The expression and significance of Epac1 and Epac2 in the inner ear of guinea pigs.

OBJECTIVE: To detect the expression of Epac1 and Epac2 in the inner ear of guinea pigs and its association with microcirculation in the inner ear. METHODS: The temporal bones of 30 healthy red-eye guinea pigs (60 ears) weighing 200-350 g were collected, then the surrounding bone wall of the cochlea was removed under a dissection microscope. Real-time quantitative PCR (RT-qPCR) and Western blot were used to detect mRNA and protein expression, respectively, of Epac1 and Epac2 in the inner ear and to compare their expression in heart, liver, kidney, intestine, and lung tissues. The specimens of the cochlea included the stria vascularis, basilar membrane, saccule, and utricles isolated under a microscope to detect the localization of Epac1 and Epac2 proteins in various parts of the inner ear through immunofluorescence staining. RESULTS: The RT-qPCR and Western blot results showed that Epac1 mRNA was universally expressed in the inner ear, heart, liver, kidneys, intestines, and lungs, and was highly expressed in the liver, kidneys, and intestines (p < 0.05 vs heart, liver, kidney, intestine; p > 0.05 vs lung). Epac2 mRNA was expressed in the inner ear and heart, but not in the liver, kidneys, intestines, or lungs (p < 0.05 vs Heart). Epac1 and Epac2 proteins were both expressed in the inner ear, heart, liver, kidneys, intestines, and lungs. The relative expression of Epac1 proteins in the inner ear was significantly different from the liver, kidneys, intestines, and lungs (p < 0.05). The relative expression of Epac2 proteins in the inner ear was significantly different from the liver, kidneys, and lungs (p < 0.05), but not from the heart (p = 0.127) or intestines (p = 0.274). Immunofluorescence staining observed under confocal microscopy indicated that Epac1 and Epac2 proteins were expressed in the stria vascularis, basilar membrane, saccule, and utricles of the inner ear. They were expressed in maginal cells, intermediate cells, and basal cells of the stria vascularis, and highly expressed in capillary endothelial cells. CONCLUSIONS: Epac1 and Epac2 mRNA and proteins were both expressed in the inner ear of guinea pigs and evenly expressed in the spiral ganglion, basilar membrane, saccule, and utricles. However, their expression in capillary endothelial cells of the stria vascularis was more obvious, suggesting that cyclic adenosine monophosphate-Epac1 signaling may play an important role in maintaining the function of the blood-labyrinth barrier and regulating the stability of microcirculation in the inner ear.

Presentation of dizziness in individuals with chronic otitis media: data from the multinational collaborative COMQ-12 study.

PURPOSE: In chronic otitis media (COM), disease chronicity and severity of middle ear inflammation may influence the development of inner ear deficits, increasing the risk of vestibular impairment. This secondary analysis of the multinational collaborative Chronic Otitis Media Questionnaire-12 (COMQ-12) dataset sought to determine the prevalence of vestibular symptoms in patients with COM and identify associated disease-related characteristics. METHODS: Adult patients with a diagnosis of COM in outpatient settings at nine otology referral centers across eight countries were included. We investigated the presence of vestibular symptoms (dizziness and/or disequilibrium) using participant responses to item 6 of a native version of the COMQ-12. Audiometric data and otoscopic assessment were also recorded. RESULTS: This analysis included 477 participants suffering from COM, with 56.2% (n = 268) reporting at least mild inconvenience related to dizziness or disequilibrium. There was a significant association between air conduction thresholds in the worse hearing ear and presence of dizziness [adjusted odds ratio (AOR), 1.01; 95% CI 1.00-1.02; p = 0.0177]. Study participants in European countries (AOR 1.53; 95% CI 1.03-2.28; p = 0.0344) and Colombia (AOR 2.48; 95% CI 1.25-4.92; p = 0.0096) were more likely to report dizziness than participants in Asian countries. However, ear discharge and cholesteatoma showed no association with dizziness in the adjusted analyses. CONCLUSION: Vestibular symptoms contribute to burden of disease in patients with COM and associates with hearing disability in the worse hearing ear. Geographical variation in presentation of dizziness may reflect financial barriers to treatment or cultural differences in how patients reflect on their health state.

Hair cell uptake of gentamicin in the developing mouse utricle.

