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Objective To evaluate otolith function by comparing the findings of the Subjective Visual Vertical, the cervical Vestibular Evoked Myogenic Potential and the ocular Vestibular Evoked Myogenic Potential tests in patients in the inter-crisis period of unilateral definite Meniere's disease. Methods The sample consisted of an experimental group (n = 22; 10 men and 12 women, mean age 47.32 ± 12.82 years) with definite unilateral Meniere's disease and a control group (n = 14; 5 men and 9 women, with a mean age of 41.64 ± 13.45 years). They all underwent vestibular evaluation by means of Subjective Visual Vertical with the bucket method and, cervical and ocular Vestibular Evoked Myogenic Potential tests. The data were collected and subjected to statistical analysis. Results The results of the comparison of the Subjective Visual Vertical, the cervical Vestibular Evoked Myogenic Potential, the ocular Vestibular Evoked Myogenic Potential and, the association of cervical Vestibular Evoked Myogenic Potential with the ocular Vestibular Evoked Myogenic Potential showed no significant difference, indicating concordance among the tests. Conclusion The identified abnormalities and the concordance between the combined proportion of the Subjective Visual Vertical, the cervical Vestibular Evoked Myogenic Potential and the ocular Vestibular Evoked Myogenic Potential findings indicate that the association of these three tests contributes to the identification of sustained and transient otolith dysfunction in the inter-crisis of unilateral definite Meniere’s disease. Level of evidence 2.

Superior semicircular canal dehiscence (SSCD) is caused by bony defects in the osseous shell of the arcuate eminence separating the labyrinth and the intracranial space. This pathologic third window causes hydroacoustic transmission resulting in debilitating symptoms. We examine the pathophysiologic association between metabolic markers, previous medical history, and SSCD symptoms before and after middle fossa craniotomy (MFC) treatment.This study was conducted between March 2011 and September 2020 with patients with SSCD who underwent MFC. We used a Fisher test to compare variables, including bilateral SSCD, second surgery, ear anomaly, osteoporosis, arthritis, vitamin D, and preoperative/postoperative symptoms, and others. Point-biserial correlation analysis was performed to test correlations between continuous variables and categorical variables.A total of 250 patients with SSCD underwent MFC repair. There was significant postoperative resolution in all symptoms (P0.0001). Laboratory 25-hydroxyvitamin D values correlated with preoperative aural fullness (rCertain metabolic markers may predict SSCD presentation before and after surgery. Previous history of osteoporosis, autoimmune conditions, or arthritis may play a role in SSCD pathophysiology and can help predict clinical outcomes. Future evaluation should take metabolic laboratory values and acquire an exact medical history.

Congenital hearing loss is a common disorder worldwide. Heterogeneous gene variation accounts for approximately 20-25% of such patients. We investigated a five-generation Chinese family with autosomal-dominant nonsyndromic sensorineural hearing loss (SNHL). No wave was detected in the pure-tone audiometry, and the auditory brainstem response was absent in all patients. Computed tomography of the patients, as well as of two sporadic SNHL cases, showed bilateral inner ear anomaly, cochlear maldevelopment, absence of the osseous spiral lamina, and an enlarged vestibular aqueduct. Such findings were absent in nonaffected persons. We used linkage analysis and exome sequencing and uncovered a heterozygous missense mutation in the PI4KB gene (p.Gln121Arg) encoding phosphatidylinositol 4-kinase β (PI4KB) from the patients in this family. In addition, 3 missense PI4KB (p.Val434Gly, p.Glu667Lys, and p.Met739Arg) mutations were identified in five patients with nonsyndromic SNHL from 57 sporadic cases. No such mutations were present within 600 Chinese controls, the 1000 genome project, gnomAD, or similar databases. Depleting pi4kb mRNA expression in zebrafish caused inner ear abnormalities and audiosensory impairment, mimicking the patient phenotypes. Moreover, overexpression of 4 human missense PI4KB mutant mRNAs in zebrafish embryos resulted in impaired hearing function, suggesting dominant-negative effects. Taken together, our results reveal that PI4KB mutations can cause SNHL and inner ear malformation. PI4KB should be included in neonatal deafness screening.

Background Superior semicircular canal dehiscence (SSCD) is a rare inner ear disorder; currently, it is unknown whether the etiopathology underlying this structural irregularity affects neighboring structures. The goal is to investigate the prevalence of bone thinning in areas of the middle cranial fossa (MCF) floor in SSCD and non-SSCD patients. Methods This retrospective study analyzed 100 patients from March 2011 to June 2017 at a tertiary referral center. 100 patients undergoing 118 SSCD repair surgeries (18 bilateral) were identified. 12 SSCD ears were excluded due to lack of pre-operative computed tomography (CT) scans or history of prior SSCD repair at an outside facility. Non-SSCD ears were identified from routinely-obtained CT scans for temporal bone fracture (fractured sides excluded) for a total of 101 ears; 26 non-SSCD ears were excluded due to lack of high-resolution imaging. Results Univariate analyses reveal that SSCD diagnosis is associated with higher rates of geniculate ganglion (GG) dehiscence compared with non-SSCD controls (42.7 vs. 24%; χ 2 (1) = 9.69, P = 0.008). Individuals with SSCD depicted significantly thinner bone overlying the geniculate ganglion (GG) (0.23 ± 1.2 mm) compared to controls (0.28 ± 1.8 mm, (t(1 6 4)) = 2.1, P = 0.04). SSCD patients presented thinner bone overlying the internal auditory canal (IAC) (0.33 ± 1.3 mm) compared to patients without SSCD (0.46 ± 1.6 mm, (t(2 5 7) = 6.4, P Conclusions The increased prevalence of dehiscence of the MCF in this cohort of SSCD patients compared to non-SSCD patients suggests that the etiology underlying SSCD affects surrounding structures.

