Thomas H. Alexander

Current Epidemiology of Meniere's Syndrome

The burden of Ménière syndrome (MS) is substantial, especially when considering the significant impact on the quality of life of those affected. Reported estimates of incidence and prevalence have varied widely due to methodological differences between studies, changes in criteria for diagnosis of MS, and differences in populations studied. Reported prevalence rates for MS range from 3.5 per 100,000 to 513 per 100,000. A recent study using health claims data for more than 60 million patients in the United States found prevalence of 190 per 100,000 with a female:male ratio of 1.89:1. The prevalence of MS increases with increasing age.

Incidence of sudden sensorineural hearing loss.

Objective Recent data regarding the incidence of sudden sensorineural hearing loss (SSNHL) in the United States is lacking. The objective of this study was to assess the current day incidence of SSNHL in the United States using data from a medical and pharmaceutical claims database containing information for more than 60 million unique patients. Study Design Population-based cross-sectional analysis. Setting Inpatient and outpatient. Patients Patients in the database are reported to be representative of the national, commercially insured population on a variety of demographic measures including age, sex, health plan type, and geographic location. Results During 2006 and 2007, the annual incidence of SSNHL was 27 per 100,000 in the United States. The incidence increased with increasing age, ranging from 11 per 100,000 for patients younger than 18 years to 77 per 100,000 for patients 65 years and older. There was an overall slight male preponderance with a male-to-female ratio of 1.07:1. This was more pronounced in patients 65 years and older, with a ratio of 1.30:1. Conclusion More than 66,000 new cases of SSNHL are seen annually in the United States. The disorder is more common in men and the elderly.

Intranasal Zinc and Anosmia: The Zinc-Induced Anosmia Syndrome

Objective: Commercial preparations of intranasal zinc gluconate gel are marketed as a remedy for the common cold. However, intranasal zinc has been reported as a cause of anosmia in humans and animals. Seventeen patients presenting with anosmia after the use of intranasal zinc gluconate are described.

Current-day prevalence of Ménière's syndrome.

Previously reported prevalence rates of Ménière’s syndrome (MS) have ranged from 3.5 per 100000 to 513 per 100000. Recent data regarding the prevalence in the United States are lacking. To further assess the current-day prevalence of MS in the US, data from a medical and pharmaceutical claims database containing information for over 60 million unique patients were analyzed. Patients in the database are representative of the national, commercially insured population on a variety of demographic measures including age, gender, health plan type, and geographic location. A 3-year period from 2005 to 2007 was analyzed. The prevalence of MS was 190 per 100000 with a female:male ratio of 1.89:1. The prevalence increased with increasing age, ranging from 9 per 100000 for patients under age 18 to 440 per 100000 for patients 65 years and older.

Safety of high-dose corticosteroids for the treatment of autoimmune inner ear disease.

Objective: To report the adverse effects associated with prolonged high-dose prednisone for the treatment of autoimmune inner ear disease (AIED). Study Design: Prospective data collected as part of a multicenter, randomized, controlled trial for the treatment of corticosteroid-responsive AIED with methotrexate. Setting: Tertiary referral centers. Patients: One hundred sixteen patients with rapidly progressive, bilateral sensorineural hearing loss. Intervention: All patients completed a 1-month course of prednisone (60 mg/d). In Phase 2, 67 patients with improvement in hearing underwent a monitored 18-week prednisone taper, resulting in 22 weeks of prednisone therapy at an average dose of 30 mg per day. Thirty-three patients were randomized to receive methotrexate in Phase 2. Thirty-four patients received prednisone and placebo. Main Outcome Measure: Adverse events (AE) in patients treated with prednisone only. Results: Of 116 patients, 7 had to stop prednisone therapy during the 1-month challenge phase due to AE. Of 34 patients, 5 were unable to complete the full 22-week course of prednisone due to AE. The most common AE was hyperglycemia, which occurred in 17.6% of patients participating in Phase 2. Weight gain was also common, with a mean increase in body mass index of 1.6 kg/m2 (95% confidence interval, 0.77-2.3) during the 22-week steroid course. Patients entering Phase 2 were followed for a mean of 66 weeks. No fractures or osteonecrosis were reported. Conclusion: Although high-dose corticosteroids are associated with known serious side effects, prospective data in the literature are limited. The present study suggests that with appropriate patient selection, monitoring, and patient education, high-dose corticosteroids are a safe and effective treatment of AIED.

Vestibular Schwannoma Quantitative Polymerase Chain Reaction Expression of Estrogen and Progesterone Receptors

Objectives/Hypothesis: Determine the role of estrogen receptor (ER) and progesterone receptor (PR) expression in sporadic and neurofibromatosis 2 (NF2)‐related vestibular schwannomas (VS). Growth and proliferation signaling in human VS tumorigenesis may play a key role in molecular therapeutic targeting. VS carry mutations of the NF2 gene encoding the tumor suppressor, merlin, which interacts with ErbB2 in Schwann cells, implicating ErbB receptors in VS tumorigenesis. ErbB receptor family members are overexpressed or constitutively activated in many human tumors, and are effective therapeutic targets in some human cancers. VS occur more frequently in women and are larger, more vascular, and demonstrate increased growth rates during pregnancy. ER and PR may play a role in ErbB pathway activation and VS progression.

Dose Effect of Intratympanic Dexamethasone for Idiopathic Sudden Sensorineural Hearing Loss: 24 mg/mL Is Superior to 10 mg/mL.