Intratympanic injection of gentamicin has proven to be an effective therapy for intractable vestibular dysfunction. However, most studies to date have focused on the cochlea, so little is known about the distribution and uptake of gentamicin by the counterpart of the auditory system, specifically vestibular hair cells (HCs). Here, with a combination of in vivo and in vitro approaches, we used a gentamicin-Texas Red (GTTR) conjugate to investigate the mechanisms of gentamicin vestibulotoxicity in the developing mammalian utricular HCs. In vivo, GTTR fluorescence was concentrated in the apical cytoplasm and the cellular membrane of neonatal utricular HCs, but scarce in the nucleus of HCs and supporting cells. Quantitative analysis showed the GTTR uptake by striolar HCs was significantly higher than that in the extrastriola. In addition, the GTTR fluorescence intensity in the striola was increased gradually from 1 to 8 days, peaking at 8-9 days postnatally. In vitro, utricle explants were incubated with GTTR and candidate uptake conduits, including mechanotransduction (MET) channels and endocytosis in the HC, were inhibited separately. GTTR uptake by HCs could be inhibited by quinine, a blocker of MET channels, under both normal and stressed conditions. Meanwhile, endocytic inhibition only reduced GTTR uptake in the CoCl2 hypoxia model. In sum, the maturation of MET channels mediated uptake of GTTR into vestibular HCs. Under stressed conditions, MET channels play a pronounced role, manifested by channel-dependent stress enhanced GTTR permeation, while endocytosis participates in GTTR entry in a more selective manner.

The preparation of dexamethasone sodium phosphate multivesicular liposomes thermosensative hydrogel and its impact on noise-induced hearing loss in the Guinea pigs.

OBJECTIVE: The aim of this study is to establish the dexamethasone sodium phosphate multivesicular liposomes thermosensative hydrogel (DEX-MVLs-Gel) drug delivery system and to analyze the pharmacodynamics, pharmacokinetics and safety of DEX-MVLs-Gel as well as to explore whether the prepared DEX-MVLs-Gel can protect the hearing in the guinea pigs following noise exposure. METHODS: DEX-MVLs formulations were constructed by double emulsion method, and the DEX-MVLs-Gel was prepared after adding P407 and P188 into the DEX-MVLs. A total of 20 adult albino guinea pigs were chosen to establish the animal models with noise-induced hearing loss. After animals were treated with DEX-MVLs-Gel at concentrations of 20, 6 and 2 mg/mL, and 5 mg/mL Dexamethasone Sodium Phosphate (DEX-P) solution, respectively, the hearing function, drug concentration in the peripheral lymph fluid, and hair cell morphology were assessed. RESULTS: The ABR threshold of the 20 mg/mL DEX-MVLs-Gel treated group at the frequencies of 4, 8, 16 and 24 kHz were measured as 47.5 ± 5.2, 48.3 ± 4.1, 55.8 ± 3.8 and 57.5 5 ± 5.2 dB SPL, respectively. Statistical significances were noted between the 20 mg/mL DEX-MVLs-Gel treated group and control group at each frequency (all P < 0.05), between the 2 mg/mL and 6 mg/mL DEX-MVLs-Gel treated groups at the frequencies of 4 and 8 kHz (both P < 0.05). High Performance Liquid Chromatography (HPLC) demonstrated that the drug concentrations in the peripheral lymph in all groups were gradually decreased on the 1st, 3rd and 7th d after intratympanic injection. Scattered hair cell loss could be observed mainly in the basal and middle turn in the saline administrated group and the 20 mg/mL DEX-MVLs-Gel administration group, and the hair cell loss was not identified in the apical turn. CONCLUSIONS: A high concentration (20 mg/mL) of DEX-MVLs-Gel exerts significant protective effects upon the guinea pigs with noise-induced hearing loss. The prepared DEX-MVLs-Gel can be effectively maintained in the peripheral lymph fluid of guinea pigs for 3-7 d and MVLs-Gel causes no obvious ototoxicity.

Grading system and surgical approaches for endolymphatic sac tumors.

