Search

To assess the clinical application of five recently published cochlear implant (CI) candidacy evaluation (CICE) referral screening tools through external validation.Retrospective cohort study.Tertiary otology/neurotology practice.Adults who underwent CICE between December 2020 and September 2021.CICE referral screening tools versus CI candidacy criteria.CICE screening tool performance, based on the ability to identify patients who met the CI candidacy criteria, was evaluated. CI candidacy criteria were defined as best-aided AzBio sentences at +10 signal-to-noise ratio and either 60% or less accuracy to reflect traditional criteria used in clinical settings or 40% or less accuracy (only patients 65 years or older) to reflect Medicare-eligible criteria.Screening criteria of proposed CICE referral tools vary widely across pure-tone average and word recognition scores. When screened by traditional criteria, the sensitivities and specificities of these referral tools varied from 40 to 77% and from 22 to 86%, respectively. When screened by Medicare-eligible criteria, sensitivities and specificities varied from 41 to 81% and from 24 to 91%, respectively. The screening tool proposed by Zwolan et al. ( Otol Neurotol 2020;41(7):895-900) demonstrated the best overall performance for traditional (Youden's J , 0.37; sensitivity, 62%; specificity, 75%) and Medicare-eligible patients (Youden's J , 0.44; sensitivity, 66%; specificity, 78%). All screening tools performed worse on the validation cohort compared with their respective development cohorts.Current tools for determining CICE referral have diverse screening criteria. These combinations of pure-tone average and word recognition score are modestly successful at identifying CI candidates.

To evaluate the impact of different open set sentence recognition tests in quiet, +10 dB signal to noise ratio (SNR), and +5 dB SNR in adult cochlear implant (CI) recipients above and below 65 years of age.Multi-institution, prospective, non-randomized, single-subject repeated measures design.Ninety six adults more than or equal to 18 years old with postlingual bilateral sensorineural hearing loss.Participants received a CI532 in one ear. Speech perception measures were evaluated before and 6-months after activation.Subjects completed consonant-nucleus-constant (CNC) words in quiet and AzBio sentences in noise using +10 and +5 dB SNR, and Montreal Cognitive Assessment (MOCA).Ninety six adult patients were enrolled (n = 70 older [≥65 yr], n = 26 younger [65 yr]). There was no difference in CNC scores (CI alone 58.4% versus 67.5%, p = 0.0857; best aided 66.7% versus 76.1%, p = 0.3357). Older adults performed worse on AzBio +10 dB SNR compared with younger patients (CI alone 37.4% versus 56.9%, p = 0.0006; best aided 51.4% versus 68.2%; p = 0.01), and in AzBio +5 dB SNR (CI alone 7.7% versus 11.2%, p = 0.0002; best aided 15.3% versus 22.3%, p = 0.0005). The magnitude of change in AzBio +10 dB SNR was significantly less in older adults in CI alone (15.3% versus 22.3%; p = 0.0493) but not best aided (21.5% versus 31.3%; p = 0.105). The magnitude of change was drastically worse in AzBio +5 dB SNR for older adults (CI alone 6.7% versus 22.1%, p = 0.0014; best aided 9.5% versus 21.5%; p = 0.0142). There was no significant difference in MOCA between the two age groups.While both older and younger patients have similar outcomes with respect to CNC word scores in quiet, the addition of noise disproportionally impacts older patients. Caution should be exercised testing the elderly in noise; testing in noise may disproportionally impact performance expectations and should be more carefully considered when used for candidacy criteria and counseling. Future studies need to further investigate the disproportionate effect of noise on candidacy testing and its impact on how elderly patients are qualified.

OBJECTIVE Describe audiologic outcomes in hearing preservation (HP) cochlear implant candidates using a slim modiolar electrode (SME). STUDY DESIGN Retrospective. SETTING Tertiary referral center. PATIENTS Two hundred three adult cochlear implant patients with preoperative low-frequency pure-tone average (LFPTA) ≤ 80 dB HL that received the SME. INTERVENTION Implantation with a SME electrode. MAIN OUTCOME MEASURES Primary outcome was postoperative HP, defined as LFPTA ≤80 dB HL. HP status was analyzed at "early" (activation or 3 mo) and "long-term" (6 or 12 mo) time frames using the patient's worst audiogram. Speech perception tests were compared between HP and non-HP cohorts. RESULTS Of the 203 HP candidates, the tip fold-over rate was 7.4%. The mean shifts in LFPTA at the "early" and "long-term" time points were 25.9 ± 16.2 dB HL and 29.6 ± 16.9 dB HL, respectively. Of 117 patients with preoperative LFPTA ≤60 dB HL, the early and long-term mean LFPTA shifts were 19.5 ± 12.3 dB HL and 32.6 ± 17.2 dB HL, respectively; early and long-term HP rates were 61.1% and 50.8%, respectively. For patients with preoperative LFPTA ≤80 dB HL, early and long-term HP rates were 45.5% and 43.7%, respectively. No significant difference was observed in postoperative speech perception performance (CNC, AzBio, HINT) at 3, 6, or 12 months between HP versus non-HP groups. CONCLUSIONS HP is feasible using the SME. While electroacoustic stimulation was not studied in this cohort, HP provided no clear advantage in speech perception abilities in this group of patients. The current reporting standard of what constitutes HP candidacy (preoperative LFPTA ≤80 dB HL) should be reconsidered.

