Management of a Complex Case of a Recurrent Paraganglioma.

Paragangliomas are rare, slow-growing, hypervascular, catecholamine-secreting neuroendocrine tumors arising from the paraganglia. Paragangliomas are rarely found in the head and neck and are typically benign, presenting as a painless, slow-growing mass. Surgical extirpation in combination with long-term surveillance has been long regarded as the standard of care; however, the advances in imaging, radiation therapy, and embolization techniques have improved diagnostic and therapeutic modalities. We present a case of an 87-year-old female who had previously undergone resection of a paraganglioma in 1998, with no evidence of disease in 2002. Eighteen years later, the patient presented to the clinic with otogenic complaints. Imaging showed an expansive mass from the jugular foramen with bone destruction and opacification within the ear canal. The patient opted for observation. The patient eventually presented to the emergency room with neurologic manifestations. Imaging showed a cerebellar abscess prompting emergency drainage. Intraoperative cultures grew Prevotella loescheii and S. intermedius, and the patient was started on 6 weeks of IV antibiotic therapy. Debulking of the paraganglioma was performed followed several months by mastoid and ear canal obliteration; however, the patient experienced complications, including dehiscence of the external auditory canal and Clostridium difficile infection. The patient was eventually treated successfully, marked by a reduction in complaints, a return to baseline activities, and imaging showing no increase in tumor size.

Transcanal Excision of Exostoses: Large Series Comparing Bone Removal Methods.

OBJECTIVE: External auditory exostosis (EAE) is a condition of progressive temporal bone growth into the external auditory canal most commonly from repeat cold water and wind exposure. Several tools have been utilized for EAE excision with varying implications for intra- and postoperative complications. However, comparisons of osteotome and microdrill are made difficult due to the few published cases and intervariability between surgeons. Furthermore, evidence is needed to analyze the safety of novel supplemental tools such as the piezoelectric bone-cutting device. STUDY DESIGN: Retrospective chart review. SETTING: Medical clinic and surgery center. METHODS: A total of 413 subjects representing 472 ears met the inclusion criteria. Of which 159 ears were operated on using osteotome alone (OA), 271 using osteotome with a drill (OD), and 42 with osteotome with piezoelectric (OP). Charts were analyzed for the most reported intraoperative complications and postoperative symptoms and complications. RESULTS: There were no significant differences in the rate of tympanic membrane perforations nor in total intraoperative complications between OA, OD, or OP. The OD group contained the only nonperforation intraoperative event. OA had the lowest or near lowest incidence of all symptoms analyzed. OA showed a significantly lower incidence of tinnitus when compared to OD and OP. CONCLUSION: We found that OA performed the best, though not statistically significant in most measures, with regard to mitigating rates of complications postsurgery. Our findings suggest OA provides lower risk intraoperatively and postoperatively for patients undergoing transcanal exostosis excision.

Totally Implantable Hearing Implant Revision Surgery Indications and Outcomes.

OBJECTIVE: Analyze indications, findings, and outcomes of revision procedures for a totally implantable active middle ear implant (AMEI) over the past 17 years. STUDY DESIGN: Retrospective review. SETTING: Single institution, private practice setting. SUBJECTS: Adult patients using a totally implantable AMEI called the Esteem middle ear implant by Envoy Medical who needed revision to their original implant between 2005 and 2022. Thirty-nine patients had 60 revision procedures. INTERVENTION: History of a totally implantable AMEI revision procedure. Indications for revision were related to either surgical wound issues or reduced device function. OUTCOME MEASURES: Device diagnostics, audiometric outcomes, and documented subjective report. RESULTS: Surgical site wound issues were readily corrected in the majority of patients; most had risk factors for wound breakdown. For sterile wound dehiscence, it was not always necessary to remove the sound processor for an extended time to promote healing. Outcomes for revision procedures because of device issues ranged from 31 to 100% success rate, depending on the cause of issue. The most common indication for revision was reduced device function caused by scar band and/or fibrotic tissue accumulation in the middle ear. CONCLUSION: Revision of fully implantable AMEI can be successful for many indications. Appropriate preoperative device troubleshooting and medical evaluation is helpful in identifying the issue and determining surgical approach. Suboptimal device function and feedback can sometimes be improved by removing tissue around the implant, although permanency of this procedure varies among patients.

Penetration of the Vestibule Following a History of Stapedectomy.

This case study describes a 72-year-old female with a history of stapedectomy 40 years prior. She presented experiencing vertigo, fogginess, and imbalance for 9 months. Computed tomography (CT) imaging revealed that the prosthesis was displaced into the vestibule by approximately 2.1 to 2.4 mm. The patient was presented with treatment options, including observation, removal, replacement of the prosthesis, and an oval window patch. The patient opted for observation as the symptoms she was experiencing did not significantly impact her quality of life. Although a stapedectomy may fail for a multitude of reasons, some of the most common causes are prosthesis displacement, especially out of the oval window into the middle ear or away from the incus. Incus necrosis may also play a substantial role in failure.

