Short and long term preservation of hearing thresholds corrected for natural hearing loss in cochlear implant recipients using a straight electrode.

The aim of this study is to investigate short and long term residual hearing preservation (HP), corrected for the natural progress of hearing loss, in cochlear implant (CI) patients receiving a straight electrode array using a round window (RW) approach. A retrospective and cross-sectional analysis on patients who received a CI with a straight electrode using a RW approach ( = 60) was performed. Audiometric data were obtained at three time points, preoperatively, at first fitting, and one year or more postoperatively.

Bilateral cochlear implantation or bimodal listening in the paediatric population: Retrospective analysis of decisive criteria.

Introduction: In children with bilateral severe to profound hearing loss, bilateral hearing can be achieved by either bimodal stimulation (CIHA) or bilateral cochlear implantation (BICI). The aim of this study was to analyse the audiologic test protocol that is currently applied to make decisions regarding the bilateral hearing modality in the paediatric population.

Methods: Pre- and postoperative audiologic test results of 21 CIHA, 19 sequential BICI and 12 simultaneous BICI children were examined retrospectively.

Phenotype of a Belgian Family With 6p25 Deletion Syndrome.

Background: The 6p25 deletion syndrome is one of the many syndromes with both hearing impairment as well as vision impairment. However, the audiometric characteristics and radiological findings of patients with 6p25 deletions are only scarcely described in literature. This study focused on characterizing the audiometric and radiological features of a Belgian family with a chromosome 6p25 deletion.

Temporal bone imaging in osteogenesis imperfecta patients with hearing loss.

Objectives/hypothesis: Osteogenesis imperfecta (OI) is an autosomal-dominant connective-tissue disorder, predominantly characterized by bone fragility. Conductive hearing loss develops in half of the OI patients and often progresses to mixed loss. Findings of computed tomography (CT) and magnetic resonance (MR) imaging of the temporal bone in the largest series of OI patients to date are presented and correlated with the audiograms.

Stapes surgery in osteogenesis imperfecta: retrospective analysis of 34 operated ears.

Intraoperative findings of stapes surgery in 34 ears from 22 patients with genetically confirmed osteogenesis imperfecta (OI) are reported, as well as the audiometric results after the longest postoperative follow-up published to date. Twenty-nine out of 34 ears underwent primary stapes surgery and 5 ears revision surgery. Postoperative audiometric follow-up ranged from 6 months to 37 years.

Association between bone mineral density and hearing loss in osteogenesis imperfecta.

Objectives/hypothesis: Osteogenesis imperfecta (OI) is a heritable connective tissue disorder, predominantly characterized by bone fragility. In half of the patients, progressive hearing loss develops, which is associated with abnormal bony changes involving the middle ear ossicles and stapes footplate. In the present study, we investigated whether the development of hearing loss in OI may be related to the overall aberrant bone quality.

Osteogenesis Imperfecta: the audiological phenotype lacks correlation with the genotype.

Background: Osteogenesis Imperfecta (OI) is a heritable connective tissue disorder mainly caused by mutations in the genes COL1A1 and COL1A2 and is associated with hearing loss in approximately half of the cases. The hearing impairment usually starts between the second and fourth decade of life as a conductive hearing loss, frequently evolving to mixed hearing loss thereafter. A minority of patients develop pure sensorineural hearing loss.

Audiologic phenotype of osteogenesis imperfecta: use in clinical differentiation.

Objectives: To describe the audiologic phenotype in osteogenesis imperfecta (OI).

Study Design: Observational study.

Setting: Tertiary referral center.

Audiometric, surgical, and genetic findings in 15 ears of patients with osteogenesis imperfecta.

Objectives/hypothesis: To provide data on the outcome of stapes surgery in patients with osteogenesis imperfecta (OI). The audiometric results of 15 ears (12 patients), in which a stapes operation was performed, are presented and compared with results from literature.

Study Design: Retrospective study.