September 7, 2012 John C. Goddard, MD; Eric R. Oliver, MD; Ted A. Meyer, MD, PhD
article
Abstract
Hallermann-Streiff syndrome, also known as oculomandibulofacial syndrome, is a rare congenital disorder affecting growth and cranial, dental, ocular, pilocutaneous, and mental development. In addition to routine audiologic testing in patients with this syndrome, high-resolution computed tomography of the temporal bones should be performed in those with documented or suspected sensorineural hearing loss. Cochlear implantation may be considered, as in other children with syndromic hearing loss and certain inner ear abnormalities, if the appropriate audiologic, psychosocial, and medical criteria are met. The current case report radiographically and clinically characterizes inner ear dysplasia in an 8-year-old patient with Hallermann-Streiff syndrome. High-resolution computed tomography of the temporal bones revealed a hypoplastic bony island between the vestibule and horizontal semicircular canals, as well as incomplete bony coverage of the posterior semicircular canal crura bilaterally. To our knowledge, this is the first report of a pediatric patient demonstrating bilateral posterior semicircular canal dehiscence.
June 4, 2012 Anil Joshi, MS(ENT), MRCS; Wendy Smith, FRCS(ORL–HNS); David A. Moffat, FRCS
article
Abstract
Spontaneous temporal meningoencephaloceles are unusual. When they do occur, they present with a variety of signs and symptoms, which can make diagnosis and management challenging. We report the interesting case of a 49-year-old woman with bilateral congenital temporal meningoencephaloceles. She presented with a 12-month history of bilateral fluctuating hearing loss, and she more recently developed right-sided acute otitis media with meningitis. The presentation of bilateral extensive tegmental defects and meningoencephaloceles with a fluctuating hearing loss and meningitis associated with acute otitis media affecting one ear and then subsequently the other ear is extremely rare and difficult to diagnose. It requires a very careful clinical and radiologic assessment. Methods of surgical repair differ depending on the size of the defects.
April 30, 2012 Joseph A. Ursick, MD; Jose N. Fayad, MD
article
Cholesteatomas are believed to form as the result of poor eustachian tube function with resultant tympanic membrane retraction and a lack of normal epithelial migration.
March 31, 2012 Jeffrey J. Nelson, MD and Parul Goyal, MD
article
Abstract
Extraorbital idiopathic pseudotumors of the skull base are very uncommon. We report the case of a 50-year-old woman who presented with left ophthalmoplegia and vision loss. Imaging studies revealed an enhancing lesion involving the left petrous apex and cavernous sinus. A transnasal endoscopic approach was used to obtain a biopsy of the left petrous apex. Pathology identified the lesion as an idiopathic pseudotumor. The patient was treated with high-dose steroids and steroid-sparing immunomodulators, and she experienced a significant improvement. To the best of our knowledge, this is the first reported case of a transnasal endoscopic approach to a biopsy of a pseudotumor involving the petrous apex. We discuss the features of this case, and we review the literature on this condition.
June 13, 2011 Sampath C. Prasad, MS, DNB, Kishore Chandra Prasad, DLO, MS, FACS, Rukma Bhandary, DLO, MS, Kumar Abhijith, MS, and Pallavi Sampath, DNB
article
Abstract
Osteomyelitis of the skull base almost always occurs in elderly patients with diabetes; however, it may occur in patients with compromised immune function regardless of their age. We present the cases of a pair of immunocompetent, 2-year-old identical twins who experienced osteomyelitis of the temporal bone almost exactly 1 year apart. An incident such as this, in this age group, has never been reported in the literature.
March 1, 2011 Zi Yang Jiang, MD and Miriam I. Saadia-Redleaf, MD
article
Abstract
Primary lymphoma of the temporal bone is an extremely rare finding in clinical practice. Although it is treated as a type of non-Hodgkin lymphoma, the absence of systemic signs and symptoms makes detection dependent on the tumor's local mass effect. In this article, we report a case of temporal bone lymphoma that caused XIIth nerve palsy. We also discuss the clinical manifestations of temporal bone lymphoma and the importance of imaging the head and neck when any idiopathic cranial nerve palsy develops.
February 1, 2011 Borlingegowda Viswanatha, MS, DLO
article
Abstract
The author conducted a retrospective review of the clinical presentation, management, and complications of temporal bone osteoma in young adolescents. The study population was made up of 9 patients-5 girls and 4 boys, aged 12 to 15 years at presentation (mean: 13.7)-who had been seen for radiologically and histopathologically proven temporal bone osteoma at the author's institution over a 9-year period. Of this group, 5 patients had extracanalicular osteoma (3 in the mastoid portion of the temporal bone, 1 in the squamous portion, and 1 in the mastoid antrum) and 4 patients had osteoma of the external auditory canal. Six of the 9 patients underwent surgical treatment; of the remainder, 1 refused surgery and 2 were managed conservatively with ongoing observation. All patients were followed for a minimum of 1 year, and no recurrences and no complications were observed during that time.
