Matched Comparison of Hearing Outcomes in Patients With Vestibular Schwannoma Treated With Stereotactic Radiosurgery or Observation.

BACKGROUND: Previous studies comparing hearing outcomes in patients managed with stereotactic radiosurgery (SRS) and a watch-and-wait strategy were limited by small sample sizes that prevented controlling for potential confounders, including initial hearing status, tumor size, and age. OBJECTIVE: To compare hearing outcomes for patients with vestibular schwannomas (VS) managed with observation and SRS while controlling for confounders with propensity score matching. METHODS: Propensity score matching was used to compare 198 patients with unilateral VS with initial serviceable hearing (99 treated with SRS and 99 managed with observation alone) and 116 with initial class A hearing (58 managed with SRS and 58 with observation), matched by initial hearing status, tumor volume, age, and sex. Kaplan-Meier survival methods were used to compare risk of losing class A and serviceable hearing. RESULTS: Between patients with VS managed with SRS or observation alone, there was no significant difference in loss of class A hearing (median time 27.2 months, 95% CI 16.8-43.4, and 29.2 months, 95% CI 20.4-62.5, P = .88) or serviceable hearing (median time 37.7 months, 95% CI 25.7-58.4, and 48.8 months, 95% CI 38.4-86.3, P = .18). For SRS patients, increasing mean cochlear dose was not related to loss of class A hearing (hazard ratio 1.3, P = .17) but was associated with increasing risk of serviceable hearing loss (hazard ratio of 1.5 per increase in Gy, P = .017). CONCLUSION: When controlling for potential confounders, there was no significant difference in loss of class A or serviceable hearing between patients managed with SRS or with observation alone.

Modern Hearing Preservation Outcomes After Vestibular Schwannoma Stereotactic Radiosurgery.

BACKGROUND: For patients with vestibular schwannoma (VS), stereotactic radiosurgery (SRS) has proven effective in controlling tumor growth while hearing preservation remains a key goal. OBJECTIVE: To evaluate hearing outcomes in the modern era of cochlear dose restriction. METHODS: During the years 2013 to 2018, 353 patients underwent Gamma knife surgery for VS at our institution. We followed 175 patients with pre-SRS serviceable hearing (Gardner-Robertson Score, GR 1 and 2). Volumetric and dosimetry data were collected, including biological effective dose, integral doses of total and intracanalicular tumor components, and hearing outcomes. RESULTS: The mean age was 56 years, 74 patients (42%) had a baseline GR of 2, and the mean cochlear dose was 3.5 Gy. The time to serviceable hearing loss (GR 3-4) was 38 months (95% CI 26-46), with 77% and 62% hearing preservation in the first and second years, respectively. Patients optimal for best hearing outcomes were younger than 58 years with a baseline GR of 1, free canal space ≥0.041 cc (diameter of 4.5 mm), and mean cochlear dose <3.1 Gy. For such patients, hearing preservation rates were 92% by 12 months and 81% by 2 years, staying stable for >5 years post-SRS, significantly higher than the rest of the population. CONCLUSION: Hearing preservation after SRS for patients with VS with serviceable hearing is correlated to the specific baseline GR score (1 or 2), age, cochlear dose, and biological effective dose. Increased tumor-free canal space correlates with better outcomes. The most durable hearing preservation correlates with factors commonly associated with smaller tumors away from the cochlea.

Temporal Bone Encephaloceles: Utility of Preoperative Imaging.

