Rachel E. Roditi

A Predictive Model of Cochlear Implant Performance in Postlingually Deafened Adults

Objective: To develop a predictive model of cochlear implant (CI) performance in postlingually deafened adults that includes contemporary speech perception testing and the hearing history of both ears. Study Design: Retrospective clinical study. Multivariate predictors of speech perception after CI surgery included duration of any degree of hearing loss (HL), duration of severe-to-profound HL, age at implantation, and preoperative Hearing in Noise Test (HINT) sentences in quiet and HINT sentences in noise scores. Consonant-nucleus-consonant (CNC) scores served as the dependent variable. To develop the model, we performed a stepwise multiple regression analysis. Setting: Tertiary referral center. Patients: Adult patients with postlingual severe-to-profound HL who received a multichannel CI. Mean follow-up was 28 months. Fifty-five patients were included in the initial bivariate analysis. Intervention(s): Multichannel cochlear implantation. Main Outcome Measures(s): Predicted and measured postoperative CNC scores. Results: The regression analysis resulted in a model that accounted for 60% of the variance in postoperative CNC scores. The formula is predCNC score = 76.05 + (−0.08 × DurHLCI ear) + (0.38 × pre-HINT sentences in quiet) + (0.04 × long sev-prof HLeither ear). Duration of HL was in months. The mean difference between predicted and measured postoperative CNC scores was 1.7 percentage points (SD, 16.3). Conclusion: The University of Massachusetts CI formula uses HINT sentence scores and the hearing history of both ears to predict the variance in postoperative monosyllabic word scores. This model compares favorably with previous studies that relied on Central Institute for the Deaf sentence scores and uses patient data collected by most centers in the United States.

Cervical vestibular evoked myogenic potentials (cVEMPs) in patients with superior canal dehiscence syndrome (SCDS)

Objective: To determine the usefulness of both amplitude and threshold data from tone-burst cervical vestibular evoked myogenic potential (cVEMP) testing for the evaluation of superior canal dehiscence syndrome (SCDS). Study Design: Case series with chart review. Subjects and Methods: Sixty-seven patients underwent cVEMP testing. We correlated mean tone burst cVEMP amplitude and threshold data with temporal bone CT findings. Patients were excluded for Ménières disease, middle ear disease, or otologic surgery. Results: Superior canal dehiscence patients had higher mean cVEMP amplitudes (SCDS 173.8 μV vs non-SCDS 69.7 μV, P = 0.031) and lower mean thresholds (SCDS 72.8 dB nHL vs non-SCDS 80.9 dB nHL) at 500 Hz. Conclusion: Patients with SCDS have larger amplitudes and lower thresholds on cVEMP testing at 500 Hz. This study supports the utility of tone burst cVEMPs for the evaluation of SCDS and is one of few large single-center studies to establish normative data.

Age: An effect modifier of the association between allergic rhinitis and Otitis media with effusion

1) To determine whether there is a significant relationship between allergic rhinitis and otitis media with effusion (OME), Eustachian tube dysfunction (ETD), or tympanic membrane retraction (TMR) in children in a nationally representative population; and 2) to determine whether age is an effect modifier of any such association because this hypothesis has yet to be tested.

Otitis Media with Effusion: Our National Practice:

Otitis media with effusion (OME) is the focus of an updated multidisciplinary clinical practice guideline published by the American Academy of Otolaryngology—Head and Neck Surgery Foundation (AAO-HNSF) and the American Academy of Pediatrics (AAP). Based on data from clinical trials, the guideline recommends against using antihistamines, antibiotics, oral steroids, and intranasal steroids for OME. To understand practice patterns related to these guidelines, we assessed nationally representative data. Despite controlling for age, sex, race/ethnicity, and other potential confounders individualized for each medication class, an increased risk of antihistamine (odds ratio [OR], 3.53), antibiotic (OR, 4.31), and intranasal steroid administration (OR, 3.58) was seen when OME was diagnosed. These analyses have demonstrated opportunities for quality improvement in the care of patients with OME, quantifying gaps in practice relevant to proposed quality measures. Education targeted according to practice setting may facilitate appropriate therapy and/or referral for definitive intervention in children with OME.

