Asymmetric Ocular Vestibular Evoked Myogenic Potentials in Pediatric Vestibular Migraine.

OBJECTIVE: Although ocular vestibular evoked myogenic potentials (oVEMP) abnormalities have been associated with vestibular migraine (VM) in adults, no studies have evaluated this in pediatric patients with VM. oVEMP asymmetry with normal cervical VEMP (cVEMP) findings may be a reliable VM biomarker in adults. We characterize VEMP results among pediatric patients with VM and benign recurrent vertigo of childhood (BRVC), a migraine precursor, and compare these results with VEMP findings from pediatric patients with nonmigrainous vestibular diagnoses. METHODS: Four hundred seventy-four pediatric patients were evaluated over a 3-year period in a multidisciplinary pediatric vestibular clinic, 139 of whom met the inclusion criteria. Records were reviewed for history, audiometry, and vestibular testing results. VEMP testing was performed with a 500-Hz tone burst. Based on adult normative data, oVEMP asymmetry was defined as greater than 33% interaural difference; cVEMP asymmetry was defined as greater than 41%. RESULTS: Eighty subjects had VM or BRVC (mean [standard deviation] 12.8 [3.8] yr; range, 4.3-18.2 yr). Fifty-nine subjects received vestibular diagnoses other than VM or BRVC (nonmigrainous group; mean [standard deviation] age, 13.0 [3.6] yr; range, 5.14-18.9 yr). A greater proportion of the VM/BRVC group demonstrated oVEMP asymmetry with normal cVEMP as compared with the nonmigrainous group (22.5% versus 10.1%; p < 0.05). Zero subjects in the VM/BRVC group demonstrated cVEMP asymmetry versus three subjects (4.9%) of the nonmigrainous group. CONCLUSIONS: VM and BRVC are notable causes of migraine-related vertigo among pediatric patients. Of VM/BRVC patients, 22.5% had oVEMP asymmetry with normal cVEMP. Similar to the adult VM population, this finding may be a useful biomarker in the right clinical setting for pediatric VM. LEVEL OF EVIDENCE: 4.

Impact of Anticholinergic Medications on Speech Perception Performance after Cochlear Implantation.

OBJECTIVE: To identify and characterize the impact of anticholinergic medications, which have known adverse effects on cognition in older adults, on speech perception after cochlear implantation. STUDY DESIGN: Retrospective cohort. SETTING: Tertiary referral center. SUBJECT POPULATION: Adult patients who underwent cochlear implantation between January 2010 and September 2020 with speech perception scores at 3, 6, and 12 months. INTERVENTIONS: Anticholinergic burden of patients' prescribed medications. MAIN OUTCOME MEASURES: AzBio speech perception scores after implantation. RESULTS: One hundred twenty-six patients had documented AzBio in quiet speech perception score at all three postactivation time points. Patients were divided into three groups by anticholinergic burden (ACB) score, including ACB = 0 (90 patients), 1 (23 patients), and ≥2 (13 patients). There was no statistically significant difference between ACB groups in audiologic performance at candidacy testing ( p = 0.77) or at 3 months after implantation ( p = 0.13). Beginning at 6 months, a lower mean AzBio was seen in patients with higher ACB scores (68% ACB = 0; 62% ACB = 1; 48.1% ACB ≥ 2; p = 0.03). At 12 months, there were further differences between the groups (71.0% ACB = 0, 69.5% ACB = 1, 48.0% ACB ≥2, p < 0.01). Controlling for the effects of age using multivariate linear regression showed persistent effects of ACB score on learning-related AzBio improvements. Comparatively, the negative impact of a single ACB score point was equivalent to nearly 10 years of aging ( p = 0.03). CONCLUSIONS: Increased ACB is associated with worse speech perception scores after cochlear implantation, an effect that persists even when accounting for patient age, suggesting that these medications may have cognitive and learning effects that reduce cochlear implant performance.

Pure tone audiometry as assessed by a commercially-available mobile phone application compared to formal audiometry.

PURPOSE: Comparison of audiometric measurements of commercially available smartphone audiogram application thresholds as compared to gold standard audiometric evaluation. MATERIALS AND METHODS: A single-institution, original contribution. Ninety consecutive adult patients presenting to a tertiary care auditory clinic with auditory complaints were evaluated using standard audiometric testing and an application-based hearing test. Correlation between app results and standard audiogram for air conduction pure tone thresholds was evaluated. RESULTS: Mimi™ (Berlin, Germany) results for audiometric thresholds were moderately correlated with standard audiogram (r = 0.51-0.68) depending on severity. The percentage of patients whose hearing loss severity on formal audiometry results were accurately reflected in the Mimi™ (app-based hearing test: ABHT)1 results ranged from 18.2 to 80 %. Among patients whose results were at the extremes of hearing performance, app and standard audiogram results were similar. ABHT yielded an overall sensitivity of 35.5 % and specificity of 97.1 % for normal hearing, and an overall sensitivity of 80 % and specificity of 96 % for severe hearing loss. CONCLUSIONS: Results from an audiometric smart phone application showed accurate categorization of hearing loss at the high and extremes as compared to standard audiometry. However, correlation of pure tone values was more variable and dependent on hearing level.