OBJECTIVE: To determine the diagnostic efficacy and clinical value of preoperative computed tomography (CT) and magnetic resonance imaging (MRI) among patients with suspected temporal bone encephaloceles (TBE). STUDY DESIGN: Retrospective chart review from 2006 to 2018. SETTING: Tertiary referral center. SUBJECTS AND METHODS: The subjects underwent surgery for a clinically suspected TBE or cerebrospinal fluid (CSF) leak. Preoperative imaging test characteristics of CT and MRI, including sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV), were calculated for TBE scans and compared with intraoperative findings. RESULTS: Fifty-seven subjects with otorrhea, middle ear effusion, hearing loss, and/or meningitis with suspected TBE or CSF leak were identified. All had preoperative CT scans, and 61% (35/57) had preoperative MRI scans. Intraoperatively, 37 of 57 patients (65%) were found to have a TBE. CT scans (17% sensitivity, 100% specificity, 100% PPV, 46% NPV) were less sensitive than MRI (58% sensitivity, 100% specificity, 100% PPV, 24% NPV) for detecting TBEs. Furthermore, the time from initial scan to operative repair was significantly longer in those who had a CT followed by MRI scan compared with CT alone or a fused CT-MRI scan (mean = 68 vs 15 days, respectively; P = .004). CONCLUSION: CT and MRI provide complementary information that may aid surgical planning. However, imaging cannot always rule out TBE. In cases with high clinical suspicion, surgical confirmation is often required for definitive diagnosis and treatment. The cost of an additional preoperative study should be considered before its use.

Transmastoid Repair of Spontaneous Cerebrospinal Fluid Leaks.

Objective Determine the efficacy of using a purely transmastoid approach for the repair of spontaneous cerebrospinal fluid (CSF) leaks and further elucidate the relationship of elevated body mass index (BMI) and skull base thickness in our patient population. Method We conducted a retrospective chart review of patients treated for spontaneous temporal bone CSF leaks at our tertiary care institution from the years 2006 to 2015. Cases were categorized as primary or secondary. We analyzed success rates, length of stay, use of lumbar drains, BMIs, and rates of meningitis. Skull base thickness was compared with BMI in each case. Results We identified 26 primary operations for spontaneous CSF leaks and 7 secondary operations. Twenty-three of 33 repairs were performed via the transmastoid approach alone with an 87% success rate (20/23). Of the10 repairs including a middle cranial fossa (MCF) or combined MCF-transmastoid approach, 2 failed for an 80% success rate (8/10). Five transmastoid repairs underwent placement of a lumbar drain versus all 10 repairs employing an intracranial exposure. Average length of stay for those undergoing a transmastoid approach (1.7 days) was significantly shorter than for patients undergoing a MCF repair (6.3 days). Four patients presented with meningitis. Average BMI was 35.3. No correlation was established between BMI and skull base thickness ( R2 = 0.00011). Conclusion The transmastoid approach is effective in the majority of cases and prevents the need for an intracranial operation, resulting in lower morbidity and a shorter length of stay. We believe that this is the preferred primary approach in most patients with spontaneous CSF leaks.

Malignant external otitis: The shifting treatment paradigm.

IMPORTANCE: Malignant external otitis (MEO) is an aggressive infection occurring in immunocompromised hosts. Increasing antimicrobial resistance is making the disease more difficult to treat. OBJECTIVE: Determine if there has been a shift in the microbiology and outcomes of MEO. DESIGN: A retrospective case series at a tertiary care institution. SETTING: Inpatient and outpatient tertiary care hospital. PARTICIPANTS: 12 cases of recent MEO were reviewed. MAIN OUTCOMES AND MEASURES: The primary outcome was progression of disease. Secondary outcomes were drug resistance and complications of MEO. RESULTS: Only 4 patients were cured of MEO. Four patients expired during the study period and at least one of these deaths was a direct result of the MEO. 7 patients developed Cranial nerve palsies, and 3 patients developed abscesses. CONCLUSIONS: Select cases of MEO now require multi-drug and long-term parenteral antibiotic therapy with extended hospital stays.

The Effects of Compensatory Auditory Stimulation and High-Definition Transcranial Direct Current Stimulation (HD-tDCS) on Tinnitus Perception - A Randomized Pilot Study.

