scholarly journals Congenital ossicular chain anomaly. Columella stapes

2021 ◽  
pp. 87-91
Author(s):  
Ostap R. Orishchak ◽  
Diana T. Orishchak ◽  
Nataliya V. Vasilyuk
Keyword(s):  
Clinical Case ◽  
Conductive Hearing Loss ◽  
Round Window ◽  
Rare Condition ◽  
Ossicular Chain ◽  
Stapes Footplate ◽  
History Of ◽  
Ear Infections ◽  
Conductive Hearing ◽  
Otoscopic Examination

Congenital anomalies of the ossicular chain are a rare condition in clinical practice. In literature described different variants of the ossicular chain anomaly from stapes footplate ankylosis to complete aplasia of the ossicular chain, oval, and round window. Possible congenital ossicular chain anomaly should be suspected in case of non-progressive conductive hearing loss with a normal ear drum during an otoscopic examination, without a history of previous ear infections or trauma. In this article, we described the clinical case of congenital ossicular chain anomaly presented as fixed incus. Stapes was presented as single, straight, broad crus that were attached to the central part of the footplate. Stapedius tendon was absent. The footplate was completely mobile. Exploratory tympanotomy was performed to confirm a diagnosis of congenital ossicular chain anomaly. The ossicular chain was reconstructed with PORP titanium prosthesis. Furthermore, a literature review was done, conclusions presented.

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.

Conductive hearing loss after head trauma: review of ossicular pathology, management and outcomes

2008 ◽  
Vol 123 (2) ◽  
pp. 177-181 ◽  
Author(s):  
O J Basson ◽  
A C van Lierop
Keyword(s):  
Hearing Loss ◽  
Head Trauma ◽  
Conductive Hearing Loss ◽  
Retrospective Chart Review ◽  
Ossicular Chain ◽  
Average Improvement ◽  
History Of ◽  
Incudostapedial Joint ◽  
Conductive Hearing

AbstractIntroduction:This paper reviews our experience of ossicular chain injuries following head trauma treated at Groote Schuur Hospital, Cape Town, South Africa.Materials and methods:We performed a retrospective chart review of all patients with a history of head trauma and a conductive hearing loss who had undergone exploratory tympanotomy. Sixteen patients were included in the study.Results:Injury was most common at the incudostapedial joint (63 per cent). Disarticulations of the icudostapedial joint were treated with cartilage interposition in all cases. Audiography showed an improvement in 12 of the patients, with an average improvement of 35 dB.Discussion:We discuss the various options available to the otologist to repair ossicular disruptions after trauma. In this series, cartilage autografts were used in most incudostapedial joint injuries, with excellent closure of the air–bone gap.Conclusion:Cartilage interposition was a very successful method of repairing incudostapedial joint dislocation in this series, at short term follow up.

scholarly journals Hearing loss in the Saethre-Chotzen syndrome

1996 ◽  
Vol 110 (10) ◽  
pp. 952-957 ◽  
Author(s):  
Robbert J. H. Ensink ◽  
Henri A. M. Marres ◽  
Han G. Brunner ◽  
Cor W. R. J. Cremers
Keyword(s):  
Hearing Loss ◽  
Middle Ear ◽  
Conductive Hearing Loss ◽  
Ossicular Chain ◽  
Middle Ear Surgery ◽  
Ear Canal ◽  
Ear Surgery ◽  
Generation Family ◽  
Ear Infections ◽  
Conductive Hearing

AbstractA three-generation family with Saethre-Chotzen syndrome and an isolated case are presented. The proband presented with conductive hearing loss. His mother and grandmother showed minor features of the syndrome including conductive hearing loss.Symptoms of the craniosynostosis syndromes can include stapes ankylosis, a fixed ossicular chain in a too small epitympanum, and small or even absent mastoids. The proband was treated with a boneanchored hearing aid (BAHA) instead of reconstructive middle ear surgery. Current literature on the results of ear surgery is reviewed. In general, reconstructive middle ear surgery should only be considered if congenital anomalies of the middle ear are the only presenting symptom. In cases with additional anomalies such as atresia of the ear canal or damage due to chronic ear infections, the outcome of reconstructive surgery to correct the anomalous ossicular chain is unsatisfactory. In such cases the BAHA is probably the best solution.

