Search

Objectives: Facial nerve schwannomas (FNSs) and vestibular nerve schwannomas (VNSs) may occupy similar locations in the internal auditory canal (IAC). Risk factors for hearing loss in patients with FNS are unknown. Similar to VNSs, we hypothesize that the size and location of FNSs do not correlate with hearing loss.Study Design: Retrospective cohort study and otopathology case review.Methods: A single institution retrospective review of all patients diagnosed with FNS was performed. Demographic data, tumor size and location, severity and type of hearing loss, and facial nerve function were analyzed. Otopathologic review of a case of FNS was also performed.Results: Thirty-three patients with FNS were identified. Mean age was 44 years, and 61% (n = 21) were female. Nine patients had tumors involving the IAC facial nerve, whereas 24 had distal tumors without IAC involvement. Eleven patients (33%) had hearing loss, and 55% of patients with sensorineural hearing loss (SNHL) had tympanic segment tumors without IAC involvement. The presence of SNHL was not associated with IAC tumor location (P = 0.21). Maximal dimensions of FNSs were 3 mm to 42 mm, without correlation between size and SNHL severity (r = -0.017, P = 0.926). Otopathologic review of an untreated 5-mm IAC FNS demonstrated disproportionate spiral ganglion and hair cell loss within the ipsilateral cochlea compared to the contralateral ear.Conclusion: Tympanic segment FNS without IAC involvement may result in SNHL. Tumor size is not associated with the presence or severity of SNHL. Otopathologic review suggests degenerative cochlear changes in FNS. Mechanisms for SNHL in schwannomas are likely multifactorial and may be unrelated to cochlear nerve compression.Level Of Evidence: 4. Laryngoscope, 127:1676-1682, 2017. [ABSTRACT FROM AUTHOR]

Objectives/Hypothesis: To determine the utility of cervical vestibular evoked myogenic potential (cVEMP) thresholds in the surgical management of bilateral superior canal dehiscence syndrome (SCDS). Study design: Retrospective review. Methods: We identified patients who underwent surgical treatment for SCDS from our database of 147 patients diagnosed with superior canal dehiscence (SCD) between 2000 and 2011 at our institution. The diagnosis of SCDS was based on clinical signs and symptoms, audiometric and cVEMP testing, and high-resolution computed tomography. Results: We identified 38 patients who underwent SCD surgery in 40 ears (2 bilateral). In seven patients with bilateral SCD, the more symptomatic ear had lower cVEMP thresholds, a larger air bone gap and a lateralizing tuning fork. In 13 patients with perioperative cVEMP testing, thresholds increased in 12 patients following primary repair, and no threshold shift was seen in one patient with persistence of symptoms after revision surgery. Audiometric data showed a significant mean decrease of the low-frequency air-bone gap and a mild (high-frequency) bone conduction loss after surgical repair. Conclusions: We found that, 1) preoperative cVEMP thresholds, the magnitude of the air-bone gap and tuning-fork testing are important to confirm the worse ear in patients with bilateral SCD, 2) elevation of cVEMP thresholds following surgery correlates with improvement of symptoms and underscores the importance of postoperative testing in patients with bilateral disease or recurrence of symptoms and, 3) SCD plugging is associated with a partial closure of the air-bone gap and a mild (high-frequency) sensorineural hearing loss. Laryngoscope, 2013 [ABSTRACT FROM AUTHOR]

