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.

scholarly journals Assess the hearing of contralateral ear in patients with unilateral chronic squamosal otitis media

2020 ◽  
Vol 6 (5) ◽  
pp. 886
Author(s):  
Raphella Khan ◽  
Anirudh Kasliwal
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Otitis Media ◽  
Conductive Hearing Loss ◽  
Sensorineural Hearing ◽  
Retraction Pocket ◽  
Mixed Hearing Loss ◽  
Contralateral Ear ◽  
The Right ◽  
Conductive Hearing

<p class="abstract"><strong>Background:</strong> Chronic squamosal otitis media can occur due to many conditions affecting the middle ear. Most common sign of developing a chronic squamosal otitis media is formation of a retraction pocket in the tympanic membrane leading to further development of a cholesteatoma and if not treated properly, may lead to development of dangerous complication in the affected ear. These etiological factors may also affect the other ear. It is therefore very necessary to assess and diagnose the contralateral ear, so that the disease can be intervened and treated at the right time, to prevent any deterioration in hearing of the contralateral ear.</p><p class="abstract"><strong>Methods:</strong> The prospective study was done in 100 patients with unilateral chronic squamosal otitis media, where the contra lateral ear was examined and assessed for any hearing loss.  </p><p class="abstract"><strong>Results:</strong> We found hearing loss in the contra lateral ear ranging from mild conductive hearing loss to sensorineural hearing loss with the maximum patients with mild conductive hearing loss (42%) and lowest in sensorineural hearing loss (1%).  </p><p class="abstract"><strong>Conclusions:</strong> In our study, 76 patients were seen with conductive hearing loss. Out of that, 42% patients were seen with mild conductive hearing loss, 30% with moderate conductive hearing loss and 4% with severe conductive hearing loss. 20% patients were seen with normal hearing. 3% patients were seen with mixed hearing loss and only 1% patient was seen with sensorineural hearing loss in contralateral ear.</p>

scholarly journals Delayed-Onset NOG Gene-Related Syndromic Conductive Deafness: A Case Report

2020 ◽  
pp. 014556132094463
Author(s):  
Huiying Sun ◽  
Yufei Qiao ◽  
Na Chen ◽  
Hua Yang ◽  
Zhiqiang Gao ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Otoacoustic Emissions ◽  
Normal Development ◽  
Conductive Hearing Loss ◽  
Newborn Hearing Screening ◽  
Delayed Onset ◽  
Conductive Deafness ◽  
Newborn Hearing ◽  
Conductive Hearing

We report a 6-year-old girl with progressive bilateral conductive hearing loss for 2 years. She passed the newborn hearing screening conducted with otoacoustic emissions testing and had a normal development of speech and language, which indicated that her deafness was delayed-onset. She also had congenital proximal interphalangeal joints. Proximal symphalangism was confirmed by genetic testing ( NOG gene: c.406C > T, p.R136C). Bilateral stapes ankyloses were proved by surgery and her hearing was improved after stapedotomy by over 30 dB. Besides, this case should remind clinicians to carefully distinguish NOG gene-related deafness from congenital ossicular malformation and pediatric otosclerosis.

Another Cause for Conductive Hearing Loss With Present Acoustic Reflexes

2008 ◽  
Vol 118 (11) ◽  
pp. 2059-2061 ◽  
Author(s):  
Charles S. Ebert ◽  
Adam M. Zanation ◽  
Craig A. Buchman
Keyword(s):  
Hearing Loss ◽  
Conductive Hearing Loss ◽  
Acoustic Reflexes ◽  
Conductive Hearing

Osteogenesis Imperfecta Congenita and Tarda: A Temporal Bone Report

1975 ◽  
Vol 84 (3) ◽  
pp. 350-358 ◽  
Author(s):  
Joan T. Zajtchuk ◽  
John R. Lindsay
Keyword(s):  
Hearing Loss ◽  
Osteogenesis Imperfecta ◽  
Temporal Bone ◽  
Histological Examination ◽  
Conductive Hearing Loss ◽  
Conductive Hearing ◽  
Osteogenesis Imperfecta Congenita ◽  
Endochondral Layer

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.

