Efficient Delivery of Adeno-Associated Virus into Inner Ear Through Trans-Stapes Route in Adult Guinea Pig.

Adeno-associated virus (AAV) are potent vectors to achieve treatment against hearing loss resulting from genetic defects. However, the effects of delivery routes and the corresponding transduction efficiencies for clinical applications remain elusive. In this study, we screened AAV vectors of three serotypes (AAV 8 and 9 and Anc80L65) into the inner ears of adult normal guinea pigs through trans-stapes (oval window) and trans-round window delivery routes .

Auditory and speech performance after unilateral cochlear implantation for cochlear nerve canal stenosis.

To explore the correlation between the width of the bony cochlear nerve canal (CNC) and long-term auditory rehabilitation after unilateral cochlear implantation (CI) in pediatric patients with congenital deafness and bilateral cochlear nerve canal stenosis (CNCS). A retrospective review was performed on 10 patients with bilateral CNCS and bilateral congenital profound hearing loss who each underwent unilateral cochlear implantation. The width of the CNC was determined on computed tomography (CT) imaging and following CI, auditory and speech performance following CI were graded using categories of auditory performance (CAP), speech intelligibility rating (SIR), and the meaningful auditory integration scale (MAIS) at 24 months following implantation.

Myoelectric characteristics of tensor palatini and collapsibility of upper airway in OSA patients with different phenotypes under DISE.

Objective: This study aims to evaluate the combination of myoelectric characteristics of tensor palatini muscle (TP) and collapsibility of upper airway in obstructive sleep apnea (OSA) patients with different external phenotypes of collapse pattern at velum level under drug-induced sleep endoscopy (DISE).

Study Design: Case series with planned data collection.

Setting: Operation room.

Cochlear Nerve Canal Stenosis: Association With MYH14 and MYH9 Genes.

The bony cochlear nerve canal transmits the cochlear nerve as it passes from the fundus of the internal auditory canal to the cochlea. Stenosis of the cochlear nerve canal, defined as a diameter less than 1.0 mm in transverse diameter, is associated with inner ear anomalies and severe to profound congenital hearing loss.

Predicting outcome of velopharyngeal surgery in drug-induced sleep endoscopy by traction velum.

Background: Outcomes of surgical management of obstructive sleep apnea-hypopnea syndrome (OSAHS) can be difficult to predict preoperatively.

Aims/objectives: To study the effect of applying traction to the velum during drug-induced sleep endoscopy (DISE) in OSAHS patients, and to describe the use of traction velum in predicting surgical success.

Materials And Methods: 41 adult surgical patients with OSA (Friedman tongue position II and III) were evaluated by DISE.

An Update on Epidemiology and Management Trends of Vestibular Schwannomas.

Objective: To determine the current epidemiology and management trends for patients with vestibular schwannomas (VS).

Study Design: Retrospective cohort study.

Setting: The Surveillance, Epidemiology, and End Results (SEER) tumor registry.

Quantifying tinnitus suppression in cochlear implantation using tinnitus interval-limited tracking.

Objectives: Quantify the effects of cochlear implants (CI) on tinnitus suppression in patients with previous cochlear implantation using a novel audiologic sequence: Tinnitus Interval Limited Tracking (TILT).

Study Design: Prospective cohort study.

Methods: Consecutive patients with tinnitus and previous cochlear implantation for profound hearing loss underwent an audiologic testing sequence called TILT.

Fluctuating Sensorineural Hearing Loss.

Background: Several otologic conditions can present with fluctuating sensorineural hearing loss, including Ménière's disease, autoimmune inner ear disease, and enlarged vestibular aqueduct. Although these 3 etiologies vary greatly, distinguishing between these conditions at initial presentation can be challenging. Furthermore, initial treatment of these conditions is often similar.

Linked-read Sequencing Analysis Reveals Tumor-specific Genome Variation Landscapes in Neurofibromatosis Type 2 (NF2) Patients.

Hypothesis: We hypothesize that genomic variants including deletions, insertions, inversions, and tandem duplications beyond the changes in tumor suppressor NF2 gene affect gene expression of tumor-specific pathways in vestibular schwannomas (VS) patients with Neurofibromatosis type 2 (NF2), thus contributing to their clinical behavior.

Background: Genomic variation could reconfigure transcription in NF2 transformation process. Therefore, genome-wide high-resolution characterization of structural variants (SV) landscapes in NF2 tumors can expand our understanding of the genes regulating the clinical phenotypes in NF2-associated VS.

'Compassionate use' protocol for auditory brainstem implantation in neurofibromatosis type 2: Early House Ear Institute experience.

Objective: To report the preliminary outcomes of auditory brainstem implantation (ABI) under a compassionate use protocol for two ABI devices that are not approved by the US Food and Drug Administration.