Objectives: Proteins in perilymph may alter the delivery profile of implantable intracochlear drug delivery systems through biofouling. Knowledge of protein composition will help anticipate interactions with delivered agents. Study Design: Analysis of mouse perilymph. Methods: Protein composition of perilymph and cerebrospinal fluid (CSF) was analyzed using a capillary liquid chromatography-mass spectrometry-based iTRAQ quantitative proteomics approach. We searched against a mouse subset of the Uniprot FASTA protein database. We sampled perilymph from the apex of the mouse cochlea to minimize CSF contamination. Results: More than 50 explicit protein isoforms were identified with very high confidence. iTRAQ reporter ions allowed determination of relative molar amounts of proteins between perilymph and CSF. Protein in perilymph was almost three times more concentrated than in CSF. More than one-third of the proteins in perilymph comprised protease inhibitors, with serpins being the predominant group. Apolipoproteins constituted 16%. Fifteen percent of the proteins were enzymes. Albumin was the most abundant single protein (14%). Proteins with relatively high perilymph/CSF ratios included broad-spectrum protease inhibitors and apolipoproteins. Discussion: Some proteins found in perilymph, such as albumin and HMW kininogen, have been implicated in biofouling through adsorption to device materials. The relatively large quantities of apolipoprotein and albumin may serve as a reservoir for acidic and lipophillic drugs. Alpha-2-glycoprotein can bind basic drugs. Conclusions: Perilymph is similar in protein composition to CSF, though amounts are 2.8 times higher. Protease inhibitors comprise the largest category of proteins. Laryngoscope, 2009 [ABSTRACT FROM AUTHOR]

Objectives: The otic capsule, when compared with other bones in the body, is unique in that it undergoes no significant remodeling of bone after development. We previously demonstrated that osteoprotegerin (OPG), which inhibits formation and function of osteoclasts, is produced at high levels in the inner ear of normal mice and secreted into the perilymph from where it diffuses into the surrounding otic capsule bone through a lacunocanalicular system. To test our hypothesis that the high level of OPG may be important in the inhibition of otic capsule remodeling, we studied the light microscopic histology of the otic capsule in OPG knockout mice for evidence of abnormal remodeling of bone. We also tested the hearing in OPG knockout mice to determine whether OPG and its influence on surrounding bone is important for auditory function.Methods: Temporal bone histopathology and pathophysiology were compared in homozygous OPG knockout mice and C57BL/6 (B6) mice, the background strain for the knockouts. Auditory function in age-matched animals from each group was evaluated at approximately 4-week intervals from 8 to 21 weeks using frequency-specific auditory brainstem responses (ABR) and distortion product otoacoustic emissions (DPOAE). After each of the last three evaluations, the cochleae from one mouse of each group were harvested, processed, and examined by light microscopy.Results: Osteoprotegerin knockout mice demonstrated abnormal remodeling of bone within the otic capsule with multiple foci showing osteoclastic bone resorption and formation of new bone. Such changes were not seen in the age-matched B6 controls. The active bone remodeling process in the knockout animals showed many similarities to otosclerosis seen in human temporal bones. Over the time period that we monitored, auditory function was significantly and progressively compromised in the knockout animals relative to B6 controls. At the earliest age of test (8 wk), the loss was apparent as a mild, high-frequency reduction in sensitivity by ABR. In contrast, DPOAE losses in the knockouts were substantial even at 8 weeks, and by 21 weeks, these losses exceeded our equipment limits. Results of ABR testing showed hearing sensitivity changes in the animals of the background strain were confined largely to the high frequencies, whereas OPG knockouts demonstrated substantial low-frequency shifts in addition to those at high frequencies.Conclusions: The histopathological and pathophysiological findings in OPG knockout mice support the hypothesis that OPG is important in the inhibition of bone remodeling within the otic capsule and the maintenance of normal auditory function. This mouse may provide a valuable animal model of human otosclerosis. [ABSTRACT FROM AUTHOR]

