Search

Objective To assess outcomes after surgery for vestibular schwannoma in patients over 70 years of age. Study design Retrospective chart review. Setting Two tertiary otology and neurotology centers. Patients and interventions Patients undergoing primary surgery for vestibular schwannoma between 2007 and 2018. Main outcome measures Postoperative complications and surgical outcomes. Results A total of 452 patients met inclusion criteria, 31 of whom (6.9%) were over 70 years of age. Age ranged from 18 to 90 years with a mean of 53 years. Elderly patients were more likely to have pre-existing hypertension (58.1% versus 34.0%, p = 0.007) and diabetes mellitus (19.4% versus 7.4%, p = 0.02). Elderly patients were less likely to undergo gross total resections of their tumors (35.5% versus 60.6%, p = 0.05) although they were not statistically significantly more likely to undergo subtotal ( 0.05). Elderly patients were also less likely to undergo second stage procedures (0% versus 9.5%, p = 0.04). There were no significant differences between elderly and non-elderly patients in the rates of any complications, ultimate facial nerve function, or duration of surgery. No patients over 70 years of age expired within 1 year of surgery. Conclusions Conservative surgery for vestibular schwannoma in appropriately selected elderly patients is appropriate and safe, given adequate consideration to risk-benefit analysis and goals of care.

OBJECTIVE Facial function is a key outcome in surgery of the cerebellopontine angle (CPA). This study describes the natural history of facial weakness after CPA surgery. STUDY DESIGN AND SETTING Retrospective study of two tertiary centers. PATIENTS AND INTERVENTION Patients undergoing surgery for CPA tumors from 2003 to 2017 with preoperatively normal facial function and without subsequent surgical or adjuvant therapy. MAIN OUTCOME MEASURE Serial facial nerve examinations using the House-Brackmann (HB) scale. RESULTS Of 301 patients examined, 149 (49.5%) had facial weakness postoperatively and 74.5% of these occurred within 24 hours. Of patients with HB-1 function within 24 hours after surgery, 95.3% had HB-1 function and 100% had HB-3 or better function at last follow-up. Of patients with HB-4 or worse function within 24 hours after surgery, 22.6% ultimately achieved HB-1 function and 83.9% ultimately achieved HB-3 or better function. By 180 days postoperatively, 83.7% of patients' facial function had stabilized. At last follow-up, 3.0% of patients had facial function poorer than HB-3, which was associated with subtotal resections (12.9% vs. 4.4% for near total and 0.6% for gross total resections, p = 0.002), aspirin use (10.6% vs. 1.6%, p = 0.001), larger tumors (p

Elevated body mass index (BMI) is a risk factor for surgical complications, but data in acoustic neuroma surgery are conflicting and limited to small single-institution studies. This work evaluates associations between BMI and complications in surgery for acoustic neuroma (AN).Retrospective review.Two tertiary otology referral institutions.Patients undergoing surgery for AN. Univariate and multivariate analysis of association between BMI and complications was performed using two-tailedBMI ranged from 18.0 kg/mThe largest series to date investigating BMI in acoustic surgery is presented. Elevated BMI is strongly associated with CSF leak and need for revision surgery but not with other complications. Obese or overweight patients undergoing acoustic neuroma resection should be counseled of their increased risk of CSF leak.

OBJECTIVE To assess complication rates in hydroxyapatite cement (HAC) cranioplasty for translabyrinthine acoustic surgery compared with historical controls. METHODS Retrospective chart review of patients undergoing de novo translabyrinthine surgery with HAC cranioplasty without concurrent adipose tissue graft or additional material between 2010 and 2017 at a tertiary otology center. RESULTS Fifty-two patients underwent HAC cranioplasty during the study period. The average length of follow-up was 30.5 months. HAC cranioplasty was associated with acceptable rates of cerebrospinal fluid leak (3.8%), wound or intracranial infection (5.8%), need for unplanned revision surgery (9.6%), and all complications (15.3%). All complications occurred within 5 months of surgery. No delayed wound infections or implant extrusions occurred. CONCLUSION HAC cranioplasty has an acceptable complication profile for translabyrinthine surgery and is a viable alternative to abdominal fat grafting without associated donor site morbidity. LEVEL OF EVIDENCE 4 Laryngoscope, 130:206-211, 2020.

