Oncogenic osteomalacia caused by phosphaturic mesenchymal tumor of the temporal bone.
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Biomedical subjects
Publications and source records attributed to C Gary Jackson.
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OBJECTIVE: To report results of revision chronic ear surgery following guidelines of the American Academy of Otolaryngology-Head and Neck Surgery and to establish expectations for infection and cholesteatoma control and hearing outcomes. STUDY DESIGN: Retrospective case review of all patients who underwent revision chronic ear surgery from January 1, 1990 to December 31, 2000. Revision chronic ear surgery included canal wall up and canal wall down procedures with ossicular chain reconstruction performed as needed. Cholesteatoma control, hearing improvement, and closure of middle ear space are main outcome measures. SETTING: Tertiary referral center. RESULTS: Cholesteatoma recurrence rate was 57% at 1 year after surgery and 14% in patients with a minimum of a 5-year follow-up. Disease control was achieved in 96% of patients. Hearing was significantly improved in all surgical groups. Closure of the air-bone gap for revision partial ossicular replacement prosthesis cases (PORP) to less than 20 dB occurred in 50% of patients. Closure of the air-bone gap to within 30 dB for revision total ossicular replacement prosthesis (TORP) occurred in 60% of patients. Canal wall down status had a significant impact on hearing results after PORPs and TORPs; patients with intact canal walls had significantly better hearing results. Diagnosis of cholesteatoma significantly impacted hearing results for TORPs but not PORPS. CONCLUSIONS: Cholesteatoma control rates after revision surgery are similar to primary cases. Significant improvement in hearing can be expected after revision chronic ear surgery. Hearing results after a revision surgery that requires a PORP is worse than primary cases and is canal wall status dependent. Closure of the middle ear space and creation of a safe dry ear can be expected after revision chronic ear surgery. SIGNIFICANCE: This is a review of a large series of exclusively revision chronic ear surgery. EBM RATING: C-4.
OBJECTIVE: To determine whether the appearance of the inner ear on T2-weighted follow-up magnetic resonance imaging correlates with hearing status after hearing-preservation surgery for vestibular schwannoma. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary referral medical center. PATIENTS: The study includes patients undergoing hearing-preservation surgery for vestibular schwannoma from 1998 to 2003. INTERVENTION: Diagnostic evaluation with magnetic resonance imaging and audiometric evaluation. MAIN OUTCOME MEASURES: Hearing results as reported in charts was correlated with appearance of membranous labyrinth on T2-weighted magnetic resonance images obtained at least 1 year after surgery. RESULTS: Twenty-nine patients were identified, 16 of whom satisfied the inclusion criteria. All 16 of the patients underwent middle fossa removal of vestibular schwannoma. Serviceable hearing according to American Academy of Otolaryngology-Head and Neck Surgery criteria was preserved in eight patients (50%). Of the eight patients without serviceable hearing, six had the cochlear nerve sacrificed at the time of surgery. All patients with serviceable hearing had normal appearing cochleovestibular signal on T2-weighted images, whereas six of eight patients (75%) with no hearing or poor hearing had abnormal low signal in the inner ear, suggesting inner ear ossification. The positive predictive value of a normal labyrinth for preserved hearing was 90%, whereas the negative predictive value of an abnormal labyrinth for no hearing was 100%. All but one patient who had the cochlear nerve sacrificed showed abnormal morphology of the labyrinth on T2-weighted magnetic resonance imaging. CONCLUSION: We describe the T2-weighted magnetic resonance findings after hearing-preservation surgery for acoustic tumor removal. Loss of inner ear signal on T2-weighted images correlates with loss of hearing postoperatively, whereas preserved inner ear signal correlates with hearing preservation after middle fossa surgery for vestibular schwannoma removal.