Objectives: To elucidate factors that may be responsible for the inhibition of remodeling of bone within the otic capsule. Methods: Expression of osteoprotegerin (OPG), receptor activator of nuclear factor kappa B (RANK), and RANK ligand (RANKL) were assayed in samples of bone obtained from the otic capsule, calvarium, and femur, and from the soft tissue within the cochlea using semiquantitative reverse transcriptase polymerase chain reaction (RT-PCR) in mice. Immunostaining was used for histologic localization of the gene products. An enzyme-linked immunosorbent assay (ELISA) was used to quantify the amount of OPG within perilymph, serum, and cerebrospinal fluid. The micro-anatomy of the interface between the otic capsule and the fluid spaces of the cochlea was investigated by brightfield and phase-contrast microscopy and by three-dimensional reconstruction in the mouse and human. Results: OPG, a powerful inhibitor of bone remodeling, was expressed at extremely high levels within the soft tissue of the cochlea and was present in the perilymph at very high concentrations. The OPG produced within the inner ear may diffuse into the surrounding otic capsule, where it may be responsible for inhibition of bone turnover. Our anatomic studies revealed an extensive system of interconnected canaliculi within the otic capsule that had direct openings into the fluid spaces of the inner ear, thus providing a possible anatomic route for the diffusion of OPG from the inner ear into the surrounding bone. Conclusion: OPG, a potent inhibitor of osteoclast formation and function, is expressed at high levels within the inner ear and is secreted into the perilymph and the surrounding bone and may serve to inhibit active bone remodeling within the otic capsule, especially immediately adjacent to the cochlea. By this means, the cochlear soft tissue may control the nature of the surrounding petrous bone. [ABSTRACT FROM AUTHOR]

Objective To develop and validate a patient-based instrument to measure both facial impairment and disability, the Facial Clinimetric Evaluation (FaCE) Scale. Study Design Prospective instrument validation. Methods Eighty-six patients with a documented history of facial paralysis completed a preliminary, 51-item instrument (alpha FaCE Scale), as well as the previously developed Facial Disability Index (FDI) and the Medical Outcomes Study Short Form 36 Item Questionnaire (SF-36). Two weeks after completing these instruments, 76 patients again completed the alpha FaCE Scale. Forty-one of the patients were also evaluated using the House-Brackmann Grading System (HBGS) and the Facial Grading System (FGS). Results Exploratory principal component factor analysis grouped 15 FaCE Scale items into 6 impairment and disability categories (domains), forming the beta FaCE Scale. Overall, the test-retest reliability of the FaCE Scale was high (Spearman's correlation coefficient ( r) = 0.88, P <.01), as were the reliability coefficients of the individual domains ( r = 0.81-0.92, P <.01). The FaCE Scale domains showed appropriate correlation to global visual analogue scale questions posed on the original alpha FaCE Scale ( r = 0.65-0.81, P <.01). Overall, the FaCE Scale showed significant correlation with HBGS and FGS scores ( r = −0.55 and 0.57, respectively; P <.01). However, not all FaCE Scale domains correlated with the HBGS and FGS scores. Conclusions A reliable and valid patient-based system to measure impairment and disability in facial paralysis has been developed. This system appears to be better than traditional, physician-graded scales for evaluating quality-of-life issues affected by facial disability. [ABSTRACT FROM AUTHOR]

Objective/Hypothesis: To determine if angiogenic growth factors including vascular endothelial growth factor (VEGF) and platelet-derived endothelial cell growth factor (PD-ECGF) are expressed in human paragangliomas. Study Design: A histopathologic and molecular examination of paraganglioma specimens obtained from surgical cases or retrieved from the Pathology Department of the Massachusetts Eye and Ear Infirmary. Methods: Fresh tumor or archival, paraffin-embedded paraganglioma specimens were analyzed by immunohistochemistry, Western blotting, and ELISA. Results: Positive immunohistochemical staining for VEGF was observed in five of nine surgical specimens and in six of eight archival specimens (11/17, or 65%). PD-ECGF immunoreactivity was detected in four of five surgical specimens and six of eight archival specimens (10/13, or 77%). The presence of PD-ECGF was confirmed by Western blot assay and ELISA confirmed the presence of VEGF in tumor extract. Conclusions: Both VEGF and PD-ECGF are expressed in paragangliomas and may contribute to the extreme vascularity of these tumors. [ABSTRACT FROM AUTHOR]

