Pediatric otosclerosis

INTRODUCTION: Endoscopic ossicular chain reconstruction (OCR) in adults has demonstrated equivalent outcomes to the traditional microscopic approach. Less data exist on endoscopic OCR outcomes in children, who have unique considerations including a smaller transcanal corridor and variable pathology. The purpose of this study was to investigate surgical and audiometric outcomes in children undergoing fully endoscopic and endoscopic-assisted OCR in both the short and long-term. METHODS: Retrospective review of all children (<17 years) who underwent endoscopic OCR at one tertiary care center between 2017 and 2021...

We present the case of a patient treated as affected by conductive hearing loss due to recurrent otitis, then as a juvenile otosclerosis, who was finally diagnosed as affected by bilateral ossicular chain fixation and enlarged vestibular aqueduct by means of cone-beam CT.

OBJECTIVE: Juvenile Otosclerosis (JO) and Congenital Stapes Footplate Fixation (CSFF) are rare ossicular chain disorders seen in the paediatric population and present with conductive hearing loss. Ongoing controversy exists regarding the role of surgical intervention in JO and CSFF given the poorer hearing outcomes and complications when compared with surgical intervention for adult otosclerosis. The objective of this study is to assess the published data on the surgical outcomes of JO and CSFF in order to guide clinicians and counsel patients on the various medical options for these disease entities...

BACKGROUND: Otosclerosis can occur during childhood, resulting in the early onset of conductive hearing loss. The approach to a child with otosclerosis can present some difficulties in terms of diagnosis and treatment, and the literature on juvenile otosclerosis (JO) is still relatively limited. AIM: To explore the current approaches to JO, in order to clear the management of this condition and evaluate the outcomes and the possible complications of surgical treatment...

OBJECTIVES: To compare the mean pre-operative air-bone gaps (ABG), mean post-operative ABGs, and extrusion rates between pediatric recipients of partial ossicular reconstruction prostheses (PORPs) and pediatric recipients of total ossicular reconstruction prostheses (TORPs) via a systematic review and meta-analysis. METHODS: A quantitative systematic review last updated on September 29, 2021 of PubMed, Scopus, and Embase databases was conducted for studies reporting mean post-operative ABGs or numbers of children with post-operative ABG ≤ 20 dB following PORP and TORP procedures in at least five children aged 0-18 years...

There has been a rapid increase in endoscopic ear surgery for the management of middle ear and lateral skull base disease in children and adults over the last decade. In this review paper, we discuss the current trends and applications of the endoscope in the field of otology and neurotology. Advantages of the endoscope include excellent ergonomics, compatibility with pediatric anatomy, and improved access to the middle ear through the external auditory canal. Transcanal endoscopic ear surgery has demonstrated comparable outcomes in the management of cholesteatoma, tympanic membrane perforations, and otosclerosis as compared to microscopic approaches, while utilizing less invasive surgical corridors and reducing the need for postauricular incisions...

OBJECTIVE: To provide an overview of the current status regarding the parallel use of the endoscope and the laser in middle ear surgery. METHODS: Comprehensive Pubmed search from 1975 to 2020 including clinical articles, of any type, reporting the combined use of a laser and an endoscope. Purely experimental and non-human studies were excluded. RESULTS: Reports on the application of the laser in pediatric and adult endoscopic middle ear surgery (EES) are increasing since 2013...

OBJECTIVES/HYPOTHESIS: Internal auditory diverticula in adults have been found to exist independent of otosclerosis, and in the presence of otosclerosis. We sought to determine the prevalence of internal auditory canal (IAC) diverticula in a pediatric cohort, to assess whether IAC diverticula are a risk factor for hearing loss, and the co-occurrence of otic capsule hypoattenuation. STUDY DESIGN: Retrospective review. METHODS: A single-site retrospective review of high-resolution temporal bones computed tomography (CT) scans including the presence and size of diverticula and hypoattenuation of the otic capsule...

The indications for cochlear implantation have gradually expanded as advancements in technology have evolved, resulting in improved audiologic outcomes for both adult and children. There remains a significant underutilization of cochlear implant technology in the United States, and recognition of the potential benefits of cochlear implantation for non-traditional indications is critical for encouraging the evolution of candidacy criteria. Adult cochlear implantation candidacy has progressed from patients with bilateral profound sensorineural hearing loss (SNHL) to include patients with greater degrees of residual hearing, single-sided deafness and asymmetric hearing, and atypical etiologies of hearing loss (eg, vestibular schwannoma, Ménière's disease, and otosclerosis)...

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...

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...

