Osteogenesis Imperfecta and hearing loss in the paediatric population

The temporal bone report of an operated case of osteogenesis imperfecta tarda is presented. Histological examination confirmed the presence of bilateral fixation of the footplate by otosclerosis as the cause of the conductive hearing loss. Fragility of bony septae in the mastoid and of the stapedial crura were observed. Sensorineural impairment in later years with a reduction in neural elements in the cochlea appear related to the extent and activity of the otosclerotic foci. Additional temporal bone reports of three cases of osteogenesis imperfecta congenita show lack of deposition of the skein-like bone in the endochondral layer, sparse bony septae in marrow spaces and deficiency of the periosteal layer. The stapedial crura were thin and in two cases both were deformed and fractured.

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Osteogenesis Imperfecta and Hearing Loss

Otolaryngology ◽

10.1177/0194599812451426a346 ◽

2012 ◽

Vol 147 (2_suppl) ◽

pp. P233-P234 ◽

Cited By ~ 1

Author(s):

Jessica Moran-Hansen ◽

Paul Esposito ◽

Ryan K. Sewell

Keyword(s):

Hearing Loss ◽

Osteogenesis Imperfecta

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Surgical options for hearing loss in patients with osteogenesis imperfecta

Acta Otorrinolaringologica (English Edition) ◽

10.1016/s2173-5735(09)70115-2 ◽

2009 ◽

Vol 60 (2) ◽

pp. 126-130

Author(s):

Manuel Sainz ◽

Juan García-Valdecasas ◽

José M. Ballesteros

Keyword(s):

Hearing Loss ◽

Osteogenesis Imperfecta ◽

Surgical Options

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Successful bone-anchored hearing aid implantation in a patient with osteogenesis imperfecta

The Journal of Laryngology & Otology ◽

10.1017/s0022215115002510 ◽

2015 ◽

Vol 129 (11) ◽

pp. 1133-1136 ◽

Cited By ~ 4

Author(s):

M B Coutinho ◽

C Marques ◽

G J Mendes ◽

C Gonçalves

Keyword(s):

Hearing Loss ◽

Case Report ◽

Osteogenesis Imperfecta ◽

Frequency Analysis ◽

Hearing Aid ◽

Resonance Frequency Analysis ◽

Implant Stability ◽

Type Iii ◽

Mixed Hearing Loss ◽

Bone Anchored Hearing Aid

AbstractObjective:To report a case of successful bone-anchored hearing aid implantation in an adult patient with type III osteogenesis imperfecta, which is commonly regarded as a contraindication to this procedure.Case report:A 45-year-old man with type III osteogenesis imperfecta presented with mixed hearing loss. There was a mild sensorineural component in both ears, with an air–bone gap between 45 and 50 dB HL. He was implanted with a bone-anchored hearing aid. The audiological outcome was good, with no complications and good implant stability (as measured by resonance frequency analysis).Conclusion:To our knowledge, this is the first recorded case of bone-anchored hearing aid implantation in a patient with osteogenesis imperfecta.

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