Recurrent dizziness, vertigo, and imbalance, as well as nausea or vomiting, can be induced by central or peripheral vestibular pathology. Vestibular migraine (VM) is a central pathology in which migraines reciprocally interact with vestibular nuclei. Vestibular neuritis and labyrinthitis (VN/L) are peripheral diseases involving inflammation of structures in the vestibular nerve or labyrinth. Because VM and VN/L in early stages can produce similar symptoms, diagnosis may require prolonged clinical evaluation. It has been suggested that differences in a patient's Vestibulo-Ocular Reflex (VOR) measured during the rotary chair step rotation test (RCS Test) can differentiate VM from peripheral pathologies, allowing for faster diagnosis. We sought to compare VOR time constants (VORTRetrospective chart review of patients seen at a hospital balance center between January 2010 and June 2019.Tertiary referral center.68 patients (mean age = 58.15, 65 % female, 35 % male) were placed into two groups based on clinical diagnosis codes: 1) VM or 2) VN/L.Primary variable of VORTPatients in the VM group demonstrated statistically significant elevated VOR TOur results support the conclusion that elevated VOR T

Superior semicircular canal dehiscence (SSCD) is a rare inner ear disorder with variable amounts of auditory and vestibular dysfunction. In addition to the absence of bone overlying the superior semicircular canal, thinning of bone in this area can also initiate the vestibulocochlear symptoms of SSCD. We evaluated normal bone thickness overlying the course of the semicircular canal using computed tomography (CT) scans and assessed correlations between bone thickness and age, gender, and location of the thinnest bone. A single-institution retrospective chart review was conducted on 133 high-resolution CT scans from 76 healthy, asymptomatic patients between ages 9 and 96 years. These CT scans of the temporal bone were obtained between January 2012 and August 2017. The superior semicircular canal dome thickness at the apex was reported with a mean of 1.25 mm for all 76 patients; the 10th percentile was 0.60 mm, and the 90th percentile was 2.08 mm. The thinnest area of bone at any location yielded a mean of 0.86 mm. The normal bone thickness overlying the superior semicircular canal does not depend on gender or age. The thinnest location was evenly distributed across the superior semicircular canal. A bone thickness of 0.40 mm or greater was present in 90% of normal patients based on CT scan measurements at the thinnest location.

Posterior semicircular canal dehiscence is a rare condition and can cause a variety of symptoms. We report three cases of children between 5 and 12 years of age with a PSCD. They all presented with different complaints as follows: Tullio phenomenon in the first case, conductive hearing loss in the second and, conductive hearing loss and pulsatile tinnitus in the third. Imaging showed in all cases a PSCD on the right side, caused by a prominent jugular bulb (high riding bulb). We describe the clinical, audiometric and radiological findings, and discuss the management and therapy. A conservative “wait and see” approach is recommended, especially with children, because of the possible complications of surgery and the possibility that the symptoms will lessen with the skull base osseous maturation.

Aim To report the effect of oval and round window reinforcement surgery performed in two patients with the Tullio phenomenon. Case 1: A male with bilateral superior canal dehiscence syndrome. Downbeat nystagmus with leftward horizontal eye movement was recorded in an electronystagmogram using a pure-tone sound of 110 dB at 2000 or 4000 Hz in the right ear. Case 2: A female who had undergone stapes surgery. Computed tomography revealed an ossicular prosthesis in the vestibule. An audiogram indicated mild to moderate hearing impairment in the right ear. Leftward horizontal nystagmus was recorded in an electronystagmogram using a pure-tone sound of 110 dB at 500 or 1000 Hz in the right ear. Surgical findings indicated that the prosthesis was inserted deep into the oval window, which was closed with thin connective tissue. Outcomes After oval and round window reinforcement surgery was performed in the right ear, and loud, pure-tone sounds elicited neither nystagmus nor dizziness in either patient.

Introduction Immune Mediated Inner Ear Disease (IMIED) is a rare form of sensorineural bilateral hearing loss, usually progressing in weeks to months and responsive to immunosuppressive treatment. Despite recent advances, there is no consensus on diagnosis and optimal treatment. Methods A review of articles on IMIED from the last 10 years was conducted using PubMed® database. Results IMIED is a rare disease, mostly affecting middle aged women. It may be a primary ear disease or secondary to autoimmune systemic disease. A dual immune response (both cellular and humoral) seems to be involved. Cochlin may be the inner ear protein targeted in this disease. Distinction from other (core common) forms of neurosensory hearing loss is a challenge. Physical examination is mandatory for exclusion of other causes of hearing loss; audiometry identifies characteristic hearing curves. Laboratory and imaging studies are controversial since no diagnostic marker is available. Conclusion Despite recent research, IMIED diagnosis remains exclusive. Steroids are the mainstay treatment; other therapies need further investigation. For refractory cases, cochlear implantation is an option and with good relative outcome.

Third window abnormalities are bony defects of the inner ear that enable abnormal communication with the middle ear and/or cranial cavity. Vestibular symptoms include vertigo and nystagmus induced by loud noises or increases in pressure. Auditory symptoms involve "pseudo-conductive" hearing loss with a low-frequency air-bone gap at audiometry, resulting from decreased air and increased bone conduction. High-resolution temporal bone computed tomography is the first-line imaging modality for evaluation of third window pathology and is critical for accurate diagnosis and management. This article reviews the fundamental mechanisms of the third window phenomenon and describes imaging findings and differential diagnosis.

Background Superior semicircular canal dehiscence (SSCD) is a rare bony defect in the petrosal temporal bone, creating a new opening between the inner ear and middle cranial fossa. Ehlers-Danlos syndrome (EDS) is an inherited connective tissue disorder characterized by a defect in collagen synthesis. Patients with EDS are more likely to have bony abnormalities, which may predispose them to developing SSCD. Case Description We report the cases of 2 women with history of EDS hypermobility type (HT). Both patients presented with bilateral auditory and vestibular symptoms, and computed tomography scan confirmed the presence of bilateral dehiscence in their superior semicircular canals. They underwent elective surgical repair via middle fossa craniotomy and report improvement in their symptoms. Conclusions Because of the rarity of both diseases and their pathophysiology, a history of EDS may predispose an individual to developing SSCD. Although further research is necessary, this report aims to improve clinical screening of patients with EDS HT by suggesting an auditory and vestibular evaluation and assessment of pertinent bony abnormalities.

Objective The purpose of this study was to investigate the development of speech skills in young children with Mondini dysplasia and age-matched deaf children with radiologically normal inner ears over a period of 5 years after cochlear implantation (CI). Methods In total, 700 congenitally severely to profoundly deaf children (281 girls and 419 boys) participated in this study. All of the participants had undergone unilateral CI surgery before 36 months of age. The participants were categorized into two groups based on the absence or presence of Mondini dysplasia in the implanted ear, as assessed via high-resolution, thin-slice computerized tomography or magnetic resonance imaging: group A comprised 592 children with radiologically normal inner ears and group B comprised 108 children with Mondini dysplasia. The Meaningful Use of Speech Scale (MUSS) and Speech Intelligibility Rating (SIR) were used to evaluate the speech performance of all young children at various time points: pre-surgery and at 1, 3, 6, 12, 24, 36, 48, and 60 months after switch-on programming. Results The mean scores of SIR and MUSS in children from both group A and group B showed significant improvements over time. No significant differences were found in the mean scores of SIR between the two groups at any time interval during the 5-year follow-up. The mean score of MUSS was significantly different between group A and group B at 12, 24, and 36 months after implantation, whereas no obvious differences were noted pre-surgery, and at 1, 3, 6, 48, and 60 months post-operation. Conclusions Young children with Mondini dysplasia develop their speech skills at a fast rate and achieve similar speech acquisition compared to age-matched children with radiologically normal inner ears 5 years post-operation. Therefore, CI is an effective intervention method for young children with Mondini dysplasia.

