Faculty Bibliography

OBJECTIVE:To review outcomes of stapes surgery in patients with concurrent otosclerosis and superior semicircular canal dehiscence. STUDY DESIGN/METHODS:Retrospective case series. SETTING:/UNASSIGNED:Tertiary referral center. PATIENTS/METHODS:Patients with concurrent otosclerosis and superior canal dehiscence, confirmed by computed tomography (CT) imaging. INTERVENTION(S)/METHODS:Stapes surgery for conductive hearing loss. MAIN OUTCOME MEASURE(S):/UNASSIGNED:Postoperative air-bone gap (ABG), as well as the number of patients in whom surgery was deemed successful (postoperative ABG <10 dB HL). RESULTS:Five patients with superior canal dehiscence and concomitant otosclerosis who underwent surgical repair were identified. Mean preoperative ABG was 29.0 ± 6.4 dB HL. Mean postoperative ABG was 13.0 ± 13 dB HL. Three patients (60%) had a successful outcome, defined as postoperative ABG less than 10. One patient experienced unmasking of superior canal dehiscence vestibular symptoms. CONCLUSIONS:Patients with concurrent otosclerosis and superior canal dehiscence appear to have a lower likelihood of successful hearing restoration following stapes surgery. Patients should be counseled accordingly. Routine preoperative CT imaging before stapes surgery may be helpful to identify patients at risk for poor outcomes.

Objective: To describe cochlear implantation (CI) outcomes for rehabilitation of hearing loss due to retrocochlear pathology and/or its treatment.
Method(s): Retrospective review between 1995 and 2019 from a single tertiary care center of all patients with retrocochlear pathology who underwent CI. Demographics, clinical history, and audiometric data were reviewed. Study endpoints include (1) logged device use, (2) ability to achieve auditory perception, and (3) word recognition score (WRS) in the CI-only condition.
Result(s): Thirty-two patients (63% of females) with retrocochlear pathology were implanted at our center. The average age at implantation was 46.9 years (SD: 19, range: 13-80). Mean duration of deafness was 4.5 years (SD: 5.0, range: 0.4-19.0). Etiology of hearing loss included VS in 24 (75%), CNS malignancy treated with radiation in 4 (13%), intralabyrinthine schwannoma in 2 (6%), head and neck malignancy treated with radiation in 1 (3%), and superficial siderosis in 1 (3%). The mean preoperative PTA was 95.8 dBHL (SD 24.7) and WRS was 7.2% (SD 13.1). Of the 24 VSs, 21 were NF2-associated and 3 were sporadic. The mean tumor size was 1.64 cm (SD: 0.6, range: 0.5-2.6 cm). At the time of CI, 11 patients had prior microsurgery, 6 patients had prior radiation to the ipsilateral tumor, and 7 patients had stable tumors without prior surgery or radiation. Device use was classified as regular (>7 hours/day) in 15 (47%), limited (<7 hours/day) in 12 (38%), and nonuse is 5 (16%). The audiometric outcomes of 26 patients are reported, as the other 6 patients have been implanted too recently for review. Auditory perception was achieved in 24/26 patients. The two patients who failed to achieve auditory perception underwent prior surgery. Open-set speech recognition (WRS > 20%) was achieved in 18 patients. Meaningful sound perception but without significant open-set speech (WRS < 20%) was seen in six patients. Altogether, the mean WRS at most recent follow-up (mean: 3.4 years, SD: 1.8) for the observation, microsurgery, and radiation cohorts was 51% (SD: 15), 36% (SD: 28), and 39% (SD: 26), respectively. Over long-term follow-up, two patients experienced decline in CI performance associated with tumor regrowth and necessitated additional surgery; both underwent explantation of the CI and successful auditory brainstem implantation. The remaining patients have demonstrated durable benefit. A multivariate analysis is presented to evaluate the effects of the following variables: duration of deafness, time interval between treatment and CI, diagnosis of NF2, treatment cohort, pathology, and status of hearing in the contralateral ear.
Conclusion(s): In appropriately selected patients, cochlear implantation is feasible for the rehabilitation of hearing loss due to retrocochlear pathology and/or its treatment. Given the heterogeneity inherent to this population, outcomes are variable. In most cases, auditory percept was achieved and over half of the patients obtained open-set speech perception, irrespective of prior management and treatment

Facial transplantation provides a functional and aesthetic solution to severe facial disfigurement previously unresolved by conventional reconstruction. Few facial allografts have been ear containing; hence, there is limited knowledge of the postoperative otologic considerations. We describe the case of a 44-year-old man who underwent transplantation of the total face, eyelids, ears, scalp, and skeletal subunits in 2015 after an extensive thermal injury. We detail the patient's transition from osseointegrated prosthetic ears to an ear-containing facial allograft, and describe the unique surgical approach and challenges encountered. Subsequent bilateral revision meatoplasties were performed, which provided relief from stenosis of the external auditory meatus. Laryngoscope, 2018.

