undergone cochlear implantation in the Shandong Provincial Hospital (Shandong, China) were recruited. The majority (n = 472) were between 0.7 and 6 years old, and the remaining (n = 63) were between 6 and 70 years old. In total, 115 mutation alleles of the three genes were screened with SNP scan assay. Of the patients, 19.44% (104/535) were found to have GJB2 mutations, and the most common allele was c.235delC, followed by c.299_300delAT and c.109G>A. SLC26A4 mutations were detected in 13.46% patients (72/535), and the most common allele was c.919-2A>G (IVS7-2A>G), followed by c.1174A>T and c.2168A>G. Seven patients (1.31%) carried mutations in mtDNA12SrRNA, with the alleles of m.1555A>G and m.1494C>T. We found the allele frequency of c.109G>A (GJB2) was relatively lower in the profound NSHL population in comparison to the moderate-to-profound ones, and the c.1174A>T (SLC26A4) relatively higher. It suggests those mutations may be connected with the degree of deafness, which needs more observations and analyses to support.

The Three-Dimensional Culture System with Matrigel and Neurotrophic Factors Preserves the Structure and Function of Spiral Ganglion Neuron In Vitro.

Whole organ culture of the spiral ganglion region is a resourceful model system facilitating manipulation and analysis of live sprial ganglion neurons (SGNs). Three-dimensional (3D) cultures have been demonstrated to have many biomedical applications, but the effect of 3D culture in maintaining the SGNs structure and function in explant culture remains uninvestigated. In this study, we used the matrigel to encapsulate the spiral ganglion region isolated from neonatal mice. First, we optimized the matrigel concentration for the 3D culture system and found the 3D culture system protected the SGNs against apoptosis, preserved the structure of spiral ganglion region, and promoted the sprouting and outgrowth of SGNs neurites. Next, we found the 3D culture system promoted growth cone growth as evidenced by a higher average number and a longer average length of filopodia and a larger growth cone area. 3D culture system also significantly elevated the synapse density of SGNs. Last, we found that the 3D culture system combined with neurotrophic factors had accumulated effects in promoting the neurites outgrowth compared with 3D culture or NFs treatment only groups. Together, we conclude that the 3D culture system preserves the structure and function of SGN in explant culture.

Expression of artemin and GFRα3 in an animal model of migraine: possible role in the pathogenesis of this disorder.

BACKGROUND: Neurotrophic factors have been implicated in hyperalgesia and peripheral levels of these molecules are altered in migraine pathophysiology. Artemin, a vasculature-derived neurotrophic factor, contributes to pain modulation and trigeminal primary afferent sensitization through binding its selective receptor GFRα3. The distribution of artemin and GFRα3 in the dura mater raises an anatomy supports that they may be involved in migraine. In this study we evaluated the expression of artemin and GFRα3 in an animal migraine model that may be relevant for migraine. METHODS: In this study, using a rat migraine model by administration of nitroglycerin (NTG), we investigated the expression of artemin in the dura mater and GFRα3 in the trigeminal ganglia (TG) by means of quantitative reverse transcription-polymerase chain reaction, western blot and immunofluorescence labeling. RESULTS: Artemin immunoreactivity was found in the smooth muscle cells of dural vasculature and GFRα3 was present in cytoplasm of TG neurons. The mRNA levels of artemin and GFRα3 were significantly elevated after NTG treatment at 2 and 4 h respectively (P < 0.05). The expression of artemin protein was increased at 4 h and continually up to 8 h in the dura mater following NTG administration (P < 0.05). The expression of GFRα3 protein was elevated at 4 h and continually up to 10 h in the TG following NTG administration (P < 0.05). CONCLUSION: The findings suggest that artemin and GFRα3 play an important role in the pathogenesis of migraine and may represent potential therapeutic targets for the treatment of migraine.

Identification of a novel missense mutation in the WFS1 gene as a cause of autosomal dominant nonsyndromic sensorineural hearing loss in all-frequencies.

