Cochlear Implantation in Adults With Asymmetric Hearing Loss: Speech Recognition in Quiet and in Noise, and Health Related Quality of Life.

OBJECTIVE: To examine the possible speech recognition and health related quality of life (HRQoL) benefits of cochlear implantation among adults with asymmetric sensorineural hearing loss. STUDY DESIGN: Retrospective chart review, single-subject design. METHODS: A total of 45 adult cochlear implant recipients with asymmetric sensorineural hearing loss where performance for the best-aided condition exceeded 60% correct open set sentence recognition in quiet, and the implanted ear met traditional candidacy criteria. End point testing of the implanted ear was evaluated with use of the Consonant-Vowel Nucleus-Consonant (CNC) word test and AzBio sentence test materials in quiet, and bimodally with the AzBio sentence test materials in noise at +5 dB signal-to-noise ratio (SNR). HRQoL was measured using the Nijmegen Cochlear Implant Questionnaire (NCIQ). RESULTS: Measured in quiet, with the non-implanted ear plugged, the average CNC word scores increased from 9.1% preoperatively to 55.7% (p < 0.01) at the 6-month post-activation test interval. Similarly, average AzBio sentence scores in quiet, with the non-implanted ear plugged, increased from 13.9% preoperatively to 73.4% (p < 0.01) at the 6-month post-activation test interval. Finally, in the bilateral/bimodal condition, the AzBio sentence score in +5 dB SNR improved from an average of 26.8% preoperatively to 52.4% (p < 0.01) at the 6-month test interval. Results of the NCIQ showed improved scores on all six subdomains. CONCLUSIONS: These data demonstrate significant benefit of cochlear implantation among a group of postlingually deafened adults whose preoperative hearing and aided speech recognition fell outside of the currently specified Food and Drug Administration candidacy guidelines. Results of this study support the evaluation of a candidate's speech recognition in noise in the best-aided condition to adequately assess candidacy for a cochlear implant.

Cochlear Implant Associated Labyrinthitis: A Previously Unrecognized Phenomenon With a Distinct Clinical and Electrophysiological Impedance Pattern.

OBJECTIVES: To report a unique clinical entity "cochlear implant associated labyrinthitis," characterized by a distinct constellation of clinical symptoms and pattern of electrode impedance fluctuations. STUDY DESIGN: Retrospective chart review. METHODS: All patients that underwent cochlear implantation between January 2014 and December 2016 were retrospectively reviewed. All subjects with acute onset dizziness, device performance decline, and characteristic erratic pattern of electrode impedances occurring after an asymptotic postoperative interval were identified and reported. RESULTS: Five patients with the above criteria were identified, representing 1.4% of all implant surgeries performed during this time. The median age at time of implantation was 71 years, and the median time interval between implantation and onset of symptoms was 126 days. All patients exhibited acute onset dizziness, subjective performance deterioration, erratic impedance pattern, and two experienced worsening tinnitus. Two of five patients underwent subsequent CT imaging, where good electrode placement was confirmed without cochlear ossification. Two of five patients received oral prednisone therapy. All patients reported a subjective improvement in symptoms and stabilization of electrode impedances. Three patients subsequently received vestibular testing, where significantly reduced peripheral vestibular function was identified. CONCLUSIONS: We describe a unique clinical entity, "cochlear implant associated labyrinthitis," characterized by a distinct constellation of clinical symptoms and corresponding electrode impedance anomalies. The exact cause for this event remains unknown, but may be related to viral illness, delayed foreign body reaction to the electrode, or a reaction to electrical stimulation. Future studies characterizing this unique clinical entity are needed to further elucidate cause and optimal management.

Health-related quality of life outcomes following adult cochlear implantation: A prospective cohort study.

INTRODUCTION: The benefits of cochlear implantation extend beyond improved speech recognition and into overall health-related quality of life (HRQoL). Several measures of HRQoL, categorized as generic or disease specific, have been used in the cochlear implant literature. The clinical utility of generic HRQoL measures have been reported to be variable by previous investigators. The degree to which HRQoL correlates to speech perception is largely unknown. METHODS: A prospective single-subject design at a large tertiary care center. Self-reported HRQoL was measured at the preoperative and 12-month post-activation test intervals. The measures of HRQoL included a generic form, (Medical Outcome Study Short Form; SF-36), and disease specific form (Nijmegen Cochlear Implant Questionnaire; NCIQ). Speech recognition was measured at the preoperative, 6- and 12-months post-activation test intervals using the Consonant-Nucleus-Consonant (CNC) monosyllabic word test. RESULTS: A total of 61 patients (mean 67 years; range 30-87 years) were included in the final analysis. Average speech recognition on the CNC word test was 10% pre-operatively, and 66.7% at 12-months post-activation. The HRQoL scores improved significantly for seven of the eight subdomains of the NCIQ, and one of the nine domains of the SF-36. CONCLUSION: Cochlear implantation significantly improves HRQoL, regardless of age. Disease specific measures, such as the NCIQ, are better able to demonstrate differences in HRQoL compared to general health surveys.

