Complicated Blastomycosis of the Skull Base Presenting as Otitis Media

Blastomyces dermatitidis is a fungus endemic to the Mississippi, Ohio, and St Lawrence River regions of the United States and Canada.1 Although pulmonary infection is its most common clinical manifestation, B dermatitidis can disseminate secondarily to any organ. We describe a case of complicated skull base osteomyelitis caused by B dermatitidis presenting as treatment-refractory otitis media.

Original Article

What If a Stapedectomy Were Not Cost-effective?

The management of otosclerosis is viewed by many as the sine qua non of an otologic practice. From the elegant otopathologic descriptions by Ádám Politzer1 in the 19th century to surgical breakthroughs by Julius Lempert2 and John Shea Jr3 in the mid-20th century, otosclerosis and its surgical management have inspired generations of students, researchers, and surgeons alike. The allure of stapedectomy may be, in part, the elegant tightrope walk to fix hearing loss along with the anticipation of a surgeon report card that arrives in the form of an audiogram. Although the surgical treatment of otosclerosis has not drastically changed since its description by Shea,3 otosclerosis and its management remain fertile ground for research, debate, and refinement. Indeed, novel 21st-century diagnoses—such as superior canal dehiscence syndrome; technological refinements, including otoendoscopy; and even inner-ear drug delivery4—appear to be viewed in connection to otosclerosis.

Original Article

Cost-effectiveness of Stapedectomy vs Hearing Aids in the Treatment of Otosclerosis

Key Points

Question  Is stapedectomy a cost-effective method of treating otosclerosis compared with hearing aids?

Findings  This cost-effectiveness analysis found that, although stapedectomy was associated with increased lifetime costs by $2978.01 compared with hearing aids, stapedectomy had an incremental cost-effectiveness ratio of $3918.43 per quality-adjusted life-year.

Meaning  This model suggests that stapedectomy is a cost-effective option for treating otosclerosis from a patient perspective.

Importance  Otosclerosis can be managed through surgical treatment, such as stapedectomy, or through hearing amplification with hearing aids. To our knowledge, there has been no cost-effectiveness analysis of these 2 treatment methods.

Objective  To determine the cost-effectiveness of stapedectomy vs hearing aid use for the treatment of otosclerosis.

Design and Setting  In this cost-effectiveness analysis, a decision tree was built to model the treatment choices for otosclerosis. The tree was run as a Markov model of a case patient aged 30 years. The model spanned the patient’s lifetime to determine total costs of management of otosclerosis with stapedectomy or hearing aids. Cost-effectiveness was measured using an incremental cost-effectiveness ratio, with a willingness to pay of $50 000 per quality-adjusted life-year (QALY) considered cost-effective. One-way sensitivity analyses were performed for all variables. A 2-way sensitivity analysis was performed for the cost of stapedectomy vs the cost of hearing aids. Probabilistic sensitivity analysis was performed to determine the likelihood that stapedectomy would be cost-effective across a range of model inputs.

Interventions  Stapedectomy vs hearing aid use.

Main Outcomes and Measures  The primary objective of this study was to determine the cost-effectiveness of stapedectomy vs hearing aids in the treatment of otosclerosis. The secondary objectives were to determine which factors are associated with the cost-effectiveness of the interventions.

Results  Stapedectomy had an estimated lifetime cost of $19 417.95, while hearing aids had an average lifetime cost of $16 439.94. Stapedectomy also had a benefit of 16.58 QALYs, and hearing aids had a benefit of 15.82 QALYs. Stapedectomy increases lifetime costs by $2978.01, with a benefit of 0.76 QALYs compared with hearing aids. The incremental cost-effectiveness ratio for stapedectomy is $3918.43 per QALY. The model was sensitive to the cost of stapedectomy and the cost of stapedectomy revision surgery. Probabilistic sensitivity analysis showed that stapedectomy was cost-effective compared with hearing aids 99.98% of the time.

Conclusions and Relevance  Stapedectomy appears to be a cost-effective option for treating otosclerosis compared with hearing aid use, from the patient perspective.

Original Article

Hearing loss weakens skills that young cancer survivors need to master reading

Researchers have identified factors that explain why severe hearing loss sets up pediatric brain tumor survivors for reading difficulties with far-reaching consequences. The findings lay the foundation for developing interventions to help survivors become better readers.