Hearing loss is the most common sensory disorder affecting 278 million people in the world, and more than 60% of hearing loss patients can be attributed to genetic causes. Although many loci have been linked to hereditary hearing impairment, most of the causative genes have not been identified as yet. The goal of this study was to investigate the cause of dominantly inherited sensorineural all-frequency hearing loss in a six-generation Chinese family. We performed exome sequencing to screen responsible candidate genes in three family members with all-frequency hearing loss and one member with normal hearing. Sanger sequencing was employed to examine the variant mutations in the members of this family and 200 healthy persons. PCR-RFLP was performed to further confirm the nucleotide mutation. A novel missense mutation c.2389G > A (GAC → AAC) in WFS1 gene was identified, which was co-segregated with the hearing loss phenotype. No mutation was found in 200 controls and the family members with normal hearing in this site. The present study identifies, for the first time, a novel mutation in WFS1 gene that causes non-syndromic hearing loss in all, rather than in low or high, frequencies.

[Diagnosis and treatment of 68 cases of traumatic facial nerve paralysis].

Objective:To summarize the clinical characteristics and therapeutic effect of traumatic facial nerve palsy. Methods:Sixty-eight cases of traumatic facial nerve palsy were retrospectively analyzed from January 2015 to May 2023. Results:The median course of disease was 33 days. The facial nerve function of the patients was grade HB-Ⅱin 2 cases, grade HB-Ⅲ in 4 cases, grade HB-Ⅳin 16 cases, grade HB-Ⅴ in 37 cases（38 ears）, and grade HB-Ⅵ in 9 cases. 42 cases occurred immediately after injury and 26 cases were delayed. CT examination of temporal bone revealed longitudinal fractures in 51 cases（52 ears） , transverse fractures in 6 cases and mixed fractures in 4 cases. No definite temporal bone fracture was found in the remaining 7 cases. The segments of facial nerve injury in 49 cases（50 ears） were geniculate ganglion and adjacent, in 7 cases were vertical segment, in 7 cases were horizontal segment, in 2 cases were horizontal segment and vertical segment; and the other 3 cases could not be evaluated. Conservative treatment with glucocorticoids was used in 23 ears and surgery was used in 46 ears. Patients were followed up 6-24 months after treatment, including 20 cases of grade HB-Ⅰ, 19 cases of grade HB-Ⅱ, 23 cases（24 ears） of grade HB-Ⅲ, 4 cases of grade HB-Ⅳ, and 1 case of grade HB-Ⅴ.One patient was lost to follow-up. After treatment, the facial nerve function of patients was significantly improved（P<0.05）, and there were significant differences between conservative treatment group and surgical treatment group in the course of facial nerve palsy, the ratio of facial palsy immediately after injury, the nerve function before treatment and the nerve function after treatment（P<0.05）. There were no significant differences in age, sex, hearing condition, temporal bone fracture, facial nerve injury segment and rate of favorable neurologic outcomes（P>0.05）. The comparison of patients with neurodegeneration rate>90% and ≤90% showed that the facial nerve function of patients with neurodegeneration rate>90% before treatment was significantly worse（P<0.05）, but there was no significant difference between the facial nerve function after treatment（P>0.05）. There was no significant difference in facial nerve function between middle fossa approach group and mastoid approach group（P>0.05）. Conclusion:Patients with traumatic facial nerve palsy should be evaluated individually. Patients with mild facial nerve palsy, low neurodegeneration rate and short course of disease can be treated conservatively and followed up closely. Patients with severe facial nerve palsy, high neurodegeneration rate and more than 6 weeks of disease can be actively considered surgery. Good prognosis can be obtained by correct evaluation and treatment.

Effect of Late-Stage Meniere's Disease and Vestibular Functional Impairment on Hippocampal Atrophy.