Objective The current investigation evaluated a novel extended release delivery system for treating inner ear diseases. The platform technology consists of a film forming agent (FFA) and microsphere component to localize and extend drug delivery within the ear. Study design Studies evaluated dissolution kinetics of microspheres with multiple encapsulates, testing of a variety of FFAs, and ability to localize to the round window membrane in mice in vivo. Setting Studies were completed at Orbis Biosciences and The University of Kansas Medical Center. Subjects In conjunction with in vitro characterization, an infrared dye-containing microsphere formulation was evaluated for round window membrane (RWM) localization and general tolerability in C57/BL6 Mus musculus for 35 days. Methods: In vitro characterization was performed using upright diffusion cells on cellulose acetate membranes, with drug content quantified by high performance liquid chromatography. Mus musculus dosing of infrared dye-containing microspheres was performed under anesthesia with a 27 GA needle and 2.0 μL injection volume Results In vitro dissolution demonstrates the ability of the FFA with microsphere platform to release steroids, proteins, peptides, and nucleic acids for at least one month, while necroscopy shows the ability of the FFA with dye-loaded microspheres to remain localized to Mus musculus RWM for the same period of time, with favorable tolerability. Conclusions: Combining FFA and microsphere for localized drug delivery may enable cost-effective, extended release local delivery to the inner ear of new and existing small molecules, proteins, peptides, and nucleic acids.

The field of hearing and deafness research is about to enter an era where new cochlear drug delivery methodologies will become more innovative and plentiful. The present report provides a representative review of previous studies where efficacious results have been obtained with animal models, primarily rodents, for protection against acute hearing loss such as acoustic trauma due to noise overexposure, antibiotic use and cancer chemotherapies. These approaches were initiated using systemic injections or oral administrations of otoprotectants. Now, exciting new options for local drug delivery, which opens up the possibilities for utilization of novel otoprotective drugs or compounds that might not be suitable for systemic use, or might interfere with the efficacious actions of chemotherapeutic agents or antibiotics, are being developed. These include interesting use of nanoparticles (with or without magnetic field supplementation), hydrogels, cochlear micropumps, and new transtympanic injectable compounds, sometimes in combination with cochlear implants.

The present case was a 38-year-old male who presented with progressive hearing loss, resulting in profound bilateral hearing loss. He had a past history of childhood medulloblastoma, which was treated with posterior fossa craniotomy and radiotherapy. A ventriculoperitoneal (VP) shunt was put in place to manage the hydrocephalus. Cochlear implantation (CI) was carried out on his right ear by a standard procedure. At CI activation, the electric impedance of the electrode was very high, and computed tomography revealed that there was no area of liquid density, suggesting depletion of the perilymph in the cochlea and vestibule. Eight months later, the impedance improved gradually, and the cochlea was filled with perilymph. Consequently, one of the causes of the pneumolabyrinth in the present case was that a scarred stenotic cochlear canaliculus secondary to surgery or radiation therapy might have prevented the CSF from filling the scala. In addition, it is also possible that the VP shunt might have altered the CSF pressure, leading to depletion of the perilymph.

The isolated anatomical position and blood-labyrinth barrier hampers systemic drug delivery to the mammalian inner ear. Intratympanic placement of drugs and permeation via the round- and oval window are established methods for local pharmaceutical treatment. Mechanisms of drug uptake and pathways for distribution within the inner ear are hard to predict. The complex microanatomy with fluid-filled spaces separated by tight- and leaky barriers compose various compartments that connect via active and passive transport mechanisms. Here we provide a review on the inner ear architecture at light- and electron microscopy level, relevant for drug delivery. Focus is laid on the human inner ear architecture. Some new data add information on the human inner ear fluid spaces generated with high resolution microcomputed tomography at 15 μm resolution. Perilymphatic spaces are connected with the central modiolus by active transport mechanisms of mesothelial cells that provide access to spiral ganglion neurons. Reports on leaky barriers between scala tympani and the so-called cortilymph compartment likely open the best path for hair cell targeting. The complex barrier system of tight junction proteins such as occludins, claudins and tricellulin isolates the endolymphatic space for most drugs. Comparison of relevant differences of barriers, target cells and cell types involved in drug spread between main animal models and humans shall provide some translational aspects for inner ear drug applications.

Introduction To evaluate the utility of positron emission tomography-computed tomography (PET/CT) as an imaging tool for the characterization of immune-mediated inner ear disease (IMIED), providing measurements of the inner ear region activity as well as detecting possible involvement of other organs. Material and methods The study included 28 patients with IMIED and 4 sex-matched and age-matched control subjects with no history of ear disease. Eighteen patients were considered to be suffering from primary IMIED and 10 patients from secondary. PET/CT scans with 18F-FDG were performed to assess systemic involvement as well as inner ear region activity. Interpretation of PET/CT scans was performed independently by two nuclear medicine physicians blinded to clinical history. In order to assess inter-rater agreement before performing the analysis of the inner ear, different Bland & Altman plots and the intraclass correlation coefficients were estimated. Results Different metabolically active foci findings were reported in 13 patients. Four patients diagnosed as primary IMIED showed thyroid and aorta activity. Regarding the inner-ear semiquantitative analysis, the inter-rater agreement was not sufficiently high. Comparisons between groups, performed using Mann–Whitney test or Kruskal–Wallis tests, showed no differences. Conclusions The study showed 18F-FDG PET/CT could be an important tool in the evaluation of IMIED as it can support the characterization of this entity providing the diagnosis of unknown or underestimated secondary IMIED. Nevertheless, we consider PET is not an adequate tool to approach the inner ear because of the small size and volume of the cochlea which makes the assessment very difficult.