New Considerations for a Totally Implantable Active Middle Ear Implant.

Totally implantable active middle ear implants (AMEI) provide full-time hearing amplification to those with moderate to severe sensorineural hearing loss. While technology in conventional hearing aids (CHA) has advanced greatly, limitations remain for people with active lifestyles, limited vision or dexterity, and hearing aid fit issues. Furthermore, direct-drive properties of AMEI are thought to provide those with inefficient middle ear transfer functions a distinct advantage in delivering prescribed sound to the cochlea, ultimately improving speech understanding with less distortion. AMEI safety, stability, and efficacy outcomes are well documented and fitting strategies continue to improve. Recent studies show how simple aided speech testing can help predict whether a patient struggling with CHA may instead benefit from an AMEI. Totally implantable AMEI continue to be a viable option for patients who cannot or will not utilize traditional hearing aids.

Reimplantation of an Extruding Cochlear Implant.

Cochlear implants have successfully improved hearing in severe and profoundly deaf patients in addition to improving their quality of life. Implant extrusion and wound infection of a cochlear implant are one of the most common postoperative complications, although it does not occur frequently (1.5%-5% of cases).1,2 We present a case of an extruding cochlear implant with dehiscence that was successfully reimplanted, a procedure of which there have been few previous reports in the literature.

Speech Perception Gap Is Predictive of an Active Middle Ear Implant Advantage.

OBJECTIVE: Evaluate whether the difference between word recognition score (WRS) obtained unaided under earphone and with a hearing aid (HA), the speech perception gap (SPgap), is predictive of performance with a totally implantable active middle ear implant (AMEI). STUDY DESIGN: Retrospective review of systematically collected data. SETTING: Private otologic practice. PATIENTS: One hundred thirty six ears (133 patients) with sensorineural hearing loss implanted with an AMEI. Subjects were 73.5% men, with mean age = 61.3 years. INTERVENTION: Previous AMEI. MAIN OUTCOME MEASURES: WRS with earphone at phonetically balanced maximum (PB-MAX), and with HA and AMEI at 50 dB HL. Differences between earphone, HA and AMEI word recognition, grouped as positive or negative speech perception gap (+SPgap/-SPgap) between two devices. RESULTS: A +SPgap (WRS poorer with device than earphone) occurred in 86.0% of patients with HA and 42.3% with the AMEI. Mean SPgap = 24.7% with HA and 3.0% with AMEI (p ≤ 0.001). With a HA +SPgap, AMEI performance was better than HA by an average difference of 23.2% WRS, with AMEI more than HA in 82.9% of subjects. With HA -SPgap, the mean difference was 1.1% correct and AMEI more than HA in 47.4% of subjects (p ≤ 0.001 and p ≤ 0.002, for mean difference and percent of subjects, respectively). The larger the +SPgap (earphone-HA), the greater the advantage of AMEI over HA (r = 0.62, p ≤ 0.001). CONCLUSION: A speech perception gap between earphone PB-MAX and aided word recognition score suggests a patient may have better speech perception with an AMEI than with his/her hearing aid. This information is useful for counseling patients who are not performing well or are unsatisfied with their hearing aid.

Comparing Light-Driven Contact Hearing Aids with Acoustic Hearing Aids: Data and Clinical Tips.

INTRODUCTION: Contact hearing aids (CHA) offer potential advantages over conventional acoustic hearing aids (AHA) in terms of sound quality, reduced occlusion, and increased high-frequency gain. This study evaluates objective and subjective outcome data for CHA compared to AHA. METHODS: Clinical outcome data were retrospectively reviewed for patients wearing CHA. Individual and group data were analyzed and then compared to unaided and AHA data. Adverse effects, device complaints, and clinical utility were documented throughout the device fitting and follow-up process. RESULTS: CHA outcomes were better than unaided hearing and AHA for all patients completing the 3-month CHA trial period (45%). However, CHA return rate was high (55%), most often due to device repair and fit issues. Two patients returned the devices due to poor hearing benefit, though both were off-label audiometric candidates. Significantly more clinical time was required from both audiologists and otolaryngologists to successfully fit and maintain maximum device benefit. CONCLUSIONS: For proper audiometric and otologic candidates, this light-driven CHA can offer significant improvements in high-frequency sound fidelity, word recognition, and sound quality compared to AHA technology. Administering questionnaires was helpful in illuminating real-world improvements. Device fit, alignment, and quality control issues are improving, though still contributing to high device return rates at this time. Operational factors should be considered when incorporating CHA into a hearing health care practice.

