Stapedotomy in osteogenesis imperfecta patients

2000 ◽  
Vol 114 (6) ◽  
pp. 424-428 ◽  
Author(s):  
E. Ferekidis ◽  
P. Stavroulaki ◽  
I. Vossinakis ◽  
J. Yiotakis ◽  
L. Manolopoulos ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Osteogenesis Imperfecta ◽  
Bone Conduction ◽  
Connective Tissue Disorder ◽  
Long Term Results ◽  
Mixed Hearing Loss ◽  
Post Test ◽  
Operative Findings ◽  
Blue Sclerae ◽  
The Impact

Osteogenesis imperfecta (OI) is a connective tissue disorder characterized by osseous fragility, blue sclerae and hearing loss. In order to assess the impact of stapedotomy on improving hearing on OI, a retrospective, one-group, pre-test-post-test design was used to compare the pre-operative and postoperative audiograms of nine OI patients, treated with stapedotomy for their mixed hearing loss. Operative findings included fixation or thickening of the stapes footplate with normal superstructure configuration and hypervascularization of the promontory mucosa. Immediate post-operative results showed a significant improvement (p<0.05) from 250–4000 Hz in air conduction and from 250–2000 Hz in bone conduction. A significant closure of the air-bone gap between 250–2000 Hz was also achieved (p<0.05). The long-term results remained satisfactory with a mean threshold shift of 8 dB HL and an almost unchanged air-bone gap. These satisfactory results and the lack of complications make stapedotomy an appealing method for the management of OI-associated hearing loss.

Audiological Findings in Osteogenesis Imperfecta

2008 ◽  
Vol 19 (08) ◽  
pp. 595-601 ◽  
Author(s):  
Joseph P. Pillion ◽  
Jay Shapiro
Keyword(s):  
Hearing Loss ◽  
Osteogenesis Imperfecta ◽  
Otoacoustic Emissions ◽  
Conductive Hearing Loss ◽  
Connective Tissue Disorder ◽  
Mixed Hearing Loss ◽  
Younger Patient ◽  
Acoustic Reflexes ◽  
The Relationship ◽  
Conductive Hearing

Background: Osteogenesis imperfecta (OI) is an inherited bone and connective tissue disorder associated with the lifelong occurrence of frequent fractures following even mild trauma. Hearing loss is frequently reported in patients with OI. Purpose: This investigation is a retrospective study of measurements of tympanometry, acoustic reflexes, and transient evoked otoacoustic emissions in a sample of patients with OI grouped according to age. The purpose of the study was to examine the relationship between the type of OI, age, and audiological findings in a sample of individuals with OI. Research Design: The study examined the correlation between audiometric measures, hearing loss of all types, and type of OI. Study Sample: Forty-one patients with OI were included in the study. The patients were divided into two groups for analysis: one group less than 20 years of age (n = 21) and the other group aged 20 and over (n = 20). Results: Hearing loss of all etiologies was observed in 62% of ears. Sensorineural or mixed hearing loss was observed in 41% and conductive hearing loss in 21% of ears. Results indicate that the younger patients with OI were subject to a greater risk of middle ear dysfunction associated with otitis media than is typical for children of comparable age. Tympanometric abnormalities associated with ossicular dysfunction were more often found in the older age group of patients. Hearing loss of all types was more prevalent in the older group of patients (88%) than in the younger patient group (38%). Conclusions: Hearing loss was not uncommon regardless of age. Therefore, close audiological monitoring of patients with OI is recommended across the age spectrum.

Piezoelectric bone conduction hearing implant Osia® - audiological and quality of life benefits

2021 ◽  
Vol 75 (4) ◽  
pp. 1-5
Author(s):  
Joanna Marszał ◽  
Renata Gibasiewicz ◽  
Magdalena Błaszczyk ◽  
Maria Gawlowska ◽  
Wojciech Gawęcki
Keyword(s):  
Quality Of Life ◽  
Hearing Loss ◽  
Hearing Aids ◽  
Bone Conduction ◽  
Speech Understanding ◽  
Mixed Hearing Loss ◽  
Wide Range ◽  
The Mean ◽  
Bone Conduction Hearing