Oral Antibiotic Use for Otitis Media with Effusion Ongoing Opportunities for Quality Improvement

Objectives (1) To evaluate the probability of antibiotic administration associated with ICD-9 diagnosis of otitis media with effusion (OME) in the absence of acute otitis media, (2) to determine whether usage varies according to visit setting, and (3) to ascertain if practice gaps are such that future practice changes might be measured. Study Design Cross-sectional analysis of an administrative database. Setting Ambulatory visits in the United States. Subjects and Methods National Ambulatory and Hospital Ambulatory Medical Care Surveys, 2005-2010; univariate, multivariate, and stratified analyses of antibiotic usage were performed. The study population was restricted to children without acute or unspecified otitis media. The primary outcome was the probability of oral antibiotic administration when OME was diagnosed. The impact of the location of service and subspecialty care was also analyzed. Results Data from 1,390,404,196 pediatric visits demonstrated that oral antibiotics were administered for 32% of visits with an OME diagnosis, even in the absence of acute otitis media (odds ratio, 4.31; 95% confidence interval: 2.88-6.44; P < .001). The highest antibiotic administration was seen in the emergency department (risk difference, 37.1%; number needed to harm, 3). No significant increased risk of antibiotic usage was seen during otolaryngology visits. Diagnoses of infections at nonotologic sites were associated with a 1.98 to 26.60 increase in odds of oral antibiotic administration. Conclusion Oral antibiotics continue to be administered in children with OME in the absence of acute infection, with risk varying by location of service. There is a potential opportunity for quality improvement through reducing antibiotic administration for pediatric OME.

Imaging case of the month: Bilateral internal auditory canal melanoma.

Neurofibromatosis type 2 (NF2) is a rare, autosomal dominant disorder resulting from mutation of the NF2 tumor suppressor gene on chromosome 22. A mutated form of the gene product, merlin, leads to a predisposition for tumors in the central and peripheral nervous system. Though many sets of diagnostic criteria exist, all agree that the finding of bilateral vestibular schwannomas confirms the diagnosis. Patients may present with tinnitus, imbalance, hearing loss or facial weakness. However, many other lesions can affect the cerebellopontine angle (CPA) including, meningiomas, epidermoid cysts, ependymomas, melanomas and metastatic lesions. This case report outlines the importance of considering the differential diagnosis in patients with bilateral CPA lesions. A 70-year-old male with long standing bilateral hearing loss and a history of cholesteatoma presented with worsened right-sided hearing. Audiometry revealed a profound right sensorineural hearing loss (SNHL) and high frequency left SNHL. Word recognition scores were 0% in the right ear and 72% in the left. Imbalance with head rotation, urinary incontinence, memory deficits and recent falls were also present. He had a history of a skin neurofibroma, but no malignancies. There was no family history of hearing loss, neurofibromatosis or cancer. Otoscopic examination and facial nerve function were normal. The head thrust test was positive with rightward rotation, but normal with rotation to the left. Magnetic resonance imaging (MRI) revealed bilateral enhancing lesions at the CPA and internal auditory canal (IAC) (Fig. 1A). The compilation of findings led to a presumptive diagnosis of NF2 with bilateral vestibular schwannomas and associated normal pressure hydrocephalus. A plan for ventriculoperitoneal (VP) shunt placement and repeat MRI to evaluate tumor growth rate was initiated. Figure 1 Panel A: T1-weighted axial MRI with gadolinium showing a 15 × 10 mm enhancing lesion in the right cerebellopontine angle and a similar but smaller (8.7 × 5.4 mm) lesion in the left CPA (arrows). Panel B: CT scan done one month after the ... Shortly after the presumed diagnosis, the patient presented with increasing confusion, unsteady shuffling gait and urinary incontinence. Imaging showed worsened ventriculomegaly with intraventricular hemorrhage, acute subdural hematoma, interval increase in right CPA lesion, an enhancing lesion of the right cerebellar hemisphere and chronic subdural hygroma (Fig. 1B). A VP shunt was placed which resolved his urinary incontinence but not the mental deficits or ataxia. Serial CT scans revealed extension of the hemorrhages, increasing edema, and mass effect. The patient died shortly afterwards from herniation due to massive acute subarachnoid hemorrhage. At autopsy, foci of metastatic melanoma were identified at the CPA and left corpus callosum. Tumor cells expressed immunohistochemical markers for vimentin, S-100 and HMB45, and were negative for pankeratin cocktail, cytokeratin 7 and cytokeratin 20 (Fig. 2). No primary site of malignancy was identified. Figure 2 Panel A: H & E stained section of tumor at the cerebellopontine angle showed pleomorphic cells with mitotic activity (arrow) but lacking cytoplasmic pigmentation, consistent with poorly differentiated neoplasm and confirmed as melanoma by diffuse ... Malignant melanoma is increasing in incidence. It comprises 1–8% of malignancies and is the third most common tumor to metastasize intracranially(1). Despite this, most metastases are to the gray matter, and CPA metastases are rare, especially without evidence of disseminated metastases. On MRI, melanoma is typically hyperintense on T1 and hypointense on T2. Amelanotic lesions, like this one, are often hypointense or isointense on T1 and hyperintense on T2, mimicking neuromas. Given their high vascularity, gadolinium enhancement is expected. These findings make it difficult to differentiate melanoma from typical benign CPA lesions on the basis of imaging alone. History of prior skin lesions, age at diagnosis and family history are crucial to proper diagnosis, and a high index of suspicion is necessary. Brackmann et al report on 8 subjects with melanoma of the CPA(2), three of which were previously reported(3). In this series, 6/8 (75%) had a history of cutaneous melanoma and one had a primary CPA tumor. Four subjects had bilateral lesions. Jacob et al. report a case of bilateral CPA melanoma in a patient with a history of cutaneous melanoma 8 years prior(4). Subarachnoid hemorrhage is relatively common with melanoma metastatic to the brain(5), and the new lesion near the corpus callosum suggested metastatic disease. The patients age at presentation and the negative family history of neurofibromatosis are further clues that, in hindsight, made the diagnosis of NF2 less likely. The current case is very unusual in that bilateral CPA metastatic melanoma was found without a prior melanoma history and without evidence of a primary lesion on autopsy. With increasing incidence of melanoma in society and better access to MRI, it is likely that we will also see an increased incidence in isolated CPA melanoma. Resection has proven successful in some cases; with several patients experiencing palliation of their CNS symptoms, and others, with primary lesions, experiencing a survival benefit(2). This identifies the importance of considering a full differential of CPA lesions when patients with bilateral lesions are identified. Melanoma should be considered with presentation at advanced age, rapid tumor growth, and subarachnoid hemorrhage.

