How common is hearing impairment in osteogenesis imperfecta?

@article{Paterson2001HowCI,
  title={How common is hearing impairment in osteogenesis imperfecta?},
  author={Colin Ralston Paterson and Elizabeth A. Monk and Susan J. Mcallion},
  journal={The Journal of Laryngology \& Otology},
  year={2001},
  volume={115},
  pages={280 - 282}
}
Hearing impairment has long been recognized as a common feature in osteogenesis imperfecta. The figures in some publications could be taken to imply that, with increasing age, the proportion of osteogenesis imperfecta patients with hearing impairment approaches 100 per cent. The incidence of hearing loss in a large survey of 1394 patients with osteogenesis imperfecta was examined. It was found that the most common age of onset was in the second, third and fourth decades of life. At the age of… 
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Hearing impairment and osteogenesis imperfecta: Literature review.
Hearing Loss in Finnish Adults with Osteogenesis Imperfecta: A Nationwide Survey
TLDR
Subjective misjudgment of hearing ability supports the need for repeated audiometry in all OI patients.
Hearing Loss in Osteogenesis Imperfecta
Vestibular dysfunction in adult patients with osteogenesis imperfecta
TLDR
It is concluded that vertigo is common in patients with OI, in most cases, it may be secondary to inner ear pathology, and in only some patients does BI explain it.
Osteogenesis imperfecta: the audiological phenotype lacks correlation with the genotype
TLDR
This study confirms that hearing loss in OI shows a strong intrafamilial variability, and further investigates the correlations between the presence and/or characteristics of the hearing loss and the underlying mutation.
High Heterogeneity of Temporal Bone CT Aspects in Osteogenesis Imperfecta Is Not Linked to Hearing Loss
TLDR
Temporal bone CT features in OI are present in a large proportion of patients, had they hearing loss or not, and might be determined more by collagen mutation type than by age or BMD.
Osteogenesis imperfecta and hearing loss: an analysis of patients attended at a benchmark treatment center in southern Brazil
TLDR
Overall, the majority of the subjects in this patient sample presented normal hearing thresholds, and hearing impairments were more prevalent in the adult group than in the adolescent or children’s groups.
Longitudinal analysis of the audiological phenotype in osteogenesis imperfecta: a follow-up study.
TLDR
Findings highlight the importance of regular audiological follow up in osteogenesis imperfecta patients, including audiometry, and measurements of acoustic admittance, acoustic stapedial reflexes and otoacoustic emissions.
Osteogenesis imperfecta
CHAPTER 18 – Osteogenesis Imperfecta
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References

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TLDR
Middle ear analysis by tympanometry and acoustic reflex analysis indicates that, although some patients with OI have a still middle ear system similar to that seen in otosclerosis, the majority display absent acoustic reflexes and increased compliance of the middle ear with notched tympansograms suggestive of anomalous ossicular articulation.
Hearing loss in patients with osteogenesis imperfecta. A clinical and audiological study of 201 patients.
TLDR
Tympanometry and acoustic reflex measurements suggested that the cause of conductive or mixed hearing loss was stapedial fixation and in a few cases ossicular discontinuity due to aplasia or fracture of the stapedia crura, and findings during stapedectomy in 32 patients confirmed these assumptions.
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An epidemiological and genetical study of osteogenesis imperfecta (OI) in Victoria, Australia confirmed that there are at least four distinct syndromes at present called OI. The largest group of…
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TLDR
Insight into the pathogenesis of these disorders will eventually permit specific therapy, prenatal diagnosis and more accurate genetic counseling for the osteogenesis imperfecta syndromes.
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TLDR
It was found that 31 patients had a hearing loss and Tympanometry was found to give unpredictable results and was unhelpful in assessing middle ear function in these patients.
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TLDR
It was clear that osteogenesis imperfecta contributed significantly to death, almost certainly to many of the respiratory deaths and to deaths from cardiac failure due to kyphoscoliosis.
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TLDR
A survey of 78 patients with osteogenesis imperfecta (OI) type IVA, the variant that causes the greatest difficulties in differential diagnosis, found an appreciable number had pale blue sclerae in early childhood and there were significantly increased incidences of bruising, nosebleeds, excessive sweating and hypermobility of joints.
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TLDR
The present study indicates that the stapedial lesion in osteogenesis imperfecta is most likely a manifestation of the generalized bone and connective tissue disorder.
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TLDR
The histopathologic findings in 8 temporal bones from 5 patients with osteogenesis imperfecta congenita showed evidence of both deficient and abnormal ossification, and the possible implication of these changes on hearing and balance is discussed.
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TLDR
A description of two families with hereditary fragility of the bones associated with grey-blue sclerotics, and in the first family with otosclerosis also; a summary of the literature of these peculiarities in relation to fragilitas ossium is presented.
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