BACKGROUND: Tinnitus correlates with elevated hearing thresholds and reduced cochlear compression. We hypothesized that reduced peripheral input leads to elevated neuronal gain resulting in the perception of a phantom sound. OBJECTIVE: The purpose of this pilot study was to test whether compensating for this peripheral deficit could reduce the tinnitus percept acutely using customized auditory stimulation. To further enhance the effects of auditory stimulation, this intervention was paired with high-definition transcranial direct current stimulation (HD-tDCS). METHODS: A randomized sham-controlled, single blind study was conducted in a clinical setting on adult participants with chronic tinnitus (n = 14). Compensatory auditory stimulation (CAS) and HD-tDCS were administered either individually or in combination in order to access the effects of both interventions on tinnitus perception. CAS consisted of sound exposure typical to daily living (20-minute sound-track of a TV show), which was adapted with compressive gain to compensate for deficits in each subject's individual audiograms. Minimum masking levels and the visual analog scale were used to assess the strength of the tinnitus percept immediately before and after the treatment intervention. RESULTS: CAS reduced minimum masking levels, and visual analog scale trended towards improvement. Effects of HD-tDCS could not be resolved with the current sample size. CONCLUSIONS: The results of this pilot study suggest that providing tailored auditory stimulation with frequency-specific gain and compression may alleviate tinnitus in a clinical population. Further experimentation with longer interventions is warranted in order to optimize effect sizes.

Endovascular Management of Sigmoid Sinus Diverticulum.

Sigmoid sinus diverticulum (SSD) is a rare vascular disorder due to dehiscence of the sigmoid plate. It may be associated with prediverticular venous sinus stenosis (SS) and usually presents as pulsatile tinnitus. The mechanism of development of the SSD and tinnitus from a sinus diverticulum and associated SS is unclear. Previous case reports have suggested that remodeling of the venous system targeting the stenosis, elimination of the diverticulum, or both, have resulted in symptom relief. We present a case of SSD with SS, treated by stenting of the stenosis along with coil embolization of the diverticulum, resulting in complete relief of symptoms. We have also reviewed the literature and discussed the evolution of management from open surgical treatment to endovascular treatment.

Identification of a novel pathogenic OTOF variant causative of nonsyndromic hearing loss with high frequency in the Ashkenazi Jewish population.

Mutations in the OTOF gene have previously been shown to cause nonsyndromic prelingual deafness (DFNB9, OMIM 601071) as well as auditory neuropathy/dys-synchrony. In this study, the OTOF NM_194248.2 c.5332G>T, p.Val1778Phe variant was identified in a large Ashkenazi Jewish family as the causative variant in four siblings with hearing loss. Our analysis reveals a carrier frequency of the OTOF c.5332G>T, p.Val1778Phe variant of 1.27% in the Ashkenazi Jewish population, suggesting that this variant may be a significant contributor to nonsyndromic sensorineural hearing loss and should be considered for inclusion in targeted hearing loss panels for this population. Of note, the degree of hearing loss associated with this phenotype ranged from mild to moderately severe, with two of the four siblings not known to have hearing loss until they were genotyped and underwent pure tone audiometry and auditory brainstem response testing. The phenotypic variability along with the auditory neuropathy/dys-synchrony, which allows for the production of otoacoustic emissions, supports that nonsyndromic hearing loss caused by OTOF mutations may be much more common in the Ashkenazi Jewish population than currently appreciated due to a lack of diagnosis.

Cervical-petrous internal carotid artery pseudoaneurysm presenting with otorrhagia treated with endovascular techniques.

Cervical-petrous internal carotid artery (CP-ICA) pseudoaneurysms are rare and have different etiologies, presentations, and treatment options. A middle-aged patient with a history of chronic otitis media presented with acute otorrhagia and was found to have a left-sided CP-ICA pseudoaneurysm. The patient was a poor surgical candidate with difficult arterial access. The pseudoaneurysm was treated with stand-alone coiling via a left brachial approach with persistent contrast filling seen only in the aneurysm neck at the end of the procedure. The patient re-presented 12 days later with repeat hemorrhage and rapid enlargement of the neck remnant, and was treated with a covered stent via a transcervical common carotid artery cut-down. A covered stent may provide a more definitive treatment for CP-ICA pseudoaneurysms compared with standalone coiling.