Objective Non-contrast MRI of the internal auditory canal (IAC) using high-resolution T2WI (T2 weighted image) has been proposed as the primary screening study in patients with sudden or asymmetric sensorineural hearing loss (ASNHL). However, there are concerns that non-contrast MRI may not detect labyrinthine pathology, specifically intralabyrinthine schwannomas (ILSs). The purpose of this study was to determine if non-contrast high-resolution T2WI alone are adequate to exclude these uncommon intralabyrinthine tumors. Methods 31 patients with ILSs and 36 patients without inner ear pathology that had dedicated MRI of the IAC performed with both non-contrast T2WI and post-contrast T1WI (T1 weighted image) were identified. Three board-certified neuroradiologists reviewed only the T2WI from these 67 cases. When an ILS was identified, its location and size were recorded. Sensitivity, specificity, and accuracy were calculated using the post-contrast T1WI as the “gold standard.” A consensus review of cases with discordant results was conducted. Results The sensitivity, specificity, and accuracy were 1.0, 1.0, and 1.0 for Observer 1; 0.84, 1.0, and 0.96 for Observer 2; 0.90, 1.0, and 0.98 for Observer 3. The 5 ILSs with discordant results were correctly identified upon consensus review. The median size of the ILSs was 4.4 mm (± 2.9 mm) and most (18/31) were intracochlear in location. Conclusion Non-contrast high-resolution T2WI alone can detect ILSs with 84–100% sensitivity, suggesting that gadolinium may be unnecessary to exclude ILSs on screening MRI. These findings have implications for reducing cost, time, and adverse events associated with gadolinium administration in patients presenting with sudden or ASNHL. Level of evidence 4

The authors provide an update on the clinical manifestations, diagnosis and various approaches to the treatment of superior semicircular canal dehiscence (SSCD). SSCD is a rare condition where the bone overlying the superior semicircular canal thins or dehisces causing characteristic clinical findings. Since this was first reported in 1998 by Minor and colleagues, there has been much advancement made in terms of diagnosis and treatment. Signs and symptoms include a wide variation of both vestibular and auditory manifestations. Diagnosis made solely on clinical signs is difficult due to how varied the presentations can be and the overlap with other otologic pathologies. High-resolution CT temporal scans have been the standard in confirming superior semicircular canal dehiscence, however, MRI FIESTA scans have recently been used to image SSCD. Additionally, audiometry and vestibular evoked myogenic potential (VEMP) testing are useful screening tools. Currently, the middle fossa approach is the most common and standard surgical approach to repair SSCD. The transmastoid, endoscopic and transcanal or endaural approaches have also been recently utilized. Presently, there is no consensus as to the best approach, material or technique for repair of SSCD. As we learn more, newer and less invasive approaches and techniques are being used to treat SSCD. We present a comprehensive review of SSCD, including clinical symptoms and presentation, histopathology, diagnosis, treatment strategies and outcomes of intervention.

Objective To elucidate the clinical features and vestibular symptoms of patients with otolith organ dysfunction in the presence of normal function of the semicircular canals. Methods We reviewed the clinical records of 277 consecutive new patients with balance disorders who underwent testing of cervical and ocular vestibular evoked myogenic potentials (cVEMPs and oVEMPs) as well as caloric testing and video head impulse testing (vHIT). Results We identified 76 patients who showed normal caloric responses and normal vHIT findings in each SCC plane, but abnormal responses in cVEMP and/or oVEMP testing. Benign paroxysmal positional vertigo (BPPV) was the most common diagnosis. 37% of patients could not be categorized into any of the established clinical entities that could cause a balance disorder and did not show sensorineural hearing loss. The most common clinical manifestation in the idiopathic cases was recurrent rotatory vertigo with a duration of 1–12 h. Conclusions The most common diagnosis of otolith organ-specific vestibular dysfunction was BPPV. The most common clinical manifestation in the idiopathic cases was recurrent rotatory vertigo. Significance Specific dysfunction of the otolith organs occurs in association with some of the undiagnosed patients with recurrent rotatory vertigo.

High resolution T2 magnetic resonance imaging (MRI) can provide exquisite detail of internal auditory canal (IAC) and cerebellopontine angle (CPA) lesions. In this retrospective case series, blinded imaging sequences were delivered to three radiologists and compared with previously archived clinical reads that were non-blinded and incorporated both T1 + C and T2 sequences together. This article demonstrates high sensitivity and specificity for high resolution T2 MRI particularly with lesions > 5 mm. This suggests a role for high resolution T2 MRI as an initial screening sequence or as a surveillance sequence.

Objectives/Hypothesis We aim to define the frequencies of anomalies of the inner ear, oval window, and round window ipsilateral to isolated non-syndromic unilateral aural atresia. Methods and materials Retrospective case series. We reviewed high resolution computed tomography scans of the temporal bones of 70 children with isolated non-syndromic unilateral congenital aural atresia. Scans were reviewed according to the Jahrsdoerfer criteria and further evaluated for anomalies of the vestibule, semi-circular canals, cochlea, internal auditory canal and vestibulocochlear nerve. Results Inner ear dysplasia was seen in two of 70 atretic ears: one with a dysmorphic lateral semicircular canal and another with a large vestibule assimilating the lateral semicircular canal. Abnormalities of the oval window and round window ipsilateral to the atresia were identified in 21% (15) and 7% (5), respectively, of the atretic ears. Oval window and round window abnormalities were associated with disproportionately lower Jahrsdoerfer scores compared to aural atresia patients without these abnormalities (P Conclusion Compared to studies that included syndromic or bilateral atresia cases, we found inner ear and oval window abnormalities less common in children with isolated non-syndromic unilateral aural atresia. However, round window anomalies seem to occur at about the same frequency.

Objective A significant proportion of children with congenital hearing loss who are candidates for cochlear implants (CIs) may have inner ear malformations (IEMs). Surgical and speech outcomes following CI in these children have not been widely reported. Methods The charts of children who were evaluated for a CI between 1/1/1986 and 12/31/2014 at a university-based tertiary level pediatric cochlear implant center were reviewed. Principal inclusion criteria included (i) age 1–18 years, (ii) history of bilateral severe to profound sensorineural hearing loss, and (iii) limited benefit from binaural amplification. Exclusion criteria included (i) underlying diagnosis of neurodevelopmental disorder and (ii) lack of follow up for speech assessment if a CI was performed. The following outcome measures were reviewed: (i) imaging findings with magnetic resonance imaging or high resolution computed tomography, (ii) intraoperative complications, and (iii) speech perception categorized as the ability to perceive closed set, open set, or none. Results The prevalence of IEMs was 27% (102 of 381), of which 79% were bilateral. Cochlear dysplasia accounted for 30% (40 of 136) of the anomalies. Seventy-eight of the 102 patients received a CI (78%). Surgery was noted to be challenging in 24% (19 of 78), with a perilymphatic gusher being the most common intraoperative finding. Cochlear dysplasia, vestibular dysplasia and cochlear nerve hypoplasia were associated with poor speech perception (open OR closed set speech recognition scores, 0–23%), although the outcomes in children with enlarged vestibular aqueduct were similar to those of children with normal inner ear anatomy (65%). Conclusions Cochlear implantation is safe in children with IEMs. However, the speech perception outcomes are notably below those of patients with normal anatomy, with the exception of when an enlarged vestibular aqueduct is present.

