Vestibular function in children with vestibulocochlear nerve aplasia/hypoplasia.

BACKGROUND: Infants and young children with vestibulocochlear nerve (VCN) hypoplasia/aplasia present with severe hearing loss and are candidates for cochlear implantation (CI). It is unknown whether vestibular function is related to CI outcome and if vestibular tests can guide the operation decision. AIMS/OBJECTIVES: Our aim was to describe the vestibular function in patients with VCN hypoplasia/aplasia before a possible CI. MATERIALS AND METHODS: Forty-two ears in 23 patients were tested between 2019 and 2022 with bone-conducted cervical vestibular evoked myogenic potentials (BCcVEMP), video head impulse test (vHIT) and miniice-water caloric test (mIWC). RESULTS: All ears could be tested with at least one vestibular test and 83% could be tested with more than one method. Twenty-nine ears (61%) showed normal function with at least one method. The presence of a normal response to any test doubled the likelihood of a measured hearing threshold after CI, the best predictors being the BCcVEMP and vHIT (p < 0.05). CONCLUSION: Canal function may represent a predictor of auditive pathway integrity with a possible favourable audiological outcome after CI operation. SIGNIFICANCE: Our results demonstrate high vestibular response rates suggesting a functioning pathway despite the radiological diagnosis.

Comorbidities and laboratory changes of sudden sensorineural hearing loss: a review.

Sudden sensorineural hearing loss (SSNHL) is defined as an abrupt hearing loss of more than 30 dB in three contiguous frequencies within 72 h. It is an emergency disease requiring immediate diagnosis and treatment. The incidence of SSNHL in Western countries' population is estimated between 5 and 20 per 1,00,000 inhabitants. The etiology of SSNHL remains unknown. Due to the uncertainty of the cause of SSNHL, at present, no specific treatment targets the cause of SSNHL, resulting in poor efficacy. Previous studies have reported that some comorbidities are risk factors for SSNHL, and some laboratory results may provide some clues for the etiology of SSNHL. Atherosclerosis, microthrombosis, inflammation, and the immune system may be the main etiological factors for SSNHL. This study confirms that SSNHL is a multifactorial disease. Some comorbidities, such as virus infections, are suggested to be the causes of SSNHL. In summary, by analyzing the etiology of SSNHL, more targeting treatments should be used to achieve a better effect.

Efficacy of intratympanic or postauricular subperiosteal corticosteroid injection combined with systemic corticosteroid in the treatment of sudden sensorineural hearing loss: A prospective randomized study.

Objectives: This study aimed to investigate the efficacy and safety of intratympanic or postauricular subperiosteal glucocorticoid injection combined with systemic glucocorticoid in the treatment of sudden sensorineural hearing loss (SSNHL). Methods: This study is a prospective randomized controlled study. This study included unilateral SSNHL patients who were hospitalized in our department between January 2020 and June 2021. Patients were randomly divided into three groups (groups A, B, and C). Patients in group A were treated with an intratympanic corticosteroid injection combined with systemic corticosteroid treatment, and patients in group B received a postauricular corticosteroid injection combined with systemic corticosteroid treatment. Patients in group C (control group) were treated with systemic corticosteroid alone. The case number of groups A, B, and C was 311, 375, and 369, respectively. Results: There was no significant difference in gender distribution, the proportion of left and right affected ears, and the average interval from onset to treatment among the three groups (P > 0.05). However, there were significant differences in their average age, distribution of audiogram type, and hearing loss levels among them (P < 0.01). Our study shows that there was no significant difference in average hearing threshold improvement before and after treatment in the three groups (P > 0.05). Regarding the complications, in group A, 33 patients (10.6%) had a transient vertigo attack during tympanic injection, which lasted for ~1-3 min. In group B, 20 patients (6.43%) complained of pain at the injection site, which disappeared after 1-3 days. No other complications occurred in all the other patients. Conclusion: The addition of intratympanic or postauricular corticosteroid to systemic steroids did not result in a significant effect on hearing recovery in SSNHL. No obvious complications occur in SSNHL patients treated with intratympanic injection or postauricular injection of corticosteroid. Clinical trial registration: [chictr.org.cn], registration number: ChiCTR2100048762.

Diagnosis, differential diagnosis, and treatment for sudden sensorineural hearing loss: Current otolaryngology practices in China.

Introduction: Although sudden sensorineural hearing loss (SSNHL) has been attempted to be understood for 70 years, diagnosis and treatment strategies still have strong heterogeneity worldwide, which are reflected in the guidelines issued by countries and the clinical practice of otolaryngologists. Methods: Questionnaires were sent to registered otolaryngologists nationwide via an online questionnaire system. We investigated the current views and clinical practices of otolaryngologists in mainland China about the diagnosis, examination, and treatment strategies of SSNHL. Results: Most otolaryngologists supported diagnostic classification via audiograms. Regional economic situation and hospital grade affected application strategies for differential diagnosis. Regarding corticosteroid therapy, 54.9% of respondents opted to discontinue the drug 5 days after systemic administration. Both intratympanic therapy and post-auricular injections were selected by more than half of the respondents as initial and salvage treatments. Discussion: Chinese otolaryngologists exhibit heterogeneity in clinical practices for SSNHL, including distinct approaches to combination therapy and local application of steroids. This study pointed out Chinese doctors' similarities, differences, and unique strategies in diagnosing and treating SSNHL and analyzed the possible reasons to help the world understand the current otolaryngology practices in China.

