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.

Sensorineural and conductive hearing loss in infants diagnosed in the program of universal newborn hearing screening

2018 ◽  
Vol 105 ◽  
pp. 181-186 ◽  
Author(s):  
Katarzyna Wroblewska-Seniuk ◽  
Piotr Dabrowski ◽  
Grazyna Greczka ◽  
Katarzyna Szabatowska ◽  
Agata Glowacka ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Conductive Hearing Loss ◽  
Hearing Screening ◽  
Newborn Hearing Screening ◽  
Universal Newborn Hearing Screening ◽  
Newborn Hearing ◽  
Conductive Hearing

Conductive Hearing Loss and Middle Ear Pathology in Young Infants Referred through a Newborn Universal Hearing Screening Program in Australia

2012 ◽  
Vol 23 (09) ◽  
pp. 673-685 ◽  
Author(s):  
Sreedevi Aithal ◽  
Venkatesh Aithal ◽  
Joseph Kei ◽  
Carlie Driscoll
Keyword(s):  
Hearing Loss ◽  
Middle Ear ◽  
Chart Review ◽  
Conductive Hearing Loss ◽  
Screening Program ◽  
Hearing Screening ◽  
Diagnostic Assessment ◽  
Newborn Hearing Screening ◽  
Newborn Hearing ◽  
Conductive Hearing

Background: Although newborn hearing screening programs have been introduced in most states in Australia, the prevalence of conductive hearing loss and middle ear pathology in the infants referred through these programs is not known. Purpose: This study was designed to (1) evaluate the prevalence of conductive hearing loss and middle ear pathology in infants referred by a newborn hearing screening program in north Queensland, (2) compare prevalence rates of conductive hearing loss and middle ear pathology in indigenous and nonindigenous infants, and (3) review the outcomes of those infants diagnosed with conductive hearing loss and middle ear pathology. Research Design: Retrospective chart review of infants referred to the Audiology Department of The Townsville Hospital was conducted. Study Sample: Chart review of 234 infants referred for one or both ears from a newborn hearing screening program in north Queensland was conducted. A total of 211 infants attended the diagnostic appointment. Review appointments to monitor hearing status were completed for 46 infants with middle ear pathology or conductive hearing loss. Data Collection and Analysis: Diagnosis of hearing impairment was made using an age-appropriate battery of audiological tests. Results were analyzed for both initial and review appointments. Results: Mean age at initial diagnostic assessment was 47.5 days (SD = 31.3). Of the 69 infants with middle ear pathology during initial diagnostic assessment, 18 had middle ear pathology with normal hearing, 47 had conductive hearing loss, and 4 had mixed hearing loss. Prevalence of conductive hearing loss in the newborns was 2.97 per 1,000 while prevalence of middle ear pathology (with or without conductive hearing loss) was 4.36 per 1,000. Indigenous Australians or Aboriginal and Torres Strait Islander (ATSI) infants had a significantly higher prevalence of conductive hearing loss and middle ear pathology than non-ATSI infants (35.19 and 44.45% vs 17.83 and 28.66%, respectively). ATSI infants also showed poor resolution of conductive hearing loss over time with 66.67% of ATSI infants reviewed showing persistent conductive hearing loss compared to 17.86% of non-ATSI infants. Medical management of 17 infants with persistent conductive hearing loss included monitoring, antibiotic treatment, examination under anesthesia, and grommet insertion. Conclusions: Conductive hearing loss was found to be a common diagnosis among infants referred through screening. ATSI infants had significantly higher rates of middle ear pathology and conductive hearing loss at birth and showed poor resolution of middle ear pathology over time compared to non-ATSI infants. Future research using a direct measure of middle ear function as an adjunct to the automated auditory brainstem response screening tool to distinguish conductive from sensorineural hearing loss may facilitate prioritization of infants for assessment, thus reducing parental anxiety and streamlining the management strategies for the respective types of hearing loss.

scholarly journals Successive ipsilateral surgery of Vibrant Soundbridge and Bonebridge devices for congenital bilateral conductive hearing loss: a case report

2020 ◽  
Vol 48 (12) ◽  
pp. 030006052097228
Author(s):  
Yujie Liu ◽  
Ran Ren ◽  
Shouqin Zhao
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Conductive Hearing Loss ◽  
Vibrant Soundbridge ◽  
Patient’S Experience ◽  
Hearing Devices ◽  
Conductive Hearing ◽  
Subjective Outcomes

The Bonebridge and Vibrant Soundbridge systems are semi-implanted hearing devices, which have been widely applied in patients with congenital conductive hearing loss. However, comparison between these two hearing devices is rare, especially in the same patient. We report a 23-year-old man who underwent successive implantation of Vibrant Soundbridge and Bonebridge devices in the same ear because of dysfunction of the Vibrant Soundbridge. We provide insight on the patient’s experience and compare the audiological and subjective outcomes of satisfaction.