Objective Guidelines exist for evaluation and management of tinnitus in adults; however lack of evidence in children limits applicability of these guidelines to pediatric patients. Objective of this study is to determine the incidence of inner ear anomalies detected on imaging studies within the pediatric population with tinnitus and evaluate if presence of hearing loss increases the rate of detection of anomalies in comparison to normal hearing patients. Methods Retrospective review of all children with diagnosis of tinnitus from 2010 to 2015 ;at a tertiary care academic center. Results 102 pediatric patients with tinnitus were identified. Overall, 53 patients had imaging studies with 6 abnormal findings (11.3%). 51/102 patients had hearing loss of which 33 had imaging studies demonstrating 6 inner ear anomalies detected. This is an incidence of 18.2% for inner ear anomalies identified in patients with hearing loss (95% confidence interval (CI) of 7.0–35.5%). 4 of these 6 inner ear anomalies detected were vestibular aqueduct abnormalities. The other two anomalies were cochlear hypoplasia and bilateral semicircular canal dysmorphism. 51 patients had no hearing loss and of these patients, 20 had imaging studies with no inner ear abnormalities detected. There was no statistical difference in incidence of abnormal imaging findings in patients with and without hearing loss (Fisher's exact test, p ;= ;0.072.) Conclusion There is a high incidence of anomalies detected in imaging studies done in pediatric patients with tinnitus, especially in the presence of hearing loss.

Chronic otitis media with cholesteatoma can cause erosion of the dense labyrinthine bone overlying the inner ear organs, and this labyrinthine fistula allows pressure or mass-induced motion of the underlying perilymphatic and endolymphatic compartment, evoking vestibular symptoms. While the mechanism of a positive fistula test, which is conducted by increasing or decreasing the external auditory canal pressure, has been well established, the mechanism underlying positional nystagmus in labyrinthine fistula has not been discussed yet. In the present study, we propose a new hypothesis accounting for positional nystagmus in labyrinthine fistula involving the lateral semicircular canal (LSCC), i.e., the change in intracranial cerebrospinal fluid pressure by position change is transmitted to the perilymphatic space, causing ampullopetal (excitatory) or ampullofugal (inhibitory) deflection of the LSCC cupula.

Objectives To analyse the ontogeny of the superior semicircular canal and tegmen tympani and determine if there are common embryological factors explaining both associated dehiscence. Methods We analysed 77 human embryological series aged between 6 weeks and newborn. Preparations were serially cut and stained with Masson's trichrome technique. Results The tegmental prolongation of tegmen tympani and superior semicircular canal originate from the same structure, the otic capsule, and have the same type of endochondral ossification; while the extension of the squamous prolongation of tegmen tympani runs from the temporal squama and ossification is directly of intramembranous type. The nuclei of ossification of the superior and external semicircular canals and accessory of tegmen collaborate in the ossification of the tegmental extension and by growth extend to the tegmental prolongation. This fact plus the fact that both structures share a common layer of external periosteum could explain the coexistence of lack of bone coverage in tegmen and superior semicircular canal. Conclusion The development of the semicircular canal and tegmen tympani could explain the causes of the association of both dehiscences.

A new type of advanced drug delivery systems is proposed: Miniaturized implants, which can be placed into tiny holes drilled into (or close to) the oval window. They consist of two parts: 1) A cylinder, which is inserted into the hole crossing the oval window. The cylinder (being longer than the depth of the hole) is partly located within the inner ear and surrounded by perilymph. This provides direct access to the target site, and at the same time assures implant fixation. 2) A cuboid, which is located in the middle ear, serving as a drug reservoir. One side of the cuboid is in direct contact with the oval window. Drug release into the cochlea occurs by diffusion through the cylindrical part of the Ear Cubes and by diffusion from the cuboid into and through the oval window. High precision molds were used to prepare two differently sized Ear Cubes by injection molding. The miniaturized implants were based on silicone and loaded with different amounts of dexamethasone (10 to 30 % w/w). The systems were thoroughly characterized before and upon exposure to artificial perilymph at 37°C. Importantly, drug release can effectively be controlled and sustained during long time periods (up to several years). Furthermore, the implants did not swell or erode to a noteworthy extent during the observation period. Drug diffusion through the polymeric matrix, together with limited dexamethasone solubility effects, seem to control the resulting drug release kinetics, which can roughly be estimated using mathematical equations derived from Fick's second law. Importantly, the proposed Ear Cubes are likely to provide much more reliable local long term drug delivery to the inner ear compared to liquid or semi-solid dosage forms administered into the middle ear, due to a more secured fixation. Furthermore, they require less invasive surgeries and can accommodate higher drug amounts compared to intracochlear implants. Thus, they offer the potential to open up new horizons for innovative therapeutic strategies to treat inner ear diseases and disorders.

The present study sought to determine whether there is a correlation between the prevalence of superior semicircular canal (SSC) dehiscence (SSCD) on temporal CT and population age. The secondary objective was to identify anatomic factors for SSCD by studying SSC diameter and its protrusion into the middle cranial fossa. The aim was to determine the acquired or congenital origin of SSCD (Minor's syndrome).A single-center retrospective radiological and anatomic study included 180 CT scans of 354 petrous parts of the temporal bone taken between January and December 2011 in a university hospital center. Bone thickness above the SSC was measured and classified in 4 grades: grade 1,2.5mm; grade 2,2.5mm: grade 3, predehiscent; grade 4, dehiscent. SSC diameter was also measured, as was the height of SSC protrusion into the middle cranial fossa.SSCD was found in 0.8% of cases and predehiscence in 12%. Patients with dehiscence were older; patients with grade 3 or 4 were significantly older than those free of dehiscence (P0.05). There was no significant difference in SSC diameter according to grade. In grade 1, protrusion was greater than in other subjects, with a significant correlation between age and reduced protrusion (P0.05).The study demonstrated a correlation between aging and SSCD prevalence. Reduced SSC roof height with age suggests that SSCD may be an acquired phenomenon, related in some way to aging of the base of the skull.

