Word recognition following implantation of conventional and 10-mm hybrid electrodes.

We compared the effectiveness of 2 surgical interventions for improving word recognition ability in a quiet environment among patients who presented with: (1) bilateral, precipitously sloping, high-frequency hearing loss; (2) relatively good auditory thresholds at and below 500 Hz, and (3) poor speech recognition. In 1 intervention (n = 25), a conventional electrode array was inserted into 1 cochlea. As a consequence, hearing was lost in the implanted ear. In the other intervention (n = 22), a Nucleus Hybrid short-electrode array was inserted 10 mm into 1 cochlea with the aim of preserving hearing in that ear. Both groups of patients had similar low-frequency hearing and speech understanding in the ear contralateral to the implant. Following surgery, both groups had significantly higher word recognition scores than before surgery. Between-group comparisons indicated that the conventional electrode array group had higher word recognition scores than the 10-mm group when stimulation was presented to the operated ear and when stimulation was presented to both ears.

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.

Retrospective analysis of outcomes after stapedotomy with implantation of a self-crimping Nitinol stapes prosthesis.

OBJECTIVE: To review hearing results after implantation of a self-crimping stapes prosthesis. STUDY DESIGN AND SETTING: Analysis of hearing results in patients implanted with a self-crimping stapes prosthesis at two academic hospitals from 2000 to 2004. RESULTS: Seventy-nine ears were divided into short-term and intermediate follow-up groups. The mean postoperative air-bone gap (ABG), preoperative minus postoperative ABG, and preoperative minus postoperative bone conduction values were 5.7, 21.4, and 4.1 dB, respectively, for the short-term group, and 6.3, 22.3, and 4.7 dB, respectively, for the intermediate group. The ABG was <10 dB in 88 percent of the short-term group and in 79 percent of the intermediate group. CONCLUSIONS: The self-crimping Nitinol stapes prosthesis provides excellent short-term and intermediate postoperative hearing results, and may overcome the limitations of stapes prostheses requiring manual crimping. SIGNIFICANCE: This paper provides evidence for the use of a self-crimping Nitinol stapes prosthesis, which may simplify hearing restoration surgery for stapes fixation.

Scalar localization of the electrode array after cochlear implantation: clinical experience using 64-slice multidetector computed tomography.

OBJECTIVE: To use the improved resolution available with 64-slice multidetector computed tomography (MDCT) in vivo to localize the cochlear implant electrode array within the basal turn. STUDY DESIGN: Sixty-four-slice MDCT examinations of the temporal bones were retrospectively reviewed in 17 patients. Twenty-three implants were evaluated. SETTING: Tertiary referral facility. PATIENTS: All patients with previous cochlear implantation evaluated at our center between January 2004 and March 2006 were offered a computed tomographic examination as part of the study. In addition, preoperative computed tomographic examinations in patients being evaluated for a second bilateral device were included. INTERVENTION: Sixty-four-slice MDCT examination of the temporal bones. MAIN OUTCOME MEASURE: Localization of the electrode array within the basal turn from multiplanar reconstructions of the cochlea. RESULTS: Twenty-three implants were imaged in 17 patients. We were able to localize the electrode array within the scala tympani within the basal turn in 10 implants. In 3 implants, the electrode array was localized to the scala vestibuli. Migration of the electrode array from scala tympani to scala vestibuli was observed in three implants. Of the 7 implants in which localization of the electrode array was indeterminate, all had disease entities that obscured the definition of the normal cochlear anatomy. CONCLUSIONS: Sixty-four-slice MDCT with multiplanar reconstructions of the postoperative cochlea after cochlear implantation allows for accurate localization of the electrode array within the basal turn where normal cochlear anatomy is not obscured by the underlying disease process. Correlating the position of the electrode in the basal turn with surgical technique and implant design could be helpful in improving outcomes.

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.

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.