Objectives To assess the accuracy of pre-operative diagnosis of masses of the cerebellopontine angle (CPA) when compared to surgical pathology. Design Retrospective chart review. Participants Patients who underwent surgery for CPA masses at two tertiary care institutions from 2007 to 2017. Main outcome measures Percent concordance between pre-operative and surgical pathologic diagnosis; sensitivity, specificity, positive predictive value, and negative predictive value for predicted diagnoses. Results Concordance between pre-operative diagnosis and surgical pathology was 93.2% in 411 sampled patients. Concordance was 57.9% for masses other than vestibular schwannoma. Prediction of vestibular schwannoma and meningioma had high positive (0.95 and 0.97, respectively) and negative (0.76 and 0.99, respectively) predictive values. Prediction of facial neuroma had sensitivity of 0.13 and positive predictive value of 0.25. Headache (p = 0.001) and facial weakness (p = 0.003) were significantly associated with different pathologic profiles. Hearing loss was associated with differences in diagnostic prediction (p = 0.02) but not with differences in surgical pathology (p > 0.05). Conclusions Comparison between pre-operative predicted diagnosis and surgical pathology for cerebellopontine angle masses is presented. Vestibular schwannoma and meningioma were effectively identified while rarer CPA masses including facial neuroma were rarely identified correctly. Clinicians caring for patients with CPA masses should be mindful of diagnostic uncertainty which may lead to changes in treatment plan or prognosis.

OBJECTIVE To examine patients with residual tumor after vestibular schwannoma (VS) resection with focus on need for further therapy, including stereotactic radiosurgery (SRS) and revision surgery. STUDY DESIGN AND SETTING Retrospective review at two tertiary otology referral centers. PATIENTS AND INTERVENTION Patients undergoing primary surgery for VS from 2007 to 2017. MAIN OUTCOME MEASURE Degree of resection and need for further treatment. RESULTS Of 289 patients undergoing surgery, 38 (13.1%) underwent subtotal resections (

Objectives/Hypothesis The cause of superior semicircular canal dehiscence (SSCD) is unknown. Because of a demonstrated association with tegmental defects and obesity, some have suggested idiopathic intracranial hypertension (IIH) could contribute by eroding the bone over the canal and resulting in SSCD. However, an association between IIH and SSCD has not previously been evaluated. Our objective was to evaluate an association between IIH and SSCD. Study Design Retrospective cohort. Methods A retrospective study was performed of opening pressures for consecutive patients presenting at a lumbar puncture clinic between August 2012 and October 2015. Imaging for patients who also had thin-sectioned computed tomography (CT) imaging was reviewed for the presence of radiographic SSCD. Association between IIH and SSCD was evaluated using the Student t test and multivariate logistic regression. Results One hundred twenty-one patients had both a lumbar puncture performed and thin-sectioned CT imaging available, of which 24 patients (19.8%) met the criteria for IIH with an opening pressure >25 cm H2O. The remaining 97 patients (80.2%) did not have elevated opening pressures and served as the control cohort. None of the 24 patients with IIH had radiographic SSCD, whereas eight of the 97 patients (8.2%) without IIH had radiographic SSCD. The average opening pressure in patients without radiographic SSCD was 20.2 cm H2O compared to 19.3 cm H2O in patients with radiographic SSCD (P = .521). In multivariate logistic regression controlling for age, body mass index, gender, and comorbidities (hypertension, diabetes, hyperlipidemia), opening pressure was not a significant predictor of radiographic SSCD. Conclusions The results of this retrospective pilot study do not suggest an association between IIH and SSCD. Level of Evidence 3b Laryngoscope, 2017

OBJECTIVE To investigate rates of obesity and obstructive sleep apnea (OSA) in patients with a diagnosis of superior semicircular canal dehiscence (SSCD). STUDY DESIGN Retrospective cohort study. SETTING Two tertiary referral centers. PATIENTS Thirty-one patients with SSCD were identified from patient records at Yale between January 1, 2003 and August 1, 2013 and from the University of Cincinnati between November 1, 2008 and November 1, 2013. The control cohort consisted of 100 consecutive adult patients who obtained high-resolution CT imaging of their temporal bones at Yale University for any reason. INTERVENTIONS CT images were reviewed by two authors in double blind fashion and patient data was analyzed statistically. MAIN OUTCOME MEASURES Rates of OSA, body mass index (BMI), and presence of tegmental defects in patients with SSCD were compared to the control cohort. RESULTS The 31 patients with SSCD demonstrated higher BMIs [SSCD avg. 31.62, standard deviation (SD) 8.6 vs. no SSCD 28.01, SD 6.3, P = 0.036], rates of OSA (SSCD 29.03% vs. no SSCD 7.00, P = 0.001), and rates of tegmental defects (SSCD avg. 64.5% vs. no SSCD 16%, P = 1.24 × 10(-7)), in comparison to the control cohort. SSCD was found in 6 of 100 consecutively reviewed adult CT scans and in 0 of 41 scans obtained in those under 17 years of age. CONCLUSIONS Patients with SSCD demonstrated higher BMIs, higher rates of OSA, and were more likely to have accompanying tegmental defects. These results may support a possible causality between increased intracranial pressure and the formation of superior semicircular canal dehiscence.

