Gingival Swelling as the Initial Manifestation of Granulomatosis with Polyangiitis.

We describe a case of granulomatosis with polyangiitis (GPA; formerly named Wegener granulomatosis) that presented initially as florid areas of gingival swelling. The patient also had upper respiratory symptoms that included sinus congestion and cough of recent onset. Clinical-pathologic correlation aided the interpretation of non-specific biopsy findings and immediate referral to an appropriate medical specialist. Treatment was rendered at an early stage of disease with a good response to date. Review of the literature indicates that gingival swelling, often with the characteristic appearance of "strawberry gingivitis" may represent the initial sign of disease in 2% of patients with GPA. Biopsy of gingival lesions often shows a non-specific histologic appearance that should be interpreted in the context of the clinical appearance and pertinent medical history. The clinical investigations that lead to definitive diagnosis and treatment are presented to facilitate the management of this uncommon but potentially fatal condition.

A novel technique of otic barotrauma management using modified intravenous cannulae.

This article provides the first detailed description and systematic evaluation of the management of otic barotrauma using modified intravenous cannulae. A 24-gauge IC cannula was modified as a tool for tympanostomy tube placement and middle ear ventilation. The medical records of 271 ears of 156 adult patients (median age 49 years) who underwent this procedure were reviewed retrospectively. Hundred and ninty-one tubes were placed for otalgia because of hyperbaric oxygen therapy, 58 tubes were inserted for air travel prophylaxis and 22 tubes were placed for management of otic barotrauma post-flight. All the patients who had this procedure for prophylaxis experienced regular otic barotrauma symptoms during air travel prior to tube placement. All patients were reviewed 6 weeks (range 2-9 weeks) post-procedure. This technique of otic barotrauma management worked effectively in 99 % of treated patients. On follow-up, 88 % of tubes were found to be extruded and non-extruded tubes were removed in clinic without any anaesthesia. 99.6 % of tympanic membrane had healed completely and spontaneously without sequelae. Given the safety, effectiveness, low risk of complications associated with this novel tympanostomy technique, it provided a simple yet effective therapeutic option for the management of otic barotrauma. Finally, this technique can be easily applied in all health settings as it only requires medical supplies readily available in hospitals, therefore there is no additional cost.

Malignant otitis externa: experience with hyperbaric oxygen therapy.

INTRODUCTION: The treatment of malignant otitis externa (MOE) with hyperbaric oxygen therapy (HBOT) remains controversial. The rarity of MOE, combined with poor access to hyperbaric facilities, explains the paucity of existing data. METHODS: We retrospectively reviewed all patients with a diagnosis of MOE referred to the Prince of Wales Hospital hyperbaric unit over a period of six years, and report one of the largest case series to date. RESULTS: From August 2001 to October 2007, 17 patients with MOE were referred, of whom 15 (88%) completed therapy, one did not tolerate HBOT and one was withdrawn due to pulmonary complications. Length of admission averaged 48 days (range 8-93 days) and three received outpatient care. Five patients had complications attributable to HBOT: acute pulmonary oedema (n = 2), seizure (n = 1), tympanic membrane perforation (n = 1) and claustrophobia (n = 1). Average time to follow up was 47 months (range 1-94 months). Twelve patients (70%) were considered cured of their disease, being disease-free at follow up, including four patients who had died of other causes but were symptom-free at the time of death. Three patients died directly from MOE (18%), one after a recurrence of their disease. Two further patients had recurrent disease, both successfully treated with a second cycle of HBOT and antibiotics. Nine patients (53%) had facial nerve palsy before commencement of HBOT, of whom four died, three from MOE, four had ongoing facial paralysis, and one resolved. CONCLUSIONS: HBOT confers minimal morbidity, but its role in MOE remains uncertain. The high mortality of MOE despite maximal therapeutic intervention highlights the need for more effective treatment protocols.

Endoscopic sublabial transmaxillary approach to the rostral middle fossa.

OBJECTIVE: The rostral middle fossa faces the temporal pole and is the endocranial anterosuperior aspect of the greater wing of the sphenoid. Standard approaches to this region, such as the subtemporal, pterional, or orbitozygomatic approaches, require significant brain retraction or manipulation of the temporalis muscle. We report an endoscopic sublabial transmaxillary approach to this cranial base region that avoids the aforementioned pitfalls. METHODS: Ten adult cadaveric half heads were used to develop the endoscopic approach and to identify the salient surgical landmarks. RESULTS: The approach was divided into three stages: entry into the maxillary sinus, entry into the infratemporal fossa, and entry into the middle fossa. A craniotomy of greater than 20 mm in diameter can be safely created in the rostral middle fossa. When coupled with image guidance, the approach provides the flexibility to tailor the size and location of the middle fossa craniotomy. CONCLUSION: Although endonasal endoscopic approaches are increasing in popularity, the middle fossa has not been adequately accessed with these techniques. The endoscopic sublabial transmaxillary approach provides safe and direct access to the rostral middle fossa, eliminating the need for brain retraction, temporalis muscle manipulation, or an external incision. The approach also permits early devascularization of cranial- or dural-based lesions.

Reversible peripheral vestibulopathy: the treatment of superior canal dehiscence.