PURPOSE: Endolymphatic sac tumors (ELSTs) are rare, low-grade adenocarcinomas arising from the endolymphatic sac. This study aims to present a novel grading system for ELSTs to determine the optimal management strategy. METHODS: We performed a retrospective analysis of 16 patients with 17 ELSTs. The tumor location and involved adjacent neurovascular structures on CT and MRI were selected to establish the grading system. RESULTS: Based on the novel grading system, grade III a tumors were most common (7/17), followed by grade I (4/17), grade II (3/17), and grade III b (3/17) tumors. Eight advanced ELSTs (grade III a and III b) received an infra-temporal fossa approach, while the other 6 early stage ELSTs (grade I and II) underwent either a retrolabyrinthine approach with posterior petrosectomy or a translabyrinthine approach combined with subtotal temporal bone resection. Hearing preservation was achieved in 2 grade I patients. Postoperative facial nerve function was HB II in 1 grade III a patient who underwent anterior facial nerve transposition and was HB III in 4 advanced patients who received facial nerve grafts with the great auricular nerve or facial-hypoglossal nerve anastomosis. The mean follow-up time was 35.1 months. Two grade III patients and 1 grade II patient had tumor recurrence during follow-up, among whom 1 grade III b patient had two cases of recurrence. CONCLUSION: A correct initial diagnosis was established in all patients after meticulous imaging studies. Surgical resection is still the first choice to manage patients with ELSTs. The novel grading system enables surgeons to select tailored surgical approaches. Long-term follow-up is necessary following surgical intervention.

Clinical features and management of Meniere's disease patients with drop attacks.

PURPOSE: The aims of the present study are to investigate the variations in clinical features, including medical history, hearing function, vestibular function, and degree of endolymphatic hydrops (EH), in Meniere's disease (MD) patients with and without drop attacks (DAs), and to examine the efficacy of intratympanic gentamicin (ITG) treatment in alleviating DAs. METHODS: In total, 177 unilateral definite MD patients, including 16 patients with DAs and 161 patients without DAs, were enrolled. The results of hearing test, vestibular-evoked myogenic potentials (VEMPs), and magnetic resonance imaging (MRI) were analyzed. Thirteen patients with DAs received a single ITG treatment and were followed up. RESULTS: The disease course of MD in the DA group was significantly longer than that in the control group (p = 0.007). MD patients with DAs had significantly greater hearing loss and worse EH than MD patients without DAs (p < 0.05). However, there was no between-group difference in vestibular function. In the study, 92.31% of refractory definite MD patients with DAs achieved satisfactory control of DAs after ITG treatment. CONCLUSIONS: MD patients with DAs tend to suffer from severe hearing loss and a significant degree of EH in the inner ear. However, the vestibular function of MD patients with DAs may not be completely abolished, but be sensitive to stimulating signals. ITG treatment, which helps to decrease vestibular sensitivity, was an effective treatment to control DAs.

A retrospective study on facial nerve schwannomas: a disease with a high risk of misdiagnosis and hearing loss.

The objective is to increase awareness of facial nerve schwannomas (FNSs). Clinical data from 32 cases with FNSs who received surgical treatment from 2005 to 2015 were reviewed retrospectively. The clinical data included age, sex, presentations, duration, facial nerve function, temporal-bone high-resolution computed tomography (CT) and magnetic resonance imaging (MRI) findings, surgical approaches, and postoperative histopathological examination. 16 men and 16 women were included, aged 7-69 years. The average age at diagnosis was approximately 44 years. The mean duration of disease was 65 months, and the mean tumor diameter was 22.4 mm. A tendency of multisegment involvement was observed in 29 FNS cases. Geniculate ganglion and tympanic segments were the most commonly involved segments. Meanwhile, the incidence of misdiagnosis of this disease was 50%. We observed that when FNSs involved the proximal portion of genicular ganglion, the hearing function tended to be worse than when the FNSs only involved the genicular ganglion and/or its distal portion (p < 0.05); in such cases, the hearing loss tended to become more severe with a longer duration of the disorder (p < 0.05). Multiple segment involvement is common in patients with FNS. We need to be more aware of the hearing function when FNSs involve the proximal portion of genicular ganglion. Misdiagnoses of FNS are common, and patients can be misdiagnosed with Bell's palsy, otitis media, or other diseases. Image studies should be conducted for differential diagnosis. Once the decision to perform surgical resection was made, reconstruction of the facial nerve should be considered.

Cochlear implant-related three-dimensional characteristics determined by micro-computed tomography reconstruction.

