Survey of Ménière's disease in a subspecialty referral practice.

OBJECTIVES: To define the prevalence of definite Ménière's disease (MD) among patients presenting with characteristic symptoms and examine the utility of published diagnostic guidelines. STUDY DESIGN AND SETTING: Retrospective review in an academic referral practice. RESULTS: The prevalence of definite MD in these 295 individuals was 64%. The next-largest group (23%) consisted of patients with only cochlear symptoms. Those initially classified as probable are usually reclassified as definite with extended follow-up. Of those with definite MD, the mean duration of disease at last follow-up was 7.6 years, 56% were female, 19% had bilateral disease, and 34% required surgical management for vertigo. CONCLUSIONS: The 1995 AAO-HNS guidelines are useful for classification of MD according to certainty of diagnosis and severity of disease, though some modifications could be considered. SIGNIFICANCE: Application of consistent diagnostic criteria is essential for epidemiological, genetic, or outcomes studies of Ménière's disease.

Otomycosis: clinical features and treatment implications.

OBJECTIVES: To determine the clinical presentation, predisposing factors, complications, and treatment outcomes of otomycosis. STUDY DESIGN AND SETTING: Retrospective review of 132 patients with a clinical diagnosis of otomycosis treated from 1998 to 2004 in an academic otology practice. RESULTS: Otalgia and otorrhea were the most common presenting complaints (48%). Prior otologic procedures increase the risk of developing otomycosis. Residual disease was observed in 13% and recurrence in 15% of the subjects. The presence of a mastoid cavity was associated with higher recurrent and residual disease rates. Topical ketoconazole, cresylate otic drops, and aluminum acetate otic drops were all relatively effective with >80% resolution rate on initial application, although topical ketoconazole had a higher resolution rate and lower rate of disease recurrence. CONCLUSIONS AND SIGNIFICANCE: Otomycosis can usually be diagnosed by clinical examination and often occurs in the setting of persistent otorrhea. Complications are not uncommon but usually resolve with application of appropriate topical antifungal agents. Eradication of disease is more difficult in the presence of a mastoid cavity.

Invasive cerebrospinal fluid cysts and cephaloceles of the petrous apex.

OBJECTIVE: To describe the presentation, diagnostic evaluation, and surgical management of petrous apex cerebrospinal fluid (CSF) cysts and cephaloceles. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Six patients with symptomatic CSF cysts or cephaloceles. INTERVENTION(S): All patients underwent operative intervention. MAIN OUTCOME MEASURE(S): Presentation, imaging characteristics, operative findings, surgical approach, resolution of symptoms, and complications. RESULTS: Six patients presented with various neurotologic symptoms including vertigo, otalgia, diplopia, meningitis, hearing loss, and retroorbital headaches. Four lesions were centered within the anterior petrous apex and were classified as a cephalocele originating from Meckel's cave. The remaining two lesions were arachnoid cysts that involved the posterior petrous apex. Cysts and cephaloceles both demonstrated bone erosion on computed tomography and were hyperintense on T2-weighted magnetic resonance imaging and isointense or hypointense on T1-weighted magnetic resonance imaging. A variety of surgical approaches was used to treat these lesions. Preoperative symptoms were improved in five of six cases. One patient developed a postoperative CSF leak that resolved with conservative measures. CONCLUSION: Petrous apex CSF cysts and cephaloceles may present with a variety of neurotologic symptoms. Imaging often helps narrow the differential diagnosis, but these lesions can still be confused with other erosive skull base lesions such as cholesterol granulomas, epidermoids, or tumors. Optimal treatment of symptomatic posterior petrous apex CSF cysts is marsupialization via a posterior fossa approach (i.e., retrosigmoid or retrolabyrinthine). A middle fossa approach with obliteration of the anterior petrous apex may be used to treat symptomatic CSF cephaloceles arising from Meckel's cave.

Adult spontaneous CSF otorrhea: correlation with radiographic empty sella.

OBJECTIVE: To determine the prevalence of radiographic empty sella in patients with spontaneous cerebrospinal fluid (CSF) otorrhea. STUDY DESIGN AND SETTING: Retrospective case series of adult patients with CSF otorhinorrhea at an academic tertiary medical center. Patients with history of skull base surgery, trauma, tumor, or chronic ear disease were excluded. Available imaging studies were reviewed with attention to the sella turcica. RESULTS: Eight patients were diagnosed with spontaneous CSF otorrhea. Five of seven patients with adequate imaging studies (71%) had a radiographic empty sella. Seven of eight patients were clinically obese, with a body mass index BMI>30 kg/m2. CONCLUSIONS: Empty sella is a common radiologic finding in patients with spontaneous CSF otorrhea. This supports the theory that increased intracranial pressure contributes to development of spontaneous CSF otorrhea. SIGNIFICANCE: Radiographic empty sella predicts elevated intracranial pressure, which may require further evaluation and treatment in patients with spontaneous CSF otorrhea. EBM RATING: C-4.

Stapes surgery in the United States.

