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Resolution of unilateral sensorineural hearing loss in a pediatric patient with a severe phenotype of Muckle-Wells syndrome treated with Anakinra: a case report and review of the literature

Overview of attention for article published in Journal of Otolaryngology -- Head & Neck Surgery, January 2018
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  • Above-average Attention Score compared to outputs of the same age (51st percentile)

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3 tweeters

Citations

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10 Dimensions

Readers on

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25 Mendeley
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Title
Resolution of unilateral sensorineural hearing loss in a pediatric patient with a severe phenotype of Muckle-Wells syndrome treated with Anakinra: a case report and review of the literature
Published in
Journal of Otolaryngology -- Head & Neck Surgery, January 2018
DOI 10.1186/s40463-018-0256-0
Pubmed ID
Authors

Cinzia Marchica, Faisal Zawawi, Dania Basodan, Rosie Scuccimarri, Sam J. Daniel

Abstract

Muckle-Wells syndrome (MWS) is a rare auto-inflammatory disease characterized by the presence of recurrent urticaria, deafness and amyloidosis. Progressive sensorineural hearing loss (SNHL) is reported to occur in up to 85% of patients occurring in the second and third decades and as early as the first decade in patients with a more severe phenotype, thus potentially having a significant impact on a child's development. IL-1 inhibitors, such as Anakinra, have been described to improve systemic inflammation, and stabilize or improve hearing status as well. However, complete resolution of hearing loss has been rarely reported. The objective of this article is to highlight the clinical presentation of a pediatric patient with a severe form of MWS and report on the complete resolution of SNHL with the use of Anakinra. A 3-year-old boy was referred to our hospital to assess for the possibility of MWS given a history of hives and recurrent episodes of fever with a family history of MWS in his mother. Of note, the patient's history was significant for conductive hearing loss, speech delay, as well as recurrent acute otitis media episodes. Genetic analysis was performed and diagnosis of MWS was confirmed due to the presence of a NLRP3 gene mutation. Further work-up demonstrated the presence of papilledema and elevation of systemic inflammatory markers for which Canakinumab was initiated. Despite initiation of this treatment, audiogram evaluation demonstrated a new right-sided SNHL. Lumbar puncture also revealed aseptic meningitis. Canakinumab was eventually discontinued and Anakinra initiated. Within 7 months of treatment with Anakinra at 5 mg/kg sc daily, resolution of the SNHL was observed. With further escalation of the Anakinra dose, there was also complete resolution of the aseptic meningitis. Progressive hearing loss is a significant finding in patients with MWS. Early screening as well as initiation of Anakinra can lead to complete resolution of SNHL even in a patient with a severe spectrum of MWS. However, as this case demonstrates, longer treatment duration and higher doses of Anakinra may be required to achieve this.

Twitter Demographics

The data shown below were collected from the profiles of 3 tweeters who shared this research output. Click here to find out more about how the information was compiled.

Mendeley readers

The data shown below were compiled from readership statistics for 25 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 25 100%

Demographic breakdown

Readers by professional status Count As %
Student > Master 4 16%
Student > Bachelor 3 12%
Student > Ph. D. Student 3 12%
Student > Postgraduate 2 8%
Student > Doctoral Student 2 8%
Other 6 24%
Unknown 5 20%
Readers by discipline Count As %
Medicine and Dentistry 10 40%
Neuroscience 2 8%
Social Sciences 1 4%
Agricultural and Biological Sciences 1 4%
Nursing and Health Professions 1 4%
Other 2 8%
Unknown 8 32%

Attention Score in Context

This research output has an Altmetric Attention Score of 2. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 23 April 2018.
All research outputs
#7,754,019
of 13,780,426 outputs
Outputs from Journal of Otolaryngology -- Head & Neck Surgery
#65
of 274 outputs
Outputs of similar age
#164,253
of 352,862 outputs
Outputs of similar age from Journal of Otolaryngology -- Head & Neck Surgery
#1
of 1 outputs
Altmetric has tracked 13,780,426 research outputs across all sources so far. This one is in the 42nd percentile – i.e., 42% of other outputs scored the same or lower than it.
So far Altmetric has tracked 274 research outputs from this source. They receive a mean Attention Score of 2.6. This one has done well, scoring higher than 75% of its peers.
Older research outputs will score higher simply because they've had more time to accumulate mentions. To account for age we can compare this Altmetric Attention Score to the 352,862 tracked outputs that were published within six weeks on either side of this one in any source. This one has gotten more attention than average, scoring higher than 51% of its contemporaries.
We're also able to compare this research output to 1 others from the same source and published within six weeks on either side of this one. This one has scored higher than all of them