UNASSIGNED: Systemic sclerosis (SSc) is a disease of a very heterogeneous clinical picture and immunological profile with progression rate that varies between individuals. Although hearing deterioration is not a complaint that comes to the fore in SSc patients, as it is not life-threatening compared to many other more severe symptoms of this disease, it can significantly impair the quality of life. Medical literature concerning this problem is rather scarce.
UNASSIGNED: In this article we systematically reviewed the medical publications concerning hearing impairment in patients with systemic sclerosis to evaluate current understanding of this complex problem. Following PRISMA guidelines a total of 19 papers were found and analysed including 11 original studies and 8 case reports.
UNASSIGNED: Although it seems that hearing impairment in SSc patients is relatively more common than in the general population, based on the analysis of available literature, no firm conclusions regarding its frequency and pathomechanism can be drawn yet. Microangiopathy leading to damage to the sensory cells of the inner ear is suspected to be the main mechanism of hearing loss, although damage to the higher levels of the auditory pathway appears to be underestimated due to incomplete audiological diagnosis.
UNASSIGNED: Undoubtedly, the reason for the difficulty in such an evaluation are the complex and still not fully elucidated pathomechanism of SSc, the individually variable dynamics of the disease and the unique heterogeneity of symptoms. Nevertheless, further studies in larger and appropriately selected groups of patients, focused more on the dynamics of microangiopathy and not solely on clinical symptoms could provide answers to many key questions in this regard.

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UNASSIGNED: To describe audiological symptoms, audiometric profile, and distortion product otoacoustic emission in symptomatic patients recovering from SARS-CoV-2 infection (positive RT-PCR test) and asymptomatic patients (negative RT-PCR test).
UNASSIGNED: An analytical cross-sectional study was conducted using data obtained from clinical charts, physical examination, audiometry, and distortion product otoacoustic emission on 40 patients [case patients (CP)] recovering from SARS-CoV-2 infection diagnosed by a positive RT-PCR test and 22 asymptomatic participants with a negative RT-PCR test [non-case (NC)].
UNASSIGNED: Sixty-two patients (mean age: 31.1 and 28.2 years in the CP and NC groups, respectively) were included. All participants were young without significant comorbidities, risk factors for hearing loss or otological history. Vertigo (5%), tinnitus (17.5%) and aural fullness/hearing loss (35%) were found in the CP group. A statistically significant difference was found in specific frequencies (1000, 4000, and 8000 Hz) and pure tone average (low and high conversational frequencies with increased threshold in the PC group compared with the NC group), which was not found in distortion product otoacoustic emission.
UNASSIGNED: Audiovestibular symptoms are frequent in symptomatic patients recovering from SARS-CoV-2 infection. SARS-CoV-2 infection was consistently associated with an increased audiometric hearing threshold at specific frequencies and low tone average.

Despite the low overall prevalence of individual rare diseases, cochlear dysfunction leading to hearing loss represents a symptom in a large proportion. The aim of this work was to provide a clear overview of rare cochlear diseases, taking into account the embryonic development of the cochlea and the systematic presentation of the different disorders. Although rapid biotechnological and bioinformatic advances may facilitate the diagnosis of a rare disease, an interdisciplinary exchange is often required to raise the suspicion of a rare disease. It is important to recognize that the phenotype of rare inner ear diseases can vary greatly not only in non-syndromic but also in syndromic hearing disorders. Finally, it becomes clear that the phenotype of the individual rare diseases cannot be determined exclusively by classical genetics even in monogenetic disorders.
Auch wenn die einzelnen Krankheitsbilder selten sind, stellen seltene Erkrankungen der Cochlea in ihrer Gänze eine doch gehäufte Entität dar, die zu Hörstörungen führt. Ein/Das Ziel des vorliegenden Referates war es, unter Berücksichtigung der Embryonalentwicklung der Hörschnecke und einer systematischen Zusammenfassung eine übersichtliche Darstellung der seltenen cochleären Erkrankungen zu ermöglichen. Auch wenn rapide biotechnologische und bioinformatische Fortschritte die Diagnose einer seltenen Erkrankung erleichtern, so kann oft nur im interdisziplinären Austausch der Verdacht einer seltenen Erkrankung erhoben werden. Trotz gleicher zugrunde liegender Mutationen kann der Phänotyp nicht nur bei den genetisch bedingten Hörstörungen sondern auch bei den syndromalen Erkrankungen stark variieren. Schließlich wird deutlich, dass der Phänotyp der einzelnen seltenen Erkrankungen nicht ausschließlich durch die klassische Genetik bestimmt werden kann.