The first reported treatment of Nager syndrome associated hearing loss with bone-anchored hearing aids: case report

2011 ◽  
Vol 126 (1) ◽  
pp. 76-78 ◽  
Author(s):  
G P Davies ◽  
I J M Johnson
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Literature Review ◽  
Current Literature ◽  
Hearing Aids ◽  
Clinical Case ◽  
Conductive Hearing Loss ◽  
First Case ◽  
Nager Syndrome ◽  
Conductive Hearing

AbstractObjective:To report the first case of treatment of Nager syndrome associated conductive hearing loss with bone-anchored hearing aids, in a three-year-old boy.Method:Clinical case report and current literature review regarding the use of bone-anchored hearing aids in the treatment of conductive hearing loss in children.Results:A three year eight month old boy with Nager syndrome was successfully treated for conductive hearing loss using bilateral bone-anchored hearing aids.Conclusion:This is the first case report of the use of bone-anchored hearing aids to treat Nager syndrome associated conductive hearing loss. Treatment was safe and successful in this case.

scholarly journals Middle Ear Adenoma: Case Report and Discussion

2014 ◽  
Vol 2014 ◽  
pp. 1-4
Author(s):  
D. Isenring ◽  
T. F. Pezier ◽  
B. Vrugt ◽  
A. M. Huber
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Middle Ear ◽  
Conductive Hearing Loss ◽  
Surgical Excision ◽  
Histological Techniques ◽  
Preoperative Ct ◽  
Middle Ear Adenoma ◽  
Conductive Hearing ◽  
Ct And Mri

Introduction. Despite modern radiological workup, surgeons can still be surprised by intraoperative findings or by the pathologist’s report.Materials & Methods. We describe the case of a 52-year-old male who was referred to our clinic with a single sided conductive hearing loss. He ultimately underwent middle ear exploration and excision of a middle ear tumour followed by second look and ossiculoplasty a year later.Results. Though preoperative CT and MRI scanning were suggestive of a congenital cholesteatoma, the pathologist’s report diagnosed a middle ear adenoma.Discussion. Middle ear glandular tumors are extremely rare and, despite numerous histological techniques, continue to defy satisfactory classification. Most surgeons advocate surgical excision though evidence of the tumour’s natural course and risk of recurrence is lacking.

Modified sleeve tympanotomy approach for removal of congenital cholesteatoma

2008 ◽  
Vol 122 (12) ◽  
pp. 1365-1367 ◽  
Author(s):  
H J Park ◽  
G H Park ◽  
J E Shin ◽  
S O Chang
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Tympanic Membrane ◽  
Middle Ear ◽  
Conductive Hearing Loss ◽  
Tympanic Cavity ◽  
Operative Techniques ◽  
Congenital Cholesteatoma ◽  
The Right ◽  
Conductive Hearing

AbstractObjective:We present a technique which we have found useful for the management of congenital cholesteatoma extensively involving the middle ear.Case report:A five-year-old boy was presented to our department for management of a white mass on the right tympanic membrane. This congenital cholesteatoma extensively occupied the tympanic cavity. It was removed through an extended tympanotomy approach using our modified sleeve technique. The conventional tympanotomy approach was extended by gently separating the tympanic annulus from its sulcus in a circular manner. The firm attachment of the tympanic membrane at the umbo was not severed, in order to avoid lateralisation of the tympanic membrane.Conclusion:Although various operative techniques can be used, our modified sleeve tympanotomy approach provides a similarly sufficient and direct visualisation of the entire middle ear, with, theoretically, no possibility of lateralisation of the tympanic membrane and subsequent conductive hearing loss.

The Use of a Custom Earmold to Prevent Recurrent External Auditory Canal Stenosis

2008 ◽  
Vol 19 (03) ◽  
pp. 233-236 ◽  
Author(s):  
Christopher Garvey ◽  
Heather Turner
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
External Auditory Canal ◽  
Conductive Hearing Loss ◽  
Canal Stenosis ◽  
Conductive Hearing

The treatment of external auditory canal (EAC) stenoses often requires the prolonged use of a stent or splint. Traditional stents often occlude the EAC, resulting in a temporary conductive hearing loss. This case report describes a custom canal earmold with a large bore used as a stent in a patient with EAC stenosis. The customized earmold stent successfully prevented restenosis, while the large bore provided ventilation and improved hearing subjectively during the stenting phase. El tratamiento de la estenosis del conducto auditivo externo (EAC) a menudo requiere del uso prolongado de un stent o una férula. Los stents tradicionales a menudo ocluyen el EAC, provocando una hipoacusia conductiva temporal. Este reporte describe un caso un molde auditivo hecho a la medida con un agujero grande utilizado como stent en un paciente con estenosis del EAC. El stent de molde auditivo a la medida fue exitoso en prevenir la re-estenosis, mientras que el gran agujero aportó ventilación y mejoró subjetivamente la audición durante la fase de mantenimiento del stent.

Bilateral conductive deafness related to erosive lichen planus

1998 ◽  
Vol 112 (4) ◽  
pp. 365-366 ◽  
Author(s):  
Ludovic Martin ◽  
Sylvain Moriniere ◽  
Marie-Christine Machet ◽  
Alain Robier ◽  
Loïc Vaillant
Keyword(s):  
Hearing Loss ◽  
Lichen Planus ◽  
Conductive Hearing Loss ◽  
External Ear ◽  
Conductive Deafness ◽  
Erosive Lichen Planus ◽  
Conductive Hearing

AbstractA case of bilateral progressive stenosis of both external auditory canals with resultant conductive hearing loss is presented. The stenosis revealed multifocal erosive and synechiant lichen planus. To our knowledge, this is the first reported case of lichen planus involvement of the external ear.

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