To explore the structural characteristics of the cochlea in three-dimensional (3D) detail using 3D micro-computed tomography (mCT) image reconstruction of the osseous labyrinth, with the aim of improving the structural design of electrodes, the selection of stimulation sites, and the effectiveness of cochlear implantation. Three temporal bones were selected from among adult donors' temporal bone specimens. A micro-CT apparatus (GE eXplore) was used to scan three specimens with a voxel resolution of 45 µm. We obtained about 460 slices/specimen, which produced abundant data. The osseous labyrinth images of three specimens were reconstructed from mCT. The cochlea and its spiral characteristics were measured precisely using Able Software 3D-DOCTOR. The 3D images of the osseous labyrinth, including the cochlea, vestibule, and semicircular canals, were reconstructed. The 3D models of the cochlea showed the spatial relationships and surface structural characteristics. Quantitative data concerning the cochlea and its spiral structural characteristics were analyzed with regard to cochlear implantation. The 3D reconstruction of mCT images clearly displayed the detailed spiral structural characteristics of the osseous labyrinth. Quantitative data regarding the cochlea and its spiral structural characteristics could help to improve electrode structural design, signal processing, and the effectiveness of cochlear implantation. Clin. Anat. 30:39-43, 2017. © 2016 Wiley Periodicals, Inc.

Factors impacting early cochlear implantation in Chinese children.

This study aimed to determine the factors that impact early cochlear implantation (CI) in Chinese children. The charts of 56 pediatric cochlear implant recipients were retrospectively reviewed. Parental interviews were conducted to collect information regarding the factors that potentially impact early CI in Chinese children. The mean age at CI was 40.98 months, and the median age was 24 months. Thirty patients received an implant before 24 months of age, and the other 26 patients received the implant after 24 months of age. By univariate logistic regression analysis, newborn hearing screening (NHS), financial burden impacted early CI significantly (p < 0.05), while the multivariate analysis indicated that only NHS significantly influenced the early CI (p < 0.05). The following factors were associated with a moderately increased risk of late implantation: lack of NHS [odds ratio (OR) = 5.833], living in a rural community (OR = 2.714), and communication barriers (OR = 2.691). Living in a rural community, financial burden and communication barriers negatively impact the age at CI, whereas NHS positively influence the age at CI. Increased awareness of these factors could promote early CI and improve the quality of life of congenitally deaf children.

Temporal bone malignancies involving the jugular foramen: diagnosis and management.

PURPOSE: To describe the diagnosis, management and outcomes of temporal bone malignancies involving the jugular foramen. MATERIALS AND METHODS: The charts of 12 patients with temporal bone malignancies involving the jugular foramen were reviewed and analyzed. RESULTS: The most frequent symptoms of temporal bone malignancies involving the jugular foramen were hearing loss (75%), otorrhea (66.67%) and otalgia (66.67%). In addition to the jugular foramen, the most invaded structure was the external auditory canal (91.67%), followed by the middle ear (75%) and facial nerve (58.33%). Four cases (33.33%) were misdiagnosed either with glomus jugulare tumor, Bell's palsy or chronic otitis media. Two patients underwent lateral temporal bone resection, whereas 10 patients underwent subtotal temporal bone resection. The mean follow-up time was 23.83 months (range, 2-85 months). The 2-year disease-free survival rate was 42.3%. CONCLUSIONS: Temporal bone malignancies involving the jugular foramen had a poor prognosis, especially in patients with squamous cell carcinoma. Misdiagnoses including glomus jugulare tumor, Bell's palsy and chronic otitis media were frequently encountered. Temporal bone malignancies involving the jugular foramen can be resected, and the choice of the surgical approach should be individualized.

Thermosensitive hydrogel containing ethosuximide-loaded multivesicular liposomes attenuates age-related hearing loss in C57BL/6J mice.