OBJECTIVE: We investigated correlations among clinical features, degree of inner ear endolymphatic hydrops (EH), and hippocampal volume (HV) in different stages of Meniere's disease (MD). METHODS: From February 2021 to April 2022, clinical data were collected from 99 patients (39 males, 60 females, mean age: 50.4 ± 10.0 [range: 26-69] years) with unilateral MD admitted to the Department of Vertigo Disease of Shandong ENT Hospital. The left and right ears were affected in 64 and 35 patients, respectively. There were 50 and 49 cases in early (Stages 1, 2) and late stages (Stages 3, 4), respectively. Fifty healthy participants were included as controls. Audiovestibular function test results, EH grading using gadolinium-enhanced magnetic resonance imaging (MRI), and HV determined on MRI were analyzed for patients at different stages of MD. RESULTS: Between-group comparisons of early and late MD revealed significant differences in the disease course, vestibular function (VF), degree of EH, and HV. There were no significant between-group differences based on age, sex, affected side, subjective degree of dizziness, hospital anxiety, or depression. Mean HV in patients with early-stage MD was correlated with the canal paresis value of the caloric test and pure tone hearing threshold, HV in late-stage patients was correlated with vestibular EH. CONCLUSION: Patients with late-stage MD exhibited severe auditory and VF impairments, increased EH, and atrophy of the HV. More advanced disease was associated with greater vestibular damage and degree of EH. LEVEL OF EVIDENCE: 3 Laryngoscope, 134:410-418, 2024.

A case of pseudogout of the temporomandibular joint with giant cell reparative granuloma of the temporal bone.

Pseudogout, also known as calcium pyrophosphate dihydrate deposition disease (CPPD) with giant cell reparative granuloma (GCRG) of the temporal bone is a rare disease, which is very easy to misdiagnose. When two diseases occur simultaneously, the pathological tissue of diseases is closely associated, which complicates clinical representation and causes enormous difficulty in diagnosis and treatment. We report a case of CPPD of the temporomandibular joint accompanied by surrounding GCRG of temporal bone in a 62-year-old male.

Vestibule Plugging for Patient with Isolated Otolith Dysfunction: A Case Report.

There is currently no satisfactory treatment for otolith dysfunctions. Here, we propose a novel surgical method, vestibule plugging (VP), and the results confirm its effectiveness and safety in treatment of otolith dysfunction. Laryngoscope, 133:1734-1736, 2023.

Surfactant Protein A Can Affect Macrophage Phagocytosis: An Important Pathogenic Mechanism of Otitis Media with Effusion.

Otitis media with effusion (OME), also known as secretory otitis media, is a common condition in otorhinolaryngology. The main manifestations include middle ear effusion and conductive hearing loss. Recently, increasing attention has been paid to the etiology of OME, wherein immune dysfunction is one important pathogenic mechanism. However, it is unknown whether changes in surfactant protein A (SPA) secretion affect the phagocytic activity of macrophages in the Eustachian tube, thereby altering pathogen clearance, during the pathogenesis of OME. In our study, an OME animal model was established and evaluated. Differences in SPA levels in Eustachian tube lavage fluid between the experimental and control groups were analyzed. Cell-based experiments revealed that SPA decreased the expression of CD64 and SYK and inhibited phagocytosis by RAW264.7 cells. By using flow cytometry and immunofluorescence, we confirmed that macrophage phagocytosis decreased with increasing SPA levels. Finally, we concluded that SPA affects macrophage function and plays a role in the occurrence and development of OME.

Relationship between electrically evoked compound action potential thresholds and behavioral T-levels in implanted children with cochlear nerve deficiency.