Intralabyrinthine schwannomas are rare tumors of the distal ends of the cochlear and vestibular nerve. Their presence can be debilitating secondary to symptoms of hearing loss, vertigo, tinnitus, and imbalance. Currently, treatment to restore hearing in those who have become profoundly deaf is not attempted. Additionally, resection in patients with functioning hearing is rare, as the surgery assures deafness. We report the first case demonstrating the feasibility of resection of an intralabyrinthine schwannoma with immediate cochlear implantation. This technique addresses the patients hearing status by taking into account advancing technology, allowing for an improved quality of life.

The cause of superior semicircular canal dehiscence (SSCD) is unknown. Because of a demonstrated association with tegmental defects and obesity, some have suggested idiopathic intracranial hypertension (IIH) could contribute by eroding the bone over the canal and resulting in SSCD. However, an association between IIH and SSCD has not previously been evaluated. Our objective was to evaluate an association between IIH and SSCD.Retrospective cohort.A retrospective study was performed of opening pressures for consecutive patients presenting at a lumbar puncture clinic between August 2012 and October 2015. Imaging for patients who also had thin-sectioned computed tomography (CT) imaging was reviewed for the presence of radiographic SSCD. Association between IIH and SSCD was evaluated using the Student t test and multivariate logistic regression.One hundred twenty-one patients had both a lumbar puncture performed and thin-sectioned CT imaging available, of which 24 patients (19.8%) met the criteria for IIH with an opening pressure25 cm HThe results of this retrospective pilot study do not suggest an association between IIH and SSCD.3b. Laryngoscope, 128:1196-1199, 2018.

The temporal bone may be affected by a variety of systemic pathology because the disease nature, location, and extent determine the symptoms. Middle ear and mastoid infections may be the initial clinical manifestation of autoimmune and acquired immunodeficiency disorders. Rituximab, an anti-CD20 chimeric antibody, has become increasingly popular as a therapeutic agent for patients with a wide range of autoimmune disorders refractory to standard treatments. Normal levels of immunoglobulin levels are usually maintained during and after rituximab therapy, and clinical trials to date have shown no statistically significant increase of serious infections among patients with autoimmune diseases being treated with rituximab (Cohen SB, Emery P, Greenwald MW, Dougados M, Furie RA, Genovese MC, et al, for the REFLEX Trial Group. Rituximab for rheumatoid arthritis refractory to anti-tumor necrosis factor therapy: results of a multicenter, randomized, double-blind, placebo-controlled, phase III trial evaluating primary efficacy and safety at 24 weeks. Arthritis Rheum. 2006;54:2793-2806. Edwards JC, Szczepanski L, Szechinski J, Filipowicz-Sosnowska A, Emery P, Close DR, et al. Efficacy of B-cell-targeted therapy with rituximab in patients with rheumatoid arthritis. N Engl J Med. 2004;350:2572-2581). However, there have been several reports of opportunistic infections associated with rituximab (Kelesidis T, Daikos G, et al. Does rituximab increase the incidence of infectious complications? A narrative review. Int J Infect Dis 2011;15:e2-e16. Teichmann LL, Woenckhaus M, Vogel C, et al. Fatal Pneumocystis pneumonia following rituximab administration for rheumatoid arthritis. Rheumatology 2008;47:1256-1257), as well as cases of it accelerating the presentation of hypogammaglobulinemia (Diwakar L, Gorrie S, et al. Does rituximab aggravate pre-existing hypogammaglobulinaemia? J Clin Pathol 2010;63:275-277). Humoral immune defects can cause persistent acute and serous otitis media, with the development of chronic suppurative otitis media refractory to medical and surgical therapy (Sasaki CT, Askenase P, Dwyer J, et al. Chronic ear infection in the immunodeficient patient. Arch Otolaryngol 1981;107:82). Here, we describe the first presentation, diagnostic workup, and treatment with intravenous immunoglobulin of chronic bilateral otomastoiditis in the setting of rituximab-induced hypogammaglobulinemia.

Objective Jugular bulb abnormalities (JBA), such as jugular bulb diverticula (JBD) or large jugular bulbs, rarely present in the middle ear. We review a large series of temporal bone histopathologic specimens to determine their prevalence and present a series of cases of JB abnormalities involving the middle ear (JBME) that shed light on the probable mechanism for their development. Patients 1,579 unique temporal bone specimens and individuals with radiographically-diagnosed JBME. Intervention Histopathologic and clinical review of temporal bone specimens and patient presentations, radiographic findings, treatments and outcomes. Main outcome measure Shared characteristics of JBME. Results There were 17 cases of JBME in 1,579 temporal bone (1.1%), of which, 15 involved the inferior mesotympanum below the level of the round window membrane (RWM), whereas 2 encroached upon the RWM or ossicles. In addition, 4 clinical cases of large JBME extending above RWM were encountered; these occurred in both sexes with ages spanning from young to old (7-66 yr). They presented with conductive hearing loss (n = 3), ear canal mass (n = 1), and intraoperative bleeding (n = 1). Radiologically, they had multiple diverticula of the JB on the side with JBME, with 1 patient demonstrating growth on serial imaging studies. All patients who underwent additional imaging had marked hypoplastic contralateral transverse sinus. Conclusion JBME abnormalities are rare, present across age groups, and may demonstrate serial growth over time. They are usually associated with multiple other diverticula within the same JB. Our clinical series suggests that JBME's development and uniquely aggressive behavior results from contralateral transverse sinus outflow obstruction.