Objective To compare outcomes in patients with idiopathic sudden sensorineural hearing loss (ISSNHL) treated with intratympanic (IT) dexamethasone (DEX) at either 10 mg/mL or 24 mg/mL. Study Design Retrospective case series. Setting Tertiary referral center. Patients Thirty-seven adults with ISSNHL. Interventions In addition to concurrent prednisone taper, patients received a series of IT DEX injections for 2 weeks with either 10 mg/mL or 24 mg/mL. Main Outcome Measure Greater than 30-dB improvement in pure-tone average (PTA). Results Baseline characteristics were similar between groups. Mean follow-up was 10 weeks. Ten (53%) of 19 patients treated with 24 mg/mL had greater than 30-dB improvement in PTA compared with 3 (17%) of 18 treated with 10 mg/mL (p = 0.0382, Fisher’s exact test). There was a trend toward improved word recognition score outcome with 24 mg/mL. The interval between onset and initiation of IT DEX significantly affected outcome, with earlier treatment resulting in greater improvement in PTA and word recognition score. Multivariate logistic regression confirmed that IT DEX dose and interval to starting treatment were both independent predictors of PTA outcome. Change in PTA was not significantly affected by age, sex, pretreatment hearing levels, or concurrent treatment with hyperbaric oxygen. Conclusion To our knowledge, this is the first demonstration of superiority of IT DEX at 24 mg/mL for the treatment of ISSNHL, with significantly better recovery of PTA. Our data suggest that treatment should be initiated as soon as possible. A prospective randomized trial to confirm the optimal dose is warranted.

Laterality of exostosis in surfers due to evaporative cooling effect.

Objectives:To correlate exostosis severity with ear canal evaporative cooling.To assess hearing and complications after canalplasty. Study Design: Retrospective chart review. Subjects and Method: A retrospective chart review from 1990 to 2007 at a university tertiary referral center. Results: Surfers from the west coast of the United States were twice as likely to have severe exostoses in the right ear compared with the left. Evaporative cooling from a predominant northerly wind direction during the coldest water temperature months in this region may contribute to this lateral bias because surfers on this coast spend most of their time facing west. Few postoperative complications were identified. No cases of facial nerve injury or entry into the temporomandibular joint occurred. Differences in preoperative versus postoperative pure-tone hearing thresholds were not significant. Conclusion: Exostosis severity seems to correspond to the ear that is more exposed to the predominant coastal wind. We propose that evaporative cooling in a cold water environment contributes to greater progression of exostoses in the ear with more exposure to the predominant wind. Exostosis removal using the postauricular approach carries a low complication rate.

Hearing preservation and vestibular schwannoma: intracochlear FLAIR signal relates to hearing level.

Objectives This study aimed to evaluate the relationship between cochlear signal on fluid-attenuated inversion recovery (FLAIR) magnetic resonance imaging (MRI) sequences and hearing in patients undergoing hearing preservation surgery for vestibular schwannoma (VS) and to demonstrate a new classification system to be used in imaging evaluation of patients with VS. Methods A search of archived surgical cases at a single institution between January 1, 2006, and January 1, 2012, revealed 51 patients who underwent hearing preservation surgery for VS. Tumor size, patient age and sex, and preoperative and postoperative pure-tone average (PTA) and speech discrimination score (SDS) were recorded. Cochleae on the affected side were examined on preoperative FLAIR sequences and classified as limited hyperintensity (LH) or extensive hyperintensity (EH). Results Mean patient age was 51 years, and mean tumor size was 1.3 cm. Preoperative FLAIR sequences were classified into LH (n = 36) and EH (n = 15) categories. Preoperative PTA and SDS were 29.5 dB (SD, 16.7), 90% (SD, 14) and 40.6 dB (SD, 13.8), 80% (SD, 25) for LH and EH, respectively. On univariate analysis, preoperative PTA was superior in the LH group (p = 0.04). There was a trend toward superior preoperative SDS and postoperative PTA in the LH group, but these differences were not statistically significant (p = 0.08 and p = 0.06, respectively). Conclusion The current study is the first to demonstrate a distinct association between cochlear FLAIR signal and pretreatment hearing levels in patients with VS. A new classification system for evaluating cochlear FLAIR signal is proposed. Improvement in FLAIR sequences will allow further investigation of this association.

Primary treatment of idiopathic sudden sensorineural hearing loss with intratympanic dexamethasone.

Purpose of reviewSystemic corticosteroids have been the mainstay of treatment of idiopathic sudden sensorineural hearing loss (ISSNHL) for over 30 years. Recently, intratympanic steroids have risen in popularity; however, there is no consensus regarding the details of their application. The purpose of this article is to review the current literature and share our opinion that intratympanic dexamethasone (alone or with systemic steroids) should be offered as primary treatment of ISSNHL. Recent findingsIntratympanic steroids have been shown to result in higher inner ear concentrations than systemic steroids in animal and human studies. Primary treatment of ISSNHL with intratympanic steroids in combination with systemic steroids has been demonstrated to result in better hearing outcomes than systemic steroids alone. Recent evidence has revealed these hearing outcomes to be dependent on the dose of intratympanic steroid. Dexamethasone may be better absorbed than methylprednisolone, and potentially offer a greater benefit. Furthermore, intratympanic steroids provide an effective alternative for patients who cannot tolerate systemic steroids. SummaryIntratympanic dexamethasone alone, or in combination with systemic steroids, provides effective treatment of ISSNHL and should be utilized as primary treatment. Further investigation is required to determine the optimal choice, concentration, and administration schedule of intratympanic steroids.