Early outcomes after cochlear implantation for adults and children with unilateral hearing loss.

OBJECTIVES/HYPOTHESIS: This study was designed to examine speech recognition and self-perceived health-related quality of life (HRQoL) received from cochlear implantation among a cohort of adults and children with a short duration of unilateral hearing loss greater than 6 months, but less than 2 years. STUDY DESIGN: Single-subject repeated measures prospective study. METHODS: This study assessed changes in speech recognition and self-perceived quality of life by prospectively analyzing data at the preoperative evaluation and at the 3-month and 6-month postactivation intervals. Measurement tools included Medical Outcomes Study Questionnaire Short Form 36, Nijmegen Cochlear Implant Questionnaire, Speech Spatial and Qualities of Hearing-Comparative, and speech recognition measures in quiet and in noise. RESULTS: Results indicated significant improvement in speech recognition, both in quiet and noise. Quality-of-life measures showed a significant increase in self-perceived benefit with disease-specific instruments, but remained constant with a generic HRQoL instrument. CONCLUSIONS: Cochlear implantation was a successful intervention for improved hearing in quiet and noise, and a self-perceived benefit for this group of adults and children with a short duration of unilateral hearing loss. LEVEL OF EVIDENCE: 4 Laryngoscope, 127:1683-1688, 2017.

Identifying and Prioritizing Diseases Important for Detection in Adult Hearing Health Care.

PURPOSE: The purpose of this research note is to identify and prioritize diseases important for detection in adult hearing health care delivery systems. METHOD: Through literature review and expert consultation, the authors identified 195 diseases likely to occur in adults complaining of hearing loss. Five neurotologists rated the importance of disease on 3 dimensions related to the necessity of detection prior to adult hearing aid fitting. RESULTS: Ratings of adverse health consequences, diagnostic difficulty, and presence of nonotologic symptoms associated with these diseases resulted in the identification of 104 diseases potentially important for detection prior to adult hearing aid fitting. CONCLUSIONS: Current and evolving health care delivery systems, including direct-to-consumer sales, involve inconsistent means of disease detection vigilance prior to device fitting. The first steps in determining the safety of these different delivery methods are to identify and prioritize which diseases present the greatest risk for poor health outcomes and, thus, should be detected in hearing health care delivery systems. Here the authors have developed a novel multidimensional rating system to rank disease importance. The rankings can be used to evaluate the effectiveness of alternative detection methods and to inform public health policy. The authors are currently using this information to validate a consumer questionnaire designed to accurately identify when pre- fitting medical evaluations should be required for hearing aid patients.

Hearing preservation among patients undergoing cochlear implantation.

INTRODUCTION: Despite successful preservation of low-frequency hearing in patients undergoing cochlear implantation (CI) with shorter electrode lengths, there is still controversy regarding which electrodes maximize hearing preservation (HP). The thin straight electrode array (TSEA) has been suggested as a full cochlear coverage option for HP. However, very little is known regarding its HP potential. METHODS: A retrospective review was performed at two tertiary academic medical centers, reviewing the electronic records for 52 patients (mean, 58.2 yr; range, 11-85 yr) implanted with the Cochlear Nucleus CI422 Slim Straight (Centennial, CO, USA) electrode array, referred to herein as the thin straight electrode array or TSEA. All patients had a preoperative low-frequency pure-tone average (LFPTA) of 85 dB HL or less. Hearing thresholds were measured at initial activation (t1) and 6 months after activation (t2). HP was assessed by evaluating functional HP using a cutoff level of 85 dB HL PTA. RESULTS: At t1, 54% of the subjects had functional hearing; 33% of these subjects had an LFPTA between 71 and 85 dB HL, and 17% had an LFPTA between 56 and 70 dB HL. At t2, 47% of the patients had functional hearing, with 31% having an LFPTA between 71 and 85 dB HL. DISCUSSION: Preliminary research suggests that the TSEA has the potential to preserve functional hearing in 54% of patients at t1. However, 22% (n = 6) of the patients who had functional hearing at t1 (n = 28) lost their hearing between t1 and t2. Further studies are needed to evaluate factors that influence HP with the TSEA electrode and determine the speech perception benefits using electric and acoustic hearing over electric alone.