OBJECTIVE: To describe functional and reconstructive results after revision lateral skull base surgery with comparison of benign and malignant lesions. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: All patients undergoing revision surgery for benign and malignant lateral skull base tumors. INTERVENTIONS: Surgical resection of recurrent lateral skull base tumors and reconstruction of resulting defects. MAIN OUTCOME MEASURES: Cranial nerve function postoperative complications. RESULTS: Forty operations for recurrent lateral skull base tumors occurred between January 1, 1987, and December 31, 2003, with follow-up of at least 1 year. Thirty-three operations were for benign lesions, 27 of which were glomus tumors. Seven operations were for malignant tumors. Fifty-eight percent of patients had preoperative cranial nerve deficits (66% of benign tumors and 14% of malignancies). The most common preoperative deficit occurred in the Xth cranial nerve. Postoperative cranial nerve deficits were seen in 95% of patients and multiple nerve deficits were seen in 75%. The most common postoperative deficits were observed in the IXth and Xth cranial nerves. Thirty-one patients had one previous procedure, six had two previous procedures, and three had three previous procedures. Abdominal fat and temporoparietal fascia were the most common reconstruction materials. There was one case of meningitis, two cerebrospinal fluid leaks, and one pseudomeningocele. There was one recurrent adenoid cystic tumor resulting in death and two partially resected glomus tumors. Subsequent procedures are discussed. CONCLUSION: Postoperative cranial deficits are more common after revision skull base surgery than after primary surgery. Complete resection without recurrence can be expected for revision skull base surgery. Modern reconstruction techniques reduce major postoperative complications and morbidity from cranial nerve deficits.
OBJECTIVE: For many years, surgery was the mainstay of therapy for medically refractory patients, but recently, transtympanic gentamicin perfusion has attracted increasing interest and is a method frequently used for treating Meniere's disease. Many otologists question the relevance of surgical treatments, and traditional options are rarely discussed or offered to patients. The purpose of this study is to describe results of labyrinthectomy, vestibular nerve section, and endolymphatic mastoid shunt surgery for patients with Meniere's disease and to compare them with published results for gentamicin perfusion. STUDY DESIGN AND SETTING: Retrospective chart review. Two hundred twenty-nine patients underwent surgery for management of Meniere's disease between January 1, 1995 and December 31, 2001. One hundred eighty-nine patients' charts had sufficient data for review. Thirty-two patients had translabyrinthine labyrinthectomies, 83 underwent suboccipital vestibular nerve sections, and 74 elected for an endolymphatic mastoid shunt. Hearing results, dizziness classification, and functional level score were determined from patient charts and telephone conversations. All results were in accordance with the guidelines of the AAO-HNS Committee on Hearing and Equilibrium for evaluation of Meniere's disease therapy. RESULTS: Audiologic results, functional level score, and dizziness classification are reported for the preoperative period and for the 18- to 24-month postoperative period for all surgical patients. These data are also reported individually for each of the 3 surgical procedures. Early postoperative data and most recent follow-up data are presented if available. CONCLUSIONS: Surgical management of Meniere's disease is a safe and viable option for patients with medically refractory disease. EBM RATING: C.
OBJECTIVES: Epidermoid cysts are the most common intracranial embryonal tumor, although they account for only 1% of all intracranial tumors. Epidermoids often spread into several intracranial compartments. Thorough preoperative surgical planning is imperative for safe epidermoid removal. This paper discusses the neurotologic management of intracranial epidermoid cysts STUDY DESIGN: Retrospective chart review. METHODS: A database search revealed 10 patients with diagnosis of intracranial epidermoid cysts between January 1, 1971 and December 31, 2003 at our institution. RESULTS: Six males and four females with ages ranging from 18 to 54 years of age underwent surgery between September 1, 1971 and November 4, 2003. The average tumor size was 3.9 cm; six originated in the cerebellopontine angle and four in the petrous apex. Six patients had a translabyrinthine approach to the tumor, two with additional transcochlear exposure. Two patients had tumors removed by way of the middle fossa approach and one through the suboccipital approach. Multiple cranial nerves were involved by tumor in all patients, including nerves III through XI. The internal carotid artery was involved by tumor in four patients. Multiple cranial nerve deficits were seen preoperatively, and facial weakness was the most common new deficit postoperatively. Eight patients required intradural access for complete tumor removal. Seven had complete tumor removal. Headaches were the most common complication. One patient had seizures postoperatively, and another had a malignant epidermoid, which resulted in death. CONCLUSIONS: Intracranial epidermoid cysts require complex surgical planning. They involve multiple cranial nerves and vascular structures. Complete resection is frequently possible with minimal new cranial nerve deficits.
