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.

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.

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.

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.

Cholesteatoma in the normal hearing ear.

OBJECTIVES/HYPOTHESIS: Surgical treatment of cholesteatoma in ears with normal or near-normal hearing represents a challenge, in that complete removal of disease may require sacrifice of the ossicular chain. Our aim was to identify the predictive factors and surgical strategies that favor hearing preservation in these patients. STUDY DESIGN: Retrospective case review. METHODS: Fifty-four procedures were performed in 50 patients with cholesteatoma and a preoperative speech reception threshold or pure-tone average (PTA) of less than 25 dB. Complete audiometric data were available in 51 cases. All patients had complete surgical removal of cholesteatoma. Whenever feasible, ossicular reconstruction was performed at the time of the initial surgical procedure. RESULTS: The median PTA changed by -3 dB, and hearing was preserved to within 10 dB of preoperative level in 72% of patients. An intact ossicular chain was found in 72% of the cases and could often be preserved at surgery. However, similar hearing outcomes resulted after intact versus reconstructed ossicular chains and in open versus closed mastoidectomies. The recidivism rate was 26%, and recidivistic cases had worse hearing outcomes. Congenital cholesteatomas and Prussak space cholesteatomas had better outcomes with respect to hearing preservation and recidivism. CONCLUSIONS: Cholesteatoma in the normal hearing ear should be treated with the same surgical priorities as all other cholesteatomas. Preventing recidivism has a significant effect on hearing preservation, whereas preserving an intact ossicular chain and maintaining an intact canal wall do not.

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.

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.

Conservative management of acoustic neuroma: a meta-analysis and proposed treatment algorithm.

OBJECTIVES/HYPOTHESES: Conservative management is a viable treatment alternative for acoustic neuroma. Using previous studies to provide evidence-based support, we have attempted to more clearly define the role of conservative management. STUDY DESIGN: Retrospective review of literature and patient charts. METHODS: Published studies on conservative management of acoustic neuroma were found using a key word search through PubMed in addition to the bibliographies of these selected studies. A spreadsheet was made to tabulate the selection criteria for conservative management, duration and frequency of follow-up, patient demographics, initial tumor size and rate of growth, change in hearing status, and the need for definitive treatment. RESULTS: A total of 21 studies comprising 1,345 patients were included in our meta-analysis. The average length of follow-up these studies was 3.2 years. The average initial tumor size was 11.8 mm (n = 900); 43% of 1,244 acoustic neuromas showed growth, whereas 57% showed either no growth or tumor regression. The average growth rate was 1.9 mm/year in 793 individuals. Hearing loss occurred in 51% of 347 individuals. In 15 studies, 20.0% of 1,001 individuals eventually failed conservative management. CONCLUSIONS: Our meta-analysis supports the role of conservative management of acoustic neuromas in properly selected patients on the basis of a slow overall rate of growth and a substantial incidence of no growth. However, the lack of predictive factors, the relatively short duration of follow-up, and the variability of inclusion criteria underscore the need for continued collection of long-term data. An algorithm for acoustic neuroma management is proposed based on initial tumor size, patient age, and hearing status.

Tuberculous otitis media: report of 2 cases on Long Island, N.Y., and a review of all cases reported in the United States from 1990 through 2003.

We report 2 cases of tuberculous otitis media that were diagnosed at Stony Brook University Hospital in New York since 1999. Both patients were women, aged 30 and 31 years. One patient had grown up in Russia, the other was a native-born American who had never left the East Coast region of the United States. Both patients had been symptomaticfor many months; one complainedof chronic otorrhea, and the other reported otorrhea, hearing loss, and discomfort. Neither patient responded to medical management, and both ultimately underwent surgery. One was diagnosed after surgical pathology revealed acid-fast bacilli on frozen-section analysis. In the other, pathology revealed chronic inflammation and granulomata, butstains were negative and her diagnosis was delayed for almost 2 years. We also review 9 other cases of tuberculous otitis media in the United States that have been reported in the literature since 1990. Our review suggests that the number of cases is rising in areas where tuberculosis is most common--that is, in major U.S. cities. Although 3 of these 9 cases occurred as reactivation disease in immigrants, most might have occurred as a result of local transmission. Clinicians should maintain a high degree of suspicion for tuberculosis in patients with chronic otitis symptoms, particularly those who are at higher risk of exposure to tuberculosis.

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.

Surgery of the inner ear with hearing preservation: serial histological changes.

OBJECTIVES/HYPOTHESIS: Surgery of the inner ear can result in hearing preservation under certain conditions, but the mechanisms responsible for hearing preservation or loss are not well understood. The specific aim of the study is to examine histological sections obtained at different time intervals after varying degrees of surgical entry into the inner ear, to understand how the cochlea is protected. The hypothesis is that internal partitioning occurs. STUDY DESIGN: Histologic examination of guinea pig inner ears by light microscopy. METHODS: Guinea pigs underwent lateral semicircular canal transection and plugging, ampullectomy, or vestibulotomy, and tone-burst auditory brainstem response thresholds at 2, 8, and 24 kHz were measured at intervals before and after surgery. Animals were killed after 1, 3, 7, or 21 or more days, and temporal bones were examined histologically. RESULTS: The histological response to surgical trauma consists of fibrosis and varying amounts of inflammation near the site of surgical entry. Cochlear hair cells are nearly always preserved, even when hearing loss occurs. Extension of the inflammatory response to the cochlea is associated with greater degrees of hearing loss. CONCLUSION: The guinea pig inner ear is capable of withstanding surgical trauma to the semicircular canals and vestibule without complete loss of cochlear function. Fibrosis creates an effective partition between the site of surgical entry and the rest of the inner ear. Cochlear preservation might be enhanced if the inflammatory response can be contained.

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.

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.

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.

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.