Pediatric otosclerosis: Case report and literature review

Salomone, Raquel; Riskalla, Paulo Emmanuel; Vicente, Andy de Oliveira; Boccalini, Maria Carmela Cundari; Chaves, A. S.; Lopes, Renata; Filho, Gilberto Bolivar Felin

doi:10.1016/s1808-8694(15)31105-8

Cited by 12 publications

(4 citation statements)

References 13 publications

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“…Salomone et al discuss a case of delayed diagnosis of juvenile otosclerosis after the conductive hearing loss in a young child was found to be refractory to grommet insertion [22].…”

Section: Discussionmentioning

confidence: 99%

Pediatric otosyphilis—An unusual cause of conductive hearing loss

Arain

Abbas

Adams

2020

Radiology Case Reports

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We present the case of a 5 year old female with a unilateral conductive hearing loss which had a relapsing and remitting course over a 3 year period. An initial noncontrast CT temporal bone study was unremarkable and a diagnosis of otitis media was made in the first instance. However, a second CT temporal bone study performed 3 years later demonstrated bilateral demineralisation of the ossicles and abnormal lucency affecting both the otic capsules. A diagnosis of otosyphilis was proposed on the basis of the imaging features and a prior medical history of previously treated congenital syphilis. With the benefit of hindsight, early pericochlear lucency was identified on the initial CT temporal bone study. There has been a steady rise of syphilis cases since the millennium with resurgence in many high income countries. Otosyphilis has a highly variable clinical presentation and there is limited data to establish the pattern of hearing loss in pediatric patients with a background of congenital otosyphilis. Temporal bone and otic capsule demineralisation carries a broad differential diagnosis including osteogenesis imperfecta, otosclerosis, Paget's disease and radiation related changes. Otosyphilis is a rare but potentially treatable cause of deafness and a high index of suspicion is required to make the diagnosis. In conjunction with a positive syphilis serology, a noncontrast temporal bone CT can aid the diagnosis and expedite the treatment.

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“…Salomone et al discuss a case of delayed diagnosis of juvenile otosclerosis after the conductive hearing loss in a young child was found to be refractory to grommet insertion [22].…”

Section: Discussionmentioning

confidence: 99%

Pediatric otosyphilis—An unusual cause of conductive hearing loss

Arain

Abbas

Adams

2020

Radiology Case Reports

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“…9 Otosclerosis is an autosomal dominant disease with 20% to 40% penetrance. 10 However, COM usually occurs sporadically. 11 Accordingly, for children with bilateral conductive deafness, stapes ankylosis, otosclerosis, and COM should be differentiated from NOG-SSDs.…”

Section: Discussionmentioning

confidence: 99%

Delayed-Onset NOG Gene-Related Syndromic Conductive Deafness: A Case Report

et al. 2020

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We report a 6-year-old girl with progressive bilateral conductive hearing loss for 2 years. She passed the newborn hearing screening conducted with otoacoustic emissions testing and had a normal development of speech and language, which indicated that her deafness was delayed-onset. She also had congenital proximal interphalangeal joints. Proximal symphalangism was confirmed by genetic testing ( NOG gene: c.406C > T, p.R136C). Bilateral stapes ankyloses were proved by surgery and her hearing was improved after stapedotomy by over 30 dB. Besides, this case should remind clinicians to carefully distinguish NOG gene-related deafness from congenital ossicular malformation and pediatric otosclerosis.

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“…10 This is found in 0.6% of temporal bones in children younger than 5 years of age, and in 4% of children between 5 and 18 years of age. 10 The disease is clinically characterized by progressive conductive and/or mixed hearing loss and tinnitus. CT is the imaging technique of choice and can demonstrate abnormalities of the oval window, which is often involved.…”

Section: Pathology Of the Middle Earmentioning

confidence: 99%

Imaging of Pediatric Hearing Loss

Tamrazi¹,

Almast²,

Mangla³

2011

neurograph

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Hearing loss in children is a prevalent disability, affecting 1 in 1000 neonates and up to 11% of school-aged children. Imaging plays a key role in the evaluation of conductive and SNHL with multiple modalities including CT and MR imaging. The purpose of this article is to review the anatomy and pathology resulting in conductive and SNHL through an imaging approach. The pathology of pediatric hearing loss includes multiple causes of both osseous and nonosseous abnormalities, which can be categorized into congenital, infectious/inflammatory, and neoplastic processes. The recognition of complex anatomy and pathology of the ear can place the radiologist in a key position in the diagnosis and overall evaluation of pediatric hearing loss and, ultimately, contribute to the management of the patient.

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Pediatric otosclerosis: Case report and literature review

Cited by 12 publications

References 13 publications

Pediatric otosyphilis—An unusual cause of conductive hearing loss

Pediatric otosyphilis—An unusual cause of conductive hearing loss

Delayed-Onset NOG Gene-Related Syndromic Conductive Deafness: A Case Report

Imaging of Pediatric Hearing Loss

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