Refractory inner ear disease refers to a spectrum of inner ear diseases that fail to achieve good therapeutic effects even after standardized treatment recommended by existing clinical practice guidelines. This disease spectrum is characterized by diverse etiological factors, high phenotypic heterogeneity, unclear subtype characteristics, unknown pathogenic mechanisms, and variable prognosis, making it difficult to define precisely. Refractory inner ear disease accounts for a significant proportion of the total population with inner ear diseases and has not been systematically summarized, refined, and deeply studied to date. This article, for the first time, clearly proposes the concept, definition, and scope of refractory inner ear disease, and elaborates on the current research status, challenges, and coping strategies for refractory inner ear disease. With the development and progress of science and technology, we believe that in the future we will witness more innovative technologies and new drugs applied to the treatment of refractory inner ear disease, bringing hope of cure to patients.
Humans ; Labyrinth Diseases/therapy*

As an effective means of local drug delivery, intratympanic administration has shown a wide range of applications in the treatment of inner ear diseases, such as Meniere's disease, sudden deafness, and immune-related inner ear disease. These diseases have complex etiologies and limited treatment effects, leading to many difficulties in clinical diagnosis and treatment. Intratympanic administration significantly enhances the therapeutic effect through bypassing the blood-labyrinth barrier and increasing the local concentration of the drug in the inner ear. This article reviews the historical development, mechanism and technical advantages of intratympanic administration, and analyzes the application effects of glucocorticoids, gentamicin and other drugs in the treatment of inner ear diseases. At the same time, the limitations and challenges of intratympanic administration in terms of drug absorption, bioavailability, individualized dose control and long-term efficacy were discussed. In the future, with the advancement of nanotechnology and precision medicine, intratympanic administration is expected to achieve higher precision and safety in the treatment of inner ear diseases, providing patients with more optimized treatment options.
Humans ; Labyrinth Diseases/drug therapy* ; Injection, Intratympanic

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

Isolated otolith dysfunction(iOD) involves a group of unexplained vestibular syndromes that manifest clinically as a sense of translation, tilting or floating, and blurred vision with head movement, with normal semicircular canal function but abnormal otolith function on laboratory vestibular testing. As vestibular medicine has gained widespread popularity in recent years, increasing attention has also been paid to iOD and case reports, clinical studies and diagnostic criteria have been published. However, there is no consensus document to guide the diagnosis of this disease in China. In this context, the Special Committee on Vertigo of China Medical Education Association organized a group of domestic experts in vestibular medicine and formulated this diagnostic consensus after thorough discussion based on the latest evidence in China and abroad, in order to promote the best clinical practice for iOD.
Humans ; Otolithic Membrane ; Vestibular Diseases/diagnosis* ; Vertigo/diagnosis* ; Vestibule, Labyrinth ; Semicircular Canals

Humans ; Cochlear Implants ; Cochlear Implantation ; Vestibule, Labyrinth ; Vestibular Function Tests ; Vestibular Diseases

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.
Humans ; Endolymphatic Hydrops/diagnosis* ; Cholesteatoma, Middle Ear/complications* ; Vertigo/complications* ; Labyrinth Diseases/complications* ; Magnetic Resonance Imaging/adverse effects* ; Semicircular Canals

Ear, Inner ; Humans ; Labyrinth Diseases

Cognition ; Humans ; Vestibular Diseases ; Vestibule, Labyrinth

Autoimmune sensorineural hearing loss is a rare disease characterized by bilateral sensorineural hearing loss, some in a progressive pattern and others in idiopathic or fluctuating pattern, often accompanied by vestibular symptoms. This disease is also known as autoimmune inner ear disease (AIED), which primarily involves the vestibulo-cochlear system. However, in some cases, it occurs in the context of systemic autoimmune disorder such as wegner granulomatosis, or the Bechet disease. Response to steroids has been the requisite clinical criterion for diagnosis. Treatment usually includes corticosteroids and immunosuppressive drugs. However, after continuous steroid treatment development of corticosteroids, resistance is common in many AIED. To patients with AIED, response to steroids is not only a criterion for diagnosis but also a criterion to test steroid-sparing therapies such as methotrexate. A 10 year-old boy presenting a fluctuating pattern of bilateral sensorineural hearing loss was treated with a cytotoxic agent in suspicion of autoimmune sensorineural hearing loss. We reviewed this case with reference to relevant publications in the medical literature.
Adrenal Cortex Hormones ; Cytotoxins ; Diagnosis ; Hearing Loss, Sensorineural ; Humans ; Labyrinth Diseases ; Male ; Methotrexate ; Rare Diseases ; Steroids

Autoimmune inner ear disease (AIED) is a rare disease, accounting for < 1% of all cases of hearing impairment or dizziness. It is characterized by sensorineural hearing loss (SNHL) or vestibular dysfunction that results from an immunemediated process. Clinical features of AIED is SNHL that progresses over weeks to month with fluctuating hearing symptoms. Because there are no diagnostic laboratory and clinical feature, response to immunosuppressive therapy were important for diagnosis of AIED. Many diseases such as sudden SNHL and Meniere disease may also mimic AIED, a broad differential must be maintained in patients suspected of having AIED. We report a case of a 46-year-old female who presented with sudden hearing loss and vertigo. We could diagnose her as AIED with systemic lupus erythematous. The symptoms were improved treated with steroids.
Diagnosis ; Dizziness ; Ear, Inner ; Female ; Hearing ; Hearing Loss ; Hearing Loss, Sensorineural ; Hearing Loss, Sudden ; Humans ; Labyrinth Diseases ; Meniere Disease ; Middle Aged ; Rare Diseases ; Steroids ; Vertigo