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.

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.

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.

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.

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.

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.

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.

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.

Flavanoid of Drynaria fortunei protects against gentamicin ototoxicity.

A flavanoid fraction (FF) from Drynaria fortunei, was investigated to see if it has the protective and ameliorative effects against gentamicin (GM) ototoxicity in guinea pigs (n = 36). Eleven (GM-group) animals received GM 100 mg/kg/day. Eleven (GMFF-group) animals received the same dose of GM but 2 days prior were dosed with FF (10 mg/kg/day) for 2 weeks. Seven (S-group) animals received saline and seven (FF-group) animals received the same dose of FF as the GMFF-group. The thresholds of tone-burst auditory evoked response (ABR) at 2 k, 8 k, and 32 k Hz were determined to be as follows: GM-group: 90 dB, 92 dB and 72 dB, GMFF-group: 30 dB, 37 dB and 38 dB, FF-group: 28 dB, 25 dB and 29 dB, S-group: 30 dB, 28 dB and 39 dB. The GM-group had a significantly higher hearing threshold than the other groups (p < 0.05). The GMFF- and FF-groups had hearing thresholds similar to the S-groups (p > 0.1). Repair of damaged hair cells was observed histologically. The percentage of the damaged outer hair cells (OHC) and inner hair cells (IHC) were determined to be as follows: GM-group: 43% and 20%, GMFF-group: 20% and 2%, FF-group: 9% and 2% and S-group: 4% and 1%. The GMFF-group showed less damage to the OHC (p > 0.05) and significantly less damage to the IHC (p < 0.05) than the GM-group. FF did not change the antimicrobial activity of GM and it did not show any intrinsic antibacterial effect. FF did not affect the kinetics of GM during the course of the experiment.

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.