Optic neuropathy of Graves disease: results of transantral orbital decompression and long-term follow-up in 215 patients.

PURPOSE: To review demographic characteristics, clinical features, and long-term outcomes of patients with optic neuropathy of Graves disease after transantral orbital decompression. DESIGN: Retrospective analysis of noncomparative interventional case series; long-term follow-up by questionnaire. METHODS: Medical record data (preoperative and postoperative assessments) were collected from patients who had transantral orbital decompression to treat Graves optic neuropathy. Responses to two follow-up questionnaires concerning patient satisfaction were evaluated. Statistical analysis (reflected as P values) compared preoperative and early postoperative (< or =182 days) data. RESULTS: Between November 1969 and May 1989, 215 patients underwent transantral orbital decompression for Graves optic neuropathy. In 205 eyes with visual acuity of 20/40 or worse before decompression, visual acuity improved by 3 Snellen lines or more in 110 (54%) (P <.001). Of 291 eyes with visual field defects preoperatively, 120 (41%) had resolution, and 126 (43%) had improvement postoperatively (P <.001). Proptosis was reduced in 350 eyes by 4.4 +/- 2.3 mm (mean +/- SD) (P <.001). In 104 eyes, disk edema resolved in 72 (69%) and improved in 28 (27%). Responses to questionnaires mailed in 1990 and 2000 showed that 76% and 88% of respondents, respectively, were subjectively satisfied with the results of orbital decompression. CONCLUSIONS: Transantral orbital decompression appeared to be effective in treating optic neuropathy of Graves disease. Patient satisfaction was high at 10-year and 20-year follow-up.

Evolution of a cochlear schwannoma on clinical and neuroimaging studies. Case report.

The authors report on a patient with a rare schwannoma that arose from the cochlear division of the vestibulocochlear nerve. Distinctively, the lesion appeared to arise from the cochlea itself and was monitored with clinical and neuroimaging studies for 12 years before it was diagnosed and treated. The atypical occurrence of schwannomas of the vestibulocochlear nerve originating in the inner ear structures underscores the high level of clinical suspicion required for the diagnosis of these lesions in patients presenting with persistent auditory and vestibular symptoms.

Management of cerebrospinal fluid leaks involving the temporal bone: report on 92 patients.

OBJECTIVE: To investigate the success of different surgical and nonsurgical techniques in the management of cerebrospinal fluid otorrhea or otorhinorrhea. STUDY DESIGN: Retrospective case analysis. METHODS: Ninety-two patients with confirmed diagnosis of cerebrospinal fluid otorrhea or otorhinorrhea treated at our institution between 1976 and 1998 were followed up long-term by retrospective chart review and patient interviews. RESULTS: Conservative treatment was successful in most cases of cerebrospinal fluid otorrhea or otorhinorrhea resulting from head injury (26 of 29 patients). In contrast, surgical intervention was required in all but 1 of 53 patients with cerebrospinal fluid otorrhea or otorhinorrhea caused surgically. The primary operative success rate was 76.9%. When relapse occurred, the interval ranged from 0 days to 24 months (mean interval, 3.9 mo; median, 1.3 mo). All leaks requiring surgery eventually were closed successfully. Surgical results were chronologically dependent. Before 1989, 11 failures occurred in 37 primary procedures. After 1989, only 4 failures occurred in 28 primary procedures. Of seven patients undergoing primary dural closure alone, three (43%) had recurrence of the leak. Closure rates were highest among patients in whom a multilayer technique for leak closure was used: combining a primary graft or sealing material such as bone wax, free muscle, or fascia for closure of the defect with additional autologous free grafts or allogenic materials. This resulted in a 2-year closure rate of 100% compared with a 2-year rate of 75.4% for patients whose primary closure was supported by a single layer of autologous or allogenic material (P =.034). Fibrin glue with primary closure alone did not have additional benefit. Postoperative meningitis occurred in two patients and was treated without sequelae. CONCLUSIONS: Conservative treatment should be reserved for cerebrospinal fluid otorrhea or otorhinorrhea resulting from head injury. Postoperative and nontraumatic cerebrospinal fluid otorrhea or otorhinorrhea should have early operative intervention. A multilayer technique combining allogenic materials with free autologous grafts is recommended.

