Changing Management of Intravestibular Schwannomas in the Era of Cochlear Implantation for Single-Sided Deafness.

Objective: Intralabyrinthine schwannomas (ILSs) are a rare cause of deafness. Patients with ILS confined to the semicircular canals and the vestibule (intravestibular schwannomas) are potential candidates for cochlear implantation for hearing rehabilitation, a new option for patients with unilateral hearing loss since the 2019 FDA approval of cochlear implant (CI) for single-sided deafness. In this report, we describe an evolving management approach for ILSs causing hearing loss.

Multicenter US Clinical Trial With an Electric-Acoustic Stimulation (EAS) System in Adults: Final Outcomes.

Objective: To demonstrate the safety and effectiveness of the MED-EL Electric-Acoustic Stimulation (EAS) System, for adults with residual low-frequency hearing and severe-to-profound hearing loss in the mid to high frequencies.

Study Design: Prospective, repeated measures.

Setting: Multicenter, hospital.

Superior Canal Dehiscence Syndrome Affecting 3 Families.

Importance: Superior canal dehiscence syndrome (SCDS) is an increasingly recognized cause of hearing loss and vestibular symptoms, but the etiology of this condition remains unknown.

Objective: To describe 7 cases of SCDS across 3 families.

Design, Setting, And Participants: This retrospective case series included 7 patients from 3 different families treated at a neurotology clinic at a tertiary academic medical center from 2010 to 2014.

Iatrogenic cholesteatoma arising at the bony-cartilaginous junction of the external auditory canal: a late sequela of intact canal wall mastoidectomy.

Objective: The objective of this study is to describe the presentation and management of a rare site of cholesteatoma recurrence at the bony-cartilaginous junction after intact canal wall (ICW) mastoidectomy.

Study Design: Retrospective case series

Setting: Tertiary referral center

Patients: Patients with cholesteatoma formation arising from the bony-cartilaginous (BC) junction of the external auditory canal (EAC) requiring surgical intervention were retrospectively identified across a 5-year period.

Intervention(s): All patients were treated surgically to eradicate the disease and reconstruct the bony defect when possible.

Surgical treatment of hearing loss when otosclerosis coexists with superior semicircular canal dehiscence syndrome.

Objective: Document a case of bilateral otosclerosis with coexisting bilateral superior semicircular canal dehiscence syndrome and the treatment of hearing loss in this setting.

Patient: A 33-year-old woman presented with bilateral mixed hearing loss; worse in the left ear. This was gradual in onset, and she denied dizziness.

Speech and language outcomes of cochlear implantation in children with isolated auditory neuropathy versus cochlear hearing loss.

Objective: Children with auditory neuropathy (AN) have variable hearing on pure tone testing, and the presence of speech and language delays often play a major role in the decision to offer cochlear implantation (CI) in this population. Despite this fact, the speech and language outcomes in this group after CI are not well described. This study compares speech and language outcomes after CI in a subset of the pediatric AN population that does not have a confounding cognitive disorder with those of their peers with cochlear hearing loss (CoHL).

Outcomes of cochlear implantation in children with isolated auditory neuropathy versus cochlear hearing loss.

Objective: Auditory neuropathy (AN) is a heterogeneous clinical entity for which the optimal method of auditory rehabilitation has been a matter of some debate. Such patients often do not receive sufficient benefit from hearing aids. Previous studies have shown that select AN patients may benefit from cochlear implantation (CI), but reported outcomes are variable and likely are a reflection of the heterogeneous patient population included under the umbrella diagnosis of AN.

Durability of hearing preservation after microsurgical treatment of vestibular schwannoma using the middle cranial fossa approach.

Object: The middle cranial fossa (MCF) approach is a microsurgical technique described as a primary option in the treatment of small, intracanalicular schwannomas involving the eighth cranial nerve. Excellent rates of complete tumor resection, hearing preservation, preservation of facial nerve function, and low complication rates have been reproduced using this technique. However, the durability of hearing preservation attained using the various treatment options has not been adequately assessed.

Squamous cell carcinoma involving the temporal bone: lateral temporal bone resection as primary intervention.

Objective: To provide an up-to-date review of treatment and outcomes of patients with squamous cell carcinoma (SCCA) involving the temporal bone.

Design: Retrospective cohort study of all patients treated at our institution for SCCA of the temporal bone between 1995 and 2007 with follow-up until 2011. Factors evaluated were demographics, presenting findings, follow-up time, previous treatment, workup, stage, surgical intervention, adjuvant therapy, histopathologic findings, recurrence, and survival.

Electrocochleography as a diagnostic and intraoperative adjunct in superior semicircular canal dehiscence syndrome.

Objective: To determine the electrocochleographic characteristics of ears with superior semicircular canal dehiscence (SSCD) and to examine its use for intraoperative monitoring in canal occlusion procedures.

Study Design: Case series.

Setting: Academic medical center.

Transcanal labyrinthectomy for intractable vertigo after unilateral cochlear implantation.

