With advances in researching the mechanisms of hereditary hearing loss (HHL) and the development of genetic engineering technology, gene therapy demonstrates enormous potential treating HHL. Adeno-associated virus mediated gene therapy has restored hearing in multiple genetic mouse models of HHL. Notably, gene therapy targeting OTOF mutations has successfully advanced to clinical trials, improve the patient’s auditory and speech functions, marking a paradigm shift in the treatment of hearing loss. In this review, we introduce the typical classifications of HHL, the progress in gene delivery tools and major gene therapy strategies, and summarize the preclinical and clinical progress in different gene types.

Background: : This study investigated the heterogeneity of unilateral Meniere's disease (MD) by comparing the clinical characteristics and laboratory results between patients with and without endolymphatic hydrops (EH), analyze the correlation between the severity of hydrops and clinical manifestations, auditory, and vestibular function impairment.

Methods:: In this retrospective study, 95 patients diagnosed with unilateral MD underwent 3D-FLAIR MRI scanning 24 hours after intratympanic gadolinium injection. Vestibular hydrops was graded on MRI using a 4-grade scale. Pure-tone audiometry, air-conducted sound and galvanic vestibular stimulation for cervical and ocular vestibular evoked myogenic potentials (ACS-cVEMP, GVS-cVEMP, ACS-oVEMP, GVS-oVEMP), caloric testing, and video head impulse test (vHIT) were performed, and their correlations with EH were analyzed.

Results: : Significant differences were found between the non-hydrops and hydrops groups in mean pure-tone average (PTA) and ACS-cVEMP response rate (p = 0.020, p = 0.023, respectively). The severity of vestibular hydrops correlated with disease duration (p = 0.001), PTA (p = 0.001), ACS-cVEMP response rate (p = 0.022), and vHIT gain of the posterior semicircular canal (p = 0.037).

Conclusion: : This study confirms the important diagnostic value of MRI-visualized EH in MD. It also identifies MD patients without detectable hydrops, suggesting the necessity of a comprehensive diagnostic approach integrating symptoms, functional tests, and imaging characteristics.

Backgrounds: : Surgical treatment of pulsatile tinnitus (PT) caused by sigmoid sinus wall anomalies (SSWA) is increasingly reported, yet short-term outcomes and their fluctuations remain poorly documented.

Objective: : To map the time-course and determinants of PT extinction after complete transmastoid resurfacing of SSWA.

Methods: : Retrospective single-center cohort (July–October 2025) of 26 consecutive patients with SSWA and positive jugular-compression test. All underwent the “Inner-Sinodural-Outer Up-down” complete air cell removal method and bone cement resurfacing. PT severity (THI-PT), subjective tinnitus (THI-ST) and aural fullness (0–10 VAS) were recorded pre-op and weekly until sustained silence. Early postop CT documented fluid/cement distribution.

Results: : 96.2% (25/26) ultimately achieved complete PT abolition. Only 38.5% were silent at 24 h; 70% displayed residual PT that declined week-by-week in all subjects by week 4 (mean THI-PT from 54.6 to 0.8; p < 0.0001). One recurrence corresponded to focal inadequate cement on CT. Transient ear fullness occurred in 100% (mean VAS peak 7.7) and resolved in 88.5% within 4 weeks; 42.3% developed self-limiting subjective tinnitus that subsided by week 5. Crackling/autophony affected 69% but did not impact quality of life and was resolved after 6 weeks.

Conclusion: : Anatomically successful resurfacing yields 96.2% PT cure in the current series, yet immediate silence is the exception. Residual PT, fullness and subjective tinnitus are expected, self-limiting consequences of postoperative fluid, mucosal inflammation and altered central gain, reliably extinguished within 4–6 weeks.

Background: : Autologous rib cartilage grafts are the most commonly used approach in auricular reconstruction. However, the unpredictable absorption rate and deformation of the cartilage framework remain important clinical challenges.

Methods: : In order to explore the mechanism of cartilage absorption, we harvested the absorbed rib cartilage framework and native rib cartilage for histological and cytological evaluation from patients with unsatisfactory primary microtia reconstruction. Histological evaluation and inflammatory and ossification markers were investigated to identify pathological changes and heterotopic ossification level of absorbed framework, as to explore environmental factors that cause cell death. Furthermore, in order to observe the internal factors of cell damage, transmission electron microscopy analysis, cell proliferation, chondrogenic ability were used to evaluate ultrastructural changes and cell function, and RNA sequencing to measure the gene expression patterns.

Results: : Considerable evidence of inflammatory cell infiltration and chondrocyte disintegration in the region near fixation materials were observed in absorbed cartilage framework, indicating that fixation materials could trigger an inflammatory response and might cause cell death. Although, chondrocytes from cartilage framework showed robust proliferation rate and strong chondrogenic potential, ultrastructural evaluation showed significantly increased in number of mitochondria (might involve in oxidative stress damage), and the expression of proinflammatory and anti-inflammatory genes seemed to dysregulate responsiveness, which may ascribe to the death of chondrocyte.

Conclusion: : The current results indicated fixation materials could trigger obvious inflammatory response and induce cell death. In such an inflammatory environment, dysregulation of inflammation-related genes and alterations in mitochondrial function in chondrocyte from absorbed framework cartilage might account for the late stage of cell death and cartilage resorption.

Background: : Deeply embedded cervical fish bones that remain undetected on direct laryngoscopy present a significant diagnostic and therapeutic challenge. When initial open neck exploration fails to locate the foreign body, patients are at risk for complications such as fistula formation. This report describes a minimally invasive salvage technique for such refractory cases.

Case presentation: : A 58-year-old man presented with persistent throat pain six weeks after fish bone ingestion, following a failed open neck exploration at another hospital. Esophagography suggested a left piriform fossa fistula, and flexible laryngoscopy revealed no visible foreign body. Thin-slice computed tomography (CT) demonstrated a 27-mm linear foreign body embedded deep to the mucosa of the left pyriform sinus. Under general anesthesia, the patient underwent a second procedure combining laryngoscopy with real-time external ultrasound guidance. The fish bone was precisely localized as a hyperechoic structure and successfully retrieved using grasping forceps.

Results: : The patient experienced immediate symptom relief post-procedure. He was managed with a nasogastric tube and intravenous antibiotics. A follow-up esophagogram at four weeks confirmed complete healing of the piriform fossa fistula, allowing for tube removal. At the three-month follow-up, the patient remained asymptomatic and had resumed a normal diet.

Discussion: : This case highlights the diagnostic difficulty of foreign bodies embedded deep within the cervical submucosa, which can elude detection even during open exploration. Ultrasound-guided endoscopic retrieval offers distinct advantages in this setting, including real-time, radiation-free localization, superior soft tissue contrast, and minimal surgical trauma. This technique enabled targeted removal and subsequent healing of an associated fistula after conventional methods had failed.

Conclusion: : Ultrasound-guided endoscopic removal is an effective and minimally invasive salvage strategy for deeply embedded cervical fish bones when open exploration is unsuccessful. It should be considered a valuable alternative to repeat open surgery in select patients with favorable anatomy for ultrasound visualization.