A 27-year-old female patient suffering endolymphatic sac tumor with intralabyrinthine hemorrhage was reported. The patient had hearing loss in the left ear with continuous tinnitus, and MRI showed the soft tissue shadow of endolymphatic sac. Considering that the tumor involved semicircular canal and vestibule,endolymphatic cyst tumor resection was performed by labyrinth route. After surgery, there was no cerebrospinal fluid leakage and facial nerve function was normal. More importantly, enhanced MRI of temporal bone showed no tumor recurrence 1 year after surgery.
Female ; Humans ; Adult ; Endolymphatic Sac/surgery* ; Neoplasm Recurrence, Local/pathology* ; Labyrinth Diseases ; Tinnitus ; Ear Neoplasms/pathology* ; Bone Neoplasms ; Hemorrhage

Necrotizing otitis externa is a progressive infectious disease involving the external auditory canal and even the skull base, which can lead to serious complications and even death if not treated in time. In this paper, the latest advances in etiology, pathogenesis, clinical manifestations, diagnosis and treatment were reviewed based on previous literature, providing reference for clinical diagnosis, treatment and future research.
Humans ; Otitis Externa/therapy* ; Skull Base/pathology* ; Ear Canal/pathology* ; Head

Humans ; Scrub Typhus ; Ear/pathology*

Humans ; Carcinoma, Squamous Cell/pathology* ; Chromosomal Proteins, Non-Histone/genetics* ; Ear, Middle/pathology* ; Poly-ADP-Ribose Binding Proteins ; Nuclear Proteins

Ear/pathology* ; Ear Diseases/therapy* ; Ear, Middle ; Humans

Objective: To collect the clinical cases of middle ear hairy polyp, and to summarize the imaging features. Methods: We retrospectively analyzed the clinical data of four cases middle ear hairy polyp confirmed by surgical and pathologic between January 2007 and January 2020 at the Affiliated Eye & ENT Hospital of Fudan University. There were three females, one male, with two left ears and two right ears, aged from 1 to 59 years. The CT and MRI imaging of the patients, and the corresponding clinical manifestations were analyzed. Results: Hairy polyps originated from tympanum in one case, originated from Eustachian tube in two cases, exhibiting recurrent otorrhea without evident inducement. The other case, hairy polyps originated from the Eustachian tube pharyngeal orifice and protruded into the nasopharyngeal cavity, with pharynx discomfort and aural fullness, endoscope showed offwhite polypoid mass with a little hair. All the four cases presented polypoid soft tissue masses on CT and MRI imaging, containing soft tissue wall and a large amount of adipose tissue, with soft tissue in the center of the mass which liked the core, and enhanced. MRI showed stratified arrangement of fat and soft tissue in the wall of the mass. Four cases all had surgical treatment, postoperative pathology examination presented that hair follicles, mature sebaceous glands and other skin appendages were found under squamous epithelium. A large amount of adipose tissue, part of muscle tissue, cartilage tissue, and some fibro-collagenous tissue were proliferated in the mass, accompanied by collagen degeneration. Conclusion: The middle ear hairy polyps has imaging characteristics, the polypoid soft tissue mass usually looks smooth and contains a large amount of adipose tissue, with a soft tissue in the center, and can be suggestively diagnosed by CT and MRI.
Adolescent ; Adult ; Child ; Child, Preschool ; Ear Diseases ; Eustachian Tube/pathology* ; Female ; Humans ; Infant ; Male ; Middle Aged ; Nasopharynx/pathology* ; Polyps/pathology* ; Retrospective Studies ; Young Adult

Child ; Choristoma ; pathology ; surgery ; Ear, Middle ; pathology ; surgery ; Female ; Hamartoma ; pathology ; surgery ; Humans ; Pharyngeal Neoplasms ; pathology ; surgery ; Salivary Glands ; pathology ; surgery