OBJECTIVES/HYPOTHESIS: Brain herniation and cerebrospinal fluid (CSF) leakage into the middle ear and mastoid are rare but described complications of chronic ear disease. This paper will discuss the presentation and management of brain herniation and/or CSF leak encountered in revision chronic ear surgery. STUDY DESIGN: Retrospective chart review. METHODS: Twelve of 1,130 cases of revision chronic ear surgery in which brain herniation or CSF leak was diagnosed were identified and analyzed. RESULTS: Ten (83%) patients' initial diagnosis was tympanic membrane (TM) perforation with cholesteatoma and two (17%) with TM perforation without cholesteatoma. Initial revision procedures included one (8.3%) tympanoplasty with canal-wall-up mastoidectomy maintaining ossicular continuity, two (17%) tympanoplasties with canal-wall-down (CWD) mastoidectomies with ossicular chain reconstruction (OCR), and nine (75%) tympanoplasties with CWD mastoidectomies without OCR. Three (25%) required a second procedure, two (17%) a third, and one (8.3%) a fourth, finally resulting in four (33%) with an ossicular reconstruction and eight (67%) without. Brain herniation and/or CSF leak were repaired by way of transmastoid and middle fossa approaches. Preoperative and postoperative pure-tone average air-bone gaps were statistically similar (33.1 and 28.1 dbHL, respectively; P = .464). CONCLUSIONS: Brain herniation and/or CSF leak appear to be rare complications of surgery for revision chronic ear disease. Their management require adherence to the principles of establishing a safe ear with hearing restoration as a secondary goal.
Glomus tumors provide unique surgical challenges for both tumor resection and defect reconstruction. Tumors with intracranial extension compound these challenges. Surgical techniques have evolved, and now, with a multidisciplinary team, single-stage surgeries are the standard. In this paper the authors will report the results of the Otology Group protocol for surgical management of glomus tumors with intracranial extension. Particular attention will be paid to prevention of cerebrospinal fluid leaks with the use of vascularized tissue for defect reconstruction.
Paragangliomas are neuroendocrine tumors derived from the extra-adrenal paraganglia of the autonomic nervous system. Within the head and neck, they are generally defined and named according to their site of origin, and may be found frequently neighboring vascular structures. Physiologic activity is rare in these neoplasms and they may exhibit patterns of inheritance which predispose their occurrence in families, often with multicentricity. These tumors generally exhibit a slow rate of growth, most often presenting asymptomatically as a space occupying mass lesion noted clinically or radiographically. The most common paraganglioma of the head and neck is the carotid body tumor followed by the jugulo-tympanic and vagal varieties. Other rare sites where this tumor may occur include; the larynx, sinonasal chambers and orbit. Diagnosis is generally made through a combination of clinical findings and radiographic studies. Magnetic resonance represents the most important imaging modality for the evaluation and characterization of suspected head and neck paraganglioma. Definitive management for these lesions should be carefully considered in relation to both tumor and patient-oriented factors, especially in regard to the potential morbidity of treatment. Surgery and radiation therapy represent the main treatment modalities for paraganglioma. The selection of treatment depends on the size, location, and biologic activity of the tumor as well as the overall fitness of the patient. Although radiotherapy may be effective in arresting growth of these tumors, rarely is the neoplasm eliminated without surgical resection. Surgery may be associated with significant morbidity, primarily as a consequence of incurring major cranial nerve injury. Patient selection (relative to age and medical condition) should be carefully considered prior to recommending aggressive surgery for paragangliomas of head and neck, especially in those patients at risk for disabling surgical morbidity.
OBJECTIVES/HYPOTHESIS: Despite the enthusiasm of recent short-term reviews, no center in the United States has published results meeting American Academy of Otolaryngology-Head and Neck Surgery guidelines with titanium-based prostheses. The purpose of the study was threefold. The first purpose was to review results with a titanium prosthesis system in cases meeting American Academy of Otolaryngology-Head and Neck Surgery reporting guidelines. The second was to compare these results with previously published results using non-titanium-based prostheses. The third was to examine the authors' results for any evidence of a "learning curve." STUDY DESIGN: Retrospective chart review was performed for the period from February 2000 to August 2001 and for the period from July 2002 to February 2003. METHODS: Of 313 cases, 130 consecutive cases were identified in the first period and 65 in the second time period. One hundred two patients had adequate follow-up for published guidelines. All cases were performed by the senior author (c.g.j.). Comparison data were obtained from a previous publication involving the senior author. RESULTS: Successful rehabilitation (<or=20 dB pure-tone average air-bone gap) of conductive hearing loss was obtained in 70% of partial ossicular chain reconstructions and 44% of total ossicular chain reconstructions when titanium prostheses were used. Comparison data revealed successful rehabilitation in 48% and 21% of non-titanium-based partial and total reconstructions, respectively. Postoperative pure-tone average air-bone gaps were not significantly different when compared with results in the period from July 2002 to February 2003. CONCLUSION: Newer titanium-based ossicular reconstruction devices represent an improvement over previously used non-titanium-based prostheses. The authors think that this improvement is realized rapidly because no learning curve existed in their data.
