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Cochlear implant fixation using a subperiosteal tight pocket without either suture or bone-recess technique

Jethanamest, Daniel; Channer, Guyan A; Moss, William J; Lustig, Lawrence R; Telischi, Fred F
Objective: To assess the complication and migration rates associated with fixation of cochlear implant receiver stimulators using a subperiosteal tight pocket without either suture fixation or bone recession. Study Design: Dual institution retrospective case review Methods: A retrospective case review was conducted at two tertiary referral centers. All patients who underwent cochlear implantation with device fixation using a subperiosteal tight pocket without suturing over the device or recessing of the receiver stimulator in bone were identified. There was a minimum follow-up period of six months. Outcome measures included intraoperative and postoperative complications, including evidence of device migration associated with interference with external device use or need for revision surgery. Other outcome measures included soft tissue flap complications. Results: Sixty-two patients were identified with a mean age of 39 years, (range 1.5-95 years). The average follow-up period was 32.6 months (range 6-120 months). Device manufacturers included Cochlear Corporation (n=44), MED-EL (n=12) and Advanced Bionics (n=6). There were no associated intraoperative complications related to subperiosteal pocket fixation of the receiver stimulator, and no cases of migration were identified. Conclusion: Fixation of the cochlear implant receiver stimulator using a subperiosteal tight pocket without either suture fixation or bone recession has been demonstrated to be feasible across a range of patient demographics and cochlear implant devices. This method of fixation appears to allow for an efficient and minimally invasive approach without compromising patient safety or device performance.
PMID: 24114870
ISSN: 0023-852x
CID: 936792

Cochlear Implant Outcomes in Neurofibromatosis Type 2: Implications for Management

Deep, Nicholas L; Patel, Evan J; Shapiro, William H; Waltzman, Susan B; Jethanamest, Daniel; McMenomey, Sean O; Roland, John Thomas; Friedmann, David R
OBJECTIVE:To describe our institutional experience with cochlear implantation (CI) for rehabilitation of hearing loss in Neurofibromatosis type 2 (NF2) patients. STUDY DESIGN/METHODS:Retrospective review between 1989 and 2019. SETTING/METHODS:Tertiary-care center. PATIENTS/METHODS:Twenty-four patients (67% female, mean age 45.6years) with NF2. Management of their ipsilateral vestibular schwannoma included microsurgery (n=12), stereotactic radiation (n=5), and observation (n=7). INTERVENTIONS/METHODS:Cochlear implantation. MAIN OUTCOME MEASURES/METHODS:Ability to obtain open-set speech, daily device usage and long-term device benefit. RESULTS:All patients achieved some degree of sound awareness with CI. Nineteen patients (79%) achieved open-set speech understanding with a mean word-recognition score of 43% (range 0-88%). Patients with tumors 1.5 cm or less demonstrated the better speech understanding, without significant differences among treatment modalities. For tumors greater than 1.5 cm, patients who underwent microsurgery had a lower rate of open-set speech understanding compared to those treated with radiation or observation. Regular daily device use in 83% of patients was found. Long-term use (>10years) was observed in several patients, though some ultimately required reimplantation with an auditory brainstem implant due to progressive tumor growth. Mean follow-up duration was 4.1 years (range 0.4-15). CONCLUSIONS:Cochlear implantation can be an effective treatment for hearing loss in NF2 patients provided the cochlear nerve is intact, regardless of prior management for the ipsilateral tumor. The degree of benefit varies and is influenced by tumor size. Management strategies that preserve the cochlear nerve maximize the interval during which a CI could be of benefit to NF2 patients.
PMID: 33351557
ISSN: 1537-4505
CID: 4726462

Cochlear Implantation in Sporadic Vestibular Schwannoma and Other Retrocochlear Pathology: A Case Series

Patel, Evan J; Deep, Nicholas L; Friedmann, David R; Jethanamest, Daniel; McMenomey, Sean O; Roland, John Thomas
OBJECTIVE:To describe outcomes with cochlear implantation (CI) for rehabilitation of hearing loss in patients with sporadic vestibular schwannomas (VS) and other retrocochlear pathologies. STUDY DESIGN/METHODS:Retrospective review. SETTING/METHODS:Tertiary-care center. PATIENTS/METHODS:Twenty three cases in 19 patients (53% men, mean age 55.8 yr) with non-neurofibromatosis type 2 related retrocochlear pathology. INTERVENTIONS/METHODS:Unilateral or bilateral CI. MAIN OUTCOME MEASURES/METHODS:Word recognition score, device usage. RESULTS:Etiology of deafness included sporadic VS (n = 9, 39%), radiation after head and neck or central nervous system (CNS) malignancy (n = 8, 35%), superficial siderosis (n = 3, 13%), neurosarcoidosis (n = 2, 9%), and pontine stroke (n = 1, 4%). Mean follow-up duration was 2.3 years (standard deviation [SD] 3.0; range, 0.2-9.4). Auditory perception was achieved in 20 out of 22 patients (91%) who have been activated. Mean WRS in patients with sporadic VS was 18% (SD 20; range, 0-44). Mean WRS in patients with non-VS retrocochlear pathology was 55% (SD 30; range, 0-94). Data logs showed 7.0 h/d of average use (SD 4.3; range, 0-13). CONCLUSIONS:Appropriately selected patients with retrocochlear pathology may benefit from CI so long as the patient has a cochlear fluid signal and an intact cochlear nerve. Patients with sporadic VS patients and normal contralateral hearing exhibited guarded outcomes with CI, whereas most patients with non-VS retrocochlear pathologies demonstrated open-set speech understanding scores comparable to or slightly worse than conventional CI candidates. Since variable performance benefit is observed with CI in patients with retrocochlear pathology, counseling is imperative to align patient expectations with realistic outcomes.
PMID: 33351558
ISSN: 1537-4505
CID: 4726472