This study is conducted to demonstrate that destructive lesions of the otic capsule by Langerhans cell histiocytosis (LCH) causing both radiographic and audiologic findings can be completely reversed with adequate treatment. Retrospective case review and analysis of clinical and imaging data were obtained as part of the diagnosis and treatment of patients with LCH of the temporal bone. With Institutional Review Board (IRB) approval, cases of LCH involving the temporal bone were searched for within the institutional databases. Criteria for inclusion was histologic diagnosis of LCH and pretreatment computed tomography (CT) demonstrating temporal bone and/or otic capsule involvement and posttreatment follow-up CT/magnetic resonance imaging (MRI) scans obtained at least 6 months after starting treatment. We report eight cases of LCH of the temporal bone with three demonstrating otic capsule involvement radiographically and/or clinically. Review of posttreatment imaging revealed all three patients had complete restoration of the bony labyrinthine architecture and near or complete restoration of their hearing. Though LCH of the temporal bone is a common site within the spectrum of the disease, involvement of the otic capsule remains rare. Here, we report the largest series of otic capsule involvement by LCH and investigate whether both architecture and hearing are recovered with appropriate treatment. Lastly, restoration of the bony architecture of the labyrinth suggests the mechanism of LCH is demineralization and not ablative.

When confronted with vertigo after an otologic procedure, a surgeon first must identify the functional status of the inner ear by performing auditory and vestibular testing. Using this information in conjunction with knowledge of the primary disease process for which the initial procedure was performed, the surgeon can make a rational selection of the procedure required to eliminate vertigo. This article outlines a systematic approach to the selection of the appropriate revision procedure and discusses the specific advantages and disadvantages of these procedures used to control vertigo.

Semicircular canal dehiscence is an important entity often presenting with symptoms of noise or pressure induced vertigo, autophony, aural fullness and conductive hearing loss. Due to its varied presentation and mimic of other otologic conditions its consideration is of increased importance. Within we report a case of right sided posterior semicircular canal dehiscence secondary to an enlarged and high jugular bulb. The results of this observation indicate a need for patients with auditory symptoms and high riding jugular bulbs to be evaluated for possible posterior semicircular canal dehiscence.

Acoustic neuroma results from abnormal proliferation of Schwann cells. These tumors originate in the region of Scarpa's ganglion at the junction of peripheral and central myelin of the vestibular nerve located in the internal auditory canal (IAC). The bony confine of the IAC houses the VII and the VIII cranial nerves. The presence of tumor mass compresses these structures. The growing tumor mass may also prolapses into the cerebellopontine angle (CPA). With continued growth, the tumor eventually compresses on the brain stem and cerebellum. Despite the benign nature of these tumors, the clinical course of this disease may be fraught with complications.

Identify and compare phenotypic properties of osteoblasts from patients with otosclerosis (OSO), normal bones (HOB), and normal stapes (NSO) to determine a possible cause for OSO hypermineralization and assess any effects of the bisphosphonate, alendronate.OSO (n = 11), NSO (n = 4), and HOB (n = 13) cultures were assayed for proliferation, adhesion, mineralization, and gene expression with and without 10(-10)M-10(-8)M alendronate.Academic hospital.Cultures were matched for age, sex, and passage number. Cell attachment and proliferation + alendronate were determined by Coulter counting cells and assaying tritiated thymidine uptake, respectively. At 7, 14, and 21 days of culture + alendronate, calcium content and gene expression by quantitative reverse transcription-polymerase chain reaction (qRT-PCR) were determined.OSO had significantly more cells adhere but less proliferation than NSO or HOB. Calcification was significantly increased in OSO compared to HOB and NSO. NSO and HOB had similar cell adhesion and proliferation rates. A dose-dependent effect of alendronate on OSO adhesion, proliferation, and mineralization was found, resulting in levels equal to NSO and HOB. All cultures expressed osteoblast-specific genes such as RUNX2, alkaline phosphatase, type I collagen, and osteocalcin. However, osteopontin was dramatically reduced, 9.4-fold at 14 days, in OSO compared to NSO. Receptor activator of nuclear factor κB ligand/osteoprotegerin (RANKL/OPG), important in bone resorption, was elevated in OSO with decreased levels of OPG levels. Alendronate had little effect on gene expression in HOB but in OSO increased osteopontin levels and decreased RANKL/OPG.OSO cultures displayed properties of hypermineralization due to decreased osteopontin (OPN) and also had increased RANKL/OPG, which were normalized by alendronate.