OBJECTIVES/HYPOTHESIS: Mastoidectomy has long been identified as an effective method of treatment for chronic ear infection. The effect of mastoidectomy on patients without evidence of active infectious disease remains highly debated and unproven. The objective in the study was to examine the impact of mastoidectomy on the repair of uncomplicated tympanic membrane perforations. STUDY DESIGN: Retrospective study of patients at tertiary referral center. METHODS: Four hundred eighty-four patients who underwent surgical repair of simple tympanic membrane perforations were identified and reviewed in a retrospective manner. Simple tympanic membrane perforations were defined as tympanic membrane perforations of any size and location without any of the following confounding variables: 1). active infection (active otorrhea, abnormal middle ear mucosa, or granulations tissue); 2). ossicular abnormalities (ossicular fixation, ossicular discontinuity, ossicular malformation, or ossicular absence); 3). cholesteatoma; or 4). prior attempt at tympanic membrane repair (prior tympanoplasty or mastoidectomy). Surgical outcome and clinical course were assessed to compare results of tympanic membrane perforation repair with and without canal wall up mastoidectomy. RESULTS: Tympanic membrane repair was equally effective in both groups at 91%. Hearing results were comparable. Development of persistent ipsilateral otological disease requiring a subsequent ipsilateral procedure was approximately twice as common in the tympanoplasty group. In the tympanoplasty group, 14.1% of patients underwent subsequent ipsilateral otological procedures, and 6.1% of patients in the tympanoplasty with mastoidectomy intact canal wall group underwent subsequent ipsilateral procedures (P <.05). The most common subsequent ipsilateral procedures were tympanoplasty, tympanostomy tube placement, tympanoplasty with mastoidectomy canal wall up, and tympanoplasty with mastoidectomy canal wall down, in that order. After including untreated tympanic membrane perforations as subsequent procedures, the adjusted rate of subsequent procedures was 15.5% in the tympanoplasty group and 12.2% in the tympanoplasty with mastoidectomy group (P >.05). CONCLUSION: Mastoidectomy was not necessary for successful repair of simple tympanic membrane perforations. However, mastoidectomy impacted the clinical course in patients by reducing the number of patients requiring future procedures and by decreasing disease progression. This suggests that even in the absence of active evidence of infection, mastoidectomy improved the underlying disease process. Combining mastoidectomy with tympanoplasty during repair of simple perforations in patients with no active evidence of infection remains an appropriate option and may be valuable in reducing the need for future surgery.
OBJECTIVE: Several previous studies have shown that muscle appearance on magnetic resonance is a sensitive indicator of muscle denervation. Previous attempts at determining preoperative indicators of final facial function after acoustic neuroma removal has been mostly unsuccessful. The goal of this study was to determine if the appearance of the facial muscles on preoperative imaging is predictive of final facial function after surgical removal of vestibular schwannomas. STUDY DESIGN: We conducted a retrospective chart and magnetic resonance review. SETTING: This study was conducted at a tertiary referral center. PATIENTS: We included all patients who underwent vestibular schwannoma removal between January 1, 1997, and December 31, 2001, with available preoperative magnetic resonance images and a minimum of 12 months follow up. INTERVENTIONS: We used translabyrinthine, middle fossa, and suboccipital approaches for tumor removal. A neuroradiologist, blinded to preoperative or final facial function after tumor removal, retrospectively reviewed preoperative magnetic resonance images. MAIN OUTCOMES MEASURES: Facial muscles were evaluated on magnetic resonance and classified as symmetric or asymmetric. Facial function was graded using the House-Brackmann scale. Preoperative facial function was noted on the preoperative physical examination. Final function was determined at least 12 months postoperatively. RESULTS: A total of 247 patients underwent tumor removal during the study period. One hundred thirty-two patients had adequate preoperative magnetic resonance images. Patients with preoperative facial muscle asymmetry seen on preoperative magnetic resonance indicating muscle atrophy had significantly worse final facial function, regardless of tumor size. CONCLUSION: The preoperative appearance of facial muscles provides valuable insight into the physiology of the facial nerve in the presence of vestibular schwannomas. Patients with pre-operative facial muscle symmetry have significantly better facial function than those with atrophy.
