Diagnostic Protocol for Detecting Otosclerosis on High-Resolution Temporal Bone CT.

OBJECTIVES: To present a systematic checklist to improve diagnosing otosclerosis (OS) on high-resolution computed tomography (HRCT) of the temporal bones and review this protocol's efficacy in diagnosing OS on HRCT. METHODS: A retrospective case series was performed at a University Referral Hospital in urban Chicago, Illinois. High-resolution computed tomographies of the temporal bone were reviewed including 17 ears in the test group with surgically confirmed OS and 21 ears in the control group surgically confirmed to not have OS. Preoperative HRCTs were evaluated by a single neuroradiologist using a systematic protocol created to assist in diagnosing OS. This looked for radiolucency at the fissula ante fenestram and pericochlear region, and new bone formation around the oval and round windows. RESULTS: The radiologist accurately diagnosed OS in all 17 test group ears and ruled out OS in all 21 control group ears using the protocol. All 17 test ears were read to have lucency at the fissula ante fenestram, 9 (53.0%) to have new bone formation, and 8 (47.1%) to have cochlear lucency. The radiologist was more confident in diagnosing OS when cochlear lucency was present with the fissula ante fenestram lucency. CONCLUSIONS: This HRCT checklist is a highly accurate tool for evaluating the presence of OS when images are reviewed in the systematic fashion described. Imaging prior to surgery aids in counseling patients, preparing surgically, and excluding other pathologies.

Stereotactic Radiosurgery and Fractionated Stereotactic Radiation Therapy for the Treatment of Uveal Melanoma.

PURPOSE: To evaluate treatment results of stereotactic radiosurgery or fractionated stereotactic radiation therapy (SRS/FSRT) for uveal melanoma. METHODS AND MATERIALS: We retrospectively evaluated 181 patients with 182 uveal melanomas receiving SRS/FSRT between 2007 and 2013. Treatment was administered with CyberKnife. RESULTS: According to Collaborative Ocular Melanoma Study criteria, tumor size was small in 1%, medium in 49.5%, and large in 49.5% of the patients. Seventy-one tumors received <45 Gy, and 111 received ≥45 Gy. Median follow-up time was 24 months. Complete and partial response was observed in 8 and 104 eyes, respectively. The rate of 5-year overall survival was 98%, disease-free survival 57%, local recurrence-free survival 73%, distant metastasis-free survival 69%, and enucleation-free survival 73%. There was a significant correlation between tumor size and disease-free survival, SRS/FSRT dose and enucleation-free survival; and both were prognostic for local recurrence-free survival. Enucleation was performed in 41 eyes owing to progression in 26 and complications in 11. CONCLUSIONS: The radiation therapy dose is of great importance for local control and eye retention; the best treatment outcome was achieved using ≥45 Gy in 3 fractions.

Long-term Results of ABI in Children With Severe Inner Ear Malformations.

OBJECTIVE: To report the long-term outcomes of children who received auditory brainstem implant (ABI) because of severe inner ear malformations. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary referral otolaryngology clinic. SUBJECTS AND METHODS: Between July 2006 and October 2014, 60 children received ABI at Hacettepe University. Preoperative work up included otolaryngologic examination, audiological assessment, radiological evaluation together with assessment of language development and psychological status. The surgeries were performed via retrosigmoid approach with a pediatric neurosurgeon. Intraoperatively, electrical auditory brainstem response was utilized. Initial stimulation was done 4 to 5 weeks postoperatively. Outcomes were evaluated with Categories of Auditory Performance (CAP), speech intelligibility rate (SIR), functional auditory performance of cochlear implant (FAPCI) and Manchester Spoken Language Development Scale scores; receptive and expressive language ages were determined. RESULTS: Sixty children who received ABI were between ages of 12 and 64 months. Thirty-five patients with follow up period of at least 1 year, were reported in means of long-term audiological and language results. The most prevelant inner ear malformation was cochlear hypoplasia (n = 19). No major complication was encountered. Majority of the patients were in CAP 5 category, which implies that they can understand common phrases without lip reading. SIR was found out to be better with improving hearing thresholds. Children with ABI were performing worse than average cochlear implantation (CI) users when FAPCI scores were compared. Patients with the best hearing thresholds have expressive vocabulary of 50 to 200 words when evaluated with Manchester Spoken Language Development Scale. There was no relationship between the number of active electrodes and hearing thresholds. The type of inner ear anomaly with the best and the worst hearing thresholds were common cavity and cochlear aperture aplasia, respectively. Patients with additional handicaps had worse outcomes. Among 35 children, 29 had closed set discrimination and 12 developed open set discrimination above 50%. It was determined that, progress of the patients is faster in the initial 2 years when compared with further use of ABI. CONCLUSION: ABI is an acceptable and effective treatment modality for pediatric population with severe inner ear malformations. Bilateral stimulation together with CI and contralateral ABI should be utilized in suitable cases.