Umbilical cord-derived mesenchymal stromal cells (UCMSCs) have potential applications in regenerative medicine. UCMSCs have been demonstrated to repair tissue damage in many inflammatory and degenerative diseases. We have previously shown that UCMSC exosomes reduce nerve injury-induced pain in rats. In this study, we characterized UCMSC exosomes using RNA sequencing and proteomic analyses and investigated their protective effects on cisplatin-induced hearing loss in mice. Two independent experiments were designed to investigate the protective effects on cisplatin-induced hearing loss in mice: (i) chronic intraperitoneal cisplatin administration (4 mg/kg) once per day for 5 consecutive days and intraperitoneal UCMSC exosome (1.2 μg/μL) injection at the same time point; and (ii) UCMSC exosome (1.2 μg/μL) injection through a round window niche 3 days after chronic cisplatin administration. Our data suggest that UCMSC exosomes exert protective effects in vivo. The post-traumatic administration of UCMSC exosomes significantly improved hearing loss and rescued the loss of cochlear hair cells in mice receiving chronic cisplatin injection. Neuropathological gene panel analyses further revealed the UCMSC exosomes treatment led to beneficial changes in the expression levels of many genes in the cochlear tissues of cisplatin-injected mice. In conclusion, UCMSC exosomes exerted protective effects in treating ototoxicity-induced hearing loss by promoting tissue remodeling and repair.

Incomplete partition type III (IP-III) is a relatively rare inner ear malformation that has been associated with a POU3F4 gene mutation. The IP-III anomaly is mainly characterized by incomplete separation of the modiolus of the cochlea from the internal auditory canal. We describe a 71-year-old woman with profound sensorineural hearing loss diagnosed with an IP-III of the cochlea that underwent cochlear implantation. Via targeted sequencing with a non-syndromic gene panel, we identified a heterozygous c.934G > C p. (Ala31Pro) pathogenic variant in the POU3F4 gene that has not been reported previously. IP-III of the cochlea is challenging for cochlear implant surgery for two main reasons: liquor cerebrospinalis gusher and electrode misplacement. Surgically, it may be better to opt for a shorter array because it is less likely for misplacement with the electrode in a false route. Secondly, the surgeon has to consider the insertion angles of cochlear access very strictly to avoid misplacement along the inner ear canal. Genetic results in well describes genotype-phenotype correlations are a strong clinical tool and as in this case guided surgical planning and robotic execution.

OBJECTIVE: To determine the effect of cochlear dimensions on cochlear implant selection in cochlear hypoplasia patients.
METHODS: Temporal bone computed tomography images of 36 patients diagnosed with cochlear hypoplasia between 2010 and 2016 were retrospectively reviewed and compared with those of 40 controls without sensorineural hearing loss.
RESULTS: Basal turn length and mid-modiolar height were significantly lower in the cochlear hypoplasia patients with subtypes I, II and III than in the control group (p < 0.001). Mid-scalar length was significantly shorter in subtype I-III patients as compared with the control group (p < 0.001). In addition, cochlear canal length (measured along the lateral wall) was significantly shorter in subtype I-IV patients than in the control group (subtypes I-III, p < 0.001; subtype IV, p = 0.002).
CONCLUSIONS: Cochlear hypoplasia should be considered if basal turn length is less than 7.5 mm and mid-modiolar height is less than 3.42 mm. The cochlear implant should be selected according to cochlear hypoplasia subgroup. It is critically important to differentiate subtype II from incomplete partition type I and subtype III from a normal cochlea, to ensure the most appropriate implant electrode selection so as to optimise cochlear implantation outcomes.