Ethosuximide is the first drug reported to protect against age-related hearing loss, but its benefits are hampered by the pronounced side effects generated through systemic administration. We prepared a thermosensitive hydrogel containing ethosuximide-encapsulated multivesicular liposomes (ethosuximide-loaded MVLs-Gel) and evaluated its functional and histological effects on age-related hearing loss in C57BL/6J mice. The MVLs-Gel showed slow sustained-release characteristics up to over 120 h. After 8 weeks of treatment, compared to the oral systemic administration of ethosuximide, intratympanic ethosuximide-loaded MVLs-Gel injection dramatically reduced the loss of age-related spiral ganglion neurons in the apical turns of the mice (low-frequency regions, p < 0.05). Correspondingly, compared to the oral systemic administration group, the intratympanic ethosuximide-loaded MVLs-Gel injection group showed significantly lower auditory brainstem response threshold shifts at stimulus frequencies of 4, 8, and 16 kHz (low-and middle-frequency regions, p < 0.05). In conclusion, intratympanic ethosuximide-loaded MVLs-Gel injection can reach the apical turn of the cochlea, which is extremely difficult with oral systemic administration of the drug. The ethosuximide-loaded MVLs-Gel, as a novel intratympanic sustained-release drug delivery system, attenuated age-related hearing loss in C57BL/6J mice.

An Improved Staging System of Adenoid Cystic Carcinoma in the External Auditory Canal.

OBJECTIVE: The purpose of this study was to define an improved staging system for adenoid cystic carcinoma (ACC) in the external auditory canal (EAC) based on biological behaviors, image findings, and the prognosis of patients with ACC in the EAC. STUDY DESIGN: A retrospective study. SETTING: A single center data. METHODS: We performed a single-institution retrospective review of 154 patients with ACC in the EAC between January 2004 and September 2021. Risk factors associated with disease-free survival (DFS) and cancer-specific survival (CSS) of ACC in the EAC were identified using univariate and multivariate cox regression analysis. Then an improved staging system was proposed and compared with the Pittsburgh-modified tumor, node, and metastasis (TNM) staging system for statistical differences in DFS and CSS. RESULTS: An improved staging system of ACC in the EAC was defined, in which stage T4 were subclassified into T4a and T4b and were statistically different from the Pittsburgh-modified TNM staging system in DFS and CSS. We also found that the dura mater, facial nerve, sigmoid sinus, deep lobe of parotid gland, and parapharyngeal space involvement were significantly associated with poor prognosis of ACC in the EAC. CONCLUSION: The improved staging system is more accurate in predicting survival prognosis than Pittsburgh-modified TNM staging system for patients with ACC in the EAC, and may provide more efficient guidance of treatment strategy. SUMMARY: The improved staging system of ACC in the EAC is more accurately to predict survival prognosis, and provide guidance of treatment plan than Pittsburgh-modified TNM staging system.

A Novel Classification System and Surgical Strategies of First Branchial Cleft Anomalies.

OBJECTIVE: To define a novel classification of first branchial cleft anomalies (FBCAs) based on the relationship between lesions and the facial nerve in terms of radiographic imaging findings and to introduce the corresponding surgical managements. METHODS: The clinical data were retrospectively reviewed. Novel classification was proposed according to the head-neck MRI findings and surgical records. FBCAs limited in the cartilaginous segment of external auditory canal (EAC) or superficial parotid gland capsule were classified as type A. Lesions close to the FN and(or) involved into the parotid gland with no scar formation and no previous parotidectomy were classified as type B. FBCAs adhered to the FN and(or) invaded the parotid gland with scar formation due to previous surgery were classified as type C. Appropriate surgery approaches was also described, which was correlated with classification. RESULTS: Fifty-one patients were included, and one patient suffered from bilateral lesions. Thirty-one, twelve, and nine lesions were classified as type A, type B, and type C FBCAs, respectively. One type A patient (1.92%) suffered from recurrence during follow-up. Temporary facial palsy (House-Brackmann II) was identified in 2 type C patients (3.85%) after surgery and recovered to normal within 2 months. One type B patient (1.92%) suffered from facial paralysis due to the FN injury and underwent facial nerve graft simultaneously. No patients with type C complained of hearing loss postoperatively. CONCLUSION: This novel classification clearly illustrates definitely relationship between lesion and the facial nerve and appropriate surgical strategies were proposed based on the novel classification. LEVEL OF EVIDENCE: 4 Laryngoscope, 2024.

TMErisk score: A tumor microenvironment-based model for predicting prognosis and immunotherapy in patients with head and neck squamous cell carcinoma.

