Hearing impairment and osteogenesis imperfecta: Literature review

Publication date: Available online 13 June 2019Source: European Annals of Otorhinolaryngology, Head and Neck DiseasesAuthor(s): F. Carré, S. Achard, I. Rouillon, M. Parodi, N. LoundonAbstractThe goal is to clarify the epidemiology of hearing loss in patients with osteogenesis imperfecta (OI), so as to improve management. A literature review analyzed data from 15 patient series. Hearing loss prevalence in OI varied widely, from 2% to 94.1%. Typically, hearing loss was conductive in young patients and sensorineural in older patients. Prevalence increased with age, but after 50 years the increase was slight, and seldom became total. Hearing loss was usually bilateral, but not necessarily symmetrical. There were no correlations between type of mutation (COL1A1 or COL1A2), prevalence, type or severity of hearing loss, or age of symptom onset; there was intra-familial variability. There was also no correlation between mutated gene, type of mutation and auditory phenotype. Frequency, type and severity of hearing loss were unrelated to other clinical parameters. Hearing loss prevalence depended on type of OI: higher in type I and lower in type IV. Incidence of otitis media was higher in children with OI, related to the associated craniofacial dysmorphia. Hearing screening before 5 years of age with long-term follow-up are recommended.
Source: European Annals of Otorhinolaryngology, Head and Neck Diseases - Category: ENT & OMF Source Type: research

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This study aimed to assess the prevalence of dental findings on panoramic radiographs (PRs) of osteogenesis imperfecta (OI) patients and correlate these results with epidemiologic and medical data.Study designA case-control study was conducted with 24 OI patients and 48 sex- and age-matched controls. Demographic, clinical, and bisphosphonate regimen-related data were recorded. The outcome variables were the presence or absence of dental alterations in PRs. Mann-Whitney U, Pearson's chi-square tests, and multinomial logistic regression (95% confidence interval) were used (significance level of 5%).ResultsOI type 4 demonstra...
Source: Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology - Category: ENT & OMF Source Type: research
ConclusionPaediatric temporal bone inflammatory processes are common and can have severe clinical sequelae. Timely intervention, facilitated by correct radiological diagnosis, can often prevent progression of disease, loss of hearing and systemic illness.
Source: Neuroradiology - Category: Radiology Source Type: research
This study aimed to assess the prevalence of dental findings on panoramic radiographs (PRs) of osteogenesis imperfecta (OI) patients and correlate these results with epidemiologic and medical data.
Source: Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics - Category: ENT & OMF Authors: Source Type: research
Conclusions: Children with OM-associated BM were prone to many problems, such as being especially ill at presentation, undergoing a difficult clinical course and showing a higher risk of complicated or fatal outcome. HIV infection and malnutrition were common in children with otorrhea, which was also associated with low socioeconomic status.
Source: The Pediatric Infectious Disease Journal - Category: Infectious Diseases Tags: Original Studies Source Type: research
Abstract Treatment of moderate and severe forms of osteogenesis imperfecta (OI) with cyclic pamidronate at the Reference Center for OI Treatment in Southern Brazil was studied. A retrospective cohort study was conducted from 2002 to 2012. Data were obtained during inpatient (drug infusion) and outpatient care. Clinical data, including the presence of blue sclerae, dentinogenesis imperfecta, history and site of the fractures, biochemical data, including calcium, phosphorus, and alkaline phosphatase levels, were systematically collected. Bone mineral density (BMD) was measured using dual energy X-ray absorptiometry (DXA). Fo...
Source: Genetics and Molecular Biology - Category: Genetics & Stem Cells Source Type: research
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Source: Journal of Bone and Mineral Research - Category: Orthopaedics Authors: Tags: Original Article Source Type: research
Abstract(1) To study the prevalence of sensorineural hearing loss among chronic renal failure patients. (2) To study the correlation between degree of hearing loss and the duration of chronic renal failure. This cross sectional study was conducted in the department of Otorhinolaryngology and Head and Neck Surgery at KAHER ’s Jawaharlal Nehru Medical College and KLEs Dr. Prabhakar Kore Hospital and Medical Research Centre, Belagavi from January 2018 to December 2018. 60 ears (30 patients) with chronic renal failure were included in the study. All 60 ears were subjected to ENT examination, pure tone audiometry and me a...
Source: Indian Journal of Otolaryngology and Head and Neck Surgery - Category: ENT & OMF Source Type: research
Authors: Kim SH, Park MK PMID: 31295993 [PubMed]
Source: Clinical and Experimental Otorhinolaryngology - Category: ENT & OMF Tags: Clin Exp Otorhinolaryngol Source Type: research
Abstract Epithelial abnormalities underpin the development of the middle ear disease, otitis media (OM). Until now, a well-characterized in vitro model of the middle ear (ME) epithelium that replicates the complex cellular composition of the middle ear has not been available. This chapter describes the development of a novel in vitro model of mouse middle ear epithelial cells (mMECs), cultured at the air-liquid interface (ALI). This system enables recapitulation of the characteristics of the native murine ME epithelium. We demonstrate that mMECs undergo differentiation into the varied cell populations seen within ...
Source: Mol Biol Cell - Category: Molecular Biology Authors: Tags: Methods Mol Biol Source Type: research
Conclusions: Olecranon fractures in the OI population occur most commonly in patients with type I OI and during early adolescence, a period of rapid growth. There is a high rate of bilateral olecranon fractures, with the contralateral fracture occurring quickly after the primary fracture. Further studies may elucidate risk factors to determine which patients are most likely to fracture the contralateral side and therefore drive treatment and potentially prevention. Level of Evidence: Level IV—retrospective cohort study.
Source: Journal of Pediatric Orthopaedics - Category: Orthopaedics Tags: Selected Topics Source Type: research
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