OBJECTIVE: To review a single institution experience with pediatric stapedotomy for juvenile otosclerosis (JO), congenital stapes footplate fixation (CSFF), or tympanosclerosis. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary referral center. PATIENTS: Pediatric patients undergoing surgery for stapes fixation from 2001 to 2017. MAIN OUTCOME MEASURES: Hearing result based on preoperative, first postoperative, and final postoperative pure-tone average air-bone gap (PTA-ABG)...

We describe a three-year-old male child who presented to the pediatrics out-patient department with a history of decrease in appetite, generalized weakness, on and off loose motions for one year, inability to walk and sit for eight months with a loss of neck holding for 14 days. On examination, the patient had a classic frog-shaped leg posture. X-rays of chest, skull, pelvis and long bones were performed which showed osteopenic bones, frontal bossing and multiple microfractures, which were classic for osteogenesis Imperfecta but the child did not have other salient features such as blue sclera, otosclerosis, and respiratory difficulty...

OBJECTIVE: To compare endoscopic and microscopic pediatric stapes surgery. STUDY DESIGN: Case series with chart review. SETTING: Two academic otology practices. SUBJECTS AND METHODS: Surgical and hearing outcomes were compared for consecutive children (<18 years) undergoing microscopic and endoscopic stapes surgery. The main outcome measure was closure of the air-bone gap (ABG) to ≤20 dB. RESULTS: Twenty-two endoscopic surgeries (17 stapedectomies, 4 stapedotomies, and 1 stapes mobilization) and 52 microscopic surgeries (30 stapedectomies, 19 stapedotomies, and 3 stapes mobilizations) were performed...

OBJECTIVES/HYPOTHESIS: Determine the utility of preoperative imaging in adult and pediatric cochlear implant candidates. STUDY DESIGN: Retrospective chart review. METHODS: Medical records of 101 consecutive adult and 20 consecutive pediatric patients who underwent 137 cochlear implantation (CI) procedures at a single institution were reviewed. RESULTS: Computed tomography (CT) was obtained preoperatively in 110 (90.9%) patients, preoperative magnetic resonance imaging (MRI) was obtained in 102 (84...

OBJECTIVES/HYPOTHESIS: To prospectively evaluate hearing outcomes in children with otosclerosis undergoing primary stapes surgery. STUDY DESIGN: A nonrandomized, nonblinded, prospective case series. METHODS: Thirty-four consecutive pediatric patients who underwent 41 primary stapedotomies for otosclerosis in a tertiary referral center were included. Patients were included when there was evidence of otosclerotic stapes fixation and they had available postoperative pure-tone audiometry...

OBJECTIVE: To compare the outcomes of 3 surgical techniques for primary stapes fixation: stapedotomy minus prosthesis (STAMP), circumferential stapes mobilization (CSM), and small fenestra stapedotomy (SFS). STUDY DESIGN: Retrospective review of 277 primary cases operated for stapes fixation from 1997 to 2007. SETTING: Tertiary academic center. PATIENTS: Consecutive adult and pediatric cases operated for conductive hearing loss because of stapes fixation...

OBJECTIVE: To compare outcomes of stapedectomy in patients with congenital stapes fixation versus juvenile otosclerosis. METHODS: A retrospective chart review was performed from January 1, 1999 until January 1, 2011 to identify patients under 18 years old who underwent a stapedectomy. Age, gender, pre- and postoperative audiograms, intraoperative findings including etiology of stapes fixation, prosthesis type, and complications were recorded. RESULTS: Twenty-two children were identified who had undergone a stapedectomy (two patients underwent sequential bilateral surgery) resulting in a total of 24 ears...

OBJECTIVE: To compare presentation, operative findings, and outcomes among pediatric patients undergoing primary stapedectomy for congenital stapes footplate fixation (CSFF) and juvenile otosclerosis (JO). STUDY DESIGN: Retrospective review. SETTING: Combined experience from 2 tertiary academic referral centers. PATIENTS: Pediatric patients with CSFF and JO. INTERVENTION: Primary stapedectomy. MAIN OUTCOME MEASURE(S): 1) Preoperative and postoperative audiometric data using the 1995 AAO-HNS reporting guidelines; 2) Notable operative findings, and postoperative complications...

BACKGROUND: Osteogenesis Imperfecta (OI) is a genetic disorder of connective tissue matrix. OI is caused by mutations that affect type I collagen. The hearing loss in OI is characterized by onset in early adulthood and can be conductive, sensorineural, or mixed. OBJECTIVES: To describe the temporal bone histopathology in 9 individuals with OI. MATERIALS AND METHODS: Four adult, 1 pediatric, and 4 infant specimens were identified. Temporal bones were removed at autopsy and studied using light microscopy...