St. Jude Children’s Research Hospital investigators led the international study, which appears today in the Journal of Clinical Oncology.

Researchers analyzed how 260 children and adolescent brain tumor survivors, including 64 with severe hearing loss, performed on skills that are the building blocks of reading. The list included information processing speed, working memory, letter-word identification and phonological skills, which include the ability to use units of sound (phonemes) to decode words.

Compared with other survivors, those with severe hearing loss experience significant declines during treatment on all eight measures included in this analysis. After accounting for the risk factors of age at diagnosis and treatment intensity, the analysis suggested that survivors with severe hearing loss struggled the most with slowed processing speed and phonological skills.

“Reading is a skill that takes a long time to learn and that we depend on for learning our entire life,” said senior and corresponding author Heather Conklin, Ph.D., a member of the St. Jude Department of Psychology. “There had been hints in the scientific literature that reading was declining in pediatric brain tumor survivors and that hearing loss may be a contributor. But this is the first study to identify the key cognitive components that lead to reading problems.”

The findings suggest that interventions should focus on improving neurocognitive and language-based skills like processing speed and phonemics before tackling more complex tasks like reading comprehension, said first author Traci Olivier, Psy.D., formerly a St. Jude postdoctoral fellow and now at Our Lady of the Lake Medical Center, Baton Rouge, Louisiana.

“Younger children, those less than 7 years old, were particularly vulnerable to declines in skills that are fundamental for reading mastery,” she said. “These children may benefit most from interventions.”

Brain tumors and hearing loss

Brain and spinal cord tumors are the second most common childhood cancers. These tumors account for about 1 in 4 newly diagnosed pediatric cancers annually.

A recent St. Jude study found that 32 percent of brain tumor patients developed severe hearing loss within several years of treatment despite treatment with a drug, amifostine, designed to protect hair cells in the inner ear that are essential for hearing.

The analysis involved 3- to 21-year-olds with medulloblastoma and other embryonal brain tumors. All patients were enrolled in a multi-site St. Jude clinical research trial and treatment that included surgery plus risk-adapted radiation treatment and chemotherapy. All had neurocognitive and hearing testing at least twice — early and later in treatment.

Next steps

The analysis proposed multiple factors, including damage to the hearing nerve caused by the tumor itself, that complicate reading mastery for pediatric brain tumor survivors with severe hearing loss. “That suggests we have an opportunity to significantly improve the quality of life for survivors by developing more effective interventions,” Conklin said.

Research is needed to determine how and when to intervene to bolster reading skills in young cancer patients. That includes tracking how cochlear implants or hearing aids affect reading and neurocognitive skills in young cancer survivors. Data on hearing aid use in this study was incomplete.

“Compared to vision loss, hearing difficulties often go undetected for longer periods. This study demonstrates the need for close audiological monitoring early in treatment so we can recognize and intervene early,” Olivier said. “Parents might not realize the impact of decreased hearing on educational outcomes.”

The other authors are Johnnie Bass, Jason Ashford, Rebecca Beaulieu, Sarah Scott, Shawna Palmer, Shengjie Wu, Arzu Onar-Thomas and Amar Gajjar, all of St. Jude; and nine researchers from research institutions in the U.S., Canada and Australia.

The research was funded in part by a grant (CA21765) from the National Cancer Institute and by ALSAC, the fundraising and awareness organization of St. Jude.

Original Article

Reconsidering Individuals With Normal Hearing

Dementia, called the “greatest global challenge for health and social care in the 21st century,”1(p2673) occurs in 47 million persons globally. This number is projected to triple by 2050. With no cure and no treatments to alter its natural history, public health prevention efforts are paramount. Hearing loss (HL) is a novel yet treatable risk factor for dementia.1 In the article by Golub and colleagues2 in this issue of JAMA Otolaryngology–Head & Neck Surgery, the importance of understanding the association between hearing and cognitive performance for older adults is highlighted. Novel to this study is the focus on adults with hearing in the normal range.

Original Article

Association of Subclinical Hearing Loss With Cognitive Performance

Key Points

Question  Is the association between hearing and cognition present among individuals who have classically defined normal hearing levels?