Objective: This report introduces a new method to control cerebrospinal fluid (CSF) otorrhea using hydroxyapatite cement (HAC) via a transmastoid approach. This technique eliminates the need for a transmastoid or middle cranial fossa approach with soft tissue repair and prolonged hospitalization caused by lumbar drainage. Study Design: Retrospective review. Methods: Thirteen cases of transmastoid repairs of CSF otorrhea using HAC from August 1996 to February 1999 were reviewed. Results: The CSF leak was controlled in every patient using HAC through a transmastoid approach. The reconstruction involved eight tegmen defects, three posterior fossa dural plate defects, and two congenital inner ear fistula secondary to Mondini malformation. Postoperative wound infection in one patient was the only complication that occurred. The average hospital stay was 48 hours. Follow-up ranged from 12 to 44 months with no recurrence of CSF otorrhea. Conclusion: The successful use of HAC to control CSF otorrhea through a transmastoid approach reduces patient morbidity by obviating the need for middle cranial fossa approaches, donor soft tissue sites, and spinal drainage.

Optokinetic nystagmus (OKN) testing is one method to determine central vestibular dysfunction. OKN may be elicited by partial visual field stimulation with a light bar (OKN-ENG) or by full visual field stimulation with rotating stripes in a rotational chair test booth (OKN-RVT). OKN-ENG and OKN-RVT were elicited in 36 healthy subjects and 48 patients with known peripheral or central vestibular disorders. Abnormal test results suggested central pathology in 29 of 36 healthy subjects with OKN-ENG versus 1 of 36 with OKN-RVT. Twenty-eight of 33 patients with peripheral pathology demonstrated abnormal OKN-ENG findings, whereas 4 of 33 had abnormal OKN-RVT results. Thirteen of 15 patients with central vestibular disorders had abnormal OKN-ENG, whereas 7 of 15 had abnormal OKN-RVT. Sensitivity and specificity of OKN-ENG were 86.7% and 17.4% versus 46.7% and 92.7%, respectively, for OKN-RVT. These findings were statistically significant (P0.00001). OKN elicited by full visual field stimulation (OKN-RVT) is a more accurate indicator of central disease than OKN elicited by partial visual field stimulation (OKN-ENG). The use of OKN-ENG to identify central vestibular dysfunction is questionable.

Yanagisawa, K., L. M. Bartoshuk, F. C. Catalanotto, T. A. Karrer, and J. F. Kveton. Anesthesia of the chorda tympani taste nerve and taste phantoms. PHYSIOL BEHAV 63(3) 329–335, 1998.—The chorda tympani nerve innervates the anterior two-thirds and the glossopharyngeal nerve, the posterior one-third, of each side of the tongue. Previous work showed that anesthesia of one chorda tympani increased the perceived intensity of quinine applied to an area innervated by the contralateral glossopharyngeal nerve, but decreased the perceived intensity of NaCl applied to an area innervated by the ipsilateral glossopharyngeal nerve. The data presented here corroborate that earlier finding and show that if both chorda tympani nerves are anesthetized, the taste of quinine is intensified and the taste of NaCl diminished at areas innervated by the glossopharyngeal on both sides of the tongue. In about 40% of the subjects, tastes occurred in the absence of stimulation (we call these tastes phantoms). The phantoms were usually localized to the posterior tongue contralateral to the anesthesia and they were abolished when a topical anesthetic was applied to the area where they were perceived. Phantoms like these may be a source of clinical dysgeusia in patients with localized taste damage. The phantoms may result because the anesthesia releases inhibition normally occurring between the central projection areas of different taste nerves.

We describe a rare complication of stereotactic radiotherapy for large acoustic neuromas in a patient with type 2 neurofibromatosis. We retrospectively reviewed the case of a 14-year-old girl who had been referred to our tertiary care center. Prior to referral, the patient had been evaluated for hoarseness. During the work-up, magnetic resonance imaging (MRI) detected two large bilateral acoustic neuromas and two bilateral jugular foramen tumors. The patient was diagnosed with type 2 neurofibromatosis, and she underwent stereotactic radiotherapy for treatment of the two acoustic neuromas; the jugular foramen tumors were not irradiated. The patient's post-treatment course was complicated by hydrocephalus and symptoms of brainstem compression, which required urgent surgical intervention. Follow-up MRI 7 months following radiotherapy demonstrated a rapid growth of the acoustic neuromas, but no appreciable change in the size of the jugular foramen neuromas. These findings suggest that the radiotherapy might have been the cause of the rapid growth of the acoustic neuromas. To our knowledge, no such report has been published in the literature, and this phenomenon might be unique. Our findings suggest that radiotherapy might not be the optimal first-line treatment for acoustic neuromas in patients with type 2 neurofibromatosis.