Tumor microenvironment (TME) is closely associated with the progression and prognosis of head and neck squamous cell carcinoma (HNSCC). To investigate potential biomarkers for predicting therapeutic outcomes in HNSCC, we analyzed the immune and stromal status of HNSCC based on the genes associated with TME using the ESTIMATE algorithm. Immune and stromal genes were identified with differential gene expression and weighted gene co-expression network analysis (WGCNA). From these genes, 118 were initially selected through Cox univariate regression and then further input into least absolute shrinkage and selection operator (LASSO) regression analysis. As a result, 11 genes were screened out for the TME-related risk (TMErisk) score model which presented promising overall survival predictive potential. The TMErisk score was negatively associated with immune and stromal scores but positively associated with tumor purity. Individuals with high TMErisk scores exhibited decreased expression of most immune checkpoints and all human leukocyte antigen family genes, and reduced abundance of infiltrating immune cells. Divergent genes were mutated in HNSCC. In both high and low TMErisk score groups, the tumor protein P53 exhibited the highest mutation frequency. A higher TMErisk score was found to be associated with reduced overall survival probability and worse outcomes of immunotherapy. Therefore, the TMErisk score could serve as a valuable model for the outcome prediction of HNSCC in clinic.

The Role of Parotid Gland Invasion in Adenoid Cystic Carcinoma of the External Auditory Canal.

OBJECTIVE: This study aimed to investigate the significance of parotid gland invasion in predicting distant metastasis of adenoid cystic carcinoma in the external auditory canal. STUDY DESIGN: Single-institution retrospective cohort study. METHODS: A retrospective review of patients with adenoid cystic carcinoma of the external auditory canal who underwent surgery was performed. Information on patient demographics, parotid gland invasion, tumor stage, perineural invasion, lymphovascular invasion, and follow-up data were collected and analyzed. RESULTS: One hundred twenty-nine patients were identified for review. Parotid gland invasion was noted in 45 patients (34.9%). Parotid gland invasion was significantly associated with tumor stage, perineural invasion, distant metastasis, and postoperative adjuvant therapy. Distant metastasis was noted in 30 patients (23.3%). Multivariate Cox proportional hazards analysis identified parotid gland invasion as an independent risk factor for predicting distant metastasis. The 5-year distant metastasis-free survival rate was 83.6% for patients without parotid gland invasion and 61.8% for patients with parotid gland invasion (p = 0.010). CONCLUSIONS: The parotid gland invasion rate is relatively high in adenoid cystic carcinoma of the external auditory canal and is significantly related to tumor stage. Parotid gland invasion is associated with worse distant metastasis-free survival. LEVEL OF EVIDENCE: 4 Laryngoscope, 134:419-425, 2024.

Preclinical and clinical studies of unrelieved aural fullness following intratympanic gentamicin injection in patients with intractable Ménière's disease.

OBJECTIVE: To clarify whether gentamicin affects vestibular dark cells in guinea pigs and relieves patients of aural fullness with intractable Ménière's disease following intratympanic administration. MATERIALS AND METHODS: Purified gentamicin-Texas Red (GTTR) was injected intratympanically in guinea pigs that were sacrificed at 1, 3, 7, 14 and 28 days. GTTR uptake was examined in hair cells, and transitional cells and dark cells in vestibular end-organs were examined. Specific attention was paid to its distribution in dark cells under confocal microscopy, and the ultrastructure of dark cells using electron microscopy, following intratympanic injection. RESULTS: Dark cells in the semicircular canals showed weak GTTR uptake at 1, 3, 7, 14 and 28 days after intratympanic injection, with no significant differences at various time points after injection. However, the adjacent transitional cells demonstrated intense GTTR uptake that was retained for at least 28 days. Ultrastructural studies demonstrated negligible characteristics associated with apoptosis or necrosis in these dark cells. The tight junctions between dark cells showed no signs of disruption at 7 or 28 days after injection. CONCLUSION: Intratympanic gentamicin has little direct impact on vestibular dark cells. CLINICAL APPLICATION: A modified low-dose titration intratympanic approach was used in 29 patients with intractable vertigo and the clinical outcomes were followed. Aural fullness following intratympanic gentamicin injection was not relieved based on our subjective scales, demonstrated by no statistically significant difference between preinjection (4.16 ± 3.08) and postinjection (3.58 ± 2.93; p > 0.05) aural fullness scores. Vertigo control was achieved in 88% of patients, with hearing deterioration identified in 16% of patients. Intratympanic gentamicin administration might not lead to relief of aural fullness in patients with intractable vertigo, although it can achieve a high vertigo control rate with some cochleotoxicity.