Introduction: Nowadays, there are many options to treat hearing-impaired patients: tympanoplastic surgery, hearing aids and a wide range of implantable devices. Objective: The aim of this study is to present the mid-term audiological and quality of life benefits after the implantation of the Osia®, a new active piezoelectric bone conduction hearing implant. Material and methods: The state of the tissues in implanted area, as well as audiological and quality of life results were analyzed six, nine and twelve months after implantation in a group of four adult patients with bilateral mixed hearing loss. Results: In all the cases, no postoperative complications were found. One year after surgery the mean audiological gain in FF PTA4 (pure tone average for 0.5, 1, 2, and 4 kHz) was 52.2±3.5 dB in comparison to the unaided situation, the mean speech understanding with Osia® in quiet was 90±8.2% for 50dB SPL, 98.8±2.5% for 65dB SPL and 100±0% for 80dB SPL, and mean speech understanding with Osia® in noise was 37.5%±23.6 for 50dB SPL, 93.8±4.8% for 65dB SPL and 98.8±2.5% for 80dB SPL. There was also an evident improvement in the quality of hearing as well as in the quality of life, measured by the APHAB (Abbreviated Profile of Hearing Aid Benefit) and the SSQ (Speech, Spatial and Qualities of Hearing Scale). Conclusions: The Osia® is an effective treatment option for patients with bilateral mixed hearing loss. The mid-term audiological and quality of life results are excellent, but further observations including bigger groups of patients and a longer follow-up are required.

Sensorineural Impairment in Unilateral Otosclerosis

1975 ◽  
Vol 84 (1) ◽  
pp. 11-15 ◽  
Author(s):  
Fred H. Linthicum ◽  
A. S. Lalani
Keyword(s):  
Hearing Loss ◽  
Bone Conduction ◽  
Significant Elevation ◽  
Bone Conduction Threshold ◽  
Mixed Hearing Loss ◽  
Temporal Bones

Audiometric findings were evaluated in a group of patients with clinical unilateral otosclerosis. A comparison was made between bone conduction threshold in the better hearing ear and the ear with the mixed hearing loss. Over 50% of the patients had a significant elevation in the bone conduction thresholds in the ear with clinical otosclerosis as compared to the uninvolved ear. A pair of temporal bones from a patient with unilateral otosclerosis and unilateral mixed hearing loss is presented.

scholarly journals Minimal invasive pocket technique for magnet bone implant hearing aid without fixation

2019 ◽  
Vol 277 (3) ◽  
pp. 715-725
Author(s):  
Pierre Dolhen ◽  
Samuel Lipski ◽  
Rachid Touijar ◽  
Juliette Van Bogaert
Keyword(s):  
Hearing Loss ◽  
Surgical Technique ◽  
Hearing Aid ◽  
Bone Conduction ◽  
Magnetic Coupling ◽  
Skin Incision ◽  
Medical Systems ◽  
Bone Implant ◽  
Mixed Hearing Loss ◽  
Baha Attract

Abstract Introduction The BAHA (bone-anchored hearing aid) Attract is a magnetic transcutaneous bone conduction device anchored into the temporal bone. The standard surgical technique for BAHA Attract is a multi-tools time-consuming process, which requires a large cutaneous incision. The objective of this study is to describe and test the feasibility of a minimally invasive pocket (MIP) technique for Magnet Bone Implant Hearing Aid (MBIHA) with a modified magnet of BAHA Attract without fixation and without any tissue reduction. We use a 3-cm vertical skin incision and a subperiosteal pocket. Method A study of 10 patients with conductive or mixed hearing loss who benefited from a MBIHA using the MIP technique is presented. The pure tone average (PTA) (dB) for air-conduction thresholds and the speech recognition threshold (SRT) (dB) in speech audiometry in quiet are calculated. The Entific Medical Systems (EMS) questionnaire and the postoperative clinical outcomes are realized. Results We found a significant improvement of 33.8 dB on average for the PTA and 44.8 dB for the SRT with MBIHA at 3 months, compared with unaided situation. No implant was removed or displaced after 2 years of follow-up. The skin condition remains intact in all the cases. Conclusion The minimally subperiosteal pocket surgical technique MIP without fixation and with tissue preservation for the MBIHA is safe, rapid and effective for patients with conductive or mixed hearing loss. It opens new perspectives of development and modify conventional concept in magnetic coupling of bone-conducted device.

Comparison of Sound Processing Strategies for Osseointegrated Bone Conduction Implants in Mixed Hearing Loss

2013 ◽  
Vol 34 (4) ◽  
pp. 598-603 ◽  
Author(s):  
Jolien B. J. Desmet ◽  
Arjan J. Bosman ◽  
Ad F. M. Snik ◽  
Peggy Lambrechts ◽  
Myrthe K. S. Hol ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Bone Conduction ◽  
Sound Processing ◽  
Mixed Hearing Loss ◽  
Processing Strategies

scholarly journals Identification of a novel heterozygous mutation in exon 50 of the COL1A1 gene causing osteogenesis imperfecta

2013 ◽  
Vol 2013 ◽  
Author(s):  
S A S Aftab ◽  
N Reddy ◽  
N L Owen ◽  
R Pollitt ◽  
A Harte ◽  
...  
Keyword(s):  
Osteogenesis Imperfecta ◽  
Health Care Professionals ◽  
Stop Codon ◽  
Connective Tissue Disorder ◽  
Heterozygous Mutation ◽  
Team Approach ◽  
List Type ◽  
Gene Coding ◽  
Metabolic Bone ◽  
Blue Sclerae