In order to study the influence of tympanic membrane lesion and ossicular erosion caused by otitis media on the hearing compensation performance of round-window stimulation, a human ear finite element model including cochlear asymmetric structure was established by computed tomography (CT) technique and reverse engineering technique. The reliability of the model was verified by comparing with the published experimental data. Based on this model, the tympanic membrane lesion and ossicular erosion caused by otitis media were simulated by changing the corresponding tissue structure. Besides, these simulated diseases' effects on the round-window stimulation were studied by comparing the corresponding basilar-membrane's displacement at the frequency-dependent characteristic position. The results show that the thickening and the hardening of the tympanic membrane mainly deteriorated the hearing compensation performance of round-window stimulation in the low frequency; tympanic membrane perforation and the minor erosion of ossicle with ossicular chain connected slightly effected the hearing compensation performance of round-window stimulation. Whereas, different from the influence of the aforementioned lesions, the ossicular erosion involving the ossicular chain detachment increased its influence on performance of round-window stimulation at the low frequency. Therefore, the effect of otitis media on the hearing compensation performance of round-window stimulation should be considered comprehensively when designing its actuator, especially the low-frequency deterioration caused by the thickening and the hardening of the tympanic membrane; the actuator's low-frequency output should be enhanced accordingly to ensure its postoperative hearing compensation performance.
Acoustic Stimulation ; Ear Ossicles ; pathology ; Finite Element Analysis ; Hearing ; Humans ; Otitis Media ; physiopathology ; Reproducibility of Results ; Round Window, Ear ; physiology ; Tomography, X-Ray Computed ; Tympanic Membrane Perforation

OBJECTIVES: To investigate the impact of labyrinthectomy and intratympanic (IT) gentamicin injections on the contralateral labyrinth, we also assessed the response of each individual semicircular canal to each IT gentamicin application. METHODS: We performed a pilot observational study on tertiary, referral, academic settings. Thirteen patients with unilateral vestibular pathology were organized into two groups, group I (seven patients) receiving IT gentamicin and group II undergoing labyrinthectomy (six patients). All patients underwent six-canal video-head-impulse test in predetermined time intervals. Patients receiving gentamicin were additionally tested 3 to 5 days after every sequential injection, until all ipsilateral canals were ablated, to determine the order of response to gentamicin. We recorded the vestibular-ocular reflex gains and the presence of covert/overt saccades for each canal. RESULTS: The posttreatment ipsilateral gains were abnormal. No patient from the gentamicin group developed abnormal contralateral responses, while patients undergoing labyrinthectomy had abnormal contralateral responses from at least one canal, even several months posttreatment. Finally, the lateral semicircular canal was the first one to be affected by IT gentamicin followed by the posterior canal: the superior canal was ablated last. CONCLUSION: In our study, labyrinthectomy had an impact on the responses recorded from the contralateral ear, while IT gentamicin ablated the ipsilateral labyrinthine function without affecting the contralateral responses, possibly because of a milder, more gradual impact. We also show for the first time the order that IT gentamicin application affects the semicircular canals, with the lateral being the first to be affected.
Ear ; Ear, Inner ; Gentamicins ; Head Impulse Test ; Humans ; Observational Study ; Pathology ; Referral and Consultation ; Reflex ; Saccades ; Semicircular Canals

BACKGROUND AND OBJECTIVES: The antioxidant ebselen will be able to limit or prevent the ototoxicity arising from 2-hydroxypropyl-β-cyclodextrin (HPβCD). Niemann-Pick Type C (NPC) disease is a disorder of lysosomal storage manifested in sphingolipidosis. Recently, it was noted that experimental use of HPβCD could partially resolve the symptoms in both animals and human patients. Despite its desirable effect, HPβCD can induce hearing loss, which is the only major side effect noted to date. Understanding of the pathophysiology of hearing impairment after administration of HPβCD and further development of preventive methods are essential to reduce the ototoxic side effect. The mechanisms of HPβCD-induced ototoxicity remain unknown, but the resulting pathology bears some resemblance to other ototoxic agents, which involves oxidative stress pathways. To indirectly determine the involvement of oxidative stress in HPβCD-induced ototoxicity, we tested the efficacy of an antioxidant reagent, ebselen, on the extent of inner ear side effects caused by HPβCD. MATERIALS AND METHODS: Ebselen was applied prior to administration of HPβCD in mice. Auditory brainstem response thresholds and otopathology were assessed one week later. Bilateral effects of the drug treatments also were examined. RESULTS: HPβCD-alone resulted in bilateral, severe, and selective loss of outer hair cells from base to apex with an abrupt transition between lesions and intact areas. Ebselen co-treatment did not ameliorate HPβCD-induced hearing loss or alter the resulting histopathology. CONCLUSIONS: The results indirectly suggest that cochlear damage by HPβCD is unrelated to reactive oxygen species formation. However, further research into the mechanism(s) of HPβCD otopathology is necessary.
Animals ; Ear, Inner ; Evoked Potentials, Auditory, Brain Stem ; Hair Cells, Auditory, Outer ; Hearing Loss ; Hearing ; Humans ; Mice ; Oxidative Stress ; Pathology ; Reactive Oxygen Species ; Sphingolipidoses ; Tight Junctions