Comparison of Outcomes of Surgical Repair of Spontaneous Temporal Bone CSF Leaks and Encephaloceles Using Bone Cement and Autologous Material.

OBJECTIVE: To compare outcomes transmastoid repair of spontaneous middle fossa cerebrospinal fluid (CSF) leak using only bone cement (BC) versus only autologous material (AM) or combined materials (CM) with both bone cement and autologous material. STUDY DESIGN: Retrospective Chart Review. SETTING: Tertiary Care Hospital. PATIENTS: Forty-three adult patients undergoing transmastoid repair of spontaneous middle fossa CSF leak between 2014 and 2020 (BC:12, AM:15, CR:16). INTERVENTIONS: Cortical mastoidectomy, identification of defect, and repair with BC (Cranios® hydroxyapatite), AM (local bone, fascia, fat, and/or cartilage), or CM (Cranios® combined with autologous materials). MAIN OUTCOME MEASURES: Successful repair without recurrent CSF leak or encephalocele throughout follow up. RESULTS: Fifty-one percent of subjects were female. Mean age at repair was 58.6 years (SD 10.9). Mean BMI was 35.4 (SD 7.6; BC:36.3, AM:36.5, CM:33.6). Forty (93%) patients had successful repair without known recurrent CSF leak or encephalocele since surgery (BC:11, 91.6%; AM:14, 93.3%; CM:15, 93.8%; p = 0.49) over a mean length of follow up of 49.6 months (BC: 37.9, AM: 59.2, CR: 49.5). The difference in mean operative time amongst the groups was faster for patients using bone cement (BC: 100.2 min, AM: 182.8, CM: 133.2; p < 0.00001). CONCLUSIONS: BC, AM, and CM techniques each demonstrate effective and sustained means of repair for middle fossa CSF leak and encephalocele, even in the presence of multiple defects. Use of isolated BC offers a significant decrease in operative time with a noninferior outcome. Active CSF leak at the time of surgery is associated with increased risk of recurrence.

Novel Surgical Technique in Active Bone Conduction: Minimally Invasive Approach to Fully Implantable Osseointegrated Implant.

We report our experience using a novel minimally invasive surgical technique for implantation of a fully implantable active bone conduction implant. This was a retrospective review of 16 adults, including 10 women and 6 men. The mean age was 54 years. Hearing loss profiles included 8 with mixed hearing loss, 5 with conductive hearing loss, and 3 with single-sided deafness. Nine patients underwent placement through the standard approach and 7 with the minimally invasive approach. There were no postoperative complications at a mean follow-up of 6.5 months (SD, 4; range, 1.5-12), and all patients received audiologic benefit with objective improvement in sound-field thresholds upon activation. Mean operative time was shorter with the minimally invasive approach (64 vs 41 minutes, P = .01). The fully implantable bone-anchored auditory implant can be effectively placed via a minimally invasive incision, with potential benefits of decreased operative time, low risk for intra- and postoperative complications, and rapid healing.

Evaluation and Management of Otalgia.

Otalgia can be broadly categorized into primary otologic causes and secondary nonotologic causes. Isolated otalgia in the absence of hearing loss, otorrhea, or abnormal otoscopic findings is typically secondary to referred pain from nonotologic causes, as the sensory nerve supply to the ear arises from 4 cranial nerves and the cervical plexus. The most common causes of primary otalgia are acute otitis media and otitis externa, whereas the most common causes of secondary otalgia are temporomandibular joint disorders and dental pathology. Persistent unilateral ear pain and other alarm symptoms warrant further evaluation for possible neoplasm.

Novel Web-Based Music Re-Engineering Software for Enhancement of Music Enjoyment Among Cochlear Implantees.