Findings  In this cross-sectional study of 6451 individuals, there was an inverse association between decreasing hearing and decreasing cognition among those classically defined as having normal hearing, after adjusting for confounders.

Meaning  The findings suggest that the association between hearing loss and impaired cognition may be present at earlier levels of hearing loss than previously recognized; the current 25-dB threshold for defining adult hearing loss may be too high.

Importance  Age-related hearing loss (HL) is a common and treatable condition that has been associated with cognitive impairment. The level of hearing at which this association begins has not been studied to date.

Objective  To investigate whether the association between hearing and cognition is present among individuals traditionally classified as having normal hearing.

Design, Setting, and Participants  Cross-sectional study of 2 US epidemiologic studies (Hispanic Community Health Study [HCHS], 2008-2011, and National Health and Nutrition Examination Study [NHANES], 1999-2000, 2001-2002, and 2011-2012 cycles). The dates of analysis were November 2018 to August 2019. Multivariable generalized additive model (GAM) regression and linear regression were used to assess the association between HL (exposure) and cognition (outcome). Participants included 6451 individuals aged 50 years or older from the general Hispanic population (HCHS [n = 5190]) and the general civilian, noninstitutionalized US population (NHANES [n = 1261]).

Exposures  Audiometric HL (4-frequency pure-tone average).

Main Outcomes and Measures  Neurocognitive performance measured by the Digit Symbol Substitution Test (DSST) (score range, 0-113), Word Frequency Test (range, 0-49), Spanish-English Verbal Learning Test (SEVLT) 3 trials (range, 5-40), SEVLT recall (range, 0-15), and Six-Item Screener (range, 0-6); higher scores indicated better cognitive performance.

Results  Among 6451 individuals, the mean (SD) age was 59.4 (6.1) years, and 3841 (59.5%) were women. The GAM regression showed a significant inverse association between hearing and cognition across the entire spectrum of hearing after adjusting for demographics and cardiovascular disease. In separate multivariable linear regressions stratified by the classic binary definition of HL, decreased hearing was independently associated with decreased cognition in adults with normal hearing (pure-tone average ≤25 dB) across all cognitive tests in the HCHS. For example in this group, a 10-dB decrease in hearing was associated with a clinically meaningful 1.97-point (95% CI, 1.18-2.75) decrease in score on the DSST. When using a stricter HL cut point (15 dB), an association was also present in NHANES. The associations between hearing and cognition were stronger or equivalent in individuals with normal hearing than among those with HL. For example, there was a 2.28-point (95% CI, 1.56-3.00) combined cohort DSST score decrease per 10-dB decrease among individuals with normal hearing vs a 0.97-point (95% CI, 0.20-1.75) decrease among those with HL, with a significant interaction term between continuous and binary hearing.

Conclusions and Relevance  An independent association was observed between cognition and subclinical HL. The association between hearing and cognition may be present earlier in HL than previously understood. Studies investigating whether treating HL can prevent impaired cognition and dementia should consider a lower threshold for defining HL than the current 25-dB threshold.

Original Article

Prolonging Life, but at What Price?

“Doc, how long have I got to live anyway if I do nothing?” This is a question from an elderly patient that is familiar to any oncologist. As the proportion of elderly patients continues to increase steadily worldwide, clinicians can expect that this will become an increasingly present scenario in medical offices. In particular, there will be a dramatic increase between 2015 and 2050 in patients currently regarded as the so-called oldest old (those 80 years and older) to more than 440 million worldwide, tripling the current population in this category.1 Whether to subject patients who have already exceeded normal life expectancy to the stressors of major surgery for an aggressive malignant condition is as much an ethical issue as it is a medical one, and clinicians are badly in need of tools to assist patients in shared decision-making. The lay press has caught wind of this issue as well; witness the title to a New York Times article published in June 2019: “The Elderly are Getting Complex Surgeries. Often It Doesn’t End Well.”2

Original Article

Treatment of Head and Neck Cancer—Sometimes, Less Is More

In this issue of JAMA Otolaryngology-Head & Neck Surgery, Saraswathula and colleagues1 used the Surveillance, Epidemiology, and End Results Program (SEER)–Medicare outcomes and claims database to compare the value of 3 different radiotherapy (RT) regimens (ie, RT alone, RT plus cisplatin, or RT plus cetuximab) administered to patients older than 65 years with locally advanced head and neck cancer (HNC) of the oral cavity, oropharynx, larynx, and hypopharynx. In this setting, RT plus cetuximab was not associated with improved survival compared with RT alone, but it was associated with an increased number of emergency department visits and increased costs. The authors concluded that cetuximab concurrently administered with RT seems to be of low value in terms of improved survival and higher costs for older patients with HNC.