Methods: A retrospective review was performed of neurofibromatosis type 2 (NF2) patients who underwent microsurgery for vestibular schwannoma (VS) and placement of either the Cochlear ABI541 or Med-El Synchrony ABIs. Peri-operative and device- related complications were reviewed.

Tinnitus Suppression After Auditory Brainstem Implantation in Patients With Neurofibromatosis Type-2.

Objective: To evaluate whether an auditory brainstem implant (ABI) can impact levels of tinnitus in neurofibromatosis type-2 (NF2) patients who have undergone translabyrinthine craniotomy for vestibular schwannoma (VS) removal and to evaluate the burden of tinnitus in these patients.

Study Design: A retrospective case series and patient survey.

Setting: Tertiary neurotologic referral center.

Symptomatic postsurgical traumatic neuromas.

Hypothesis: Growing nerve fibers from the stumps of amputated sensory nerves can form traumatic neuromas within inner or middle ear postsurgical fibrosis and may produce symptoms commensurate with the normal function of the nerve involved, that is, balance or pain.

Background: Microscopic traumatic neuromas have been identified in postoperative middle and inner ear fibrosis in the temporal bones of patients complaining of intractable pain or imbalance.

Methods: Postsurgical temporal bones having inner or middle ear traumatic neuromas were reviewed.

Treatment of recurrent and residual glomus jugulare tumors.

Residual and recurrent glomus jugulare tumors are rare but challenging. Treatment options include microsurgical resection, stereotactic radiotherapy, a combination of modalities, and "observation." Choice of treatment must be made on a case-by-case basis, considering patient age, health status, location and size of tumor, status of the lower cranial nerves, and, of course, patient desire.

Distribution of nonvestibular cranial nerve schwannomas in neurofibromatosis 2.

Objective: To describe the prevalence and location of cranial nerve schwannomas, other than bilateral vestibular schwannoma, in patients with neurofibromatosis 2 (NF2). The NF2 Natural History Consortium prospectively gathered cranial magnetic resonance imaging for 83 patients across 3 annual evaluations. The time between the first and last evaluation was approximately 3 years.

Management of cerebrospinal fluid leaks after acoustic tumor removal.

Objective: To present a logical algorithm for management of postoperative cerebrospinal fluid (CSF) leak that occurs after acoustic tumor removal, and to describe a method for eustachian tube resection.

Clinical Presentation: We present an algorithm in the form of a flow chart, describe middle fossa craniotomy for eustachian tube resection, and present three cases in which this technique was used.

Technique: For CSF leak, pressure dressing at the wound and bed rest for the patient are advised; lumbar drain is indicated if the leak does not resolve.

Facial palsy and fallopian canal expansion associated with idiopathic intracranial hypertension.

Objective: Describe neurotologic findings associated with idiopathic intracranial hypertension (IIH).

Study Design: Retrospective.

Setting: Tertiary referral center.

Prevention and treatment of cerebrospinal fluid leak after translabyrinthine acoustic tumor removal.

Objective: To determine the incidence rate of cerebrospinal fluid (CSF) leak after translabyrinthine acoustic tumor removal using titanium mesh cranioplasty and to compare with previous series and historical controls.

Study Design: Database analysis with historical controls.

Setting: Tertiary referral neurotologic private practice.

Controversies in building a management algorithm for vestibular schwannomas.

Purpose Of Review: The present review examines the various mainstream treatment options, benefits and risks, and controversies involved in developing a management algorithm for treatment of vestibular schwannoma.

Recent Findings: Advances in microsurgery and radiosurgery have made tremendous contributions to management of vestibular schwannoma; however, considerable controversy still exists. The auditory and facial nerve functional outcomes have improved with use of intraoperative monitoring for vestibular schwannoma removal and with lower radiosurgery doses; however, risks to the facial and auditory nerves still exist.

Meniere's disease: prevalence of contralateral ear involvement.

Objective: Determine the prevalence and time interval for conversion from unilateral to bilateral involvement in Meniere's disease and cochlear hydrops.

Study Design And Setting: Retrospective chart review in a tertiary otologic referral center.

Patients: 232 patients diagnosed with Meniere's Disease (n=186) or cochlear hydrops (n=46) between 1959 and 2001, who visited the clinic over a five-year period between 1997-2001 and have at least 2 audiograms more than 12 months apart.

Surgical salvage after failed irradiation for vestibular schwannoma.

Objectives/hypothesis: Compare vestibular schwannoma (VS) surgical outcome between patients with prior irradiation and those not previously treated.

Study Design: Retrospective review with matched control group.

Methods: Review of tumor adherence to the facial nerve, facial nerve grade, and complications in 38 patients with radiotherapy as a primary procedure before VS surgical removal and a matched random sample of 38 patients with primary surgery.