Readaptation of the vestibulo-ocular reflex relieves the mal de debarquement syndrome.

The mal de debarquement syndrome (MdDS), a continuous feeling of swaying, rocking, and/or bobbing, generally follows travel on the sea. The associated symptoms cause considerable distress. The underlying neural mechanisms are unknown, and to date there have been no effective treatments for this condition. Results in monkeys and humans suggested that MdDS was caused by maladaptation of the vestibulo-ocular reflex (VOR) to roll of the head during rotation. We studied 24 subjects with persistent MdDS (3 males, 21 females; 19.1 ± 33 months). Physical findings included body oscillation at 0.2 Hz, oscillating vertical nystagmus when the head was rolled from side-to-side in darkness, and unilateral rotation during the Fukuda stepping test. We posited that the maladapted rocking and the physical symptoms could be diminished or extinguished by readapting the VOR. Subjects were treated by rolling the head from side-to-side while watching a rotating full-field visual stimulus. Seventeen of the 24 subjects had a complete or substantial recovery on average for approximately 1 year. Six were initially better, but the symptoms recurred. One subject did not respond to treatment. Thus, readaptation of the VOR has led to a cure or substantial improvement in 70% of the subjects with MdDS. We conclude that the adaptive processes associated with roll-while-rotating are responsible for producing MdDS, and that the symptoms can be reduced or resolved by readapting the VOR.

Cervical-petrous internal carotid artery pseudoaneurysm presenting with otorrhagia treated with endovascular techniques.

Cervical-petrous internal carotid artery (CP-ICA) pseudoaneurysms are rare and have different etiologies, presentations, and treatment options. A middle-aged patient with a history of chronic otitis media presented with acute otorrhagia and was found to have a left-sided CP-ICA pseudoaneurysm. The patient was a poor surgical candidate with difficult arterial access. The pseudoaneurysm was treated with stand-alone coiling via a left brachial approach with persistent contrast filling seen only in the aneurysm neck at the end of the procedure. The patient re-presented 12 days later with repeat hemorrhage and rapid enlargement of the neck remnant, and was treated with a covered stent via a transcervical common carotid artery cut-down. A covered stent may provide a more definitive treatment for CP-ICA pseudoaneurysms compared with standalone coiling.

High-resolution microendoscope images of middle ear cholesteatoma and surrounding tissue: evaluation of interobserver concordance.

OBJECTIVE: Investigate how accurately otolaryngologists could differentiate between images obtained with high-resolution microendoscopy (HRME) of ex vivo cholesteatoma specimens and surrounding middle ear epithelium. STUDY DESIGN: HRME images of surgically resected cholesteatoma and middle ear epithelium were obtained and otolaryngologists classified these images. SETTING: Tertiary medical center. SUBJECTS AND METHODS: Resected cholesteatoma and middle ear epithelium were stained with a contrast agent, proflavine, and HRME images were captured. Specimens were sent for standard histopathology and compared with HRME images. Quality-controlled images were used to assemble a training set. After viewing training images, otolaryngologists without prior cholesteatoma HRME experience reviewed and classified test images. RESULTS: Ten cholesteatoma and 9 middle ear specimens were collected, of which 17 representative cholesteatoma and 19 middle ear epithelium images were extracted for a testing set. Qualitative analysis for concordance between HRME images and histological images yielded a strong correlation between modalities. The mean accuracy of all reviewers in correctly identifying images was 95% (95% confidence interval [CI], 92%-98%). The sensitivity to correctly detect cholesteatoma images was 98% (95% CI, 93%-100%), and the specificity was 92% (95% CI, 87%-97%). The Fleiss kappa interrater reliability score was 0.83, (95% CI, 0.77-0.89). CONCLUSIONS: Medical professionals can quickly be trained to accurately distinguish between HRME images of cholesteatoma and normal middle ear epithelium, both of which have distinct imaging characteristics. Real-time HRME optical imaging can potentially improve the results of otologic surgery by allowing for extirpation of cholesteatomas while eliminating residual disease.

Inner ear disorders.