Cervical VEMP threshold response curve in the identification of Ménière's disease.

PURPOSE: To investigate the sensitivity/specificity of a shift upward in the most sensitive frequency of the cervical vestibular evoked myogenic potential (cVEMP) threshold-response curve in the identification of Ménière's disease (MD). A secondary purpose was to investigate the clinical characteristics that had an impact on the sensitivity/specificity and to adjust the criteria for a positive shift upward in the cVEMP curve to maximize performance of the test. RESEARCH DESIGN: A retrospective review of patients diagnosed with MD and those without MD. STUDY SAMPLE: Two hundred ninety-four patients met the inclusion criteria of symptom complaints of spontaneous events of vertigo and a full vestibular and balance evaluation with cVEMP threshold-response curve testing. Two hundred six of these patients were diagnosed with MD, and 88 patients were determined to be non-MD. DATA COLLECTION AND ANALYSIS: Review of the patients' medical records was used to extract data on the results of the cVEMP curve, age, gender, duration from time of onset of spontaneous events, pure tone average from hearing test, and water caloric asymmetry. Student's t-test, χ² test, receiver operating characteristic (ROC) curve with area under the curve (AUC), Pearson correlation coefficient, and sensitivity/specificity from 2 × 2 tables were all used in the analysis. RESULTS: Basic sensitivity/specificity for a shift upward in the most sensitive frequency to 1000 Hz in the cVEMP threshold-response curve was 0.47/0.64 respectively. Clinical characteristics that were found to have a significant impact on the sensitivity/specificity were age equal to or above 60 yr and a caloric asymmetry ≥25%. Various combinations of age and caloric with the requirement of a shift upward in the cVEMP curve most sensitive frequency to 1000 Hz resulted in significant but modest improvements in sensitivity/specificity. However, the overall performance was not shown acceptable for routine clinical use with maximum sensitivity at 0.73. Therefore, placing an emphasis on specificity over sensitivity results showed specificity of 0.95 for those under 60 yr and 0.90 for those 60 yr of age or older with sensitivity at 0.20, but only in the context of a ≥25% caloric asymmetry. CONCLUSIONS: We recommend the use of the shift upward to 1000 Hz with a caloric asymmetry as the clinical protocol to maximize the use of the cVEMP threshold-response curve for assistance in the identification of MD, in the context of a ≥25% caloric asymmetry. This implies that if the test is negative no interpretation of identification of MD can be made. If the test is positive the results can be used to increase the argument for MD since the probability of the result being a false positive is only 5-10%.

Pediatric cerebellopontine angle and internal auditory canal tumors: clinical article..

OBJECT: The aim in this study was to describe the clinical presentation, differential diagnosis, and risk for neurofibromatosis Type 2 (NF2) in pediatric patients presenting with cerebellopontine angle (CPA) and internal auditory canal (IAC) tumors. METHODS: The authors conducted a retrospective study at a tertiary care academic referral center. All patients with an age ≤ 18 years who had presented with an extraaxial CPA or IAC tumor between 1987 and 2012 were included in the study cohort. Data regarding symptoms, diagnosis, tumor characteristics, and NF2 status were collected and analyzed. RESULTS: Sixty patients (55% female, 45% male) harboring 87 tumors were identified. The mean age at diagnosis was 12.8 years (median 14.0 years, range 0.9-18.9 years). Schwannomas were the most commonly identified lesions (57 of 87 tumors, including 52 vestibular, 3 facial, and 2 trigeminal schwannomas), followed by meningiomas (5 of 87) and epidermoid cysts (4 of 87). Six malignant tumors were diagnosed, including small-cell sarcoma, squamous cell carcinoma, malignant meningioma, atypical rhabdoid-teratoid tumor, endolymphatic sac tumor, and malignant ganglioglioma. Headache, followed by hearing loss and imbalance, was the most common presenting symptom, whereas dysphagia, otalgia, and facial pain were uncommon. Neurofibromatosis Type 2 was diagnosed in 20 (61%) of 33 patients with vestibular schwannoma (VS), while the other 13 patients (39%) had sporadic tumors. Nineteen of the 20 patients with NF2 met the diagnostic criteria for that disorder on initial presentation, and 15 of them presented with bilateral VS. At the last follow-up, 19 of the 20 patients subsequently diagnosed with NF2 demonstrated bilateral VSs, whereas 1 patient with a unilateral VS and multiple other NF2-associated tumors has yet to demonstrate a contralateral VS to date. Only 1 patient presenting with an isolated unilateral VS and no family history of NF2 demonstrated a contralateral VS on subsequent radiological screening. CONCLUSIONS: Cerebellopontine angle and IAC tumors in the pediatric population are rare. There are several noteworthy differences between the adult and pediatric populations harboring these lesions. While VS is the most common pathology in both age groups, the lesion was found in only 60% of the pediatric patients in the present study. Unlike in adults, VSs in the pediatric population were associated with NF2 in over one-half of all cases. The majority of pediatric patients with NF2 fulfilled the diagnostic criteria at initial presentation; however, approximately 7% of patients presenting with a seemingly sporadic (no family history of NF2) unilateral VS will meet the criteria for NF2 later in life. Finally, malignancies account for a significantly higher percentage (10%) of cases among pediatric patients. These findings underscore the importance of early screening and close radiological follow-up and may be helpful in patient counseling.