Etiological analysis of patients with sudden sensorineural hearing loss: a prospective case-control study.

Sudden sensorineural hearing loss (SSNHL) is a multifactorial emergency disease. Until now, the etiology of SSNHL is still unknown. Previous studies regarding the etiology of SSNHL are clinical studies depending on clinical data collection and analysis. Due to the insufficient sample size or various selective bias in clinical studies, the results of these studies may be inaccurate. This prospective case-control study aimed at exploring the possible etiology and risk factors of SSNHL. We enrolled 255 SSNHL patients and 255 sex-, age- and residence-matched non-SSNHL subjects in the control group. Our study shows that there was no significant difference in the prevalence of comorbidities including hypertension and diabetes, as well as the incidence of smoking and drinking habits between the case and control groups (P > 0.05). In addition, the peripheral blood white blood cell count, neutrophil count, platelet-to-lymphocyte ratio (PLR) and fibrinogen level of the case group were significantly higher than those in the control group (P < 0.05). These findings suggest smoking, drinking, hypertension and diabetes may not be related to the onset of SSNHL. However, hypercoagulable state and inner ear vascular microthrombosis related to an elevated fibrinogen level might be the risk factors of the disease. In addition, inflammation play an important role of SSNHL onset.Trial Registration: Chinese Clinical Trial Registry. Registration number: ChiCTR2100048991.

Effects of lesions of the organ of corti on hearing.

BACKGROUND: Lesions causing changes in the microstructure of the organ of Corti may lead to hearing impairment. AIMS/OBJECTIVES: The aim of this study was to investigate the effect of various structural lesions on the organ of Corti and the auditory function. METHODS: A finite element method of the cochlea and the organ of Corti were established based on computed tomography scanning and anatomical data. We evaluated the accuracy of the model by comparing the simulation results to reported experimental data. We simulated and analyzed the impact of the lesions on the sound-sensing function of the cochlea by adjusting the biomaterial parameters of each component of the cochlea. RESULTS: In the explored frequency range, the stereocilia and outer hair cells and basilar membrane sclerosis resulted in 23.4%, 47.2%, and 57.8% reduction of basilar membrane displacement, respectively. Lesions of the basilar membrane and stereocilia and outer hair cells in the Corti caused a hearing response curve shift to higher frequencies and a decrease of the amplitude of the basilar membrane. CONCLUSIONS AND SIGNIFICANCE: Lesions of the internal structure of the Corti cause diminished movement of basement membrane and decreased sensorial function, which ultimately lead to hearing loss.

The relationship between clinical characteristics and magnetic resonance imaging results of Ménière disease: a prospective study.

Ménière disease (MD) is an idiopathic inner ear disorder, and endolymphatic hydrops (EH) being considered to be its pathological basis. Currently, there is no gold standard for diagnosing MD. Previous study has reported visualized EH using MRI by intratympanic gadolinium-based contrast media (GBCM) administration (IT-Gd) in patients with MD, and this technique was gradually established for MD diagnosis. However, few studies reported their diagnostic sensitivity and specificity in clinical application. This prospective study aimed at investigating the clinical characteristics and magnetic resonance imaging (MRI) results of patients with MD, and analyzing the relationship between clinical results and MRI findings in MD patients. Our study shows that the diagnostic sensitivity and specificity of MRI were 79.2% and 80.7% respectively. Moreover, there was no significant correlation between hearing levels and cochlear grading scores, nor vestibular grading scores. The duration of disease was not significantly associated with cochlear or vestibular grading scores. These findings suggest that IT-Gd MRI offers reliable radiological diagnostic criteria for MD.

Methodological aspects of testing vestibular evoked myogenic potentials in infants at universal hearing screening program.

Motor development in infants is dependent upon the function of the inner ear balance organ (vestibular organ). Vestibular failure causes motor delays in early infancy and suboptimal motor skills later on. A vestibular test for newborns and infants that is applicable on a large scale, safe and cost effective is in demand in various contexts: in the differential diagnosis of early onset hearing loss to determine forms associated with vestibular failure; in early hearing habilitation with cochlear implant, indicating the vestibular predominant side; and in the habilitation of children affected by motor skill disorders, revealing the contribution of a vestibular failure. This work explored the feasibility of cervical vestibular evoked myogenic potentials (VEMP) in conjunction with newborn universal hearing screening program. VEMP was measured after the hearing tests and was evoked by bone-conducted stimuli. Moreover, stimulus delivery was regulated by neck muscle activity, with infants rested unconstrained in their parents´ arms and with the head supported by the operator´s hand. This VEMP protocol showed a high level of feasibility in terms of test viability and result reproducibility. VEMP integrated into the newborn hearing screening program may represent a practical method for large-scale assessment of balance function in infants.