Objective To evaluate the prevalence, surgical management, and audiologic impact of pulsatile tinnitus caused by sigmoid sinus dehiscence. Study design and setting Retrospective chart review at a tertiary care hospital. Patients Adults with unilateral pulsatile tinnitus attributable to sigmoid sinus dehiscence who underwent resurfacing between January 2010 and January 2020. Interventions Transmastoid sigmoid resurfacing. Main outcome measures Resolution of pulsatile tinnitus; audiologic outcomes; complications; tinnitus etiologies. Results Nineteen patients (89.4% women) had surgery for suspected sigmoid sinus dehiscence. The mean dehiscence size was 6.1 mm (range, 1-10.7 mm). Eight patients had concurrent sigmoid sinus diverticulum and one patient also had jugular bulb dehiscence. Only two patients (10.5%) had the defect identified by radiology. Low-frequency pure-tone average, measured at frequencies of 250 and 500 Hz, showed a significant median improvement of 8.8 dB following resurfacing (18.8 dB versus 10.0 dB, p = 0.02). The majority of patients had complete resolution of pulsatile tinnitus (16/19, 84.2%). Of those without complete resolution, two patients had partial response and one patient had no improvement. There were no significant complications. Of 41 consecutively tracked patients with a pulsatile tinnitus chief complaint, sigmoid pathology represented 32% of cases. Conclusions Sigmoid sinus dehiscence represents a common vascular cause of pulsatile tinnitus that, if properly assessed, may be amenable to surgical intervention. Sigmoid sinus resurfacing is safe, does not require decompression, and may improve low-frequency hearing. Radiographic findings of dehiscence are often overlooked without a high index of clinical suspicion. Its relationship with transverse sinus pathology and idiopathic intracranial hypertension remain unclear.

OBJECTIVE Hearing loss remains a significant morbidity for patients with vestibular schwannomas (VS). A growing number of reports suggest audibility with cochlear implantation following VS resection; however, there is little consensus on preferred timing and cochlear implant (CI) performance. DATA SOURCES A systematic literature search of the Ovid Medline, Embase, Scopus, and clinicaltrails.gov databases was performed on 9/7/2018. PRISMA reporting guidelines were followed. STUDY SELECTION Included studies reported CI outcomes in an ear that underwent a VS resection. Untreated VSs, radiated VSs, and CIs in the contralateral ear were excluded. DATA EXTRACTION Primary outcomes were daily CI use and attainment of open-set speech. Baseline tumor and patient characteristics were recorded. Subjects were divided into two groups: simultaneous CI placement with VS resection (Group 1) versus delayed CI placement after VS resection (Group 2). DATA SYNTHESIS Twenty-nine articles with 93 patients met inclusion criteria. Most studies were poor quality due to their small, retrospective design. Group 1 had 46 patients, of whom 80.4% used their CI on a daily basis and 50.0% achieved open-set speech. Group 2 had 47 patients, of whom 87.2% used their CI on a daily basis and 59.6% achieved open-set speech. Group 2 had more NF2 patients and larger tumors. CI timing did not significantly impact outcomes. CONCLUSIONS Audibility with CI after VS resection is feasible. Timing of CI placement (simultaneous versus delayed) did not significantly affect performance. Overall, 83.9% used their CI on a daily basis and 54.8% achieved open-set speech.

Objective To demonstrate successful surgical management of vestibular schwannomas via an exclusively endoscopic transcanal transpromontorial approach (EETTA). Patients Four patients with vestibular schwannomas. Interventions Surgical excision via EETTA. Main outcomes Technique refinements, tumor access, complete tumor removal, and patient morbidity. Results Three tumors were Koos grade I and one tumor was Koos grade II. All ears had non-serviceable hearing prior to surgery. The EETTA enabled access to the internal auditory canal and porus acousticus as well as limited access to the cerebellopontine angle. Gross total tumor resection was achieved in all cases. There were no intraoperative or postoperative complications and the mean hospital duration was 2.8 days. After a mean follow-up of 5.0 months, all cases had a good facial nerve outcome. Conclusions The EETTA can be successfully used for the management of small vestibular schwannomas in ears without serviceable hearing. Additional studies are needed to fully elucidate the risk-benefit profile of this minimally invasive approach.