A 10-year review of otic lichen planus: the Mayo Clinic experience.

IMPORTANCE: Lichen planus is an autoimmune inflammatory dermatosis that typically affects the skin but can also involve the stratified squamous epithelium of the external auditory canals and tympanic membranes. Here we report our experience with the clinical presentation, diagnosis, and management of otic lichen planus. OBSERVATIONS: We retrospectively reviewed medical records from January 1, 2001, through May 31, 2011, of patients with a diagnosis of otic lichen planus. Nineteen cases were identified (mean age at diagnosis, 57 years; 15 women). The most common concerns were persistent otorrhea and hearing loss. Other symptoms included plugging, pruritus, tinnitus, pain, and bleeding. The mean symptom duration was 4.0 years (n = 13). Most patients responded well to topical tacrolimus within several months. One patient had a dramatic positive response to rituximab. CONCLUSIONS AND RELEVANCE: Otic lichen planus can lead to persistent hearing loss and should be considered in the differential diagnosis of relentless otorrhea and external auditory canal stenosis. In our experience, topical tacrolimus is the best primary treatment, but alternative therapies could be instituted in severe cases. Early recognition of the nonspecific symptoms of otic lichen planus may lead to prompt treatment and avoidance of irreparable late sequelae.

The prevalence and clinical course of facial nerve paresis following cochlear implant surgery.

OBJECTIVES/HYPOTHESIS: To describe the prevalence, clinical course, and outcomes of facial nerve paresis following cochlear implantation and to identify variables associated with poor definitive facial nerve function. STUDY DESIGN: Retrospective cohort study with systematic literature review. METHODS: All patients who underwent cochlear implantation between January 1990 and December 2010 at a single tertiary academic referral center were reviewed. Data including clinical presentation, intraoperative findings, onset, severity, management, and outcomes of all patients who experienced facial nerve paresis following cochlear implantation were recorded. RESULTS: Eight hundred eighty-eight cochlear implants (282 pediatric, 606 adult) were performed in 768 patients. Eleven patients with postoperative facial nerve paresis were identified. Ten patients (1.1%) developed delayed-onset paresis and had complete recovery within 6 months of surgery, whereas a single patient (0.1%) demonstrated immediate onset paresis and experienced incomplete return of facial nerve function. Seventeen additional cases were identified in the literature and were summarized. CONCLUSIONS: Facial nerve paresis following cochlear implantation is rare. Most cases demonstrate a delayed onset and have complete recovery within months of surgery. Delayed onset facial nerve paresis following cochlear implantation heralds an excellent prognosis, whereas immediate onset facial paresis prognosticates a poorer outcome. In the absence of medical contraindications, corticosteroid therapy should be considered in facial paresis following cochlear implant surgery.

Outcomes comparing primary pediatric stapedectomy for congenital stapes footplate fixation and juvenile otosclerosis.

