Stanley Pelosi

Glomus tympanicum: a review of 115 cases over 4 decades.

Objective To characterize the clinical presentation, surgical management, and outcomes of a large consecutive cohort of patients with glomus tympanicum (GT) tumors managed at a single tertiary referral group over 4 decades. Study Design Retrospective review. Setting Tertiary neurotological referral center. Subjects and Methods All patients underwent surgical treatment of histopathologically confirmed GT between January 1973 and March 2014. Audiometric outcomes were reported according to AAO-HNS guidelines, and tumor stage was described using the Glasscock-Jackson classification system. Results There were 115 patients (90.4% women; mean age, 55.2 years) who met the inclusion criteria; 38 (33.0%) cases of GT were stage I, 51 (44.3%) stage II, 10 (8.7%) stage III, and 16 (13.9%) stage IV. There were 108 (93.9%) patients who underwent gross total removal, while 7 (6.1%) received less than complete resection for advanced disease that was adherent to the petrous carotid artery, facial nerve, stapes footplate, or round window. Two patients who underwent gross total resection experienced transient facial paresis, and 1 had internal carotid injury with stroke. No patients had been diagnosed with recurrent disease at a mean follow-up of 30.4 months. Conclusion Surgery remains the treatment of choice for GT, providing a high rate of tumor control and resolution of aural symptoms with a low risk of complications. The surgical approach and extent of resection should be tailored to the patient. Gross total resection can be performed in over 90% of patients; however, leaving a limited adherent tumor remnant on the facial nerve or carotid artery should be considered with advanced infiltrative disease to prevent unnecessary morbidity.

Fibrous dysplasia of the temporal bone: A review of 66 cases

Fibrous dysplasia is a condition of nonmalignant osseous change and may occur in a monostotic or polyostotic pattern, the latter potentially being associated with McCune‐Albright syndrome. Symptoms are highly variable and dependent upon lesion location and size.

Clinical characteristics associated with isolated unilateral utricular dysfunction.

PURPOSE The ocular vestibular evoked myogenic potential (oVEMP) represents the sound-induced activation of extraocular muscles and is believed to originate from the utricle and superior vestibular nerve. Isolated unilateral oVEMP abnormalities with otherwise normal balance function test (BFT) results have not yet been characterized in a large patient series, and their clinical significance remains unclear. MATERIALS AND METHODS Retrospective review of adult patients with vestibular complaints at a tertiary academic neurotologic referral center was performed. Patients with isolated unilateral oVEMP abnormalities were identified. The prevalence of vestibular symptoms and results of the Dizziness Handicap Inventory (DHI) and Hospital Anxiety and Depression Scale (HADS) were compared between these patients and those with normal BFT results. RESULTS Thirty-one adult patients with isolated unilateral oVEMP abnormalities were identified (71% female, mean age 48 ± 14 years). Presenting complaints included vertigo in 53%, non-vertiginous dizziness in 68%, postural instability in 52%, and swaying/rocking sensation in 13%. Significant differences were observed in the percentage of patients with postural instability (p=0.046) and swaying/rocking sensation (p=0.04) when comparing the abnormal oVEMP group to patients with a normal BFT battery. No differences were observed when comparing other symptoms, age, gender, diagnoses, and DHI/HADS scores between groups. CONCLUSION This is the largest series to date reporting on patients with isolated unilateral oVEMP abnormalities. Our results suggest this population may demonstrate an increased prevalence of postural instability and swaying/rocking sensation. Other measures of postural stability may further characterize the vestibular impairments associated with isolated unilateral utricular dysfunction.

Adenomatous Tumors of the Middle Ear

Adenomatous tumors are an uncommon cause of a middle ear mass. Clinical findings may be nonspecific, leading to difficulties in differentiation from other middle ear tumors. Controversy also exists whether to classify middle ear adenoma and carcinoid as separate neoplasms, or alternatively within a spectrum of the same pathologic entity. Most adenomatous middle ear tumors are indolent in behavior, with a benign histologic appearance and slowly progressive growth. The mainstay of treatment is complete surgical resection, which affords the greatest likelihood of cure.