Surgical outcomes of 43 cases with adenoid cystic carcinoma of the external auditory canal.

PURPOSE: To evaluate surgical outcomes for adenoid cystic carcinoma (ACC) of the external auditory canal (EAC). METHODS: Forty-three patients with ACC of the EAC in Eye and ENT Hospital of Fudan University were analyzed retrospectively for survival. The patients were staged according to the modified Pittsburgh staging system. Thirteen patients with T1 stage underwent local resection (LR), 6 patients with T1 stage underwent lateral temporal bone resection (LTBR), and 8 patients with T1 stage underwent LTBR including superficial parotidectomy (SP). Two patients with T2 stage underwent LTBR, and 1 patient with T2 stage underwent LTBR+SP. Three patients with T3 stage underwent LTBR. One patient with T4 stage underwent LTBR, two patients with T4 stage underwent subtotal temporal bone resection (STBR), and 7 patients with T4 stage underwent LTBR+SP. RESULTS: Of all patients that underwent surgery, 13 died of their primary cancers during the follow-up time. The 5-year survival rates of patients with T stages 1 through 4 were 85%, 67%, 67%, and 30%, respectively. There was statistically significant difference in 5-year survival rate between T1 and other stages (T2, T3, T4) using the log-rank test (p<0.05). There was significant difference in 5-year survival rate between T4 and other stages using the log-rank test (p<0.05). The 5-year survival rates after LR, LTBR or LTBR plus SP for T1 were 77%, 87% and 100%, respectively. The 5-year survival rates after LTBR, STBR or LTBR plus SP for T4 were 0%, 50% and 29%, respectively. The 5-year survival rates for 19 patients with clear surgical margins and 24 patients with positive margins were 89% and 54%, respectively. The 5-year survival rates of patients with radiotherapy and without radiotherapy were 62% and 86%, respectively. CONCLUSION: An en bloc resection including superficial parotidectomy is favored in an effort to produce negative surgical margins for ACC of the EAC. Adjunctive radiotherapy is used for patients with positive margins and in advanced lesions.

The misdiagnosis of external auditory canal carcinoma.

External auditory canal (EAC) carcinomas are frequently misdiagnosed. The aim of this study was to conduct a review of misdiagnosed cases and analyze the factors involved. This study was a retrospective assessment. Eighteen of 44 EAC carcinoma cases seen at the Eye and ENT Hospital were misdiagnosed. All medical records were retrospectively analyzed for the age, sex, presenting symptoms, type of misdiagnosis, computed tomographic (CT) or magnetic resonance imaging (MRI) findings, stage of the cancer, surgical approach, histopathological examination, adjunctive therapy (postoperative radiotherapy) and outcomes of treatment. Six cases were misdiagnosed as otitis media, five cases were misdiagnosed as otitis externa, and two cases were misdiagnosed as external auditory canal cholesteatomas. Other misdiagnoses were stenosis of the EAC, ear neuralgia, furuncle of the EAC, benign neoplasm of the EAC and pre-auricular fistula. Our analyses suggest that a biopsy should be conducted to obtain a histopathological diagnosis if an EAC carcinoma is suspected, in case otitis media or otitis externa does not respond to routine anti-bacterials. Head and neck MRI should be used to explore the involvement of soft tissues. Patients with bloody ear discharge and otalgia, particularly with temporal bone erosion seen in a CT scan, are highly likely to have a malignant carcinoma of the temporal bone.

[A case report of middle ear cholesteatoma complicated with labyrinthine fistulaand delayed endolymphatic hydrops].

Delayed endolymphatic hydrops （DEH） is a rare disease that causes vertigo and is often misdiagnosed as other vertigo diseases. This article reports on a patient with vertigo who was easily misdiagnosed. The patient was a middle ear cholesteatoma complicated by labyrinthine fistula （LF）; however, his vertigo was episodic vertigo, which could not be explained solely by LF causing labyrinthitis. The possibility of endolymphatic hydrops was suspected, which was confirmed by inner ear magnetic resonance gadolinium imaging. This is the first reported case of middle ear cholesteatoma complicated by LF and DEH. The patient underwent surgical resection of the cholesteatoma and three semicircular canal obstructions at the same time. During two years postoperative follow-up, the patient did not experience a recurrence of vertigo. When diagnosing vertigo diseases, a careful history of vertigo is of utmost importance.