OBJECTIVE: To compare presentation, operative findings, and outcomes among pediatric patients undergoing primary stapedectomy for congenital stapes footplate fixation (CSFF) and juvenile otosclerosis (JO). STUDY DESIGN: Retrospective review. SETTING: Combined experience from 2 tertiary academic referral centers. PATIENTS: Pediatric patients with CSFF and JO. INTERVENTION: Primary stapedectomy. MAIN OUTCOME MEASURE(S): 1) Preoperative and postoperative audiometric data using the 1995 AAO-HNS reporting guidelines; 2) Notable operative findings, and postoperative complications. RESULTS: Forty-four pediatric ears met inclusion criteria (27 CSFF, 17 JO). Patients with CSFF presented with a more significant hearing loss (mean PTA 52 dB versus 42 dB; p = 0.04), underwent surgery at a younger age (12.2 versus 16.3 yr; p < 0.001), and more commonly had coincident ossicular malformations (37% versus 0%; p = 0.004). Subjects with JO demonstrated a smaller postoperative ABG (mean 8.8 dB versus 17.2 dB; p = 0.04), although both groups experienced a statistically significant improvement following surgery. Mean bone conduction thresholds remained stable for both groups. There were no instances of profound sensorineural hearing loss, perilymph gusher, facial nerve paresis, or tympanic membrane perforation. CONCLUSION: When performed by an experienced surgeon, stapedectomy is safe and effective in managing carefully selected pediatric patients with CSFF and JO. CSFF is associated with a more severe hearing loss at presentation and concurrent ossicular anomalies are common. Both groups experience substantial benefit from stapedectomy, although ABG closure rates are superior in patients with JO. These data may be helpful in preoperative assessment and patient counseling.

Primary inner ear schwannomas: a case series and systematic review of the literature.

OBJECTIVES/HYPOTHESIS: To describe the natural history of primary inner ear schwannomas (PIES) and evaluate management outcomes and relationship between PIES location, clinical presentation, and time to diagnosis. STUDY DESIGN: Retrospective chart review and systematic review of the literature. METHODS: Vestibular schwannoma confined to or arising from the inner ear were included. PIES classification was based on anatomic subsite(s) involved. Detailed clinical history and outcomes were recorded. RESULTS: In a systematic review (1933-2011), including 14 patients from the authors' institution (1999-2009), a total of 72 studies comprising 234 patients were evaluated. Mean follow-up was 32.8 ± 39.1 months (range, 0-183 months). The cochlea was the most commonly involved subsite (51%). Hearing loss was the most frequent presenting symptom (99%). Vertigo and abnormal balance were more common among tumors involving the vestibular system (P < .01). Average delay between symptom onset and diagnosis was 7.0 ± 8.0 years (median, 5 years; range, 0-40 years). Recent onset hearing loss was more likely to elicit an earlier diagnosis (P = .01). The majority of patients were observed without treatment (53%). Tumor progression was seen in 52% of patients. CONCLUSIONS: PIES are rare tumors and most commonly involve the cochlea. Tumor location is often associated with clinical presentation and correlates with delay between symptom onset and diagnosis. A watch-and-scan approach is the management strategy of choice in the absence of intractable vertigo or extensive tumor growth. The majority of patients report stable or improved symptoms over time, regardless of treatment.

Use of supramaximal stimulation to predict facial nerve outcomes following vestibular schwannoma microsurgery: results from a decade of experience.

OBJECT: The goal of vestibular schwannoma surgery is tumor removal and preservation of neural function. Intraoperative facial nerve (FN) monitoring has emerged as the standard of care, but its role in predicting long-term facial function remains a matter of debate. The present report seeks to describe and critically assess the value of applying current at supramaximal levels in an effort to identify patients destined for permanent facial paralysis. METHODS: Over more than a decade, the protocol for stimulating and assessing the FN during vestibular schwannoma surgery at the authors' institution has consisted of applying pulsed constant-current stimulation at supramaximal levels proximally and distally following tumor resection to generate an amplitude ratio, which subtracted from 100% yields the degree to which the functional integrity of the FN "dropped off" intraoperatively. These data were prospectively collected and additional variables that might impact postoperative FN function were retrospectively reviewed from the medical record. Only patients with anatomically intact FNs and > 12 months of follow-up data were analyzed. RESULTS: There were 267 patients available for review. The average posterior fossa tumor diameter was 24 mm and the rate of long-term good (House-Brackmann Grade I-II) FN function was 84%. Univariate logistic regression analysis revealed that prior treatment, neurofibromatosis Type 2 status, tumor size, cerebellopontine angle extension, subjectively thinned FN at the time of operation, minimal stimulation threshold, percent dropoff by supramaximal stimulation (SMS), and postoperative FN function all correlated statistically (p < 0.05) with long-term FN function. When evaluating patients with significant FN weakness at the time of hospital discharge, only the percent dropoff by SMS remained a significant predictor of long-term FN function. However, the positive predictive value of SMS for long-term weakness is low, at 46%. CONCLUSIONS: In a large cohort of patients, the authors found that interrogating intraoperative FN function with SMS is safe and technically simple. It is useful for predicting which patients will ultimately have good facial function, but is very limited in identifying patients destined for long-term facial weakness. This test may prove helpful in the future in tailoring less than gross-total tumor removal to limit postoperative facial weakness.