Analysis of intersubject variations in intracochlear and middle ear surface anatomy for cochlear implantation.

Hypothesis We hypothesize that surface landmarks surrounding the round window typically used to guide electrode placement during cochlear implantation (CI) exhibit substantial variability with respect to intracochlear anatomy. Background Recent publications suggest that both atraumatic electrode insertion and electrode location within the scala tympani can affect auditory performance after CI. However, current techniques for electrode insertion rely on surface landmarks alone for navigation, without actual visualization of intracochlear structures other than what can be seen through a surgically created cochleostomy. In this study, we quantify how well the position of intracochlear anatomy is predicted by surface landmarks surrounding the round window. Methods Structures representing middle ear surface and intracochlear anatomy were reconstructed in &mgr;CT scans of 10 temporal bone specimens. These structures were then reoriented into a normalized coordinate system to facilitate measurement of inter-subject anatomical shape variations. Results Only minor intersubject variations were detected for intracochlear anatomy (maximum deviation, 0.71 mm; standard deviation, 0.21 mm), with greatest differences existing near the hook and apex. Larger intersubject variations in intracochlear structures were detected when considered relative to surface landmarks surrounding the round window (maximum deviation, 0.83 mm; standard deviation, 0.54 mm). Conclusion The cochlea and its scala exhibit considerable variability in relation to middle ear surface landmarks. While support for more precise, atraumatic CI electrode insertion techniques is growing in the otologic community, landmark guided insertion techniques have limited precision. Refining the CI insertion process may require the development of image-guidance systems for use in otologic surgery.

Factors Associated With Recurrence of Squamous Cell Carcinoma Involving the Temporal Bone

Objective: This study aimed to better identify factors associated with recurrence of squamous cell carcinoma (SCC) involving the temporal bone. Methods: A retrospective study was conducted at a tertiary hospital. Sixty patients who were diagnosed over a 10-year period with SCC involving the temporal bone and underwent surgical resection were analyzed. All patients were staged based on the University of Pittsburgh staging system. Demographic, intraoperative, and pathologic data were analyzed with respect to recurrence. Results: Thirteen (21.7%) patients were T1, 8 (13.3%) T2, 7 (11.7%) T3, and 32 (53.3%) T4. Eighteen patients (30.0%) recurred in the study period. The mean time to recurrence was 5.8 months. Tumors originating in the skin overlying the parotid gland and the external auditory canal had higher recurrence rates than those from the auricle/postauricular skin and temporal bone (P = .05). Direct parotid and perineural spread accounted for 15.0% of all routes of temporal invasion but resulted in 22.2% of all recurrences (P = .04). Increased N stage was statistically associated with increased risk of recurrence (P = .01). Cervical, as compared to perifacial and parotid, lymph node involvement was associated with increased risk of recurrence (odds ratio = 6.91; 95% confidence interval, 1.11–42.87). Conclusion: We have identified multiple factors that are associated with increased recurrence of SCC involving the temporal bone.

Evolving considerations in the surgical management of cholesteatoma in the only hearing ear.