OBJECTIVES: To present a framework for the diagnosis and treatment of inner ear disorders, with an emphasis on problems common to neuro-rehabilitation. INTRODUCTION: Disorders of the inner ear can cause hearing loss, tinnitus, vertigo and imbalance. Hearing loss can be conductive, sensorineural, or mixed; conductive hearing loss arises from the ear canal or middle ear, while sensorineural hearing loss arises from the inner ear or auditory nerve. Vertigo is a hallucination of motion, and is the cardinal symptom of vestibular system disease. It should be differentiated from other causes of dizziness: gait imbalance, disequilibrium, lightheadedness (pre-syncope). Vertigo can be caused by problems in the inner ear or central nervous system. METHODS: The diagnosis of inner ear disorders begins with a targeted physical examination. The initial work-up of hearing loss is made by audiometry, and vertigo by electronystagmography (ENG). Supplemental tests and MRI are obtained when clinically indicated. RESULTS: The clinical pattern and duration of vertigo are the most important clinical features in the diagnosis. Common inner ear causes of vertigo include: vestibular neuritis (sudden, unilateral vestibular loss), Meniere's disease (episodic vertigo), benign paroxysmal positional vertigo (BPPV), and bilateral vestibular loss. Common central nervous system causes of vertigo include: post concussion syndrome, cervical vertigo, vestibular migraine, cerebrovascular disease, and acoustic neuroma. CONCLUSION: A basic knowledge of vestibular physiology, coupled with a understanding of common vestibular syndromes, will lead to correct diagnosis and treatment in most cases.

Mastoid osteoma: A case report and review of the literature.

We describe the case of a 22-year-old woman who presented with a slowly growing osseous lesion of the mastoid cortex. On computed tomography, the lesion was found to involve the mastoid cortex, with which it demonstrated similar attenuation. The indications for treatment in this case were the patient's sensation of a mass effect, the encroachment of the mass onto the external auditory meatus, and a cosmetic deformity. The tumor was removed in its entirety via a postauricular approach. Findings on histopathologic examination were consistent with a compact osteoma. Mastoid osteomas are rare, benign tumors. If their growth significantly occludes the meatus, they may cause cosmetic deformities, conductive hearing loss, and recurrent external ear infections. Several other osseous lesions of the temporal bone should be considered in the differential diagnosis. The etiology of mastoid osteomas is poorly understood. Surgical management can be undertaken with minimal postoperative morbidity.

Optical imaging with a high-resolution microendoscope to identify cholesteatoma of the middle ear.

OBJECTIVES/HYPOTHESIS: High-resolution optical imaging is an imaging modality that allows visualization of structural changes in epithelial tissue in real time. Our prior studies using contrast-enhanced microendoscopy to image squamous cell carcinoma in the head and neck demonstrated that the contrast agent, proflavine, has high affinity for keratinized tissue. Thus, high-resolution microendoscopy with proflavine provides a potential mechanism to identify ectopic keratin production, such as that associated with cholesteatoma formation, and distinguish between uninvolved mucosa and residual keratin at the time of surgery. STUDY DESIGN: Ex vivo imaging of histopathologically confirmed samples of cholesteatoma and uninvolved middle ear epithelium. METHODS: Seven separate specimens collected from patients who underwent surgical treatment for cholesteatoma were imaged ex vivo with the fiberoptic endoscope after surface staining with proflavine. Following imaging, the specimens were submitted for hematoxylin and eosin staining to allow histopathological correlation. RESULTS: Cholesteatoma and surrounding middle ear epithelium have distinct imaging characteristics. Keratin-bearing areas of cholesteatoma lack nuclei and appear as confluent hyperfluorescence, whereas nuclei are easily visualized in specimens containing normal middle ear epithelium. Hyperfluorescence and loss of cellular detail is the imaging hallmark of keratin, allowing for discrimination of cholesteatoma from normal middle ear epithelium. CONCLUSIONS: This study demonstrates the feasibility of high-resolution optical imaging to discriminate cholesteatoma from uninvolved middle ear mucosa based on the unique staining properties of keratin. Use of real-time imaging may facilitate more complete extirpation of cholesteatoma by identifying areas of residual disease. Laryngoscope, 2012.