OBJECTIVE: Superior canal dehiscence (SCD) is a recently described disorder that results from absence of bone over the superior semicircular canal. We have reviewed 30 cases of SCD found at our institution and report their presentation, workup, and response to therapy. STUDY DESIGN: Retrospective chart review of all patients diagnosed with SCD from 1999 to 2004 at the University of Utah. RESULTS: Thirty patients were identified with SCD. Patients presented with chronic disequilibrium (63%), Tullio's phenomenon (41%), pressure evoked vertigo (44%), hearing loss (30%), and pulsatile tinnitus (7%). ENG performed early in our series revealed abnormal nystagmus with sound presentation, Valsalva, or tympanogram; however, history and CT examination alone was used to identify this condition in most of our patients. Twenty-seven of the 30 patients had some symptoms related to SCD; the other 3 were found to have incidental SCD on CT examination. Of these patients, 14 had severe enough symptoms to warrant operative intervention. All, but one had resolution of their symptoms after completion of intervention. CONCLUSIONS: Superior canal dehiscence is a highly treatable form of vestibulopathy once recognized. When patients present with typical symptoms, workup with CT is reliable and accurate. Surgical intervention results in reversal of symptoms in most cases with low morbidity. EBM RATING: C-4.

Necrotizing fasciitis of the face: a rare but dangerous complication of dental infection.

Necrotizing fasciitis of the face is extremely rare. However, dentists should be familiar with the presentation of this condition because of the suddenness of its onset, the rapidity of its spread, the resulting drastically disfiguring morbidity and the high rate of mortality associated with it. In this paper, we describe the presentation and treatment of a 57-year-old woman with necrotizing fasciitis of the face and neck due to dental causes and discuss factors in the management of this life-threatening condition.

Congenital bifurcation of the intratemporal facial nerve.

Anomalies of the course of the facial nerve have been reported in association with middle and inner ear malformations. Bifurcation of its intratemporal portion is a rare malformation in which focal splitting of one or more facial nerve segments occurs. We describe the CT appearance of this anomaly and discuss its possible embryology. Facial nerve bifurcation is important to recognize in patients undergoing evaluation for congenital hearing loss and other congenital ear malformations.

Development of a novel eighth-nerve intraneural auditory neuroprosthesis.

OBJECTIVES/HYPOTHESIS: Cochlear nerve stimulation using a linear array of electrodes, the cochlear implant, has become an accepted treatment for profound deafness. Major limitations of this technology are high threshold of stimulation, poor performance in a noisy background, cross-talk between electrodes, unsatisfactory channel selectivity, and variable reconstruction of frequency space. A novel auditory neuroprosthesis is proposed that is expected to overcome these problems by implanting an array of three-dimensional microelectrodes, the Utah Electrode Array, directly into the cochlear nerve. STUDY DESIGN: We have conducted acute, extending for up to 12 hours and semichronic, extending for up to 52 hours, electrophysiological experiments, radiologic and histologic studies in 12 cats. METHODS: The electrically evoked auditory brainstem response was used as a means to characterize the threshold, dynamic range, and stability of cochlear nerve stimulation through the implanted Utah Electrode Array neuroprosthesis. Plain film, computed tomographic, and histological studies were conducted to determine the result of the implant. RESULTS: The electrically evoked auditory brainstem response thresholds were approximately one to two orders of magnitude lower than those evoked with conventional cochlear implants. We were able to close the cochleostomy, bring the cat into normal anatomical position, and obtain stable electrically evoked auditory brainstem responses for up to 52 hours. Plain film and computed tomographic studies indicated that the Utah Electrode Array neuroprosthesis was in the intended position in the nerve. Histological studies did not reveal hemorrhage or significant damage to the nerve. CONCLUSION: Because the presented stimulation paradigm appears to significantly mitigate some of the problems of conventional cochlear implants, it may offer a new therapeutic approach to profound deafness.

Imaging findings of cochlear nerve deficiency.

BACKGROUND AND PURPOSE: High-resolution T2-weighted fast spin-echo MR imaging provides excellent depiction of the cisternal and intracanalicular segments of the vestibulocochlear and facial nerves. Absence or reduction in caliber of the cochlear nerve (deficiency) has been described in association with congenital sensorineural hearing loss (SNHL). Depiction of cochlear nerve integrity may be important for diagnosis and management of SNHL. METHODS: We retrospectively reviewed high-resolution T2-weighted fast spin-echo MR images of 22 patients examined for SNHL who had deficiency of the cochlear nerve. Images were evaluated for the presence and comparative size of the component nerves (facial, cochlear, superior vestibular, and inferior vestibular nerves), relative size of the internal auditory canal (IAC), and any associated inner ear abnormalities. The clinical history, results of the clinical examination, and audiometric findings were reviewed for each patient. RESULTS: Deficiency of the cochlear nerve was observed in 12 patients with congenital SNHL and in 10 patients with acquired SNHL. Hypoplasia of the IAC was observed in association with congenital deficiency of the cochlear nerve in 11 of 12 patients. Deficiency of the cochlear nerve was observed in association with acoustic schwannoma in two cases and with acquired labyrinthine abnormalities in seven cases. Hypoplasia of the IAC was not observed in association with acquired SNHL. CONCLUSION: Deficiency of the cochlear nerve can be shown by high-resolution T2-weighted fast spin-echo MR imaging. Deficiency may be observed in association with congenital or acquired SNHL and may be important in the assessment of patients for cochlear implantation. Hypoplasia of the IAC is an indicator of congenital cochlear nerve deficiency.