The efficacy of surgery in controlling infection in 272 tympanomastoidectomy procedures for chronic otitis media (COM) was assessed by means of a four-point rating scale that incorporated both symptoms and signs, such as the presence or absence of otorrhea and granulation tissue. Of the 272 procedures, 170 were performed for COM with cholesteatoma and 102 were for active COM with granulation tissue but no cholesteatoma. Forty-seven percent were primary procedures, and 53% were revisions. Minimum follow-up was 12 months for all cases, with a mean of 30 months. Adequate control of infection occurred in 248 (91%) of the 272 cases. Of the 24 cases (9%) that developed persistent infection, 10 were controlled with a combination of oral and topical antibiotics and/or delayed skin grafting in the office. Thus overall satisfactory control of infection was achieved in 258 of 272 cases (95%). The outcome was influenced by the diagnostic category of COM: COM with cholesteatoma did significantly better than COM with granulation tissue ( P = 0.02). The outcome was not influenced by the following variables: primary versus revision surgery, canal wall-up versus canal wall-down surgery, and extent of disease. The results suggest that active COM with granulation tissue may be more difficult to control than COM with cholesteatoma. [ABSTRACT FROM AUTHOR]

Messenger ribonucleic acid (mRNA) for actin was detected in celloidin-embedded archival human temporal bone sections with reverse transcription polymerase chain reaction (RT-PCR). Actin mRNA was detected in 10% of sections analyzed. One possible reason for this modest detection incidence is enzymatic degradation of RNA by exogenously introduced ribonucleases (RNases). We have identified steps of the temporal bone processing protocol for archival storage in which exogenous RNases could be introduced to the tissue, and have verified that the bone sections are exposed to these enzymes. We have demonstrated that implementing precautions to minimize exogenous RNase contamination during processing improves recovery of intact RNA. This study indicates that although gene expression analysis of archival human temporal bones may be limited by enzymatic degradation of RNA, simple modification of processing protocol can improve yield of informative data. [ABSTRACT FROM AUTHOR]

Seventy-four revision stapedectomies performed consecutively over 10 years (1986 to 1995) were reviewed retrospectively. The most common intraoperative findings were incus erosion, prosthesis displacement, and oval window closure. Incus erosion was more frequently associated with multiple revisions. The postoperative results were reported using the conventional method (postoperative air minus preoperative bone) as well as the guidelines recently published by the American Academy of Otolaryngology-Head and Neck Surgery (postoperative air minus postoperative bone), with success rates of postoperative air-bone gap closure to within 10 dB after revision surgery of 51.6% and 45.6%, respectively. Patients with persistent conductive hearing loss(large residual air-bone gaps) after primary stapedectomy had poorer postrevision hearing results. Sensorineural hearing loss (defined as a drop in bone pure-tone average of more than 10 dB) occurred in four cases (5.4%). The number of revision surgeries, variations in operative techniques using laser or drill, and the ossicle to which the prosthesis was attached did not statistically affect the postoperative air-bone gaps. These results were compared with previously published data. [ABSTRACT FROM AUTHOR]

Copyright of Laryngoscope is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Temporalis muscle transposition is a reliable surgical technique for the reanimation of patients with long-standing facial paralysis. It is often employed when facial nerve reinnervation via crossover or cable grafting is not possible. Temporalis muscle transposition can also be used for the immediate treatment of complete facial paralysis due to insults leaving the facial nerve anatomically intact but requiring a prolonged recovery time (more than 1 year). Because temporalis muscle transposition does not interfere with neuronal regeneration, it may be employed early in the management of complete facial paralysis when recovery is predicted to be extended and incomplete. The authors report their experience with early temporalis muscle transposition in the management of facial paralysis in 56 patients with an anatomically intact facial nerve. More than 90% of these patients achieved improved symmetry at rest as well as purposeful movement at the corner of the mouth. In the last 30 patients, the temporoparietal fascial flap was simultaneously harvested and successfully used to obliterate the donor site defect. In conjunction with the immediate implantation of a gold weight in the ipsilateral upper eyelid, this approach to the early management of facial paralysis helps reduce the period of facial disability from years to weeks in a select group of patients. [ABSTRACT FROM AUTHOR]

Copyright of Laryngoscope is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

