Faculty Bibliography

OBJECTIVES: To investigate the characteristics associated with survival in esthesioneuroblastoma and to determine whether the modified Kadish staging system can predict outcome. DESIGN: Retrospective population-based cohort study. SUBJECTS: All patients in the Surveillance, Epidemiology, and End Results tumor registry diagnosed as having esthesioneuroblastoma (1973-2002). MAIN OUTCOME MEASURES: The modified Kadish stage and the overall and disease-specific survival rates were determined. RESULTS: The cohort included 311 patients with a mean age of 53 years and a unimodal age distribution. The overall 5- and 10-year survival rates were 62.1% and 45.6%, respectively. The modified Kadish staging system was applied to 261 patients. Kaplan-Meier analysis showed the overall and disease-specific survival rates at 10 years to be 83.4% and 90%, respectively, for patients with stage A disease; 49% and 68.3% for patients with stage B disease; 38.6% and 66.7% for patients with stage C disease; and 13.3% and 35.6% for patients with stage D disease. Log-rank test comparisons found Kadish stage (P<.01), treatment modality (P<.002), lymph node status (P<.01), and age at diagnosis (P<.001) to be significant predictors of survival. Cox regression analysis confirmed that Kadish stage remained a significant predictor of disease-specific survival. CONCLUSION: The modified Kadish staging system, lymph node status, treatment modality, and age are useful predictors of survival in patients who present with esthesioneuroblastoma

Pediatric autoimmune neuropsychiatric disorder associated with streptococcal infections (PANDAS) has been primarily described in the neurology and psychiatry literature. The symptoms of this syndrome typically are a range of obsessive compulsive disorders and neuromuscular tics. The otolaryngologist occasionally becomes involved with these children when it is deemed that chronic tonsil infections are the source. We report here on a child diagnosed with PANDAS who presented with severe ventricular hyperfunction and adductor spasmodic dysphonia. She was treated with botulinum toxin, which resulted in a significant improvement in subjective voice as well as reduced jitter and shimmer on objective voice measurements.

BACKGROUND: The aim of this study was to determine the incidence, outcomes, and risk factors for synechia formation after endoscopic sinus surgery (ESS) and middle turbinate medialization with and without FloSeal. METHODS: A retrospective review was performed of patients who underwent primary ESS with middle turbinate medialization, with or without the placement of FloSeal. Medialization was performed with the placement of an absorbable conchopexy suture and silastic splint. Operative variables and outcomes were analyzed to identify risk factors for synechia formation. RESULTS: One hundred thirty-five patients underwent medialization alone and 37 patients underwent medialization with placement of FloSeal. Overall, synechia formation was noted in 16 patients (9.3%). A statistically significant higher incidence of synechia formation was noted in patients who underwent middle turbinate medialization with the placement of FloSeal versus medialization alone (18.9% versus 6.7%). The incidences of intraoperative complications (6.2% versus 4.7%) and postoperative complications (6.2% versus 7%) were similar between patients with and without synechia, respectively. Patients experiencing synechia, however, underwent a statistically significant higher rate of revision procedures (25% versus 5.1%). CONCLUSION: Despite adequate prevention with middle turbinate medialization, synechia formation after ESS may result in higher rates of revision procedures. The placement of FloSeal in conjunction with middle turbinate medialization may result in a higher incidence of synechia formation

Severe hearing loss during early development is associated with deficits in speech and language acquisition. Although functional studies have shown a deafness-induced alteration of synaptic strength, it is not known whether long-term synaptic plasticity depends on auditory experience. In this study, sensorineural hearing loss (SNHL) was induced surgically in developing gerbils at postnatal day 10, and excitatory synaptic plasticity was examined subsequently in a brain slice preparation that preserves the thalamorecipient auditory cortex. Extracellular stimuli were applied at layer 6 (L6), whereas evoked excitatory synaptic potentials (EPSPs) were recorded from L5 neurons by using a whole-cell current clamp configuration. In control neurons, the conditioning stimulation of L6 significantly altered EPSP amplitude for at least 1 h. Approximately half of neurons displayed long-term potentiation (LTP), whereas the other half displayed long-term depression (LTD). In contrast, SNHL neurons displayed only LTD after the conditioning stimulation of L6. Finally, the vast majority of neurons recorded from control prehearing animals (postnatal days 9-11) displayed LTD after L6 stimulation. Thus, normal auditory experience may be essential for the maturation of synaptic plasticity mechanisms