Approximately 3 in 1000 children in the US under 4 years of age are affected by hearing loss. Currently, cochlear implants represent the only line of treatment for patients with severe to profound hearing loss, and there are no targeted drug or biological based therapies available. Gene replacement is a promising therapeutic approach for hereditary hearing loss, where viral vectors are used to deliver functional cDNA to “replace” defective genes in dysfunctional cells in the inner ear. Proof-of-concept studies have successfully used this approach to improve auditory function in mouse models of hereditary hearing loss, and human clinical trials are on the immediate horizon. The success of this method is ultimately determined by the underlying biology of the defective gene and design of the treatment strategy, relying on intervention before degeneration of the sensory structures occurs. A challenge will be the delivery of a corrective gene to the proper target within the therapeutic window of opportunity, which may be unique for each specific defective gene. Although rescue of pre-lingual forms of recessive deafness have been explored in animal models thus far, future identification of genes with post-lingual onset that are amenable to gene replacement holds even greater promise for treatment, since the therapeutic window is likely open for a much longer period of time. This review summarizes the current state of adeno-associated virus (AAV) gene replacement therapy for recessive hereditary hearing loss and discusses potential challenges and opportunities for translating inner ear gene replacement therapy for patients with hereditary hearing loss.

Introduction Pneumolabyrinth is defined as the presence of air within the inner ear causing cochleovestibular signs and symptoms. Twenty-seven cases of pneumolabyrinth are published in the literature of which only two were described as delayed pneumolabyrinth as a complication of stapes surgery. Case report A 45-year-old male patient presented with vertigo after Valsalva maneuver 12 years after stapedectomy. He was diagnosed with delayed pneumolabyrinth on high resolution computed tomography and underwent an exploratory tympanotomy with surgical repair. Discussion Delayed pneumolabyrinth is a rare complication of stapes surgery and should be considered in patients presenting with vestibulocochlear symptoms even many years later. An exploratory tympanotomy is recommended for patients diagnosed with pneumolabyrinth and having a sensorineural hearing loss and/or persistent vertigo.

INTRODUCTION: Congenital malformations and acquired lesions of the inner ear are characterised by small structural changes in this region. In recent decades, treatment options have improved considerably. At the same time, there has been a great advancement in diagnostic methods, obtaining high-resolution labyrinth images. Currently, we use a 64-multislice computed tomography scanner in spiral mode (Brilliance 64 Phillips, Eindhoven, the Netherlands), with an overlap of 0.66 mm and an interval of 0.33 mm, 120 KV and 300 mA. The magnetic resonance images were taken with Signa HDxt 1.5 and 3.0 T units (GE Healthcare, Waukesha, WI, USA). We reviewed the radiological features of the lesions affecting the inner ear. They are classified as congenital (labyrinth malformation and statoacoustic nerve deficiencies) or acquired (otospongiosis, labyrinthitis, Meniere's disease, inner ear haemorrhage, intralabyrinthine schwannoma and endolymphatic sac tumour). CONCLUSION: Magnetic resonance imaging and computed tomography play an essential role in diagnosing patients with inner ear pathology. The technique selected should be chosen depending on the clinical setting. In a generic way, tomography is the method of choice for the study of traumatic pathology or otospongiosis. When tumour or inflammatory pathology is suspected, magnetic resonance is superior. In cases of congenital malformation, both techniques are complementary.

This article reviews the relevant anatomy, pathophysiology, clinical presentation, radiographic differential, management, outcomes, and rehabilitation of patients with intralabyrinthine schwannomas.

We herein report a case of posterior semicircular canal dehiscence (SCD) syndrome who had been audiologically followed up for eight years. The patient originally had sensorineural hearing loss. The audiogram had gradually transformed to pure conductive hearing loss. The posterior SCD was identified in CT scan. The reported case showed the possibility to distinguish the mechanism at play underlying the typical conductive hearing loss in SCD patients by tracing the transition of the hearing loss pattern. This information is of much help to predict the hearing outcomes if surgical intervention were chosen for the treatment.

Objective High jugular bulb (HJB) can erode inner ear structures creating a jugular bulb related inner ear dehiscence (JBID). The aim of this study was to analyze the relationship between the position of jugular bulb (JB) and JBID using high-resolution computed tomography (HRCT). Material and methods In this retrospective study HRCT images of 552 ears of 276 patients with hearing loss, otogenic vertigo, tinnitus or idiopathic peripheral facial nerve paralysis were analyzed. HJB type-1 was defined when JB dome reached above the inferior part of the round window, but was below the inferior edge of the internal acoustic meatus (IAM). HJB type-2 was defined when the dome of JB was higher than the inferior edge of IAM. The frequencies and types of HJB were evaluated. JBID for each HJB type was determined. Frequencies of JBID eroding the vestibular aqueduct (VA), the cochlear aqueduct and the posterior semicircular canal were examined. Results HJB type-1 and HJB type-2 were found in 19% (105/552) and in 15.8% (87/552) of studied ears. JBID showed to be in 3.8% (21/552) of all ears. 90.5% (19/21) of JBID revealed eroding of VA. Jugular bulb related cochlear aqueduct dehiscence and jugular bulb related posterior semicircular canal dehiscence were found in one ear each. The frequency of jugular bulb related vestibular aqueduct dehiscence (JBVAD) in patients with HJB reaching above IAM was higher than in patients with HJB lower than IAM. Conclusions HJB is common, but JBID is rare. JBID prevalently erodes VA. HJB rising above IAM is most at risk to show JBVAD.

Because of its lack of ionizing radiation, MR imaging is increasingly used for patients with cardiovascular disease, including young women. However, the risks related to the MR environment need to be acknowledged and prevented. For women, there are unique gender-related safety issues that are important to address in cardiovascular MR examinations. This article familiarizes radiologists with MR safety issues and current, evidence-based recommendations for specific situations such as pregnancy or lactation and imaging of women who have pelvic gynecologic devices such as intrauterine devices. Practical algorithms to minimize risk and increase MR safety for these women are suggested.

Active middle ear implants (AMEIs) have been available for a number of years and yet most radiologists have never heard of them. Some bear a striking resemblance to cochlear implants whereas others are more similar to conventional hearing aids. The aims of this review are to provide an introduction as to the types of implants available, how they work and when they are indicated. Also, to highlight important pre-operative imaging features that can influence surgery and to consider the role of imaging in the post-operative setting. As patient choice increases, it becomes more likely that radiologists will encounter these devices in daily practice and knowledge of them may prove useful.