OBJECTIVE CHARGE syndrome is associated with a variety of temporal bone anomalies and deafness. The lack of surgical landmarks and facial nerve irregularities make cochlear implantation in this population a challenging endeavor. This study aims to describe a safe and efficacious transcanal approach for cochlear implantation that obviates the need to perform a mastoidectomy and facial recess. PATIENTS Three children with profound hearing loss secondary to CHARGE syndrome. INTERVENTION Transcanal cochlear implantation with closure of the ear canal via a modified Rambo meatoplasty. MAIN OUTCOME MEASURE(S) Retrospective chart review of temporal bone anomalies associated with CHARGE syndrome, technical nuances of this transcanal approach, and cochlear implant outcomes. RESULTS The mean patient age was 2.5 years (range 1.5-3.8 yr). Two were male and two were left ears. All patients had a hypoplastic mastoid, semicircular canal aplasia, and had some degree of cochlear dysplasia. A full cochlear implant insertion was achieved in all cases, even in the presence of grossly abnormal middle ear and facial nerve anatomy. There were no intraoperative or postoperative complications. The mean follow-up was 12.4 months (range, 3.9-25.2 mo). All three patients use their device daily. Their guardians report improved vocalization and environmental awareness. CONCLUSIONS The modified Rambo transcanal approach provides a safe corridor for cochlear implantation in patients with CHARGE syndrome. This approach minimizes the anatomical variations associated with the syndrome and may reduce the risk of electrode extrusion. Implant outcomes in this patient population remain highly variable based on the patient's global cognitive capacity.

Objective To evaluate the audiometric outcomes following endoscopic ossicular chain reconstruction (OCR). Study design Retrospective case series. Setting Two tertiary referral centers. Patients Sixty two ears with ossicular discontinuity. Intervention(s) Endoscopic and microscopic OCR in patients with ossicular discontinuity. Main outcome measures Bone and air pure-tone averages (PTA), air-bone gap (ABG), and word recognition scores (WRS). Results Sixty two ears were included for analysis. Patients that underwent ossiculoplasty were subdivided based on prosthesis type (total ossicular replacement prosthesis [TORP] and partial ossicular replacement prosthesis [PORP], primary and staged ossiculoplasties, and surgical approach [microscopic and total endoscopic]). Forty two ears required PORP reconstructions, while 20 ears required TORP reconstructions. The microscope was used to reconstruct the ossicular chain in 31 cases, while an exclusive endoscopic approach was used in the remaining 31 patients. Controlling for the prosthesis, there were no significant postoperative differences in bone PTA, air PTA, and ABG between primary and staged ossiculoplasties, or surgical approach. Conclusions Controlling for the type of prosthesis, there were no significant differences in hearing outcomes with respect to staged ossicular chain reconstruction or whether the endoscope or microscope was used for visualization. Thus, in this series, endoscopic OCR yields similar audiometric outcomes when compared with microscopic OCR.

Objective Describe the diagnosis and management of spontaneous lateral skull base cerebrospinal fluid (CSF) leaks that originate from the posterior fossa. Study design Retrospective case review. Setting Tertiary university hospital. Patients Adult patients from 2005 to 2015 who underwent surgical repair of a spontaneous lateral skull base CSF leak with intraoperative confirmation of a posterior fossa leak source. Intervention Surgical repair. Main outcome measures CSF leak resolution. Results Five patients had CSF leaks from the posterior fossa. The mean age at presentation was 54 years old (range, 19-79), the mean body mass index (BMI) was 32.6 (standard deviation [SD], 8.4), and the mean follow-up length was 34.6 months (SD, 19.4). Presentations did not differ from CSF leaks through middle fossa defects, including three patients with a history of meningitis and all patients with clear otorrhea following tympanostomy tube placement. All patients had resolution of the leak after surgical repair, but two patients required revision surgery for persistent leaks and one patient had a postoperative infection. Surgical approaches included one middle fossa, two transmastoid, one combined middle fossa/transmastoid, and one transcanal. Radiographic studies suggested a posterior fossa source in all cases but findings were often subtle. Conclusion Posterior fossa CSF leaks represent a rare subset of spontaneous lateral skull base leaks. Diligent radiographic review and intraoperative assessment of the posterior fossa plate are crucial. The size and location of the defect dictates the optimal surgical approach. Surgeons should consider a posterior fossa source in failed repairs or when the initial surgery did not fully evaluate the posterior fossa plate.