Inhibition of MEK pathway in vestibular schwannoma cell culture.

OBJECTIVES/HYPOTHESIS: The purpose of this study was to evaluate the Ras GTPase (Ras) to extracellular signal-regulated kinase (ERK) pathway in vestibular schwannoma (VS) cell cultures and patient excised schwannoma tumors. Mitogen-activated protein kinase kinase (MEK) inhibitor CI-1040 (PD184352) was utilized to evaluate the effect of specific MEK inhibition on benign schwannoma cell culture proliferation and apoptosis. STUDY DESIGN: Prospective evaluation of human schwannoma cell lines and tumors. METHODS: Western blotting was completed with phospho-antibodies for proteins in the Ras-ERK pathway. Increasing concentrations of CI-1040 were utilized in schwannoma cell cultures to evaluate cell proliferation and apoptosis. Proliferation was measured with the 3-[4,5-dimethylthiazol-2-yl]-2,5-diphenyl tetrazolium bromide proliferation assay, and apoptosis was monitored with flow cytometry of annexin V/propidium iodide-stained cells. RESULTS: The most consistent Ras-ERK pathway alterations were found in phospho-MEK and ERK. Phospho-MEK was not overexpressed in the schwannoma cell lines, but six out of 10 VS showed significant increases compared to benign Schwann cell controls. Similarly, nine of 10 VS tumors showed increased phospho-ERK expression. CI-1040 showed significantly reduced schwannoma cell proliferation at the 50 and 100 µM (IC(50) 20 µM and 30 µM) concentrations when compared to carrier only controls in two out three schwannoma cell lines. The remaining schwannoma cell line was relatively refractory to the antiproliferative effects of CI-1040 at doses up to 100 µM (IC(50) 58 µM). Cumulative data of four separate schwannoma cell lines demonstrated that apoptosis was increased in treated schwannoma cells at CI-1040 concentrations of 50 and 100 µM at 72 hours. CONCLUSIONS: There is overexpression of phosphorylated (activated) proteins in the Ras-ERK pathway in schwannoma cultures and tumors as compared to benign human Schwann cell culture controls. MEK inhibitor, CI-1040, created significantly decreased schwannoma cell proliferation and increased apoptosis in cell culture. These data justify the use of MEK inhibitors in animal treatment studies of VS.

Auditory and vestibular symptoms and chronic subjective dizziness in patients with Ménière's disease, vestibular migraine, and Ménière's disease with concomitant vestibular migraine.

OBJECTIVE: To compare presentations of Ménière's disease (MD), vestibular migraine (VM), and Ménière's disease plus vestibular migraine (MDVM), with and without comorbid chronic subjective dizziness (CSD). STUDY DESIGN: Retrospective review with diagnosis confirmed by consensus conference of investigators using published criteria for MD, VM, and CSD. SETTING: Ambulatory, tertiary dizziness clinic. PATIENTS: Approximately 147 consecutive patients with diagnoses of MD, VM, or MDVM, with/without comorbid CSD. INTERVENTIONS: Diagnostic consultation. MAIN OUTCOME MEASURES: Similarities and differences between diagnostic groups in demographics; symptoms; and results of neurotologic, audiometric, and vestibular laboratory assessments. RESULTS: Seventy-six patients had MD, 55 MD alone. Ninety-two patients had VM, 71 VM alone. Twenty-one patients had MDVM, representing about one-quarter of those diagnosed with MD or VM. Clinical features thought to differentiate VM from MD were found in all groups. Twenty-seven patients with VM (38%) had ear complaints (subjective hearing loss, aural pressure, and tinnitus) during episodes of vestibular symptoms and headache, including 10 (37%) with unilateral symptoms. Conversely, 27 patients with MD alone (49%) had headaches with migraine features that did not meet full IHS diagnostic criteria, migrainous symptoms (photophobia, headache with vomiting), or first-degree relative with migraine. Including MDVM patients, 59% (45/76) of all patients with MD had migrainous features. Thirty-two patients had CSD; most (29; 91%) were in the VM group. CONCLUSION: Comorbidity was common between MD and VM, and their symptoms overlapped. More specific diagnostic criteria are needed to differentiate these diseases and address their coexistence. CSD co-occurred with VM but was rarely seen with MD.