The relationship between post-traumatic ossicular injuries and conductive hearing loss: A 3D-CT study

2017 ◽  
Vol 44 (5) ◽  
pp. 333-338 ◽  
Author(s):  
Olivier Maillot ◽  
Arnaud Attyé ◽  
Claire Boutet ◽  
Kamel Boubagra ◽  
Romain Perolat ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Conductive Hearing Loss ◽  
3D Ct ◽  
Post Traumatic ◽  
The Relationship ◽  
Conductive Hearing

Conductive hearing loss: investigation of possible inner ear origin in three cases studies

2002 ◽  
Vol 116 (11) ◽  
pp. 942-945 ◽  
Author(s):  
Hamad Al Muhaimeed ◽  
Yousry El Sayed ◽  
Abdulrahman Rabah ◽  
Abdulrahman Al-Essa
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Conductive Hearing Loss ◽  
Round Window ◽  
The Third ◽  
Conductive Deafness ◽  
Mixed Hearing Loss ◽  
Stapedial Reflex ◽  
Conductive Hearing ◽  
Inner Ear Disorders

This is a report of three cases of mixed hearing loss that resulted from inner ear disorders. Two cases were unilateral and the third was bilateral. The diagnosis was based on the findings of normal middle and external ears in association with the absence of round window reflexes. The contralateral stapedial reflex was present in the two unilateral cases. This is the first documentation of conductive deafness due to inner ear abnormality. This diagnosis should be considered in cases of conductive hearing loss if the middle and external ears are normal. More studies are needed to establish the pathophysiology of this entity.

Bone Conduction Implants: Transcutaneous vs. Percutaneous

1992 ◽  
Vol 106 (1) ◽  
pp. 68-74 ◽  
Author(s):  
Phillip S. Wade ◽  
Jerry J. Halik ◽  
Marshall Chasin
Keyword(s):  
Clinical Trial ◽  
Hearing Loss ◽  
Pure Tone ◽  
Conductive Hearing Loss ◽  
Bone Conduction ◽  
Mixed Hearing Loss ◽  
Hearing Devices ◽  
Bone Anchored Hearing Aid ◽  
Bone Conduction Hearing ◽  
Conductive Hearing

Clinical experience with transcutaneous bone conduction implants has demonstrated that they are most beneficial for patients with purely conductive hearing loss in at least one ear. Percutaneous bone conduction implants, however, have been reported to provide adequate benefit for patients with mixed hearing loss with bone conduction pure-tone averages up to 45 db hl (Tjellstrom, 1989). The results of 24 Xomed Audiant osseointegrated bone conduction hearing devices (including a clinical trial on two patients using a new, larger magnet [Neodynium Iron Boron]), plus the results of eleven patients implanted and fitted with the percutaneous bone-anchored hearing aid are reported. Aided results with these devices will be presented. In addition, general comparisons of benefit obtained with the two devices will be made for patients who exhibit similar hearing losses. Finally, a direct comparison will be made on two patients who have undergone both implant procedures.

Audiologic and Otologic Clinical Manifestations of Loeys-Dietz Syndrome: A Heritable Connective Tissue Disorder

2021 ◽  
pp. 019459982110088
Author(s):  
Jun W. Jeon ◽  
Julie Christensen ◽  
Jennifer Chisholm ◽  
Christopher Zalewski ◽  
Marjohn Rasooly ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Connective Tissue ◽  
Sensorineural Hearing Loss ◽  
Tympanic Membrane ◽  
Conductive Hearing Loss ◽  
Wide Spectrum ◽  
Clinical Manifestations ◽  
Connective Tissue Disorder ◽  
Sensorineural Hearing ◽  
Conductive Hearing