Objective To describe a contemporary, pragmatic approach to managing cholesteatoma in the only hearing ear. Study Design Retrospective case series. Setting Single tertiary referral center. Patients All patients that underwent cholesteatoma surgery, having profound hearing loss in the contralateral ear. Intervention(s) Cholesteatoma surgery. Main Outcome Measure(s) Surgical strategy, preoperative and postoperative audiometric outcomes, short- and long-term complications, recidivism. Results Twenty-eight patients met criteria, representing 0.25% of all chronic ear surgeries performed between 1970 and 2012. Patients undergoing surgery in the latter half of the study underwent intact canal wall procedures and ossicular chain reconstruction more frequently despite having similar severities of disease. All patients with inner ear fistula underwent an open-cavity operation. In the early postoperative period, 86% of ears had stable or improved hearing levels, and all patients maintained preoperative bone conduction thresholds. At a mean follow-up of 48 months, 79% of patients maintained stable or improved pure tone thresholds, whereas 2 subjects experienced delayed sensorineural hearing loss and 2 experienced isolated declining speech discrimination. Notably, 3 of the latter 4 patients were diagnosed with labyrinthine fistula and had undergone radical mastoidectomy. None of the patients who received an intact canal wall tympanomastoidectomy experienced worsening bone conduction thresholds, whereas 1 subject demonstrated a delayed decline in speech discrimination and another recurred. Conclusion It is commonly held that the radical or classic modified radical mastoidectomy is the procedure of choice when managing cholesteatoma in the only hearing ear while intact canal wall techniques are contraindicated. Over the last 20 years, we have adopted a less-rigid, functional approach favoring intact canal wall procedures in the absence of inner ear fistula rather than unequivocally committing to an open cavity. This strategy has been influenced by advancements in preoperative evaluation, increasing familiarity and refinement of closed-cavity techniques, postoperative imaging surveillance options, and the potential for cochlear implant “salvage” in the rare case of profound hearing loss. Based on the current series, this approach appears safe when performed by an experienced surgeon, and reliable long-term patient follow-up is maintained.

Outcomes comparing primary pediatric stapedectomy for congenital stapes footplate fixation and juvenile otosclerosis.

Objective To compare presentation, operative findings, and outcomes among pediatric patients undergoing primary stapedectomy for congenital stapes footplate fixation (CSFF) and juvenile otosclerosis (JO). Study Design Retrospective review. Setting Combined experience from 2 tertiary academic referral centers. Patients Pediatric patients with CSFF and JO. Intervention Primary stapedectomy. Main Outcome Measure(s) 1) Preoperative and postoperative audiometric data using the 1995 AAO-HNS reporting guidelines; 2) Notable operative findings, and postoperative complications. Results Forty-four pediatric ears met inclusion criteria (27 CSFF, 17 JO). Patients with CSFF presented with a more significant hearing loss (mean PTA 52 dB versus 42 dB; p = 0.04), underwent surgery at a younger age (12.2 versus 16.3 yr; p < 0.001), and more commonly had coincident ossicular malformations (37% versus 0%; p = 0.004). Subjects with JO demonstrated a smaller postoperative ABG (mean 8.8 dB versus 17.2 dB; p = 0.04), although both groups experienced a statistically significant improvement following surgery. Mean bone conduction thresholds remained stable for both groups. There were no instances of profound sensorineural hearing loss, perilymph gusher, facial nerve paresis, or tympanic membrane perforation. Conclusion When performed by an experienced surgeon, stapedectomy is safe and effective in managing carefully selected pediatric patients with CSFF and JO. CSFF is associated with a more severe hearing loss at presentation and concurrent ossicular anomalies are common. Both groups experience substantial benefit from stapedectomy, although ABG closure rates are superior in patients with JO. These data may be helpful in preoperative assessment and patient counseling.

Implantable Hearing Devices: The Ototronix MAXUM System

For many hearing-impaired individuals, the benefits of conventional amplification may be limited by acoustic feedback, occlusion effect, and/or ear discomfort. The MAXUM system and other implantable hearing devices have been developed as an option for patients who derive inadequate assistance from traditional HAs, but who are not yet candidates for cochlear implants. The MAXUM system is based on the SOUNDTEC Direct System technology, which has been shown to provide improved functional gain as well as reduced feedback and occlusion effect compared to hearing aids. This and other implantable hearing devices may have increasing importance as future aural rehabilitation options.

Historical Development of Active Middle Ear Implants

Active middle ear implants (AMEIs) are sophisticated technologies designed to overcome many of the shortcomings of conventional hearing aids, including feedback, distortion, and occlusion effect. Three AMEIs are currently approved by the US Food and Drug Administration for implantation in patients with sensorineural hearing loss. In this article, the history of AMEI technologies is reviewed, individual component development is outlined, past and current implant systems are described, and design and implementation successes and dead ends are highlighted. Past and ongoing challenges facing AMEI development are reviewed.