Program Description: Every time the ear surgeon operates, a significant number of events occur and are anticipated. Many of these are routine and predictable. However, sometimes the unexpected occurs, which can be during the preoperative assessment, during surgery, or after surgery. Treatment outcomes are dependent on the surgeon’s handling of unexpected findings or occurrences as well as the decisions made regarding what is best for the patient. This miniseminar presents cases of 4 common otologic diagnoses where the surgeon was faced with unanticipated situations that challenged his diagnostic and surgical skills; where every decision made affected the final outcome. The disease entities presented are Meniere’s disease, otosclerosis, chronic otitis media with cholesteatoma, and chronic otitis media without cholesteatoma. In each case presented, the panelist will discuss his decisions regarding whether or not to image the patient, whether or not to recommend surgery, what was found intraoperatively, and ...

Revision mastoid surgery carries increased risk of facial nerve injury as compared with primary surgery because of distortion of normal temporal bone anatomy caused by progressive disease and/or previous surgical manipulation. When confronted with a revision procedure, the otologist must combine knowledge of facial nerve anatomy and surgical skills to obtain a successful surgical outcome. The facial nerve must be identified at a distance from the disease, and then disease must be exenterated with constant visualization of the facial nerve in the involved region of the temporal bone. This can only be accomplished if the otologist understands the course of the facial nerve in the temporal bone and its related anatomy. This article will review the salient features of the surgical anatomy of the facial nerve and describe approaches to the facial nerve used in canal wall up and canal wall down revision surgery.

Studies using anesthesia provide insights into oral phantoms. The chorda tympani nerve is accessible for anesthesia at two sites. First, the chorda tympani leaves the tongue with the lingual nerve (CN V) and the two travel through the pterygomandibular space. The inferior alveolar nerve, which conveys pain from the lower teeth, passes through the same space; thus dental anesthesia abolishes taste and touch as well as pain. Secondly, the chorda tympani passes through the middle ear after separating from the lingual nerve, so injection of an anesthetic just under the skin near the ear drum anesthetizes taste but not touch. Using both procedures, we showed that anesthesia of the chorda tympani intensifies tastes evoked from the contralateral rear of the tongue, the area innervated by the glossopharyngeal nerve (Lehman et al., 1995; Yanagisawa et al., 1998). This finding supports the earlier evidence of Halpern and Nelson (1965) for central inhibitory connections between the chorda tympani and glossopharyngeal nerves. This inhibition acts as a constancy mechanism: when one nerve is damaged, its input to the central nervous system (CNS) is reduced, releasing inhibition on other taste structures and thus compensating for the loss of input from the damage. During our anesthesia experiments, about half of the subjects developed taste phantoms typically localized to the contralateral rear of the tongue (Yanagisawa et al., 1998). This suggested that clinical taste phantoms (i.e. dysgeusia) might be the result of localized taste damage. Indeed, we have found taste damage in patients reporting taste phantoms (Bartoshuk et al., 2002).

Permanent obliteration of the eustachian tube via the middle ear traditionally has met with limited success. Combinations of muscle, fascia, adipose tissue, bone fragments, and inert material such as Proplast have been used to seal the middle ear from the nasopharynx by inciting a fibrous reaction within the lumen of the eustachian tube. Long-term follow-up has demonstrated repneumatization of the middle ear cleft in the majority of cases, indicating failure of the obliteration technique. This report describes a technique for successful long-term obliteration using hydroxyapatite cement. This new biomaterial possesses osseointegrative and osseoconductive properties that result in permanent obliteration by producing new bone formation within the lumen of the eustachian tube. This technique eliminates the complication of cerebrospinal fluid rhinorrhea in lateral skull base procedures that expose the eustachian tube to cerebrospinal fluid.

Objectives: To describe the indications for successful use of hydroxyapatite cement (HAC) in temporal bone surgery. Study Design: Retrospective case review. Methods: One hundred nine temporal bone defects related to surgical approaches to the skull base, infection, neoplasms, or congenital defect in 102 adults and children were corrected using HAC over a 10-year period. The results and complications were obtained through retrospective chart review. Results: Ninety-seven percent of temporal bone defects were successfully repaired using HAC, and the reconstruction remained stable over the course of this study. There was one case of postoperative cerebrospinal fluid leak. Wound infection occurred in three patients, which required reoperation and removal of the biomaterial. Conclusions: HAC is a biomaterial that should be used as the primary method to reconstruct temporal bone defects. Proper use of this biomaterial results in restoration of the integrity of the temporal bone and elimination of cerebrospinal fluid leak as a postoperative complication.

