Robert C. O’Reilly

Posterior fossa arachnoid cysts can mimic Meniere’s disease

Arachnoid cysts constitute 1% of all intracranial space-occupying lesions. In the posterior fossa, they typically produce vague, nonspecific symptoms. However, a subset of these lesions can produce signs and symptoms indistinguishable from those of Menieres disease. We discuss the clinical and laboratory features of 2 cases of posterior fossa arachnoid cysts mimicking Menieres disease as well as the substantial resolution of symptoms in 1 patient after cysto-peritoneal shunt. Posterior fossa arachnoid cyst must be considered in the differential diagnosis of patients presenting with signs and symptoms of Menieres disease.

The Role of Gastric Pepsin in the Inflammatory Cascade of Pediatric Otitis Media

IMPORTANCE Otitis media is characterized as an ongoing inflammation with accumulation of an effusion in the middle ear cleft. The molecular mechanisms underlying the pathogenesis, particularly the inflammatory response, remain largely unknown. We hypothesize that aspiration of gastric contents into the nasopharynx may be responsible for the initiation of the inflammatory process or aggravate a preexisting condition. OBJECTIVE To investigate the correlation of gastric pepsin A with inflammatory cytokines, bacterial infection, and clinical outcomes. DESIGN, SETTING, AND PARTICIPANTS Prospective study of 129 pediatric patients undergoing myringotomy with tube placement for otitis media at a tertiary care pediatric hospital. MAIN OUTCOMES AND MEASURES Ear samples were tested for pepsin A; cytokines interleukin (IL)-6, IL-8, and tumor necrosis factor; and bacterial culture inoculation. Data were analyzed by descriptive statistics and regression analysis to identify risk factors for the presence of pepsin A and to correlate pepsin A levels with cytokine levels, infection status, and clinical outcomes. RESULTS Of the 129 patients, 199 ear samples were obtained; 82 samples (41%) and 64 patients (50%) were positive for pepsin A as measured by immunoassay. Pepsin A positivity correlated with age younger than 3.0 years (mean [SD], 2.3 [2.1] years in the positive group vs 3.3 [3.0] years in the negative group) and with all 3 cytokine levels (mean [SD] tumor necrosis factor, 29.5 [45.9] pg/mL in the positive group vs 13.2 [21.6] pg/mL in the negative group; IL-6, 6791.7 [9389.1] pg/mL in the positive group vs 2849.9 [4066.3] pg/mL in the negative group; and IL-8, 6828.2 [8122.3] pg/mL in the positive group vs 2925.1 [3364.5] pg/mL in the negative group [all P < .05]); however, logistic regression analysis showed that only IL-8 (odds ratio, 3.96; 95% CI, 1.3-12.0; P = .02) and age (odds ratio, 3.83; 95% CI, 1.2-12.7; P = .03) were significant independent variables. No statistically significant association was found with other parameters. Multiple linear regressions revealed that the levels of pepsin A were correlated with IL-8 levels (R2 = 0.248; P < .001) and the need for second or third tubes 6 to 12 months after the first (R2 = 0.102; P = .006). The presence of pepsin A in the middle ear was not associated with increased bacterial infection. Interleukin 8 was independent and significantly associated with both pepsin A levels and bacterial infection (R2 = 0.144 and 0.263, respectively; P = .001 for both). CONCLUSIONS AND RELEVANCE Extraesophageal reflux as indicated by the presence of pepsin A is closely involved in the middle ear inflammatory process and may worsen the disease in some children; however, a proof of cause and effect between extraesophageal reflux and middle ear inflammation requires further investigation.

Hearing loss in children with mitochondrial disorders.

OBJECTIVE At least 1-5 children per 1000 suffer from congenital hearing loss, and 50% of these cases can be attributed to genetic causes. It has been estimated that 1% of pre-lingual hearing loss is due to mutations in mitochondrial DNA. Previous literature reports audiometric data for few patients, usually less than 20 per study. The goal of this study was to characterize the hearing loss associated with mitochondrial mutations and determine whether previously characterized patterns of hearing loss in these patients (progressive, sensorineural, high frequency losses) are found in our population as well. METHODS An IRB-approved retrospective chart review of the electronic medical records in the Nemours/Alfred I. dupont Hospital for Children system from January 2004 to October 2009 (a five-year period) was undertaken using ICD-9 codes 277.87 (mitochondrial disorder) and 359.89BA (mitochondrial myopathy). These 149 records were then evaluated for audiologic data, resulting in 26 charts with both a mitochondrial disorder and hearing evaluation. RESULTS Of 26 patients with known mitochondrial disorders and audiometric documentation, 15 (58%) had hearing loss, and 11 patients had normal hearing (42%). Ten patients had sensorineural hearing loss (38%), two patients had conductive hearing loss (7.7%), one patient had a mixed hearing loss (3.8%), and two patients had an as yet undefined hearing loss (ABR had not yet been performed at the time of this study) (7.7%). CONCLUSION In comparison with previous studies, generally including less than 20 patients, this is one of the largest collections of audiometric data on children with mitochondrial disorders. Unlike prior studies describing a progressive, sensorineural loss across all frequencies or mainly affecting high frequencies, the hearing loss in our patients was more variable including low frequency losses, mid-frequency losses, and conductive losses and was often not progressive or even improved. Our overall 38% rate of sensorineural hearing loss correlates well with previous case series; this study clearly justifies the use of routine audiometric screening in children with mitochondrial disorders, including use of ABR and OAEs as ASND can be seen in this population, as well as repeat testing over time to evaluate for progression.