OBJECTIVE: A contemporary analysis of trends in the surgical management of otosclerosis in the United States is presented. DATA SOURCES: U.S. population demographics were obtained from the U.S. Census Bureau. Surgical case data was obtained from publications of the National Center for Health Statistics. Physician population statistics are published by the American Medical Association. DATA EXTRACTION: Available estimates for otologic operations are interpreted according to clinical setting used for data acquisition and surgical practice patterns. Linear regression of included data defines trends in operations performed. Population statistics are used to define changes in the incidence of surgical cases. CONCLUSIONS: The number of stapedectomy cases has declined over the past 30 years. During this same interval, the U.S. population (including the white population) and the number of surgeons has increased. This indicates that the incidence of surgical otosclerosis is declining. Although the true etiology of the decline is uncertain, widespread immunization for measles is a plausible hypothesis.

Coronal computed tomography prevalence of superior semicircular canal dehiscence.

OBJECTIVES: The relatively new clinical entity superior canal dehiscence syndrome (SCDS) is diagnosed by clinical symptoms and signs. Coronal computed tomography (CT) has been used to confirm the diagnosis. A consecutive series of temporal bone CT scans was reviewed to define the prevalence of a dehiscent-appearing superior semicircular canal. STUDY DESIGN AND SETTING: Temporal bone CT scans performed over a 2-year period at a university-based tertiary referral center were reviewed independently by 3 individuals. Scans were excluded if coronal images were not obtained or reconstructed from axial images. Prevalence figures for dehiscent-appearing superior semicircular canal were determined by consensus. Medical records of selected individuals with a dehiscent-appearing canal were reviewed for study indications and otologic symptoms. RESULTS: A dehiscent-appearing superior semicircular canal was seen in 9% of studies. Correlation among examiners was greater than 94%. Medical records indicated symptoms suggestive of or compatible with the diagnosis of SCDS in rare cases. CONCLUSION: The prevalence of a dehiscent-appearing superior semicircular canal on coronal CT of the temporal bones performed with 1.0-mm collimation is substantially greater than that predicted by temporal bone histologic study. Clinical symptoms compatible with the diagnosis were seldom recorded, suggesting low specificity. The high sensitivity and low specificity of CT scan create a risk for overdiagnosis of SCDS if the coronal CT scans are not correlated with clinical symptoms.

Inflammatory pseudotumor of the temporal bone.

OBJECTIVE: To characterize the clinical presentation, imaging characteristics, intraoperative findings, and key histopathologic features of inflammatory pseudotumors of the temporal bone. Findings from an index case are presented, and the literature is reviewed for comparison. STUDY DESIGN: Retrospective case review. SETTING: University tertiary referral center. PATIENTS: Cases were identified by review of surgical specimens from the temporal bone and lateral skull base with histopathologic confirmation. A single case was identified at our institution. Nine additional cases were identified in the literature; clinical features were reviewed. INTERVENTION: Of reported cases, treatment consisted of complete surgical excision in eight cases and subtotal excision in one. The index patient underwent surgical excision with postoperative corticosteroid therapy for adjacent meningeal involvement, after histopathologic interpretation. Corticosteroids were administered to one patient with residual microscopic tumor, and external beam radiotherapy was used for residual/recurrent disease in one case. RESULTS: The lesions were typically locally aggressive with extensive bony erosion. Three cases (33%) demonstrated labyrinthine and otic capsule involvement. Four cases (44%) involved the facial nerve. Characteristic histopathologic features included fibroblastic proliferation and a mixed inflammatory cell infiltrate in all cases. Mitotic figures, nuclear pleomorphism, and necrosis were rare or nonexistent. CONCLUSIONS: Inflammatory pseudotumors of the temporal bone are rare but aggressive lesions. Therapy should consist of surgical excision with steroids reserved for residual or intracranial disease or in patients in whom surgery is not an option. These lesions must be differentiated from other infectious, granulomatous, and neoplastic lesions on the basis of histopathologic and immunohistochemical findings.

Delayed-onset facial paralysis after vestibular neurectomy.

OBJECTIVES/HYPOTHESIS: Delayed-onset facial paralysis (beginning more than 3 d after the procedure) has been described as a complication of many different types of otological procedures. The incidence of this problem in vestibular neurectomy and the relationship to surgical approach are detailed in the study. STUDY DESIGN: Retrospective case review. METHODS: In the setting of a tertiary referral center, vestibular neurectomy was performed in 70 individuals with disabling vertigo unresponsive to medical therapy who elected vestibular neurectomy. The main outcome measure was incidence of delayed onset facial paralysis. RESULTS: Delayed-onset facial paralysis was significantly more common after the middle fossa (18%) and translabyrinthine (11%) approaches compared with the retrosigmoid approach (0%). CONCLUSIONS: Surgical approach influences the incidence of delayed-onset facial paralysis. Measures to prevent this complication such as prophylactic antiviral medication or labyrinthine segment decompression may be considered in middle fossa and translabyrinthine operations.