Objective To demonstrate the efficacy of intact canal wall procedure coupled with a second-stage exploration for the treatment of cholesteatoma. Design Retrospective case study of patients with cholesteatomas treated with staged surgical extirpation. A minimum of 6 months' postoperative follow-up time was required for inclusion into the study. Setting Tertiary academic referral center. Patients A total of 35 adult and pediatric patients, ranging from 9 to 65 years of age, who underwent 2-stage procedures for removal of cholesteatomas. Interventions Two-stage procedures, separated by 6 months, performed with posterior tympanotomy approaches. Main Outcome Measures The presence or absence of cholesteatoma on second-stage look and the subsequent surgical treatment for recurrent cholesteatoma. The overall hearing results after the completion of the 2-staged procedure were calculated. Results Disease was controlled in 26 (74%) of the patients. Residual and/or recurrent cholesteatomas were found in 9 (26%) of the patients during the second-stage operation. Of these patients, 5 (14% of the total group) ultimately required conversion to canal-wall-down procedure. Average hearing gain at the completion of the second-stage procedure was 9 dB. Conclusions A planned 2-stage procedure that uses the posterior tympanotomy approach for the control of cholesteatoma is an effective technique. This approach offers significant potential for hearing preservation and restoration.

Acoustic neuroma results from abnormal proliferation of Schwann cells. These tumors originate in the region of Scarpa's ganglion at the junction of peripheral and central myelin of the vestibular nerve located in the internal auditory canal (IAC). The bony confine of the IAC houses the VII and the VIII cranial nerves. The presence of tumor mass compresses these structures. The growing tumor mass may also prolapses into the cerebellopontine angle (CPA). With continued growth, the tumor eventually compresses on the brain stem and cerebellum. Despite the benign nature of these tumors, the clinical course of this disease may be fraught with complications.

Despite advances in neurological, reconstructive, and endoscopic sinus surgery, sphenoethmoid cerebrospinal fluid (CSF) fistulae continually pose difficult management problems. Standard surgical techniques for fistulae closure succeed approximately 78% to 90% of the time. To improve this success rate, hydroxyapatite cement (HAC), a Food and Drug Administration–approved substance for cranial defect repair, was applied to this problem in a clinical setting. Twenty-one patients with spontaneous, posttraumatic, or postoperative CSF leaks of the sphenoid sinus, cribriform plate, or ethmoid region were treated with HAC. Study participants were prospectively accrued at 5 tertiary care medical centers in the eastern United States. The CSF leaks of all 21 patients treated with HAC were successfully sealed by its initial application. The sites of CSF leakage included the nasal cavity (n = 2) and sphenoid sinus (n = 19). Fifteen of the patients had previously undergone a failed repair by standard methods. There have been no recurrent CSF leaks with a maximum follow-up of 72 months, and an average follow-up of 36 months. All patients have survived to date. The only HAC-related morbidity was the extrusion of the HAC when placed in the nasal cavity. Hydroxyapatite cement is an effective method of repair for postoperative, posttraumatic, and spontaneous sphenoid CSF leaks. The efficacy of HAC in sealing the CSF leak was unaffected by previous attempts at leak closure by standard methods or by its origin. Hydroxyapatite cement should not be applied transnasally for the treatment of an ethmoid region fistula owing to its high probability of extrusion. Correct patient selection and technical familiarity with HAC are necessary for successful application.

Individuals who have sustained considerable damage to parts of the taste system often fail to experience changes in everyday taste experience. The two halves of the tongue are independently innervated: the chorda tympani (branch of the facial or VIIth cranial nerve) innervating the anterior two-thirds and the glossopharyngeal (IXth cranial nerve) innervating the posterior one-third. Anesthesia of the chorda tympani nerve on one side produced increased taste intensities for some stimuli on the area innervated by the glossopharyngeal nerve on the other side. Because this effect occurs across the midline and taste projects ipsilaterally, the effect must occur in the central nervous system (CNS). This supports Halpern and Nelson's release-of-inhibition hypothesis that the area to which the chorda tympani projects in the CNS must normally inhibit that of the glossopharyngeal nerve. Anesthesia of the chorda tympani abolishes that inhibition and leads to perception of increased taste intensities from areas innervated by the glossopharyngeal nerve.

Educational objectives: To recognize the common causes of failure of canal wall down mastoidectomy and to develop a systematic approach for successful modified radical mastoidectomy.