A cochlear implant (CI) is a surgically implanted device for the treatment of severe to profound sensorineural hearing loss in children and adults. It works by transducing acoustic energy into an electrical signal, which is used to stimulate surviving spiral ganglion cells of the auditory nerve. The past 2 decades have witnessed an exponential rise in the number of CI surgeries performed. Continual developments in programming strategies, device design, and minimally traumatic surgical technique have demonstrated the safety and efficacy of CI surgery. As a result, candidacy guidelines have expanded to include both pre and postlingually deaf children as young as 1 year of age, and those with greater degrees of residual hearing. A growing proportion of patients are undergoing CI for off-label or nontraditional indications including single-sided deafness, retrocochlear hearing loss, asymmetrical sensorineural hearing loss (SNHL) in adults and children with at least 1 ear that is better than performance cut-off for age, and children less than 12 months of age. Herein, we review CI design, clinical evaluation, indications, operative technique, and outcomes. We also discuss the expanding indications for CI surgery as it relates to lateral skull base pathology, comparing CI to auditory brainstem implants, and address the concerns with obtaining magnetic resonance imaging (MRI) in CI recipients.

Intralabyrinthine schwannomas (ILS) are rare tumors that frequently cause sensorineural hearing loss. The development and increased use of magnetic resonance imaging in recent years have facilitated the diagnosis of these tumors that present with otherwise nondiscriminant symptoms such as tinnitus, vertigo, and hearing loss. The following is a review of the presentation, pathophysiology, imaging, and treatment with a focused discussion on auditory rehabilitation options of ILS.

Children with hearing loss and additional disabilities can benefit from cochlear implants and other implantable auditory devices. Although each individual child must be evaluated, and families uniquely counseled on goals and realistic expectations, overall many gains and benefits are possible in this population. In this article, an overview of the considerations for this group is discussed and outcomes are reviewed, including auditory and speech measures as well as benefits in other skills and quality of life.

BACKGROUND:Measurement of the angular depth of insertion (aDOI) of cochlear implant electrode arrays has numerous clinical and research applications. Plain-film radiographs are easily obtained intraoperatively and have been described as a means to calculate aDOI. CT imaging with 3D reformatting can also be used for this measurement, but is less conveniently obtained and requires higher radiation doses, a particular concern in pediatrics. The extent to which plain-film and 3D CT image-based measurements are representative of the true position of the electrode within the cochlea is unknown. METHODS:Cochlear implantation was performed on 10 cadaveric temporal bones. Five bones were implanted with perimodiolar electrodes (Contour Advance TM, Cochlear, Sydney, Australia) and five were implanted with lateral wall electrodes (Slim Straight, Cochlear). The insertion depths of the electrodes were varied. Each bone was imaged with a radiograph and CT. aDOI was measured for each bone in each imaging modality by a neurotologist and a neuroradiologist. To obtain a 'gold standard' estimate of aDOI, the implanted temporal bones were embedded in an epoxy resin and methodically sectioned at 100 μm intervals; histologic images were captured at each interval. A 3D stack of the images was compounded, and a MATLAB script used to calculate aDOI of the most apical electrode. Measurements in the three modalities (radiograph, CT, and histology) were then compared. RESULTS:The average aDOI across all bones was similar for all modalities: 423° for radiographs, 425° for CT scans, and 427° for histology, indicating that neither imaging modality resulted in large systematic errors. Using the histology-measured angles as a reference, the average error for CT-based measures (regardless of whether the error was in the positive or negative direction) was 12°, and that for radiograph-based measures was 15°. This small difference (12 vs 15° error) was not statistically significant. CONCLUSION/CONCLUSIONS:Based on this cadaveric temporal bone model, both radiographs and CTs can provide reasonably accurate aDOI measurements. In this small sample, and as expected, the CT-based estimates were more accurate than the radiograph-based measurements. However, the difference was small and not statistically significant. Thus, the use of plain radiographs to calculate aDOI seems judicious whenever it is desired to prevent unnecessary radiation exposure and expense.

Introduction The aims of this study are to investigate a possible correlation between the time point at which peak hydraulic pressure and peak force on the cochlear wall appears during insertion of a cochlear implant electrode and to investigate whether a difference exists in maximum hydraulic pressure and maximum force on the cochlear wall during a fast and slow insertion, a manual and automatic insertion and an electrode insertion into a narrow or wide round window (RW) opening. Material and methods Twenty fresh frozen human temporal bones were used. Intracochlear hydraulic pressure and force on the cochlear wall were recorded during round window insertions of a straight electrode array with different insertion speeds, different insertion methods and with different widths of the opening of the RW. Results A statistical signifcant correlation between the time point at which peak hydraulic pressure and peak force on the cochlear wall appears was found (r=0.91, p<0.001). Furthermore, a slow insertion speed showed a higher hydraulic pressure and a higher force on the cochlear wall compared to a fast insertion speed (p<0.001). No statistically signifcant effect of insertion method or the width of the opening of the RW was found on hydraulic pressure and on force on the cochlear wall. Conclusions Peak hydraulic pressure and peak force on the cochlear wall during electrode insertion seems to appear at approximately the same time. Furthermore, a slow insertion speed seems to result in a higher intracochlear hydraulic pressure and a higher force on the cochlear wall