Complementary and Integrative Treatments: Otitis Media

Acute otitis media is diagnosed frequently in childhood with peak incidence between 6 and 15 months of age. It is the most common reason for physician visits and antibiotic prescriptions in children, with an excess of

Tongue entrapment in aluminum water bottle: Discussion of removal and airway management

100 spent per episode. In this article we discuss current concepts of complementary and alternative medicine as they apply to otitis media. A thorough literature review shows that, whereas many of these modalities are promising, few have been assessed with randomized controlled trials. Finally, we provide an algorithm that may be used in assessing and treating patients with acute otitis media.

Clinical characteristics of pediatric granular myringitis.

Tongue entrapment in a bottle neck is rare, with few cases reported in the literature. Reported here is the case of a child who presented to the Emergency Department with her tongue entrapped in an aluminum bottle. This case is unique in that the patient had a delayed presentation resulting in significant tongue edema, requiring advanced methods for removal and airway management. These include sedated mechanical removal of the strangulating object and transnasal fiberoptic intubation. We review published cases and the options for removal of an entrapped tongue from a bottle neck, and we discuss the importance of airway management.

Auditory nerve function following cochleitis

Objective To determine the clinical characteristics of children with granular myringitis. Study Design Case series with chart review of children with granular myringitis. Setting Tertiary care children’s hospitals in Delaware and Florida. Subjects and Methods From July 1, 2006, to June 30, 2011, 15 patients were identified with granular myringitis based on International Classification of Diseases, Ninth Revision code 384.1 (10 male, 5 female; 10 left-sided, 5 right-sided). Results Average age at onset was 10 years (range, 4-18 years). Average length of symptoms was 21 months (range, 3-48 months). Thirteen of the 15 patients (87%) had myringotomy tube placement prior to diagnosis of myringitis, and 11 had a previous perforation (73%). Eleven myringoplasties were performed on 8 (53%) patients prior to the onset of granular myringitis (5 fascia, 2 fat, 2 cartilage, 1 paper patch, and 1 at an outside hospital that was unknown). A total of 27 otolaryngologic surgical procedures were done on 14 of 15 patients. Ten patients (67%) had audiometry performed, and 6 (40%) had ear cultures. Medical treatment included ciprofloxacin and dexamethasone otic drops in all patients, oral antibiotics in 4 patients, and additional topical agents in 8 patients. Two patients had laser resurfacing, and 1 patient had surgical curettage. Five patients had long-term remission, 8 had intermittent remission, and 2 had no remission of their symptoms. Conclusion Granular myringitis is a chronic, recalcitrant disease typically preceded by myringotomy and tube placement or myringoplasty/tympanoplasty. Medical and surgical intervention is varied and often does not lead to permanent resolution of the disease.

Management of First Branchial Cleft Anomalies via a Cartilage-Splitting Technique

We present a case of temporary cochlear nerve dysfunction due to endocochlear inflammation with subsequent recovery. Retrospective case review at a pediatric tertiary care hospital. A nine-year-old male presented seven years post-cochlear implantation with an electrode array extruded into the external auditory canal. Upon exploration in the operating room, turbid perilymph from the scala tympani was discovered. A new electrode array was implanted. The patient had no discernible neural response telemetry (NRT) responses or auditory perception immediately following the procedure. Continuous but varying stimulation was continued postoperatively. Two months after implantation, he began having some auditory awareness; subsequent mapping resulted in the activation of a limited number of channels. Nine months following reimplantation, NRT demonstrated responses in three channels. Additionally, functional gain testing revealed sound awareness levels in the mild-hearing-loss range and the patient was able to detect and repeat all six Ling sounds. Continuous cochlear nerve stimulation at various levels may aid in function recovery after endocochlear inflammation.

Build a Better Ear: What’s New in Chronic Ear Disease

First branchial cleft anomalies are uncommon lesions that often present as periauricular infections. They have high recurrence rates, due in part to scarring secondary to prior infections and their management. These lesions have a close relationship with the facial nerve, and most authors recommend its identification and dissection because of this relationship. Nonetheless, facial nerve palsy has been reported in up to 15% of cases. We describe a novel technique for the management of first branchial cleft anomalies. Such lesions that presented in an infra- or postauricular location were approached via an incision through the cartilage of the pinna, between the tragus and antitragus. This technique affords direct access to the lesion without the need for facial nerve dissection. Six patients were treated. Five had prior surgery, including 3 with previous attempts at excision. There were no complications. The median follow-up was 35 months. One patient developed a recurrence.

Atypical presentation of geniculate ganglion venous malformation in a child: Conductive hearing loss without facial weakness

Program Description: There is a growing interest in implementing surgical techniques and using novel materials for tympanic membrane perforation repair. Bioactive materials, cartilage and fascia grafts derived from the recipient patient, novel partial ossicular replacement prostheses and total ossicular replacement prostheses, and novel technologies (ie, otoendoscopy) are in continuous evolution, striving for higher quality and longer duration and stability in otologic surgery grafting. The presenters will provide descriptions and show videos demonstrating the function of these different materials and techniques. In addition, a non-committee panel will present an evidence-based medicine (EBM) algorithm for treatment:”What Literature says—an EBM decision Algorithm as Key to Success.” Educational Objectives: 1) Describe the variety of grafting materials, protheses, and different techniques available for surgical repair of tympanic membrane perforations. 2) Discuss the advantages and limitations of using these technologies and materials. 3) Assess the validity of the studies presented to evaluate the efficacy of these surgical tools.