Hydroxyapatite cement, a new biomaterial presently under clinical investigation, has been demonstrated to have potentially wide application in cranial reconstruction. We describe our experience with this biologic bone cement in the reconstruction of suboccipital craniectomy defects in seven patients after vestibular schwannoma removal. With up to 2-year follow-up, cranial bone integrity has been reestablished in five patients. Dissolution of cement has occurred in dependent areas and appears to be technique related. Cranial bone contour appears dependent on the amount of cement used. The frequency of debilitating postoperative headache was reduced in these patients when compared to patients who had no reconstruction of the craniectomy defect. Reconstruction of the bony defect after suboccipital craniectomy with hydroxyapatite cement is not only useful to restore cranial contour, but also appears to reduce some of the functional deficits attributed to this surgical approach.

Patients often fail to notice taste loss even when their taste systems are severely damaged. Pfaffmann [1,2] presented his own case of Ramsey-Hunt’s syndrome (cranial nerve damage produced by the virus responsible for chicken pox) at a meeting of the Association for Chemoreception Sciences. The virus destroyed taste on the left side of his mouth yet he had no subjective awareness of loss in everyday taste experience.

Referred otalgia is a challenging symptom, with the burden on the physician to identify the source. Only by careful patient history and physical examination can all causes in this extensive differential diagnosis be excluded. In the absence of primary otologic pathology, referred pain from a head and neck carcinoma must be considered foremost and ruled out.

Methods and structures for placing sensing and driver assemblies in a middle ear of a patient are disclosed. Methods and structures according to various embodiments of the invention provide minimally invasive techniques and devices that facilitate placement of sensing/driver assemblies in a middle ear using a trans-canal implantation technique. Certain embodiments include placement of a longitudinal body through the ear canal for mounting in the middle ear, and for supporting a sensing/driving assembly subsequently coupled thereto.

Otitis media is the most common infectious disease seen in the pediatric population. Hearing loss is a well-recognized sequela of otitis media, whether acute or chronic. In acute cases, hearing generally returns as the resulting effusion resolves, which may take up to several months. Hearing loss in chronic otitis media often resolves during quiescent stages of the disease; persistent hearing loss that fails medical management is best treated through surgical intervention. Tympanosotomy tube insertion effectively recovers hearing in cases of chronic middle ear effusions, whereas tympanoplasty and ossiculoplasty, often in conjunction with mastoidectomy, are necessary for chronic draining ears. Hearing loss may occur in the absence of otitis media. In such cases the clinician must be aware of the potential ramifications of the hearing loss for the child as well as the proper method of evaluating such a patient. Background During the first 5 years of childhood, the ability to hear is related intimately to the degree of speech and language acquisition as well as to the development of emotional, social, and academic skills. It is estimated that a normal-hearing 5-year-old child has a vocabulary of 5000 to 26 000 words, whereas a profoundly hearing-impaired child has a vocabulary of 200 words. The effects on reading ability are equally astounding, with the average reading achievement for hearing-impaired 16-year-olds at the third- or fourth-grade level.

As the identification of patients with small acoustic neuromas and salvageable hearing increases, intraoperative auditory nerve monitoring has been used increasingly in an attempt to improve the hearing preservation rate. Far-field recordings obtained by brainstem auditory evoked potentials (BAEP), at times enhanced by electrocochleography, have become a standard method of intraoperative auditory nerve assessment. To evaluate the usefulness of this monitoring technique, the hearing preservation results of a series of unmonitored acoustic tumor removals were compared to a series of patients monitored via the standard brainstem auditory evoked potentials. With comparable average tumor sizes, 4 of 7 unmonitored patients had hearing preserved at preoperative levels compared to 4 of 9 monitored patients. Neither preoperative BAEP assessments nor absolute tumor size were predictive of hearing preservation. This report brings into question the effectiveness of far-field intraoperative BAEP monitoring during acoustic tumor resection and suggests that direct auditory nerve monitoring may be more appropriate.

Unlike the eventual resolution of facial paralysis in most patients with intact facial nerves, delayed hearing return after acoustic tumor resection is rare. This discrepancy in recovery has been ascribed to the inherent resilience of the facial nerve (a special visceral efferent nerve) to injury versus the cochlear nerve (a special somatic afferent nerve). In the presence of an intact cochlear nerve, hearing loss has been attributed to transection or spasm of the internal auditory artery or to direct mechanical trauma to the cochlear nerve during manipulation of the tumor. The possibility of a reversible conduction block in the cochlear nerve has not been considered. A review of three instances of delayed spontaneous hearing recovery several months after acoustic tumor resection suggests that a conduction block phenomenon can exist. This report describes the pathophysiology and the possible intraoperative predisposing features of this condition.

A case of Pierre Robin syndrome (mandibular hypoplasia, cleft palate, and glossoptosis), associated with a unilateral choanal atresia in a full-term infant, is described. The pathogenesis of this combined anomaly is discussed through review of the embryologic development of the craniofacial structures. Diagnostic and management alternatives are reviewed.