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OBJECTIVES/HYPOTHESIS: Stapes fixation combined with fixation, absence, or malformation of the malleus-incus complex requires an uncommon surgical reconstruction and offers a unique combination of challenges and hazards. This situation may occur in the presence of severe tympanosclerosis, otosclerosis, congenital ossicular malformations, and revision surgery for either stapedectomy or chronic ear disease. In previous reports, this procedure has been grouped with total ossicular reconstruction without much distinction. However, the challenges unique to this problem deserve special consideration. The present report offers a treatment plan for a group of patients requiring reconstruction of the entire ossicular conduction mechanism including removal of the stapes footplate. STUDY DESIGN: Retrospective review. METHODS: Three thousand three hundred fifty (3350) charts of patients requiring total ossicular replacement prostheses (TORPs) were reviewed. Of this group of patients, only 21 of 3350 patients from 1977 to 1999 required TORP placement and removal of the stapes footplate. The patients were followed for an average period of 50 months. RESULTS: Hearing results indicated an overall improvement in the air-bone gap of 10 dB, with 52% achieving an air-bone gap of less than 20 dB. Of the 21 cases, 5 revision surgeries were performed. Three were performed because of a displaced TORP (14.2%). and 2 were performed because of extruded TORPs (9.5%). CONCLUSIONS: Reconstruction of the entire ossicular conduction mechanism including removal of the stapes footplate can be successfully achieved with improvement of the air-bone gap of less than 20 dB. Hearing results and extrusion rates are comparable to reported results of TORP placement on a mobile footplate. Successful stapedectomy and simultaneous ossicular chain reconstruction can be performed as a single or staged procedure. Special attention is paid to avoid intrusion of the prosthesis into the vestibule.
OBJECT: Historically poor outcomes have been characteristic in patients with lateral skull base malignancies. As advances in skull base surgical techniques have been made, complete resection has increasingly been achieved. This has resulted in improved survival rates and local tumor control. METHODS: The authors performed a retrospective review of 95 patients treated for lateral skull base malignancies. The mean age of the patients was 49.4 years. There were 44 females and 51 males. The mean follow-up period was 50 months. Resection was performed in all patients, and postoperative radiotherapy was undertaken in 54% of the cases. Local disease control was maintained in 73% of the patients. Tumor involvement of the facial nerve and intracranial tumor extension did not jeopardize the rate of local control. CONCLUSIONS: Despite the fact that technical advances in skull base surgery have resulted in a higher incidence of complete tumor resection and improved survival rates, a respect for the poor prognosis historically associated with lateral skull base malignancies should be maintained and treatment should be appropriately aggressive.
OBJECTIVE: Chronic ear surgery is difficult. The management of such a disease either with or without cholesteatoma and in an only hearing ear is particularly challenging. Consequences of disease or unintended outcomes of therapy can both result in patient lifestyle alterations of major proportion. This report offers a diagnostic and treatment plan for chronic otitis media with and without cholesteatoma in the only hearing ear. METHODS: More than 10,000 charts of patients with chronic otitis media were retrospectively reviewed. Twenty-seven charts of patients with chronic otitis media in an only hearing ear who underwent surgical treatment were identified. The patients were followed for an average time of 43 months. RESULTS: Overall, the chronic otitis media was well controlled, and there was no change in the average discrimination or hearing thresholds when comparing preoperative and postoperative results. CONCLUSIONS: Chronic otitis media with and without cholesteatoma in an only hearing ear can be treated successfully with hearing preservation. Canal wall down tympanomastoidectomy is performed in most cases. Manipulation of the ossicular chain is avoided, and cholesteatoma that lies over a potential fistula is exteriorized.