Summary A 19-year-old woman was diagnosed with osteogenesis imperfecta (OI). She had sustained numerous low-trauma fractures throughout her childhood, including a recent pelvic fracture (superior and inferior ramus) following a low-impact fall. She had the classical blue sclerae, and dual energy X-ray absorptiometry (DEXA) bone scanning confirmed low bone mass for her age in the lumbar spine (Z-score was −2.6). However, despite these classical clinical features, the diagnosis of OI had not been entertained throughout the whole of her childhood. Sequencing of her genomic DNA revealed that she was heterozygous for the c.3880_3883dup mutation in exon 50 of the COL1A1 gene. This mutation is predicted to result in a frameshift at p.Thr1295, and truncating stop codon 3 amino acids downstream. To our knowledge, this mutation has not previously been reported in OI. Learning points OI is a rare but important genetic metabolic bone and connective tissue disorder that manifests a diverse clinical phenotype that includes recurrent low-impact fractures. Most mutations that underlie OI occur within exon 50 of the COL1A1 gene (coding for protein constituents of type 1 pro-collagen). The diagnosis of OI is easily missed in its mild form. Early diagnosis is important, and there is a need for improved awareness of OI among health care professionals. OI is a diagnosis of exclusion, although the key diagnostic criterion is through genetic testing for mutations within the COL1A1 gene. Effective management of OI should be instituted through a multidisciplinary team approach that includes a bone specialist (usually an endocrinologist or rheumatologist), a geneticist, an audiometrist and a genetic counsellor. Physiotherapy and orthopaedic surgery may also be required.

scholarly journals The Bonebridge BCI 602 Active Transcutaneous Bone Conduction Implant in Children: Objective and Subjective Benefits

2021 ◽  
Vol 10 (24) ◽  
pp. 5916
Author(s):  
Katarzyna B. Cywka ◽  
Henryk Skarżyński ◽  
Bartłomiej Król ◽  
Piotr H. Skarżyński
Keyword(s):  
Hearing Loss ◽  
Word Recognition ◽  
Middle Ear ◽  
Bone Conduction ◽  
Free Field ◽  
Subjective Assessment ◽  
Congenital Defects ◽  
Mixed Hearing Loss ◽  
Speech Reception ◽  
Bone Conduction Implant

Background: the Bonebridge hearing implant is an active transcutaneous bone conduction implant suitable for various types of hearing loss. It was first launched in 2012 as the BCI 601, with a newer internal part (BCI 602) released in 2019. With the new size and shape, the BCI 602 can be used in patients previously excluded due to insufficient anatomical conditions, especially in patients with congenital defects of the outer and middle ear. Objectives: the purpose of this study is to evaluate the objective and subjective benefits of the new Bonebridge BCI 602 in children who have hearing impairment due to conductive or mixed hearing loss. Safety and effectiveness of the device was assessed. Methods: the study group included 22 children aged 8–18 years (mean age 14.7 years) who had either conductive or mixed hearing loss. All patients were implanted unilaterally with the new Bonebridge BCI 602 implant. Pure tone audiometry, speech recognition tests (in quiet and noise), and free-field audiometry were performed before and after implantation. Word recognition scores were evaluated using the Demenko and Pruszewicz Polish Monosyllabic Word Test, and speech reception thresholds in noise were assessed using the Polish Sentence Matrix Test. The subjective assessment of benefits was carried outusing the APHAB (Abbreviated Profile of Hearing Aid Benefit) questionnaire. Results: after implantation of the Bonebridge BCI 602 all patients showed a statistically significant improvement in hearing and speech understanding. The mean word recognition score (WRS) changed from 12.1% before implantation to 87.3% after 6 months. Mean speech reception threshold (SRT) before implantation was +4.79 dB SNR and improved to −1.29 dB SNR after 6 months. All patients showed stable postoperative results. The APHAB questionnaire showed that difficulties in hearing decreased after implantation, with a statistically significant improvement in global score. Pre-operative scores (M = 35.7) were significantly worse than post-operative scores at 6 months (M = 25.7). Conclusions: the present study confirms that the Bonebridge BCI 602 is an innovative and effective solution, especially for patients with conductive and mixed hearing loss due to anatomical ear defects. The Bonebridge BCI 602 system provides valuable and stable audiological and surgical benefits. Subjective assessment also confirms the effectiveness of the BCI 602. The BCI 602 offers the same amplification as the BCI601, but with a smaller size. The smaller dimensions make it an effective treatment option for a wider group of patients, especially children with congenital defects of the outer and middle ear.

Sign in / Sign up

Export Citation Format

Share Document