Force and pressure measurements in temporal bones

Snels, Chantal; Roland, John Thomas; Treaba, Claudiu; Jethanamest, Daniel; Huinck, Wendy; Friedmann, David R; Dhooge, Ingeborg; Mylanus, Emmanuel
PURPOSE/OBJECTIVE:Some cochlear implant (CI) patients lose their residual hearing during surgery. Two factors that might play a role in residual hearing loss are the change in intracochlear hydraulic pressure and force on the cochlear wall during electrode insertion. The aim of this study is to investigate whether a difference in peak hydraulic pressure and peak force on the cochlear wall exists during a CI electrode insertion with different insertion techniques. MATERIALS AND METHODS/METHODS:Twenty fresh frozen temporal bones were used. Hydraulic pressure and force on the cochlear wall were recorded during straight electrode insertions with 1) slow versus fast insertion speed, 2) manual versus automatic insertion method and 3) round window approach (RWA) versus extended RWA (ERWA). RESULTS:When inserting with a slow compared to a fast insertion speed, the peak hydraulic pressure is 239% (95% CI: 130-399%) higher with a RWA and 58% (95% CI: 6-137%) higher with an ERWA. However, the peak force on the cochlear wall is a factor 29% less (95% CI: 13-43%) with a slow insertion speed. No effect was found of opening and insertion method. CONCLUSIONS:As contradictory findings were found for hydraulic pressure and force on the cochlear wall on insertion speed, it remains unclear which insertion speed (slow versus fast) is less traumatic to inner ear structure.
PMID: 33440250
ISSN: 1532-818x
CID: 4746972

First Report of Bilateral External Auditory Canal Cochlin Aggregates ("Cochlinomas") with Multifocal Amyloid-Like Deposits, Associated with Sensorineural Hearing Loss and a Novel Genetic Variant in COCH Encoding Cochlin

Basu, Atreyee; Boczek, Nicole J; Robertson, Nahid G; Nasr, Samih H; Jethanamest, Daniel; McPhail, Ellen D; Kurtin, Paul J; Dasari, Surendra; Butz, Malinda; Morton, Cynthia C; Highsmith, W Edward; Zhou, Fang
Pathogenic variants in COCH, encoding cochlin, cause DFNA9 deafness disorder with characteristic histopathologic findings of cochlin deposits in the inner and middle ears. Here, we present the first case of deafness associated with bilateral external auditory canal (EAC) cochlin deposits, previously unreported evidence suggestive of cochlin-derived amyloid formation, and a novel COCH variant. A 54-year-old woman presented with progressive sensorineural hearing loss and bilateral EAC narrowing by subcutaneous thickening. Excision and histologic evaluation of tissue from both EACs showed paucicellular eosinophilic aggregates containing multiple Congo red-positive foci with yellow and green birefringence under crossed polarization light microscopy. Mass spectrometry performed on both the Congo red-positive and Congo red-negative areas identified cochlin as the most abundant protein, as well as a low abundance of universal amyloid signature peptides only in the Congo red-positive areas. Peptides indicative of a canonical amyloid type were not detected. Electron microscopy showed haphazard, branched microfibrils (3-7 nm in diameter) consistent with cochlin, as well as swirling fibrils (10-24 nm in diameter) reminiscent of amyloid fibrils. Cochlin immunohistochemical staining showed positivity throughout the aggregates. Sequencing of the entire COCH gene coding region from the patient's blood revealed a novel variant resulting in a non-conservative amino acid substitution of isoleucine to phenylalanine (c.1621A>T, p.I541F) in the vWFA2 domain at the protein's C-terminus. Our findings reveal a new pathologic manifestation of cochlin, raise the possibility of previously undescribed cochlin-derived amyloid formation, and highlight the importance of thoroughly investigating all aggregative tissue findings in the practice of diagnostic pathology.
PMID: 31493294
ISSN: 1936-0568
CID: 4092562

Stapes Surgery Outcomes in Patients With Concurrent Otosclerosis and Superior Semicircular Canal Dehiscence

McClellan, Joseph; Nguyen, Anthony; Hamilton, Bronwyn; Jethanamest, Daniel; Hulla, Timothy E; Gupta, Sachin
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.
PMID: 32472923
ISSN: 1537-4505
CID: 4468452

Exoscope and Personal Protective Equipment Use for Otologic Surgery in the Era of COVID-19

Gordon, Steven A; Deep, Nicholas L; Jethanamest, Daniel
PMID: 32423361
ISSN: 1097-6817
CID: 4443902

Cochlear implantation in patients with neurofibromatosis type 2 and other retrocochlear pathology: A review of 32 cases over 25 years [Meeting Abstract]

Deep, N L; Patel, E; Shapiro, W H; Waltzman, S B; Jethanamest, D; McMenomey, S O; Roland, J T; Friedmann, D R
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
ISSN: 2193-6331
CID: 4387112

Otologic considerations in a full face transplant recipient

Cammarata, Michael J; Jethanamest, Daniel; Rodriguez, Eduardo D
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.
PMID: 30582171
ISSN: 1531-4995
CID: 3560332

Cochlear Implantation: An Overview

Deep, Nicholas L; Dowling, Eric M; Jethanamest, Daniel; Carlson, Matthew L
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.
PMID: 30931225
ISSN: 2193-6331
CID: 3783822