Cochlear implantation in patients with neurofibromatosis type 2: variables affecting auditory performance.

OBJECTIVE: To investigate cochlear implant performance outcomes among patients with Neurofibromatosis type 2 (NF2). STUDY DESIGN: Retrospective case series, patient questionnaire, and systematic review of the literature. SETTING: Tertiary academic referral center. PATIENTS: All patients with NF2 having an anatomically intact ipsilateral cochlear nerve who underwent cochlear implantation (CI). INTERVENTION(S): Cochlear implantation. MAIN OUTCOME MEASURES: Postimplantation audiometric performance and patient perceived benefit. RESULTS: Ten patients met study criteria. The median duration of follow-up after CI was 42 months (mean, 46.9 mo; range, 12-97 mo). Five patients received previous microsurgical resection of their ipsilateral vestibular schwannoma, 4 underwent previous stereotactic radiosurgery, and 1 patient had no tumor treatment before CI. Nine subjects achieved sound awareness, 6 attained open-set speech recognition and 7 are daily users. Variables including prolonged auditory deprivation, cochlear ossification, unfavorable electrical promontory stimulation testing, and useful contralateral hearing were associated with poor cochlear implant performance. No statistical associations were found between open-set recognition capacity and previous tumor management strategy, surgical approach, or ipsilateral tumor size. CONCLUSION: Cochlear implantation is an attractive alternative to auditory brainstem implantation for hearing rehabilitation in patients with NF2. Approximately 70% of patients achieve open-set speech discrimination, many scoring at the ceiling of audiometric testing. Given a favorable risk profile and superior audiometric outcomes, CI should be strongly considered in patients with nonserviceable hearing who have an anatomically intact cochlear nerve, whereas auditory brainstem implantation should be reserved for patients with evidence of cochlear nerve loss. Akin to conventional cochlear implant recipients, prolonged hearing loss, unfavorable electrophysiological testing, and cochlear ossification may predict poor subject performance. Finally, useful hearing in the contralateral ear may present a barrier to daily device use.

Reimplantation with a conventional length electrode following residual hearing loss in four hybrid implant recipients.

HYPOTHESIS: Revision surgery using a newer-generation conventional length cochlear implant electrode will provide improved speech perception in patients that initially underwent hybrid electrode implantation and experienced post-operative loss of residual hearing and performance deterioration. CLINICAL PRESENTATION: We present four patients who experienced delayed post-operative hearing loss following implantation with the Nucleus Hybrid S8 device and underwent reimplantation with the Nucleus Freedom or Nucleus 5 device using the Contour Advance array. Pure-tone thresholds and speech perception data were retrospectively reviewed. INTERVENTION: Four subjects underwent reimplantation with the Nucleus Freedom or Nucleus 5 device after experiencing deteriorating performance related to delayed acoustic hearing loss. Comparison of pre-revision performance to the most recent post-revision performance demonstrated improved speech perception performance in all subjects following reimplantation. CONCLUSIONS: A small percent of patients will experience a significant loss of residual low-frequency hearing following hybrid implantation thereby becoming completely reliant on a shorter electrode for electrical stimulation. In the current series, reimplantation with a conventional length electrode provided improved speech perception performance in such patients. Revision surgery with a conventional length electrode should be considered in 'short electrode' recipients who experience performance deterioration following loss of residual hearing.

Magnetic resonance imaging surveillance following vestibular schwannoma resection.

OBJECTIVES/HYPOTHESIS: To describe the incidence, pattern, and course of postoperative enhancement within the operative bed using serial gadolinium-enhanced magnetic resonance imaging (MRI) following vestibular schwannoma (VS) resection and to identify clinical and radiologic variables associated with recurrence. STUDY DESIGN: Retrospective cohort study. METHODS: All patients who underwent microsurgical resection of VS between January 2000 and January 2010 at a single tertiary referral center were reviewed. Postoperative enhancement patterns were characterized on serial MRI studies. Clinical follow-up and outcomes were recorded. RESULTS: During the last 10 years, 350 patients underwent microsurgical VS resection, and of these, 203 patients met study criteria (mean radiologic follow-up, 3.5 years). A total of 144 patients underwent gross total resection (GTR), 32 received near-total resection (NTR), and the remaining 27 underwent subtotal resection (STR); 98.5% of patients demonstrated enhancement within the operative bed following resection (58.5% linear, 41.5% nodular). Stable enhancement patterns were seen in 24.5% of patients, regression in 66.0%, and resolution in only 3.5% of patients on the most recent postoperative MRI. Twelve patients recurred a mean of 3.0 years following surgery. The average maximum linear diameter growth rate among recurrent tumors was 2.3 mm per year. Those receiving STR were more than nine times more likely to experience recurrence compared to those undergoing NTR or GTR (P < .001). Nodular enhancement on the initial postoperative MRI was associated with a 16-fold increased risk for future recurrence compared to those with linear patterns (P = .008). Among those with nodular enhancement on baseline postoperative MRI, a maximum linear diameter of ≥ 15 mm or volume of ≥ 0.4 cm(3) was associated with an approximate five-fold increased risk for future growth (P < .02). CONCLUSIONS: Persistent nonspecific radiologic enhancement within the postoperative field is common, making the diagnosis of tumor recurrence challenging. Factors including completeness of resection and baseline postoperative MRI findings provide valuable information regarding risk for recurrence, which may assist the clinician in determining an appropriate postoperative MRI surveillance schedule. Future studies using standardized terminology and consistent study metrics are needed to further refine surveillance recommendations.