Totally Implantable Active Middle Ear Implants.

The Envoy Esteem and the Carina system are the 2 totally implantable hearing devices. The Esteem is designed for patients with bilateral moderate to severe sensorineural hearing loss who have an unaided speech discrimination score of greater than and equal to 40%. The Carina system is designed for patients with moderate to severe sensorineural hearing loss or those with mixed hearing loss. The Esteem offers a technologically advanced method to provide improvements in hearing and is available in the United States, whereas the Carina system is currently not available in the United States.

Totally implantable active middle ear implant: Hearing and safety results in a large series.

OBJECTIVES: To evaluate hearing performance and safety measures in a large group of totally implantable active middle ear implant (AMEI) wearers. METHODS: Retrospective case review of 172 ears (166 patients) with sensorineural hearing loss undergoing implantation of a totally implantable AMEI. Pure-tone air and bone thresholds, pure-tone average, speech reception threshold (SRT), and word recognition scores (WRS) at phonetically balanced maximum and at 50 dB (WRS50) were assessed at baseline unaided, with a hearing aid (baseline aided [BLA]), with the implant at activation, and for best implant postoperative measurement. Adverse events were reviewed. RESULTS: Mean length of follow-up was 35.7 months. Compared to unaided, the implant provided significant gain at all frequencies through 6 kHz. Amount of gain was significantly better with the implant than BLA at 1,500 and 2,000 Hz (P ≤ .001). Implant SRT was also significantly improved compared to BLA (29.9 dB vs. 38.5 dB, P ≤ .001). At a 50-dB presentation level, WRS was significantly better with the implant than BLA (65.6% vs. 45.5%, P ≤ .001). Bone conduction thresholds were not affected by the implant. The most common adverse event was taste disturbance, which occurred in 39.1%. In subjects with preimplant tinnitus, 56.1% experienced resolution of their tinnitus. Revision surgery was required in 15.7%, and 4% were ultimately explanted. CONCLUSION: The implant provided significant hearing benefit compared to unaided and to the subjects' own hearing aids in most test categories, with minimal adverse events. Many subjects experienced complete resolution of their tinnitus after implantation. LEVEL OF EVIDENCE: 4 Laryngoscope, 128:2872-2878, 2018.

Totally implantable hearing system: Five-year hearing results.

OBJECTIVES/HYPOTHESIS: 1) To provide long-term hearing outcome measures of a totally implantable hearing system (implant) and compare to the baseline unaided (BLU) and baseline aided (BLA) conditions, and 2) discuss relevant safety measures. STUDY DESIGN: Prospective, nonrandomized, multicenter, single-subject-as-own-control design. METHODS: Fifty-one subjects with mild to severe sensorineural hearing loss were implanted between 2008 and 2009 and enrolled in this postmarket approval study in the setting of private and hospital-based practices. Forty-nine of these subjects completed the 5-year study, which included annual follow-ups. Primary effectiveness endpoints were speech reception threshold (SRT) and word recognition scores at 50 dB (WRS50s). Secondary effectiveness endpoints were WRSs and the Abbreviated Profile of Hearing Aid Benefit (APHAB) scores. Adverse Device Effects (ADEs) and Serious Adverse Device Effects (SADEs) reported during the study period and a comparison of bone conduction scores are submitted as safety measures. RESULTS: Compared to the BLA condition, 1) SRT scores were improved at every annual follow-up; 2) WRS50s were better in 49%, and the same in 41% at the 5-year follow-up; 3) WRSs were improved by 17% at the 5-year follow-up; and 4) APHAB scores were improved in most subscales at every annual follow-up. There were three SADEs in three subjects and 15 ADEs in 11 subjects. Bone conduction scores increased by 3.7 dB at the 5-year follow-up. Average battery life was 4.9 years. CONCLUSIONS: The implant compared favorably to the subjects' hearing aid throughout the 5-year period in all of the areas measured and was found to be safe. LEVEL OF EVIDENCE: 2b. Laryngoscope, 128:210-216, 2018.

The Envoy Esteem implantable hearing system.

This article discusses the Envoy Esteem implantable hearing system, a completely implantable hearing device. The device is indicated for patients older than 18 years with stable moderate to severe sensorineural hearing loss and good speech discrimination. The device is placed through an intact canal wall tympanomastoidectomy with a wide facial recess approach. The implant is typically activated at 6 to 8 weeks postoperatively and usually requires several adjustments for optimal performance. The sound processor/battery lasts 4.5 to 9.0 years and can be replaced through a minor outpatient procedure.