The surgical results in 78 recent cases of total removal of unilateral acoustic neuroma in which an attempt was made to preserve cochlear function have been added to the authors' previous series of 66 cases to evaluate the factors influencing the ability to preserve useful hearing. Useful hearing was defined by speech reception threshold no poorer than 70 dB and a discrimination score of at least 15%. Analysis using a logistic regression model showed that certain preoperative clinical parameters such as tumor size, speech discrimination score, and gender were significantly correlated with hearing outcome. Favorable outcome was significantly correlated with smaller tumor size, higher preoperative speech discrimination score, and male sex. From this data, an explicit formula was devised for predicting hearing outcome for an individual patient. In four cases with useful hearing preserved, there was improvement of greater than 15 percentage points in speech discrimination scores.While preoperative auditory brainstem responses were not predictive of hearing preservation, monitoring of intraoperative auditory evoked potentials was predictive of hearing outcome in selected cases. Specifically, when wave V was unchanged at the end of the operation, even if it may have been transiently lost during surgery, useful hearing was invariably preserved. [ABSTRACT FROM AUTHOR]

Objectives: Proteins in perilymph may alter the delivery profile of implantable intracochlear drug delivery systems through biofouling. Knowledge of protein composition will help anticipate interactions with delivered agents., Study Design: Analysis of mouse perilymph., Methods: Protein composition of perilymph and cerebrospinal fluid (CSF) was analyzed using a capillary liquid chromatography-mass spectrometry-based iTRAQ quantitative proteomics approach. We searched against a mouse subset of the Uniprot FASTA protein database. We sampled perilymph from the apex of the mouse cochlea to minimize CSF contamination., Results: More than 50 explicit protein isoforms were identified with very high confidence. iTRAQ reporter ions allowed determination of relative molar amounts of proteins between perilymph and CSF. Protein in perilymph was almost three times more concentrated than in CSF. More than one-third of the proteins in perilymph comprised protease inhibitors, with serpins being the predominant group. Apolipoproteins constituted 16%. Fifteen percent of the proteins were enzymes. Albumin was the most abundant single protein (14%). Proteins with relatively high perilymph/CSF ratios included broad-spectrum protease inhibitors and apolipoproteins., Discussion: Some proteins found in perilymph, such as albumin and HMW kininogen, have been implicated in biofouling through adsorption to device materials. The relatively large quantities of apolipoprotein and albumin may serve as a reservoir for acidic and lipophilic drugs. Alpha-2-glycoprotein can bind basic drugs., Conclusions: Perilymph is similar in protein composition to CSF, though amounts are 2.8 times higher. Protease inhibitors comprise the largest category of proteins.

Objective: Present four new cases of carcinoid tumor of the middle ear, two of which developed late recurrences and regional metastases. Review the literature to identify the clinical features, rate of recurrence, and incidence of metastasis of carcinoid tumor of the middle ear., Study Design: Retrospective chart review., Setting: Tertiary referral hospital., Patients: Eligibility criteria consist of a diagnosis of carcinoid tumor of middle ear., Intervention: Surgical excision of primary and metastatic disease., Main Outcome Measure: Clinical characteristics, rate of recurrence, and incidence of metastasis of carcinoid tumor of the middle ear., Results: Forty-six patients with carcinoid tumor of the middle ear are included in this report, 42 patients were identified from a review of the literature, and 4 new patients are presented. The most common presenting symptom was hearing loss. Surgical excision was the treatment with radical mastoidectomy being the most common procedure. Ten (22%) patients developed locally recurrent disease, and four (9%) developed regional metastases., Conclusions: Carcinoid tumor of the middle ear is an infrequent cause of a middle ear mass, with only 46 cases published. Despite previous assertions of benignancy, the findings of this study suggest that carcinoid tumor of the middle ear is indeed a potential low-grade malignancy with documented metastatic potential. Almost all middle ear adenomatous tumors ("adenoma" and "carcinoid") show evidence of neuroendocrine differentiation, and so at least some middle ear carcinoids ("adenomas") appear to represent well-differentiated neuroendocrine carcinomas. Presentation and symptoms are consistent with a middle ear mass and rarely include carcinoid syndrome. Surgical treatment is recommended and tailored to the extent of disease. Patients with carcinoid tumor of the middle ear require indefinite follow-up for possible recurrence or metastasis.