UNASSIGNED: No cochlear otosclerosis in infants with congenital bilateral SNHL has been reported.
UNASSIGNED: We report an infant male with bilateral cochlear otosclerosis, suggesting that cochlear otosclerosis may be a congenital disease and to further analyze the etiology of and genetic expression in congenital bilateral cochlear otosclerosis. We also describe the clinical characteristics and experience of patients with bilateral cochlear otosclerosis treated with cochlear implants (CIs).
UNASSIGNED: Seven patients, including an infant, who were diagnosed with cochlear otosclerosis underwent CI surgery. Their medical records, audiological and radiological results, surgical procedures, and CI outcomes were collected and reviewed.
UNASSIGNED: The median age at hearing loss was 38 years, ranging from 0 to 47 years. The child had bilateral hearing loss at birth and received a CI at 1 year of age. He also had growth retardation and was diagnosed with 3q+/3p- syndrome. All patients (8 ears) had better postoperative auditory performance than that preoperatively.
UNASSIGNED: Although cochlear otosclerosis often starts at middle age and progresses slowly, it may be a congenital disease that is related to chromosome abnormality. This disease presents with SNHL or MHL, and treatment with a CI is beneficial.

BACKGROUND: Hyperinsulinemia is the most common metabolic change associated with cochleovestibular diseases.
OBJECTIVE: We aimed to investigate the auditory functions in hyperinsulinemic individuals.
METHODS: A total of 164 patients were included in this case-control study. While 76 patients with insulin resistance (homeostasis model assessment of insulin resistance [HOMA-IR] of ≥2.5) constituted the case group, 88 patients with HOMA-IR values of <2.5 constituted the control group of the study.
METHODS: The 75 g oral glucose tolerance test, blood biochemistry tests, hormonal analysis, audiological assessment, electrocochleography (EcochG), and transient evoked otoacoustic emissions (TEOAE) testing were performed.
METHODS: One-way analysis of variance and Kruskal-Wallis analysis of variance were used for the comparison of the metabolic and ear parameters in the normal glucose tolerance (NGT), impaired fasting glucose (IFG), and impaired glucose tolerance (IGT) groups. The chi-square test was used to compare nominal variables. Spearman and Pearson correlation coefficients were used for the correlation analyses of continuous variables.
RESULTS: The pure tone audiometry at 0.5, 1, 2, and 4 kHz was better in the case group than in the control group. A positive correlation was found between HbA1c and right ear 0.5, 1, 4, and 8 kHz threshold values and left ear 2, 4, 6, and 8 kHz threshold values. A negative correlation was found between HbA1c and speech discrimination scores. The right ear 1.00 and 2.83 kHz TEOAE measurements in the individuals with NGT were found higher than those in patients with IGT, and the 1.42 kHz TEOAE measurements and reproducibility were found higher than those in patients with IFG. The left ear 1.00 and 1.42 kHz TEOAE measurements of the IGT patients were found lower than those of IFG and NGT patients.
CONCLUSIONS: We showed that hearing was worsening in hyperinsulinemic patients and prediabetic conditions were related to hearing function impairment.

Bilateral sensorineural deafness and unilateral cochlear ossification have rarely been described in patients with chronic myeloid leukemia (CML). A 21-year-old man presented to a hospital with right-sided sudden hearing loss and tinnitus. He was diagnosed with CML. Five days later, sudden hearing loss appeared in the other ear. Abnormality of the right-sided inner ear structure was revealed by preoperative magnetic resonance imaging; honeycomb-like cochlear ossification was observed during cochlear implant surgery in the right ear. The patient\'s auditory performance exhibited significant improvement after bilateral cochlear implantation in our hospital. Hematological disorders must be considered in patients with sensorineural hearing loss. Cochlear implantation is feasible in patients with CML who exhibit sensorineural deafness, but cochlear ossification should be carefully evaluated by means of preoperative imaging examinations.