Objective: Document the use of transcanal labyrinthectomy to treat disabling attacks of vertigo after unilateral cochlear implantation.

Patient: A 46-year-old woman with severe-profound bilateral sensorineural hearing loss secondary to enlarged vestibular aqueducts underwent cochlear implantation for her right ear with a Nucleus Freedom device. The surgery was uneventful, and postoperative imaging confirmed that the electrode was positioned properly.

Cochlear implantation in the presence of a programmable ventriculoperitoneal shunt.

Objective: Document challenges of cochlear implantation in the presence of a ventriculoperitoneal shunt (VPS) with a programmable valve.

Patient: A 7-month-old, postmeningitic, hydrocephalic infant with profound sensorineural hearing loss.

Intervention: Ventriculoperitoneal shunt revision with a nonprogrammable valve and bilateral cochlear implantation.

Autoantibodies to recombinant human CTL2 in autoimmune hearing loss.

Objectives/hypothesis: Choline transporter-like protein 2 (CTL2), a 68-72 kDa inner-ear membrane glycoprotein, is a candidate target antigen in autoimmune hearing loss (AIHL). The objective of this study was to test recombinant human CTL2 as a potential target for the detection of human autoantibodies in patients with AIHL.

Study Design: In vitro assay development.

Role of electrically evoked auditory brainstem response in cochlear implantation of children with inner ear malformations.

Objective: To evaluate the usefulness of promontory electric auditory brainstem response (EABR) testing in children with inner ear malformations before cochlear implantation indicated by postoperative speech performance.

Study Design: Retrospective analysis.

Setting: Tertiary academic cochlear implant center.

Facial nerve outcomes in middle cranial fossa vs translabyrinthine approaches.

Objective: To compare the final facial nerve outcomes between middle cranial fossa (MCF) vs translabyrinthine (TL) resection of size-matched vestibular schwannomas.

Study Design And Setting: Retrospective case review at a tertiary care hospital. All patients who underwent resection utilizing either MCF or TL approaches with tumors 18 mm or smaller and complete data were included in the analysis.

Corticosteroid response and supporting cell antibody in autoimmune hearing loss.

Objective: To determine whether antibodies to supporting cells are associated with response to corticosteroids in patients with autoimmune sensorineural hearing loss.

Design: Prospective analysis of antibody to inner-ear antigens.

Setting: Collaborating otology practices in Pennsylvania, Michigan, and Indiana.

Prediction of long-term facial nerve outcomes with intraoperative nerve monitoring.

Objective: Evaluate the utility of a statistical equation using two independent intraoperative monitoring parameters in predicting long-term facial nerve function.

Study Design: Retrospective case review.

Setting: Tertiary care hospital.

Cochlear implantation in the chronically diseased ear.

Purpose Of Review: The presence of chronic ear disease in patients with profound SNHL presents a challenge to the otologic surgeon and was considered, in the past, a contraindication to cochlear implantation. This review discusses options for cochlear implantation in patients with chronically diseased ears.

Recent Findings: Several management options are available for cochlear implantation in patients with chronic suppurative otitis media (CSOM) and severe to profound sensorineural hearing loss (SNHL).

Effects of trigeminal ganglion stimulation on the central auditory system.

A projection from the trigeminal ganglion to the ventral cochlear nucleus (VCN) of the guinea pig was recently described. The synaptic terminals of this projection terminate in the granule and magnocellular regions of the VCN. Stimulation of this projection has been shown to result in activation of neurons of the ventral cochlear nucleus.

Identification and characterization of choline transporter-like protein 2, an inner ear glycoprotein of 68 and 72 kDa that is the target of antibody-induced hearing loss.

The Kresge Hearing Research Institute-3 (KHRI-3) antibody binds to a guinea pig inner ear supporting cell antigen (IESCA) and causes hearing loss. To gain insight into the mechanism of antibody-induced hearing loss, we used antibody immunoaffinity purification to isolate the IESCA, which was then sequenced by mass spectroscopy, revealing 10 guinea pig peptides identical to sequences in human choline transporter-like protein 2 (CTL2). Full-length CTL2 cDNA sequenced from guinea pig inner ear has 85.

Cochlear implantation in prelingually deaf children with ossified cochleae.

Objective: To evaluate the effects that degree of cochlear ossification has on performance of prelingually deafened children who receive cochlear implants.

Study Design: A matched-pairs analysis comparing speech perception results obtained 6 and 24 months after implant by children with ossified and nonossified cochleae. Additionally, long-term performance was evaluated in patients with follow-up periods longer than 24 months.

External auditory canal closure in cochlear implant surgery.

Objective: To evaluate surgical techniques and complications associated with external auditory canal (EAC) closure in cochlear implant surgery.

Study Design: Retrospective case review.

Setting: Tertiary referral center with a large cochlear implant program.

Cochlear implantation in chronic suppurative otitis media.

Objective: To evaluate management options for cochlear implantation in patients with chronic suppurative otitis media.

Study Design: Retrospective case review.

Setting: Tertiary referral center with a large cochlear implant program.