Oval window atresia: a novel surgical approach and pathognomonic radiological finding.

OBJECTIVES: The facial nerve usually occupies the oval window area in patients with oval window atresia. During exploration, if the facial nerve is discovered to lie in the oval window area, this is usually regarded as a contraindication for further surgical intervention. The aim of the present paper is to demonstrate the preoperative pathognomonic radiological sign and describe a new surgical approach for this difficult situation. METHODS: 3 patients and 4 ears were operated due to conductive hearing loss by the same surgeon in a tertiary referral center. Their clinical presentation, radiological findings, surgical findings and final outcomes were evaluated and correlated. RESULTS: Surgical findings were identical in all 4 ears: facial nerve was running over the oval window and tympanic portion was completely dehiscent. Incus long arm was medially displaced due to abnormal development of the stapes suprastructure. In each ear a successful vestibulotomy and teflon piston placement was achieved. Preoperative mean air-bone gap of 47.5dB was improved to 21.5dB. There were no complications. CONCLUSION: Oval window atresia is a rare middle ear anomaly usually regarded as a contraindication for surgical intervention. In this study we present a novel surgical approach with succesful results. However the best approach is to inform the family by showing the nerve on tomography, showing the operation video, informing the family about the sensorineural hearing loss and letting the family choose the treatment option.

Early computed tomography findings of the inner ear after stapes surgery and its clinical correlations.

OBJECTIVE: Pneumolabyrinth resulting from temporal bone trauma and stapes luxation has been associated with sensorineural hearing loss (SNHL). The principal purpose of this study was to determine the incidence and volume of pneumolabyrinth after stapedotomy in which iatrogenic perilymphatic fistula is created and to also correlate this with possible hearing loss and vertigo. STUDY DESIGN: Prospective study. SETTING: Tertiary referral center. PATIENTS AND METHODS: Fifty stapedotomy patients were operated on for otosclerosis, and of those 50, 20 underwent high-resolution computed tomography (CT) on the first day, 10 on the third day, and 20 on the seventh day. The patients followed up regarding SNHL and vertigo that could develop postoperatively, and the correlation of such complications with HRCT findings was examined. RESULTS: The 20 patients who had high-resolution CT (HRCT) on the first day all presented with pneumolabyrinth, and none of the 20 patients who underwent HRCT on the seventh day had pneumolabyrinth. Postoperatively, 92% of the patients had less than 20 dB and 62% had less than 10 dB air-bone gap. None of the patients had SNHL or persistent vertigo. There was no correlation between pneumolabyrinth and hearing loss or vertigo. CONCLUSION: Pneumolabyrinth is a radiological sign of perilymphatic fistula and has no effect on sensorineural hearing loss and vertigo. Observing pneumolabyrinth during the early postoperative stage should not necessarily implicate a complication; however, pneumolabyrinth after the first week supported with the clinical symptoms of perilymphatic fistula would be a meaningful finding.