OBJECTIVES: Cochlear implant (CI) listeners experience diminished music perception and enjoyment from a variety of patient-related and implant-related factors. We investigate the hypothesis that patient-directed music re-engineering may enhance music enjoyment with CI. STUDY DESIGN: Prospective cohort study. SETTING: Academic cochlear implant center. SUBJECTS AND METHODS: A multidisciplinary team of neurotologists, audiologists, and a sound/audio engineer collaborated with a web developer to create a music re-engineering application. Experienced adult CI listeners rated original excerpts from five major genres of music on enjoyment using a visual analog scale (VAS). Subjects were then allowed to re-engineer the original by adjusting treble frequencies, bass frequencies, percussion emphasis, and reverberation and again rated on enjoyment. RESULTS: Total of 46 subjects, with a mean age of 57.6 years (SD = 16; range, 18-90) participated in the study. User-mixed audio was rated higher across all measures of enjoyment than original recordings (mean difference +0.92; p < 0.05, CI [0.22, 1.62]), an effect that was seen across all genres except for country music. Subjects preferred louder bass frequencies (mean difference +7.1 dB; p < 0.01, CI [2.15, 24.3]) and more reverberation (mean difference +6.6 ms; p < 0.01, CI [2.85, 10.7]). Re-engineered music increased enjoyment in 57%, and 79% reported an interest in being able to mix music of their own choosing. CONCLUSION: User-directed music re-engineering increases music enjoyment for CI listeners. The cochlear implantee preferred heightened bass, reverberation, and treble across musical genres. These findings support the implementation of patient-directed music re-engineering to enhance music enjoyment with technology that is readily available today.

Predictors of Postoperative Electrode Deactivation Among Adult Cochlear Implantees.

OBJECTIVE: To characterize postoperative electrode functionality after adult cochlear implantation; to identify rationale and risk factors for electrode deactivation. STUDY DESIGN: Retrospective Chart Review. SETTING: Academic Cochlear Implant Center. SUBJECT POPULATION: Five hundred nineteen cochlear implants in 433 adult patients over 5 years. INTERVENTIONS: Unilateral or bilateral cochlear implantation. MAIN OUTCOME MEASURES: Rate of electrode deactivation after adult cochlear implantation. RESULTS: One hundred twenty (27.7%) patients experienced electrode deactivation postoperatively, involving a total of 447 electrodes. The most common reasons for deactivation were bothersome nonauditory symptoms (n = 170, 38.0%), perceived benefit by patients (n = 64, 14.3%), and bothersome auditory symptoms (n = 60, 13.4%). Four hundred nineteen (93.7%) of involved electrodes remained deactivated at most recent follow-up, whereas 28 (6.3%) were able to be reactivated. Deactivation was most likely to occur within the first 4 weeks after activation (n = 90 patients,75.0%; p < 0.01). Among affected patients, the average number of electrodes deactivated was 3.44 (range 1-13; SD 2.50). Age was not associated with electrode deactivation. CONCLUSIONS: While 98% of cochlear implants had full insertions, more than a quarter of implantees may experience electrode deactivation postoperatively for a multitude of reasons, with bothersome nonauditory symptoms most prevalent. Deactivation of five or more electrodes and simultaneous deactivation of two or three electrodes seems to increase the odds of subsequent device failure. However, deactivation encompasses a wide range of issues that likely include patient factors, surgical technique, and device-specific issues. Prognosis varies greatly at the individual level and further evaluation is required to better identify the issues underlying deactivation and identify true predictors of failure.

Impact of Reconstruction With Hydroxyapatite Bone Cement on CSF Leak Rate in Retrosigmoid Approach to Vestibular Schwannoma Resection: A Review of 196 Cases.

OBJECTIVE: To assess the impact of reconstructive technique on the incidence of cerebrospinal fluid (CSF) leak following retrosigmoid approach to acoustic neuroma resection. STUDY DESIGN: Retrospective case series. SETTING: Academic medical center. PATIENTS: A total of 1,200 patients with acoustic neuromas presented to our institution from 2005 to 2018. Of these, 196 patients underwent surgical resection via a retrosigmoid approach. INTERVENTION: At our institution, internal auditory canal (IAC) reconstruction following a retrosigmoid approach was performed with bone wax and muscle plug or Norian hydroxyapatite bone cement from 2005 to 2013. Starting in 2014, a newer model of bone cement, Cranios hydroxyapatite, was used exclusively for reconstruction. MAIN OUTCOME MEASURES: Rates of CSF leak were evaluated across different methods of IAC reconstruction and types of bone cement. Patients whose leaks were attributable to the craniectomy site were excluded from analysis. RESULTS: The postoperative CSF leak rate among patients who did not receive bone cement for IAC reconstruction was 15.6% (n.5). The leak rate amongst patients who received Norian bone cement was 6.3% (n.4). After introduction of Cranios bone cement, the total leak rate decreased to 1% (n.1). Compared with all other types of closure, Cranios had a significantly reduced rate of postoperative CSF leak (p < 0.005). The leak rate following Cranios versus Norian was also significantly reduced (p < 0.05). Leak rate was not affected by tumor size (p.0.30) or age (p.0.43). CONCLUSION: CSF leak rate following acoustic neuroma resection was significantly reduced by introduction of Cranios hydroxyapatite bone cement.