Objective Loeys-Dietz syndrome (LDS) is a rare genetic connective tissue disorder resulting from TGF-ß signaling pathway defects and characterized by a wide spectrum of aortic aneurysm, arterial tortuosity, and various extravascular abnormalities. This study describes the audiologic, otologic, and craniofacial manifestations of LDS. Study Design Consecutive cross-sectional study. Setting Tertiary medical research institute. Methods Audiologic and clinical evaluations were conducted among 36 patients (mean ± SD age, 24 ± 17 years; 54% female) with genetically confirmed LDS. Cases were categorized into genetically based LDS types 1 to 4 ( TGFBR1, TGFBR2, SMAD3, TGFB2, respectively). Audiometric characteristics included degree and type of hearing loss: subclinical, conductive, mixed, and sensorineural. Results LDS types 1 to 4 included 11, 13, 5, and 7 patients, respectively. In LDS-1, 27% had bilateral conductive hearing loss; 9%, unilateral mixed; and 36%, subclinical. In LDS-2, 38% had conductive hearing loss and 38% subclinical. In LDS-3 and LDS-4, 40% and 43% had bilateral sensorineural hearing loss, respectively. Degree of hearing loss ranged from mild to moderate. Bifid uvula was observed only in LDS-1 (55%) and LDS-2 (62%). Submucosal/hard cleft palates were primarily in LDS-1 and LDS-2. Posttympanostomy tympanic membrane perforations occurred in 45% (10/22 ears) of LDS-1 and LDS-2. There were 4 cases of cholesteatoma: 3 middle ear (LDS-1 and LDS-2) and 1 external ear canal (LDS-3). Conclusion Conductive hearing loss, bifid uvula/cleft palate, and posttympanostomy tympanic membrane perforation are more common in LDS-1 and LDS-2 than LDS-3 and LDS-4, while sensorineural hearing loss was present only in LDS-3 and LDS-4. These LDS-associated key clinical presentations may facilitate an early diagnosis of LDS and thus prompt intervention to prevent related detrimental outcomes.

scholarly journals Significance of Pseudo-conductive Hearing Loss and Positional Nystagmus on Perilymphatic Fistula: Are they related to third window effects?

2021 ◽  
Author(s):  
Jong Sei Kim ◽  
Se-Eun Son ◽  
Min Bum Kim ◽  
Young Sang Cho ◽  
Won-Ho Chung
Keyword(s):  
Hearing Loss ◽  
Clinical Features ◽  
Conductive Hearing Loss ◽  
Hearing Threshold ◽  
Positional Nystagmus ◽  
Horizontal Semicircular Canal ◽  
Perilymphatic Fistula ◽  
The Relationship ◽  
Conductive Hearing ◽  
Lower Frequencies

Objectives. Clinical presentation is critical to identify suspected perilymphatic fistula (PLF). To explain characteristic of PLF, it was hypothesized that a third window lesion might be involved in the pathomechanism. The purpose of this study is to investigate clinical features in PLF and figure out the relationship of third window effect and PLF. Methods. Sixty patients underwent surgical exploration under suspicion of PLF and reinforecmenet of oval window and round window was performed. Clinical features including demographics, pure tone audiometry (PTA), and videonystagmography (VNG) were evaluated preoperatively and 1 month after operation. Surgical outcomes were analyzed according to the improvement of hearing and vestibular symptoms and signs. The conductive components of PTA (air-bone gap (ABG)) were measured, and the relationship between ABG closure after surgery and hearing improvement was analyzed. In addition, the postoperative subjective dizziness was assessed by interview in clinic. The change of positional nystagmus was analyzed according to ABG closure and hearing improvement.Results. ABG at lower frequencies (LFABG, 250 Hz, 500 Hz, 1,000 Hz) was present in 27 patients (45%). Postoperatively, the PTA was significantly improved after surgical repair. Among the patients with preoperative LFABG (n=27), 15 (55.5%) showed postoperative ABG closure and significant improvement in PTA at all frequencies compared with the patients without ABG closure (P=0.012). The subjective dizziness improved in 56 patients (91.8%). Positional nystagmus was found in 45 of 49 patients. Multiple canal involvement was more common than single canal (67% vs. 33%). The horizontal semicircular canal was most commonly involved, followed by the posterior and anterior canals. Postoperatively, the positional nystagmus disappeared, or the number of involved canals decreased in 22 of 34 (64.7%) patients. Conclusions. Pseudo-conductive hearing loss at the lower frequencies and positional nystagmus originating from multiple semicircular canals were common findings in PLF. Surgical reinforcement of the RW and OW improved hearing threshold accompanied by closure of ABG. The third window lesion might explain these clinical features that indicate PLF.

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