Implications of minimizing trauma during conventional cochlear implantation.

OBJECTIVE: To describe the relationship between implantation-associated trauma and postoperative speech perception scores among adult and pediatric patients undergoing cochlear implantation using conventional length electrodes and minimally traumatic surgical techniques. STUDY DESIGN: Retrospective chart review (2002-2010). SETTING: Tertiary academic referral center. PATIENTS: All subjects with significant preoperative low-frequency hearing (≤70 dB HL at 250 Hz) who underwent cochlear implantation with a newer generation implant electrode (Nucleus Contour Advance, Advanced Bionics HR90K [1J and Helix], and Med El Sonata standard H array) were reviewed. INTERVENTION(S): Preimplant and postimplant audiometric thresholds and speech recognition scores were recorded using the electronic medical record. MAIN OUTCOME MEASURE(S): Postimplantation pure tone threshold shifts were used as a surrogate measure for extent of intracochlear injury and correlated with postoperative speech perception scores. RESULTS: : Between 2002 and 2010, 703 cochlear implant (CI) operations were performed. Data from 126 implants were included in the analysis. The mean preoperative low-frequency pure-tone average was 55.4 dB HL. Hearing preservation was observed in 55% of patients. Patients with hearing preservation were found to have significantly higher postoperative speech perception performance in the CI-only condition than those who lost all residual hearing. CONCLUSION: Conservation of acoustic hearing after conventional length cochlear implantation is unpredictable but remains a realistic goal. The combination of improved technology and refined surgical technique may allow for conservation of some residual hearing in more than 50% of patients. Germane to the conventional length CI recipient with substantial hearing loss, minimizing trauma allows for improved speech perception in the electric condition. These findings support the use of minimally traumatic techniques in all CI recipients, even those destined for electric-only stimulation.

Management of eustachian tube dysfunction with nasal steroid spray: a prospective, randomized, placebo-controlled trial.

OBJECTIVE: To determine the efficacy of intranasal aqueous triamcinolone acetonide in treating the tympanometric signs and symptoms of eustachian tube dysfunction, such as otitis media with effusion and negative middle ear pressure. DESIGN: Randomized, placebo-controlled, double-blind prospective clinical trial. SETTING: Tertiary referral clinic. PATIENTS: Adults (≥18 years) and children (6-17 years) presenting with otitis media with effusion, negative middle ear pressure, or both. INTERVENTIONS: The 2 treatment arms consisted of aqueous triamcinolone or matching placebo administered once daily intranasally for 6 weeks. All subjects underwent tympanometry, otologic examination, and completion of a symptom questionnaire before and after treatment. MAIN OUTCOME MEASURES: Resolution of abnormal tympanometry and change in symptom scores (severity and frequency). RESULTS: Ninety-one patients presenting from September 1, 2005, through December 31, 2008, with otitis media with effusion or with negative middle ear pressure were enrolled and randomly assigned to treatment or placebo in a double-blind manner. No statistically significant difference in normalization of abnormal tympanometric signs was demonstrated with the active treatment arm compared with placebo on either a per-patient basis (19% vs 32%; P = .18) or a per-ear basis (22% vs 35%; P = .15). There was also no significant difference in the overall poststudy symptom score between the 2 treatment arms, after adjusting for the prestudy overall symptom score in an analysis of covariance model (P = .27). CONCLUSION: These findings do not support the use of intranasal steroid sprays to treat the manifestations of eustachian tube dysfunction. Trial Registration clinicaltrials.gov Identifier: NCT00279916.