Psychologic profile of tinnitus patients using the SCL-90-R and Tinnitus Handicap Inventory.

OBJECTIVE: To correlate the Tinnitus Handicap Inventory and the Symptom Checklist-90-R results on a group of tinnitus patients and to compare the average scores of the Tinnitus Handicap Inventory and SCL-90-R for help-seeking and non-help-seeking patients with tinnitus. STUDY DESIGN: A prospective study in which tinnitus patients were administered the Tinnitus Handicap Inventory and the Symptom Checklist-90-R. SETTING: Tertiary referral center. PATIENTS: Patients with tinnitus seeking audiological services. INTERVENTIONS: Rehabilitative. MAIN OUTCOME MEASURES: Results of Tinnitus Handicap Inventory and Symptom Checklist-90-R. RESULTS: Fifty-three consecutive patients having tinnitus were administered the Tinnitus Handicap Inventory and the Symptom Checklist-90-R. There was a significant correlation between the Symptom Checklist-90-R and the Tinnitus Handicap Inventory (rs = 0.43). Furthermore, 25% of these patients scored abnormally high on the Symptom Checklist-90-R, which is substantially more than the general medical population of patients. CONCLUSIONS: Based on this sample of 53 patients, the Symptom Checklist-90-R does seem to be a useful tool in identifying distress among tinnitus patients. The Global Severity Index of the Symptom Checklist-90-R has a defined cutoff score indicating significant distress levels, which makes it a useful screening tool for identifying those who would benefit from psychologic or psychiatric intervention.

Preliminary ossiculoplasty results using the Kurz titanium prostheses.

OBJECTIVE: Limitations in biocompatibility and hearing improvement with ossicular chain reconstruction prostheses are addressed with new, lightweight titanium prostheses designed to maximize visualization of the capitulum and footplate regions. The effectiveness of these new prostheses is being tested in a prospective multicenter study. STUDY DESIGN: Prospective case series. SETTING: Multicenter (8 sites), primarily tertiary private practice or academic otologic clinics. PATIENTS: A convenience sample of 31 patients undergoing ossiculoplasty, with 16 partial ossicular chain reconstructions using the Bell prosthesis and 15 total reconstructions using the Aerial prosthesis. INTERVENTION: Ossiculoplasty using new Kurz titanium prostheses. Cartilage was interposed between the tympanic membrane and the prosthesis. MAIN OUTCOME MEASURES: Air-bone gap for pure tone average and 3,000 and 4,000 Hz, assessed preoperatively and 3 months, 6 months, and 12 months postoperatively; percent of patients obtaining an air-bone gap of

Autoimmune inner ear disease: diagnostic and therapeutic approaches in a multidisciplinary setting.

Autoimmune Inner Ear Disease (AIED) is a clinical syndrome of uncertain pathogenesis. It is associated with bilateral rapidly progressive hearing loss. The hearing loss may be associated with vestibular symptoms. Autoimmunity has been proposed as the pathogenesis of this sort of hearing loss, although the mechanism of the disease is poorly understood. It is well accepted that the endolymphatic sac is an immunocompetent organ and circulating autoantibodies against inner ear antigens have been reported, as have viral antigens in the endolymph, although the sensitivity, specificity, and roles of those antibodies in a disease process are poorly defined. We will describe the clinical aspects of the disease, the histopathology, the immunologic indicators, the types of presentation, both from the audiologic and vestibular point of view, clinical trials for treatment and the follow-up. One of our conclusions is that many of these patients respond favorably to the treatment Methotrexate.

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.

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.

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.