Drug-induced ototoxicity, as a toxic side effect, is an important issue needed to be considered in drug discovery. Nevertheless, current experimental methods used to evaluate drug-induced ototoxicity are often time-consuming and expensive, indicating that they are not suitable for a large-scale evaluation of drug-induced ototoxicity in the early stage of drug discovery. We thus, in this investigation, established an effective computational prediction model of drug-induced ototoxicity using an optimal support vector machine (SVM) method, GA-CG-SVM. Three GA-CG-SVM models were developed based on three training sets containing agents bearing different risk levels of drug-induced ototoxicity. For comparison, models based on naïve Bayesian (NB) and recursive partitioning (RP) methods were also used on the same training sets. Among all the prediction models, the GA-CG-SVM model II showed the best performance, which offered prediction accuracies of 85.33% and 83.05% for two independent test sets, respectively. Overall, the good performance of the GA-CG-SVM model II indicates that it could be used for the prediction of drug-induced ototoxicity in the early stage of drug discovery.

Dizziness is a frequent indication for neuroimaging within the outpatient and emergency setting with variable diagnostic yield. The majority of persistent, recurrent, and isolated dizziness can be managed clinically. However, it may be difficult to distinguish a benign peripheral aetiology from a central cause, particularly in the emergency setting. We review the relevant anatomy, differential diagnosis, and key imaging features of central and peripheral causes of dizziness, as well as the literature for the diagnostic yield in acute and outpatient settings.

Objective For diagnostic purposes and a better understanding of the pathophysiology of inner ear hearing disorders it would be of great interest to have parameters available that indicate inner ear hypoxia. In animal studies typical hypoxia-related alterations of the 2f1–f2 distortion product otoacoustic emissions (DPOAE) such as a reversible level decrease and destabilization could be demonstrated. The goal of this study was to investigate whether these hypoxia-associated alterations can also be observed in humans because this might help develop a new diagnostic tool for patients with inner ear disorders. Methods In 16 volunteers DPOAE levels were continuously measured at first under normal room air conditions, during and after 8.5 h of oxygen deprivation (13% O2) and during re-oxygenation. Saturation of oxygen of arterial blood (SaO2) was monitored. Results The mean SaO2 during the hypoxic interval was 78%. A significant decrease in DPOAE level under hypoxia occurred in five different test persons at one or more frequencies (f2 = 1, 1.5, 2, 3, and 4 kHz). A destabilization of the DPOAE level with considerable fluctuations during hypoxia was observed in nine subjects at one or more frequencies. Furthermore, the so called ‘post hypoxia effect’ could be observed in five participants. Conclusion The observations made here have been described similarly in animal studies and seem to be characteristic of metabolic disorders of the cochlea caused by hypoxia. To our knowledge, this is the first study to examine DPOAE level alterations over time in humans under conditions of normobaric hypoxia. If DPOAE destabilization is observed in a clinical setting in patients with certain inner ear hearing disorders hypoxia can be suspected as one underlying pathophysiological cause which might influence treatment decisions.

Objective Although many reports describe the short-term hearing outcomes of surgically managed labyrinthine fistulae, the long-term results remain unknown. We reviewed the long-term postoperative hearing outcomes of 14 ears of patients with cholesteatoma and labyrinthine fistulae. Methods Between 1996 and 2010, 84 patients with cholesteatoma and labyrinthine fistula underwent tympanoplasty at Hyogo College of Medicine Hospital. Fistulae were located in the lateral semicircular canal in all patients and in the superior semicircular canal in one. Fourteen patients were followed up for more than 5 years. Results The postoperative air-bone gap was ≤10 dB in one patient, between 11 and 20 dB in seven, between 21 and 30 dB in four, and ≥31 dB in two. Mean bone-conduction hearing levels on the operated side had deteriorated by 3, −1 and −2 dB at 1, 2 and 4 kHz, respectively at 1 year postoperatively, and by 8, 6 and 2 dB at 1, 2 and 4 kHz, at 5 years postoperatively. Bone-conduction hearing levels at 1 and 2 kHz were significantly deteriorated at 5 years postoperatively, compared with baseline and 1 year (P

Summary Introduction: Temporal bone fracture is a common complication of high-energy cranial trauma. Labyrinth involvement is rare, but there is a risk of perilymphatic rupture that is often underes- timated on initial clinical examination due to the predominance of neurological and/or somatic symptoms. Case report: A patient presented with overlooked perilymphatic fistula, decompensated by hyperpressure effort due to poorly adapted management. Discussion: Following a review of the literature on post-traumatic pneumolabyrinth, overall management (from diagnosis to treatment, via prevention advice) was analyzed. A constructive critique of the patient's treatment was thus made. Conclusion: We argue for a systematic management protocol in cranial trauma with temporal bone fracture, comprising ENT examination, millimetric-scale cross-sectional imaging of the fracture site, and standardized counseling to prevent late complications. © 2013 Published by Elsevier Masson SAS.

Background Post-decompression shock with plasma volume deficit is a very rare event that has been observed under extreme conditions of hypobaric and hyperbaric exposure in aviators and professional divers. Case Report We report a case of severe hypovolemic shock due to extravasation of plasma in a recreational scuba diver presenting with inner ear decompression sickness. Impaired endothelial function can lead to capillary leak with hemoconcentration and hypotension in severe cases. This report suggests that decompression-induced circulating bubbles may have triggered the endothelial damage, activating the classic inflammatory pathway of increased vascular permeability. Conclusion This observation highlights the need for an accurate diagnosis of this potentially life-threatening condition at the initial presentation in the Emergency Department after a diving-related injury. An elevated hematocrit in a diver should raise the suspicion for the potential development of capillary leak syndrome requiring specific treatment using albumin infusion as primary fluid replacement.

Purpose of the study The aim of the present study was to evaluate the ototoxicity of Burow solution. Procedures Compound action potentials (CAPs) of the eighth nerve were measured before and 30 minutes after the application of the Burow solution in the middle ear cavity. Results Use of the original Burow solution (pH 3.5) for 30 minutes caused a significant reduction of click sounds. A 2-fold diluted Burow solution (pH 4.4) for 30 minutes caused no reduction in CAP threshold. Burow solution, pH adjusted to 4.5, caused no changes in CAP threshold at 30 minutes. At 24 hours, Burow solution (pH 3.5) caused complete abolition of CAP. Conclusions Burow solution is ototoxic in the guinea pig when applied in the middle ear cavity for 30 minutes or longer. In the clinical settings, it is advisable to avoid allowing the solution to contact the round window for extended times.