Original Article

Care Value for Older Patients Receiving Radiotherapy With or Without Cisplatin or Cetuximab for Head/Neck Cancer

Key Points

Question  What is the value of cetuximab chemoradiotherapy compared with radiotherapy alone for older patients with locoregionally advanced head and neck cancer?

Findings  In this cohort study of 1091 older patients with stages III to IVB head and neck cancer, radiotherapy with cetuximab treatment was not associated with improved survival compared with radiotherapy alone, but radiotherapy with cisplatin was associated with improved survival. Cetuximab use was associated with higher Medicare spending but not with higher rates of inpatient admission compared with radiotherapy alone, unlike radiotherapy with cisplatin.

Meaning  Despite performing better on some Medicare quality metrics, cetuximab appears to be of low value for older patients with advanced disease; composite metrics should be used in the future to assess care quality within the context of outcomes and treatment cost.

Importance  Clinicians frequently use radiotherapy with cetuximab over radiotherapy only or radiotherapy with cisplatin because of a perceived survival and tolerability advantage, but scant data are available to support this perception.

Objective  To measure the 3 aspects of value (quality, outcomes, and cost) in older patients receiving radiotherapy only, radiotherapy with cisplatin, or radiotherapy with cetuximab for locoregionally advanced head and neck cancer.

Design, Setting, and Participants  For this cohort study, patient records were obtained from the Surveillance, Epidemiology, and End Results Program (SEER)–Medicare outcomes and claims database from January 1, 2004, to December 31, 2014. Participants were 65 years or older; received a diagnosis between 2006 and 2013 of stages III to IVB head and neck cancer; had only 1 cancer on record; and did not undergo surgical intervention. Data analysis was conducted from February 5, 2018, to March 27, 2019.

Exposures  Patients were divided into exposure arms on the basis of their first-line therapy or identified chemoradiotherapy and radiotherapy regimen.

Main Outcomes and Measures  Overall survival was analyzed by propensity score matching Cox proportional hazards regression models, quality by measuring 90-day emergency department (ED) visit and inpatient admission rates, and costs by assessing 90-day total Medicare spending.

Results  The overall cohort included 1091 patients, of whom 815 (74.7%) were male; the mean (SD) age was 73.9 (6.6) years. Patients receiving radiotherapy with cisplatin had higher overall survival compared with those receiving radiotherapy only (adjusted hazard ratio [HR], 0.64; 95% CI, 0.47-0.87). This finding was not seen in patients receiving radiotherapy with cetuximab (adjusted HR, 0.95; 95% CI, 0.75-1.20), compared with the radiotherapy only group, and it persisted after stratifying patients by age. The ED visit (adjusted incidence rate ratio [IRR], 1.72; 95% CI, 1.30-2.30) and inpatient admission (adjusted IRR, 1.48; 95% CI, 1.12-1.98) rates in the 90 days after treatment start were higher in patients receiving radiotherapy with cisplatin compared with those treated with radiotherapy only. Patients receiving radiotherapy with cetuximab had a higher rate of ED visits (adjusted IRR, 1.38; 95% CI, 1.05-1.82) compared with those in the radiotherapy only group. The 90-day after-treatment spending for patients receiving radiotherapy with cetuximab was $48 620 (95% CI, $46 466-$50 775) compared with $33 009 (95% CI, $31 499-$34 519) for radiotherapy with cisplatin and $27 622 (95% CI, $25 118-$30 126) for radiotherapy only.

Conclusions and Relevance  In this cohort study, no survival difference, a higher rate of ED visits but not of inpatient admissions, and higher spending were observed in patients receiving radiotherapy with cetuximab compared with patients receiving radiotherapy only. The findings suggest that radiotherapy alone should be maintained as a treatment arm in evaluation of novel therapeutics for locoregionally advanced head and neck cancer in older and sicker patients.

Original Article