Failure and Revision Surgery After Cochlear Implantation in the Adult Population: A 10-year Single-institution Retrospective and Systematic Review of the Literature.

OBJECTIVE: To characterize failure rate and etiology after cochlear implantation; to identify predictors and describe outcomes after implant failure. STUDY DESIGN: Retrospective chart review and systematic review of the literature using PubMed and Embase. SETTING: Academic Cochlear Implant Center. SUBJECT POPULATION: Four hundred ninety-eight devices in 439 distinct adult patients. INTERVENTIONS: Unilateral or bilateral cochlear implantation. MAIN OUTCOME MEASURES: Implant failure rate and etiology. RESULTS: A total of 32 devices (5.9%) failed in 31 patients encompassing the following failure types in accordance with the European Consensus Statement of Cochlear Implants: 17 device failures (53.1%), 11 failures due to performance decrement/adverse reactions (34.4%), and 4 medical reasons (12.9%). There was no significant difference in age, sex, or manufacturer between patients with and without failures. Twenty-five percent of patients with failure leading to explantation had childhood onset of deafness compared to 12.1% of patients with adult-onset hearing loss (OR = 2.42; p = 0.04). Performance decrement/adverse reaction patients had an older average age at implantation compared to device failure patients (mean 68.5 yr 95% CI: 59.9-77.1 vs mean 47.6 yr, CI: 39.9-55.3, p < 0.01). There was no significant difference in time to failure, sex, or device manufacturer between the different types of failures. Twenty-nine patients who experienced CI failure underwent a revision surgery, while the remaining two opted for explantation without reimplantation. One patient who underwent revision surgery subsequently presented with a second failure and underwent a second revision, which was successful.In our systematic review, 815 citations were reviewed, and 9 studies were selected for inclusion. Overall failure rate across all studies was 5.5%. Device failure was the leading cause of failure in the majority (6/9) of studies, accounting for 40.8% of all failures. Medical reasons were the second leading cause at 33.6%, followed by performance decrement/adverse reaction (20.9%) and other (4.8%). CONCLUSIONS: Cochlear implant failure is a rare phenomenon. Childhood-onset of hearing loss appears to be associated with an increased risk of overall failure. Older patients are at increased risk for performance decrement/adverse reaction. Revision surgery success rates remain very high and patients with failure of any cause should be offered explantation with concurrent reimplantation.

Impact of Underlying Diagnosis on Speech and Quality of Life Outcomes After Cochlear Implantation for Single-Sided Deafness.

OBJECTIVE: Our objective was to compare outcomes in speech and quality of life in those undergoing cochlear implantation for single-sided deafness (SSD), with the aim to characterize the clinical impact of underlying diagnosis in the affected ear and pre-operative hearing status. STUDY DESIGN: Prospective case series. SETTING: Academic Cochlear Implant Center. PATIENTS: 42 adult patients implanted with the diagnosis of SSD. INTERVENTIONS: Patients were evaluated at 3-, 6-, and 12-months post-operatively using AZBio sentence and speech, and consonant-nucleus-consonant (CNC) depending on appropriate testing level. Our previously validated Comprehensive Cochlear Implant Quality of Life (CCIQ) questionnaire was administered. MAIN OUTCOME MEASURES: Speech perception, quality of life. RESULTS: Subjects were stratified by the underlying diagnosis: Meniere's Disease (MD; n = 10), sudden sensorineural hearing loss (SSNHL; n = 13), and Other (eg TBI, acoustic neuroma, progressive, noise-induced; n = 19). Mean preoperative PTA of the implanted ear was 82dB ±â€Š17; that of the nonimplanted ear was 32dB ±â€Š17. SSNHL and MD demonstrated the highest speech perception score at 3 months (93 and 95%), and "Other" demonstrated the lowest scores at 88%. All 3 groups demonstrated nadir in speech scores at 6 months before improving at 12 months, but the "Other" diagnoses maintained the lowest speech testing across all time points. All 3 groups reported improved quality of life on CCIQ. CONCLUSIONS: Subjects with SSNHL and MD demonstrate excellent speech perception and quality of life outcomes after cochlear implantation for SSD. Subjects with "Other" diagnoses underlying their SSD demonstrated lower scores on speech testing but nonetheless reported improved quality of life.