Spiral ganglion neurons (SGNs) provide afferent innervation to the cochlea and rely on contact with hair cells (HCs) for their survival. Following deafferentation due to hair cell loss, SGNs gradually die. In a rat culture model, we explored the ability of prosurvival members of the Bcl-2 family of proteins to support the survival and neurite outgrowth of SGNs. We found that overexpression of either Bcl-2 or Bcl-xL significantly increases SGN survival in the absence of neurotrophic factors, establishing that the Bcl-2 pathway is sufficient for SGN cell survival and that SGN deprived of trophic support die by an apoptotic mechanism. However, in contrast to observations in central neurons and PC12 cells where Bcl-2 appears to promote neurite growth, both Bcl-2 and Bcl-xL overexpression dramatically inhibit neurite outgrowth in SGNs. This inhibition of neurite growth by Bcl-2 occurs in nearly all SGNs even in the presence of multiple neurotrophic factors implying that Bcl-2 directly inhibits neurite growth rather than simply rescuing a subpopulation of neurons incapable of extending neurites without additional stimuli. Thus, although overexpression of prosurvival members of the Bcl-2 family prevents SGN loss following trophic factor deprivation, the inhibition of neurite growth by these molecules may limit their efficacy for support of auditory nerve maintenance or regeneration following hair cell loss

HYPOTHESIS: Delivery of math1 using an adenovector (Admath1.11D) results in vestibular hair cell regeneration and recovery of balance function in ototoxin-treated adult mice. BACKGROUND: Loss of peripheral vestibular function is associated with disease processes such as vestibular neuronitis, aminoglycoside ototoxicity, and aging. Loss of vestibular hair cells is one of the mechanisms underlying balance dysfunction in all of these disorders. Currently, recovery from these diseases relies on central vestibular compensation rather than on local tissue recovery. Overexpression of the mammalian atonal homologue math1 has been demonstrated to induce generation of hair cells in neonatal organ of Corti cultures and in the guinea pig cochlea in vivo and could thus provide an approach to local tissue recovery. METHODS: Admath1.11D was applied to cultures of aminoglycoside-treated macular organs or in vivo in a mouse aminoglycoside ototoxicity model. Outcome measures included histologic examination, immunohistochemistry, swim testing, and evaluation of the horizontal vestibulo-ocular reflex. RESULTS: Delivery of math1 resulted in the generation of vestibular hair cells in vitro after aminoglycoside-mediated loss of hair cells. Math1-treated mice showed recovery of the vestibular neuroepithelium within 8 weeks after Admath1.11D treatment. Assessment of animals after vector infusion demonstrated a recovery of vestibular function compared with aminoglycoside-only-treated mice. CONCLUSION: Molecular replacement of math1 may provide a therapeutic means of restoring vestibular function related to vestibular hair cell loss.

OBJECTIVE: We describe our surgical posterior transpetrosal technique, particularly the transcrusal variant for lesions involving the upper and middle clivus, petroclival regions, and lesions that involve both the posterior and middle fossae. METHODS: An outline of the posterior transpetrosal technique involved, particularly the transcrusal variant, is described. Important superficial landmarks are identified, and a radical mastoidectomy is performed. The antrum is identified and entered, and, upon completion of the mastoidectomy and when Trautman's triangle is defined, the temporal and suboccipital craniotomies are completed. After bone flap elevation, dura opening, and incision along the middle fossa dura, the superior petrosal sinus is ligated and cut. Tentorium cut completion is at the incisura posterior to the trochlear nerve. Watertight dural closure and standard flap replacement and skin closure complete the technique. RESULTS: Clival exposure and the degree of temporal bone resection increase. Operative freedom also increases with increased temporal bone resection, especially when going from the retrolabyrinthine to transcrusal variants. Little is gained in terms of operative freedom and exposure of the clivus with resection of additional temporal bone beyond that of the transcrusal variant, and resection carries the cost of increasing morbidity, especially with respect to VIIth and VIIIth nerve function. CONCLUSION: The posterior transpetrosal approach and the transcrusal variant provide a lateral operative corridor to lesions of the upper and middle clivus. The transcrusal variant provides increased exposure and operative freedom similar to that provided by the transcochlear approach while minimizing cranial nerve morbidity.