Surgical treatment of Bell's palsy: current attitudes.

OBJECTIVES/HYPOTHESIS: To learn the current management of Bell's palsy among practicing otologists and neurotologists and to better define the role of surgical decompression of the facial nerve in the treatment of Bell's palsy. STUDY DESIGN: Survey questionnaire. METHODS: We conducted a survey of members of the American Otological Society and the American Neurotology Society to learn their current practices in the treatment of Bell's palsy. RESULTS: Eighty-six neurotologists responded out of 334 surveys (26%). The majority of respondents obtain magnetic resonance imaging and electrical testing for new patients and treat with a combination of steroids and antiviral agents. More than two thirds of respondents would recommend surgery to patients who met the established electrophysiologic criteria (electroneuronography <10% normal, no spontaneous motor unit action potentials on electromyography within 10 days of onset of complete paralysis). However, only half believe that surgical decompression should be the standard of care, and only half would use a standard middle fossa approach. Lack of evidence was the most commonly cited reason for not recommending surgery. Several respondents wrote that they would leave the option of surgery to the patient. Most important, one third of neurotologists have not performed a surgical decompression for Bell's palsy in the last 10 years, and 95% perform less than one procedure per year. CONCLUSIONS: Disagreement persists among practicing otologists about the role of surgical decompression for Bell's palsy. More convincing clinical evidence will be needed before there is widespread consensus regarding the surgical treatment of this condition.

Cerebrospinal fluid leaks of temporal bone origin: selection of surgical approach.

Cerebrospinal fluid leaks of the temporal bone are rare, often occult, and sometimes challenging to localize and repair. This is a retrospective study of eight patients with spontaneous cerebrospinal fluid leak and six patients with cerebrospinal fluid leak or encephalocele discovered during chronic ear surgery who were treated in a tertiary medical center over a 5-year period. All received preoperative temporal bone computed tomography, and six also underwent magnetic resonance imaging, one computed tomography cisternography, and one radionuclide cisternography. All patients initially underwent a transmastoid surgical approach. Additional exposure was necessary in three patients; two underwent middle fossa craniotomy and another required minicraniotomy. Primary surgical repair was successful in six of the eight patients with spontaneous leaks and in all six chronic ear patients. Both recurrences required intradural middle fossa repair. An individualized approach should be taken for repair of temporal bone cerebrospinal fluid leaks. In this series, most were successfully repaired in a single stage using a transmastoid or combined approach. The transmastoid approach provides information about the precise size and location of the dural defect. A primary transcranial approach is needed for defects that are multiple, located in the petrous apex, and in revision cases.

Facial Nerve Grading System 2.0.

OBJECTIVE: To present an updated version of the original Facial Nerve Grading Scale (FNGS), commonly referred to as the House-Brackmann scale. STUDY DESIGN: Controlled trial of grading systems using a series of 21 videos of individuals with varying degrees of facial paralysis. RESULTS: The intraobserver and interobserver agreement was high among the original and revised scales. Nominal improvement is seen in percentage of exact agreement of grade and reduction of instances of examiners differing by more then one grade when using FNGS 2.0. FNGS 2.0 also offers improved agreement in differentiating between grades 3 and 4. CONCLUSION: FNGS 2.0 incorporates regional scoring of facial movement, providing additional information while maintaining agreement comparable to the original scale. Ambiguities regarding use of the grading scale are addressed.

Petrous apex aspergillosis as a long-term complication of cholesterol granuloma.

Aspergillus infection of the petrous apex is a rare and devastating condition. To date, only two such cases have been reported, which resulted from direct extension of chronic Aspergillus otitis media. We present a case of petrous apex aspergillosis occurring years after surgical drainage of a petrous apex granuloma cyst. Because of the potential lethal nature of this condition, aggressive surgical therapy should be considered early in this illness and may provide the best chance for survival.