Normative data of incus and stapes displacement during middle ear surgery using laser Doppler vibrometry.

OBJECTIVES: To report normative data for incus and stapes motion using laser Doppler vibrometry (LDV) during middle ear surgery and to discuss possible limitations of the procedure. STUDY DESIGN: Institutional review board-approved, retrospective study of data from patients undergoing the Envoy Esteem implantable device at 3 institutions. SETTING: Quaternary referral health system. ELIGIBILITY CRITERIA: patients sucsessfully implanted with an Esteem device. METHODS: Data from 70 patients undergoing the Envoy Esteem procedure were reviewed. Sound at 100 dB and 50 frequencies ranging from 125 to 8,000 Hz were used during the procedure. LDV was performed to measure the displacment of the body of the incus and the posterior crus of the stapes to assess whether there was sufficient ossicular mobility to allow for implantation. RESULTS: The average displacement of the ossicles for all 70 patients was collected and analyzed. The trend was an average displacement around 100 nm from 125 to 500 Hz for both the incus and stapes with a linear decline starting at 1,000 Hz for the incus and 500 Hz for the stapes, with slightly greater displacement of the stapes at higher frequencies. CONCLUSION: This is the first article to report in vivo measures of ossicular mobility. These data help to understand the micromechanics of ossicular motion as well as the use and limitations of LDV. This information may lead to a prescreening process for implanted middle ear devices that function by overdriving the stapes.

Profound high-frequency sensorineural hearing loss treatment with a totally implantable hearing system.

OBJECTIVE: This study seeks to assess the efficacy of the Envoy Esteem totally implantable hearing device in treating profound high-frequency sensorineural hearing loss. PATIENTS: Five patients with profound high-frequency hearing loss participating in a prospective, multi-center, nonrandomized Food and Drug Administration clinical trial. INTERVENTION: Implantation with Esteem totally implantable hearing device and comparison to baseline unaided and aided conditions. MAIN OUTCOME MEASURE: Speech reception threshold and word recognition scores (WRS) at 50 dB HL presentation level. RESULTS: Preoperative speech reception threshold improved from an unaided 65 dB and aided 48 dB average to 26 dB with the Esteem at 12 months. WRS at 50 dB scores improved from an unaided 10% and aided 23% average to 78% postoperatively. CONCLUSION: The Esteem totally implantable middle ear hearing device provides appreciable functional gain and improvement in WRS to rehabilitate hearing in patients with a profound high-frequency sensorineural hearing loss.

Envoy Esteem Totally Implantable Hearing System: phase 2 trial, 1-year hearing results.

OBJECTIVES: (1) To assess outcomes of the Envoy Esteem Totally Implantable Hearing System as measured by hearing results compared with preimplant baseline unaided (BLU) and best-fit aided conditions (BLA) and (2) to determine safety of the device. STUDY DESIGN: Prospective, nonrandomized, multicenter, subject-as-own-control, US Food and Drug Administration (FDA) trial. SETTING: Private practice and hospital-based. SUBJECTS AND METHODS: Between January 2008 and August 2009, an FDA trial was performed at 3 sites. Fifty-seven subjects with bilateral, mild to severe sensorineural hearing loss, with discrimination greater than 40%, were implanted. Implanted components were (1) a sound processor and (2) 2 piezoelectric transducers (a sensor and a driver). A sound processor was implanted in the temporal bone. Transducers were coupled to the ossicles. Devices were activated 2 months postimplant. Hearing results were compared with ipsilateral BLU and BLA. RESULTS: Speech reception thresholds (SRTs) improved from BLA of 41.2 dB to 29.4 dB with the Esteem (P ≤ .001). Word recognition score (WRS) at 50 dB hearing level (HL) improved from BLA of 46.3% to 68.9% with the Esteem. Pure tone averages improved by 27 ± 1 dB (confidence interval, 30-25). There were no changes in bone conduction. QuickSIN results showed no change. There were 6 serious adverse device effects: 2 wound infections (1 resolved medically, 1 required explantation), 1 delayed facial paralysis that resolved with medication, and 3 revisions due to limited benefit. CONCLUSION: Phase 2 results at 12 months post implant demonstrated that (1) hearing results with the device are statistically superior to baseline best-fit hearing aids for SRT and WRS and (2) the device is safe.

The management of pediatric cholesteatoma.

Pediatric cholesteatoma can be congenital or acquired. The two types appear to be separate and distinct entities based on molecular studies and clinical course. Pediatric cholesteatoma behaves differently from cholesteatomas in the adult. This may have more to do with anatomic and physiologic differences than with the molecular structure of the cholesteatoma. Treatment requires an individualized approach taking into account the experience of the operative surgeon and the high risk of recidivism of this disease.