It is challenging to program children with cochlear nerve deficiency (CND) due to limited auditory and speech abilities or concurrent neurological deficits. Electrically evoked compound action potential (ECAP) thresholds have been widely used by many audiologists to help cochlear implant programming for children who cannot cooperate with behavioral testing. However, the relationship between ECAP thresholds and behavioral levels of cochlear nerve in children with CND remains unclear. This study aimed to investigate how well ECAP thresholds are related to behavioral thresholds in the MAP for children with CND. This study included 29 children with CND who underwent cochlear implantation. For each participant, ECAP thresholds and behavioral T-levels were measured at three electrode locations across the electrode array post-activation. The relationship between ECAP thresholds and behavioral T-levels was analyzed using Pearson's correlation coefficient. The results showed that ECAP thresholds were significantly correlated with behavioral T-levels at the basal, middle, and apical electrodes. ECAP thresholds were equal to or higher than the behavioral T-levels for all tested electrodes, and fell within MAP's dynamic range for approximately 90% of the tested electrodes. Moreover, the contour of the ECAP thresholds was similar to the contour of T-levels across electrodes for most participants. ECAP thresholds can help audiologists select stimulation levels more efficiently for children with CND who cannot provide sufficient behavioral response.

Systematic Hearing Performance Evaluation Process for Adolescents with Cochlear Implantation at Early Ages.

Cochlear implant (CI) provision is the most effective clinical treatment to restore hearing performance in individuals with profound sensorineural hearing loss (SNHL). It has been successful in providing improved speech perception outcomes, especially in quiet environments. However, speech perception performance within complex environments, lexical tone recognition, and music perception have been shown to only improve with newer fine structure coding strategies or related techniques. Therefore, the methods used to assess hearing performance in noisy environments, lexical tone recognition, and music perception are of vital importance. These assessments must reflect the postoperative outcomes and also provide guidance for the programming, rehabilitation, and application of new coding strategies. In this study, hearing performance in simple and complex situations was evaluated before and after upgrading to a fine structure strategy. The participants were a cohort of Mandarin-speaking adolescents, who were experienced CI users. The comprehensive clinical workflow involved assessments of speech in quiet conditions, speech in noisy conditions, lexical tone recognition, and music perception. This battery of tests is explained in detail, from the coding strategy to the test methods, including the test process, environment, device, material, and order. The details that require special attention are discussed, such as the position of the participants, the angle of the loudspeaker, the intensity of the sound, the noise type, the practice test, and the way of answering questions. Each test step, method, and material for speech, lexical tone, and music perception is presented in detail. Finally, the clinical results are discussed.

Foreign Body Granuloma Originating From the Lateral Process of Malleus: A Case Series of 19 Children.

OBJECTIVE: A special presentation of foreign body granuloma originating from the lateral process of the malleus (FBGLP) was noted in the absence of a history of foreign body entry into the external auditory canal (EAC). This study reported the clinical features, pathology, and prognosis of patients with FBGLP. DESIGN: Retrospective study. SETTING: Shandong Provincial ENT Hospital. PATIENTS: Nineteen pediatric patients (age, 1-10 yr) with FBGLP. INTERVENTIONS: Clinical data were collected from January 2018 to January 2022. MAIN OUTCOME MEASURES: Clinicopathologic characteristics of the patients were analyzed. RESULTS: All patients had an acute course, and were within 3 months of ineffective medical treatment. The most common symptoms were suppurative (57.9%) and hemorrhagic (42.1%) otorrhea. FBGLP imaging examinations demonstrated a soft mass blocking the EAC without bone destruction and occasionally concomitant effusion in the middle ear. The most common pathologic findings were foreign body granuloma (94.7%,18/19), granulation tissue (73.7%, 14/19), keratotic precipitate (73.7%, 14/19), calcium deposition (63.2%, 12/19), hair shafts (47.4%, 9/19), cholesterol crystals (5, 26.3%), and hemosiderin (15.8%, 3/19). Foreign body granuloma and granulation tissue showed higher expression levels of CD68 and cleaved caspase-3 than did the normal tympanic mucosa, whereas Ki-67 levels were similarly low in all tissues. The patients were followed up for 3 months to 4 years without recurrence. CONCLUSION: FBGLP is caused by endogenous foreign particles in the ear. We recommend the trans-external auditory meatus approach for FBGLP surgical excision, as this shows promising outcomes.