Superior semicircular canal dehiscence syndrome (SSCDS) is a set of symptoms, related to vestibular and/or auditory, which dues to a dehiscence of bone at the superior semicircular canal. We reported an extremely rare case with idiopathic bony dehiscence at the lateral semicircular canal, which presents the similar symptoms with SSCDS, and performed a pertinent literature review. A 76-year-old male patient complained experiencing vertigo and autophony caused by loud noises, with a sign of horizontal eye movements for the stimuli of loud noise to his right ear. HRCT scanning presented a solitary 2.0 mm bony defect at the right lateral semicircular canal. For a chief complaint of sound-/pressure-induced vertigo and/or oscillopsia and/or auditory symptoms, semicircular canal dehiscence syndrome should be considered. Then the doctor could presume the location of the dehiscence via the way of the oscillopsia. If the dehiscence lying the lateral semicircular canal, in clinical radiology, both axial and coronal images to be produced though the lateral semicircular canal plane in a parallel and perpendicular way should be carefully assessed for making diagnosis.

This article presents a detailed case report of a patient who was diagnosed with superior canal dehiscence at 37 years of age, but who had a suspicious history for that syndrome from at least 10 years of age. The authors hypothesize several reasons for this late diagnosis, with the goal of helping pediatricians, otolaryngologists, and neurologists consider this syndrome in their differential diagnosis of children, adolescents, or adults experiencing dizziness.

Cerebellar lesions may present with gravity-dependent nystagmus, where the direction and velocity of the drifts change with alterations in head position. Two patients had acute onset of hearing loss, vertigo, oscillopsia, nausea, and vomiting. Examination revealed gravity-dependent nystagmus, unilateral hypoactive vestibulo-ocular reflex (VOR), and hearing loss ipsilateral to the VOR hypofunction. Traditionally, the hypoactive VOR and hearing loss suggest inner-ear dysfunction. Vertigo, nausea, vomiting, and nystagmus may suggest peripheral or central vestibulopathy. The gravity-dependent modulation of nystagmus, however, localizes to the posterior cerebellar vermis. Magnetic resonance imaging in our patients revealed acute cerebellar infarct affecting posterior cerebellar vermis, in the vascular distribution of the posterior inferior cerebellar artery (PICA). This lesion explains the gravity-dependent nystagmus, nausea, and vomiting. Acute onset of unilateral hearing loss and VOR hypofunction could be the manifestation of inner-ear ischemic injury secondary to the anterior inferior cerebellar artery (AICA) compromise. In cases of combined AICA and PICA infarction, the symptoms of peripheral vestibulopathy might masquerade the central vestibular syndrome and harbor a cerebellar stroke. However, the gravity-dependent nystagmus allows prompt identification of acute cerebellar infarct.

Resumen Introduccion La via intratimpanica para el tratamiento de enfermedades laberinticas es un procedimiento poco agresivo y ambulatorio, que maximiza las concentraciones de farmaco en la coclea y minimiza su difusion sistemica. A traves de esta revision se propone actualizar las tecnicas de aplicacion y los resultados que ofrecen los diferentes farmacos para las enfermedades cocleovestibulares. Se expone nuevas perspectivas futuras en cuanto al desarrollo de farmacos y de la terapia genica. Material y metodo Se analiza la literatura hasta la fecha segun las bases medicas MEDLINE y EMBASE. Se ha considerado los articulos relacionados con la tecnica de instilacion, los resultados en el tratamiento esteroideo y aminoglucosido (gentamicina) y articulos aislados sobre nuevos farmacos o vias de administracion. Resultados La terapia esteroidea ha demostrado su eficacia en los sintomas cocleovestibulares como pauta de rescate tras corticoterapia intravenosa en la sordera subita y en el control de la enfermedad de Meniere. La administracion de gentamicina intratimpanica en pautas a demanda aporta un adecuado control del vertigo en el 80% de los pacientes y un riesgo de hipoacusia del 0–25% en la enfermedad de Meniere. Conclusiones La via intratimpanica es un procedimiento eficaz en el control de enfermedades cocleovestibulares, como la sordera subita y la enfermedad de Meniere. Junto con el tratamiento esteroideo y gentamicina, perspectivas futuras podrian ampliar las indicaciones de su uso y un nuevo arsenal terapeutico farmacologico y genico.

Summary Objective Osteoma occurs almost exclusively in the head and neck region, only rarely developing into the internal auditory canal. We report an incidental finding of a case in the radiological evaluation of a patient with left hemifacial spasm. Patients and methods A 79-year-old woman consulted for left hemifacial spasm associated with left anacusis. Symptoms occurred up to 30 years prior to the first radiological investigations. Computed tomographic (CT) and magnetic resonance (MRI) images were taken. Results MRI demonstrated a left internal auditory canal lesion with spontaneous hypointense signal on T1- and T2-weighted images. CT showed the lesion as a typically dense and opaque osteoma. It measured 0.6 cm in maximum size. No surgery was performed because of the slow-growing features of the lesion. Clinical monitoring was recommended. Conclusion Osteomas are diagnosed incidentally in asymptomatic patients. Our case was symptomatic and raised the question of surgical management. This article discusses the presentation and management associated with this exceptional osteoma location.

Intratympanic drug delivery for labyrinth diseases is a non-aggressive outpatient procedure where drugs reach high concentrations in the cochlea and minimum systemic diffusion. The aim of this review is to update the delivery techniques and report on the results obtained with different substances in cochleovestibular disorders. New perspectives in drug development and gene therapy are discussed.We have analyzed the literature published to date using the MEDLINE and EMBASE databases. The categories chosen for the review where the delivery techniques, the results using corticosteroids and aminoglucosides (gentamicin) and isolated papers related with new drugs or pathways to introduce the substance in the inner ear.Intratympanic steroid therapy has been shown to be effective for cochleovestibular symptoms after failure of systemic steroids for sudden deafness and for control of Ménière's disease. Intratympanic gentamicin using a titration method showed vertigo control in 80% of the patients with a 0-25% risk of hearing impairment in Ménière's disease.Intratympanic delivery is an effective procedure for the control of cochleovestibular disorders such as sudden deafness and Ménière's disease. Future perspectives could increase the indications for steroid and gentamicin treatment and open the door to new drugs and gene therapy.