June 30, 2010 Dare V. Ajibade, BA, Iwao K. Tanaka, MD, Kapila V. Paghdal, PharmD, MD, Neena Mirani, MD, Huey-Jen Lee, MD, and Robert W. Jyung, MD
article
Abstract
We report the case of a 41-year-old man who presented with progressive right-sided ear pressure, otalgia, hearing loss, tinnitus, and intermittent otorrhea. Computed tomography and magnetic resonance imaging detected a soft-tissue mass in the right mastoid with intracranial invasion and erosion through the tegmen tympani and mastoid cortex. Histopathologic examination was consistent with an inflammatory pseudotumor (plasma cell granuloma). These lesions rarely occur in the temporal bone. When they do, they are locally destructive and can erode bone and soft tissues. Aggressive surgery is recommended as a first-line treatment, with adjunctive steroid or radiotherapy reserved for residual or refractory disease. Our patient subsequently experienced multiple recurrences, and his treatment required all of these modalities. At the most recent follow-up, he was disease-free and doing well.
March 1, 2010 Deepika Sareen, MBBS, Ashwani Sethi, MS, Sumit Mrig, MBBS, Sonu Nigam, MD, and A.K. Agarwal, MS
article
Abstract
We report the case of an 11-year-old girl who presented with a soft-tissue mass that filled the left external auditory canal and a discharge that resembled chronic suppurative otitis media. The patient underwent mastoid exploration with complete excision of the mass. Findings on the excision biopsy were consistent with a myxoma of the temporal bone. At follow-up 2 years postoperatively, the patient remained disease-free. To the best of our knowledge, this is only the 12th case of a myxoma of the temporal bone to be reported in the English-language literature.
April 30, 2009 K. Asif Ahmed, MD, David Allison, MD, Wesley S. Whatley, MD, and Rakesh K. Chandra, MD
article
Abstract
We conducted a retrospective study of the utility of angiography in the evaluation of patients with temporal bone fractures. Our study population was made up of 64 patients-58 males and 6 females, aged 14 to 75 years (mean: 35.3)-with a temporal bone fracture who had presented to a level I trauma center over a 1-year period. Records were reviewed and data were obtained on the mechanism of injury; the type of fracture; associated injuries, particularly neurocranial injuries detected on computed tomography (CT) of the head; and any angiographic findings that might have been obtained. The primary outcomes measures were the type of treatment administered (conservative vs. surgical) and mortality. Patients were assigned to 1 of 4 groups according to CT results and angiographic findings, if any: normal CT and no angiogram (group 1; n = 12), abnormal CT and no angiogram (group 2; n = 28), abnormal CT and an abnormal angiogram (group 3; n = 9), and abnormal CT and a normal angiogram (group 4; n = 15). Conservative treatment was administered to all 12 patients in group 1 and to 9 patients (60%) in group 4; surgical treatment was provided to two-thirds of the patients in both group 2 and group 3. Mortality was low in group 1 (n = 0), group 3 (n = 1; 11%), and group 4 (n = 1; 7%), but high in group 2 (n = 10; 36%). In fact, the key finding of this study was that mortality in the group with an abnormal CT and no angiogram (group 2) was significantly higher than mortality in the group with an abnormal CT and an abnormal angiogram (group 3) (p = 0.02), even though the injuries in the 2 groups were similarly severe and their management was similarly aggressive. We conclude that current guidelines for angiography may need to be expanded to include all patients who have CT evidence of neurocranial injury in order to detect those vascular injuries that need aggressive management and thus lower overall mortality.
October 31, 2008 Husain A. Sattar, MD, Dorise L. Yang, MD, Aliya N. Husain, MD, Miriam I. Redleaf, MD, and Vijay S. Dayal, MD
article
Abstract
We report the case of a 71-year-old woman with multiple benign lung paragangliomata and a benign glomus jugulare paraganglioma in one temporal bone that mimicked a malignancy. The patient's lung lesions did not regress with chemotherapy. Subsequent histologic markers suggested several very slowly dividing tumors. We review the patient's medical course and pathology from both sites. A finding of multiple lung paragangliomata should raise the suspicion of a multicentric rather than malignant tumor. Before any chemotherapeutic regimen is initiated, a thorough physical examination of the head and neck should be performed, and biopsy material should be tested for markers of cell division.
June 30, 2008 Borlingegowda Viswanatha, MS, DLO
article
Abstract
Extracanalicular osteomas of the temporal bone are rare, slow-growing, benign neoplasms. Although they may occur anywhere in the temporal bone, they are most common in the mastoid area. Often, symptoms are negligible and treatment is unnecessary. However, surgical removal is indicated when growth of the osteoma causes distressing symptoms or cosmetic issues. The patient described in this report had no symptoms but underwent surgical excision of a mastoid osteoma for cosmetic reasons. At 4-month follow-up, she remains asymptomatic and recurrence-free.