Medications for Allergic Rhinitis An Opportunity for Quality Improvement

Objectives Adherence to the allergic rhinitis clinical practice guideline is being considered as a potential focus for national performance metrics. To help inform this discussion, we assessed patient- and clinician-reported medication administration among nationally representative populations of patients with allergic rhinitis. Study Design Cross-sectional analyses. Setting and Subjects Home health assessments, ambulatory visits. Methods Participants in the National Health and Nutrition Examination Survey and the National Ambulatory Medical Care Survey / National Hospital Ambulatory Medical Care Survey were assessed. The primary outcomes were the percentage of patients reporting receipt of antihistamines and/or nasal steroids among those with allergy-related symptoms and the percentage for whom a clinician administered these medications when diagnosing allergic rhinitis. Secondary outcomes included assessments of those with worse quality of life, confirmatory allergy testing, and leukotriene receptor antagonist use. Results Within the National Health and Nutrition Examination Survey, an estimated 29.2 million patients were diagnosed with “hay fever,” while 92.2 million were diagnosed with “allergies.” Patients with symptoms of allergic rhinitis reported that antihistamines or nasal steroids were prescribed in 21.1% to 24.0% of cases. Leukotriene receptor antagonists were given to 1.7% of those without asthma or use of other allergy medications. Within the National Ambulatory Medical Care Survey / National Hospital Ambulatory Medical Care Survey, observations representing 149.5 million visits for allergic rhinitis demonstrated that nasal steroids were administered in 29.6% of cases, while nonsedating and sedating antihistamines were given in 22.4% and 17.2%, respectively. Conclusions Despite a high prevalence of allergic rhinitis, per patient report and clinician entry, a substantial number of affected patients do not receive antihistamines and nasal steroids.

The Influence of Age on the Relationship Between Allergic Rhinitis and Otitis Media

Purpose of ReviewTo examine the relationship between otitis media, allergic rhinitis, and age.Recent FindingsOtitis media and allergic rhinitis are prevalent conditions with a controversial relationship. Some data suggest that these entities are significantly associated, either through allergic rhinitis inducing Eustachian tube dysfunction or through allergic pathophysiology simultaneously occurring intranasally and in the ear. Other studies, however, have refuted this relationship. For example, treatment with antihistamines does not reliably improve OME, making causation and association challenging to establish. Age may have an effect on the nature of the relationship between allergic rhinitis and otitis media, by impacting both the individual conditions and their association. Epidemiological, immunological, and adenoidal studies have suggested that differences occur with age, and this review encapsulates the related data and publications. We begin by evaluating how allergic rhinitis and otitis media each are affected by age, then evaluate the role that age may have in the relationship between the two conditions. Adult and pediatric literature are evaluated so as to include the full impact of age across patients’ lifespan.SummaryAge induces changes in immunity, patterns of inflammation, and susceptibility to both allergic rhinitis and otitis media with effusion. Age may also be an effect modifier which impacts the nature of the relationship between these two conditions. The influence of age on the association between these highly prevalent conditions remains a topic of active study.

Grade repetition and parents' perception of hearing loss: An analysis of data from children in the United States.

To determine whether parent‐perceived hearing problems are associated with grade repetition among children in the United States.

Epidermoid cyst of the zygomatic root.

Intracranial cysts are common findings on both computed tomography (CT) and magnetic resonance imaging (MRI) and represent a wide range of diagnoses. A cyst can be defined as a spherical growth with an epithelial lining, forming a cavity that is filled with fluid, cellular products, or debris. Histo