Objective: To assess the outcomes of patients undergoing surgical management of superior semicircular canal dehiscence (SSCD)., Study Design: Retrospective review., Methods: The medical records of all patients undergoing surgical treatment for SSCD at our institution between 2000 and 2004 were reviewed., Results: Eleven patients underwent unilateral operative management via a middle fossa approach. Ten patients were treated successfully by canal plugging and one unsuccessfully by canal re-roofing. Plugging of SSCD provided resolution of sound- and pressure-induced nystagmus, autophony, and conductive hearing loss (HL). One patient experienced a mild high-frequency sensorineural HL and two patients experienced both a mild high-frequency sensorineural HL and a reduction in vestibular function. Two additional patients underwent exploration for SSCD but were found to have a thin layer of bone overlying the canal., Conclusions: Plugging of the SSCD, while efficacious in alleviating the symptoms of the disease, may cause loss of labyrinthine function beyond the superior canal.

Objectives/hypothesis: Although hearing loss is common in MELAS (syndrome of mitochondrial encephalopathy, lactic acidosis and stroke-like episodes), the histopathology of the temporal bone has not been reported. The majority of cases of MELAS are linked to a mitochondrial DNA (mtDNA) mutation at nucleotide 3243. In MELAS, normal mtDNA and mutant mtDNA coexist in a heteroplasmic manner. The purpose of the study was to report the otopathological findings from two patients with MELAS and quantitative mtDNA analysis in the inner ear of one of these patients., Study Design: Basic scientific histopathological examination and quantitative mtDNA analysis of the temporal bone., Methods: Temporal bones were embedded in celloidin and sectioned for light microscopic study. Graphic reconstruction of the cochlea was performed using the method described by Schuknecht. For quantitative mtDNA analysis, total DNA from the membranous part of the inner ear was collected, amplified by polymerase chain reaction, and digested with the restriction enzyme. The percentage of mutant/total mtDNA was measured by the ratio of fluorescence intensity., Results: Histopathological examination revealed severe degeneration of the stria vascularis and degenerative change of spiral ganglion cells in both patients. The quantitative DNA studies showed that the proportion of mutant to wild-type mtDNA was similar in both histologically affected and histologically unaffected tissues within the inner ear., Conclusion: Dysfunction of the stria vascularis and spiral ganglion cells causes sensorineural hearing loss in MELAS.

Objectives/hypothesis: For many complex surgical procedures, larger hospital or surgeon caseload is associated with better patient outcome. We examined the volume-outcome relationship for surgical excision of acoustic neuromas., Study Design: Retrospective cohort study., Methods: The Nationwide Inpatient Sample (1996 to 2000) was used. Multivariate regression analyses were adjusted for age, sex, race, payer, geographic region, procedure timing, admission type and source, medical comorbidities, and neurofibromatosis status., Results: At 265 hospitals, 2643 operations were performed by 352 identified primary surgeons. Outcome was measured on a four-level scale at hospital discharge: death (0.5%) and discharge to long-term care (1.2%), to short-term rehabilitation (4.4%), and directly to home (94%). Outcomes were significantly better after surgery at higher-volume hospitals (OR 0.47 for fivefold-larger caseload, P <.001) or by higher-volume surgeons (OR 0.46, P <.001). Of patients who had surgery at lowest-volume-quartile hospitals, 12.3% were not discharged directly home, compared with 4.1% at highest-volume-quartile hospitals. There was a trend toward lower mortality for higher-volume hospitals (P =.1) and surgeons (P =.06). Of patients who had surgery at lowest-caseload-quartile hospitals, 1.1% died, compared with 0.6% at highest-volume-quartile hospitals. Postoperative complications (including neurological complications, mechanical ventilation, facial palsy, and transfusion) were less likely with high-volume hospitals and surgeons. Length of stay was significantly shorter with high-volume hospitals (P =.01) and surgeons (P =.009). Hospital charges were lower for high-volume hospitals (by 6% [P =.006]) and surgeons (by 6% [P =.09])., Conclusion: For acoustic neuroma excision, higher-volume hospitals and surgeons provided superior short-term outcomes with shorter lengths of stay and lower charges.