Congenital stapedial suprastructure fixation with normal footplate mobility: case report

2009 ◽  
Vol 124 (6) ◽  
pp. 680-683 ◽  
Author(s):  
J H Lee ◽  
S H Jung ◽  
H C Kim ◽  
C H Park ◽  
S M Hong
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Rare Case ◽  
Conductive Hearing Loss ◽  
Branchial Arch ◽  
Fallopian Canal ◽  
Stapes Footplate ◽  
The Right ◽  
Conductive Hearing

AbstractObjective:We report a case of bilateral conductive hearing loss caused by stapedial suprastructure fixation with normal footplate mobility.Case report:A 50-year-old woman had suffered hearing loss in both ears since childhood. Exploratory tympanotomy revealed immobility of the stapes due to a bony bridge between the stapedial suprastructure and the fallopian canal. The incus was missing, while the malleus handle was minimally deformed. The mobility of the stapes footplate was normal. Post-operatively, the hearing in the right ear improved both subjectively and audiographically, while that in the left ear did not improve because of footplate subluxation during surgery.Conclusion:This is a rare case of congenital stapedial suprastructure fixation with normal footplate mobility. In this patient, development of the second branchial arch was arrested. When performing exploratory tympanotomy for stapedial fixation, one must keep in mind that normal footplate mobility is possible.

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.

A rare case of primary otoscleroma of the middle ear

2012 ◽  
Vol 126 (12) ◽  
pp. 1276-1277
Author(s):  
A R Kakeri ◽  
A H Patel
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Middle Ear ◽  
World Literature ◽  
Rare Case ◽  
Conductive Hearing Loss ◽  
Histopathological Examination ◽  
Suppurative Otitis Media ◽  
The World ◽  
Conductive Hearing

AbstractObjective:We report an extremely rare case of primary otoscleroma.Method:We present a case report and a review of the world literature concerning otoscleroma.Results:An adult woman presented with chronic suppurative otitis media with tubotympanic disease and conductive hearing loss. On mastoid exploration, dark granulations were seen, which were identified as otoscleroma on histopathological examination. The patient responded well to streptomycin.Conclusion:To the best of our knowledge, this is the first report of primary otoscleroma in the world literature. This case indicates that Frisch's bacillus can also spread to the middle ear.

Malleo-incudal osteoma: an unexpected finding during surgery for presumed otosclerosis

2011 ◽  
Vol 125 (9) ◽  
pp. 968-969 ◽  
Author(s):  
K Ramdoo ◽  
O T Dale ◽  
R C D Herdman
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Literature Review ◽  
Clinical Diagnosis ◽  
Rare Case ◽  
Conductive Hearing Loss ◽  
Ossicular Chain ◽  
Unexpected Finding ◽  
Conductive Hearing

AbstractObjective:To report a rare case of a symptomatic malleo-incudal osteoma, and to highlight the difficulties in making the clinical diagnosis.Method:Case report and literature review.Results:Malleo-incudal osteoma is a rare cause of unilateral conductive hearing loss. Its symptoms may mimic those of other otological causes of conductive hearing loss, such as otosclerosis.Conclusion:This case report highlights the challenges involved in establishing a clinical diagnosis of malleo-incudal osteoma. It also emphasises the importance of assessing the mobility of the divided ossicular chain during a planned stapedectomy.

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.

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>

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.

scholarly journals Otosclerosis. 2. rész. Diagnózis

2019 ◽  
Vol 160 (51) ◽  
pp. 2007-2011
Author(s):  
Balázs Liktor ◽  
Andor Hirschberg ◽  
Bálint ifj Liktor ◽  
Tamás Karosi
Keyword(s):  
Hearing Loss ◽  
Tympanic Membrane ◽  
Preoperative Diagnosis ◽  
Conductive Hearing Loss ◽  
Clinical Suspicion ◽  
Otic Capsule ◽  
Human Temporal Bone ◽  
Stapes Footplate ◽  
Bony Lesions ◽  
Conductive Hearing

Abstract: Otosclerosis is a bone remodeling disorder affecting exclusively the human temporal bone which causes small bony lesions in the otic capsule. The symptoms depend on the location and the extent of the otosclerotic foci. Hence, clinically the most relevant sign is the conductive hearing loss due to the stapedial otosclerosis with fixation of the stapes footplate. In many cases, the specific anamnestic features, the age of presentation and usually the absence of tympanic membrane pathology can provide a strong clinical suspicion for otosclerosis. Although audiometric and imaging examinations and VEMP testing can confirm our preoperative diagnosis, the histolopathologic examination of the removed stapes footplate is the most accurate way to determine the diagnosis. Orv Hetil. 2019; 160(51): 2007–2011.

scholarly journals A Start Codon Variant in NOG Underlies Symphalangism and Ossicular Chain Malformations Affecting Both the Incus and the Stapes

2019 ◽  
Vol 2019 ◽  
pp. 1-5
Author(s):  
Nathan R. Lindquist ◽  
Eric N. Appelbaum ◽  
Anushree Acharya ◽  
Jeffrey T. Vrabec ◽  
Suzanne M. Leal ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Conductive Hearing Loss ◽  
Ossicular Chain ◽  
Start Codon ◽  
Imaging Data ◽  
Hearing Thresholds ◽  
Stapes Footplate ◽  
Multiple Abnormalities ◽  
Protein Variants ◽  
Conductive Hearing

We performed exome sequencing to evaluate the underlying molecular cause of a patient with bilateral conductive hearing loss due to multiple ossicular abnormalities as well as symphalangism of the fifth digits. This leads to the identification of a novel heterozygous start codon variant in the NOG gene (c.2T>C:p.Met1?) that hinders normal translation of the noggin protein. Variants in NOG lead to a spectrum of otologic, digit, and joint abnormalities, a combination suggested to be referred to as NOG‐related‐symphalangism spectrum disorder (NOG‐SSD). Conductive hearing loss from such variants may stem from stapes footplate ankylosis, fixation of the malleoincudal joint, or fixation of the incus short process. In this case, the constellation of both stapes and incus fixation, an exceptionally tall stapes suprastructure, thickened long process of the incus, and enlarged incus body was encountered, leading to distinct challenges during otologic surgery to improve hearing thresholds. This case highlights multiple abnormalities to the ossicular chain in a patient with a start codon variant in NOG. We provide detailed imaging data on these malformations as well as surgical considerations and outcomes.

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.

Sign in / Sign up

Export Citation Format

Share Document