Ossicular Discontinuity and Exostoses in Proteus Syndrome: A Case Report

2005 ◽  
Vol 114 (3) ◽  
pp. 242-246
Author(s):  
Joni K. Doherty ◽  
Dennis R. Maceri
Keyword(s):  
Hearing Loss ◽  
High Frequency ◽  
Conductive Hearing Loss ◽  
Ossicular Chain ◽  
Typical Feature ◽  
Proteus Syndrome ◽  
Frequency Range ◽  
Pure Tone Average ◽  
Ossicular Chain Reconstruction ◽  
Conductive Hearing

Proteus syndrome (PS) is a rare hamartomatous disorder characterized by mosaic overgrowth of multiple tissues that manifests early in life and is progressive. The presence of unilateral external auditory canal exostoses in a patient who is not a swimmer or surfer is suggestive of PS. However, hearing loss is not a typical feature. Here, we describe exostoses and ossicular discontinuity with conductive hearing loss in a patient with PS. The treatment consisted of canalplasty and ossicular chain reconstruction. A postoperative reduction was demonstrated in the patient's air-bone gap, from 21 dB to 13 dB for the pure tone average (four frequencies) and from 41 dB to 15 dB in the high-frequency range (6,000 to 8,000 Hz). Causes of ossicular discontinuity are discussed. Routine annual audiometric and otolaryngological evaluation should be considered in all patients with temporal bone inyolvement of PS.

INDEX OF SUSPICION

1993 ◽  
Vol 14 (5) ◽  
pp. 191-193
Author(s):  
Geeta Berera ◽  
Frank B. Magill ◽  
Melanie Oblender
Keyword(s):  
Early Diagnosis ◽  
Conductive Hearing Loss ◽  
Speech Development ◽  
Therapeutic Implications ◽  
Pressure Equalization ◽  
Ear Infections ◽  
Case Presentations ◽  
Conductive Hearing ◽  
Serous Otitis Media ◽  
Audiologic Evaluation

This section of Pediatrics in Review reminds clinicians of those conditions that can present in a misleading fashion and require suspicion for early diagnosis. Emphasis has been placed on conditions in which early diagnosis is important and that the general pediatrician might be expected to encounter, at least once in a while. The reader is encouraged to write possible diagnoses for each case before turning to the discussion, which is on the following page. We invite readers to contribute case presentations and discussions. Case 1 Presentation The parents of a 6-year-old boy would like your opinion regarding a tonsillectomy and adenoidectomy for their son. His speech development has been delayed, and even now, despite 2 years of therapy, he is difficult to understand. The child had experienced recurrent ear infections for which pressure equalization tubes were placed 4 years ago. Physical examination reveals bilateral serous otitis media and a bifid uvula. The tonsils appear normal for his age. His speech has a hypernasal quality. An audiologic evaluation reveals bilateral conductive hearing loss. You see a pattern that suggests an underlying condition with important therapeutic implications. Case 2 Presentation A 6-week-old infant is brought to the emergency room because of bleeding from the umbilicus for 8 hours.

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 Post Inflammatory Medial Canal Fibrosis: A Case Report

2013 ◽  
Vol 2 (2) ◽  
pp. 69-71
Author(s):  
A Kumar ◽  
K Santosh
Keyword(s):  
Conductive Hearing Loss ◽  
Fibrous Tissue ◽  
External Auditory Meatus ◽  
Solid Core ◽  
Medial Part ◽  
Review Of The Literature ◽  
Soft Tissue Density ◽  
History Of ◽  
Conductive Hearing ◽  
Bony Erosion

Medial canal fibrosis is an interesting type of acquired meatal atresia that is characterized by formation of a solid core of fibrous tissue in the medial part of the external auditory meatus abutting the tympanic membrane. A review of the literature showed that many different terms have been used interchangeably to report the same or similar condition. This is a case of medial canal fibrosis being reported to emphasize the importance in diagnosing this rare but easily treatable disease. A 16 yrs old female presented with bilateral conductive hearing loss & history of recurrent rhinitis & sinusitis. CT Temporal bone showed soft tissue density lesions in bilateral bony EAC (External auditory canal) with no bony erosion & normal middle ear. A diagnosis of Medial canal fibrosis was given. The patient was operated & biopsy of the specimen came out to be inflammatory granulation tissue. Nepalese Journal of Radiology; Vol. 2; Issue 2; July-Dec. 2012; 69-71 DOI: http://dx.doi.org/10.3126/njr.v2i2.7689

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 Neuroendocrine Adenoma of the Middle Ear (NAME)

2009 ◽  
Vol 88 (4) ◽  
pp. 874-879 ◽  
Author(s):  
Karen Leong ◽  
Marian M. Haber ◽  
Venu Divi ◽  
Robert T. Sataloff
Keyword(s):  
Hearing Loss ◽  
Otitis Media ◽  
Middle Ear ◽  
Conductive Hearing Loss ◽  
Carcinoid Tumors ◽  
Rare Tumor ◽  
Pathologic Findings ◽  
History Of ◽  
Conductive Hearing ◽  
Recurrent Otitis Media

Neuroendocrine adenoma of the middle ear (NAME) is a rare tumor. We report a case of NAME, the clinical and pathologic findings of which illustrate the biologic behavior of adenomatous tumors of the middle ear and their relationship with rare carcinoid tumors of the middle ear. A 29-year-old man presented with a history of recurrent otitis media, right conductive hearing loss, and aural fullness. The tumor was removed in its entirety. Otolaryngologists should be familiar with this unusual but important entity.

Inversion of the stapedial reflex in ossicular chain lesions

1986 ◽  
Vol 100 (4) ◽  
pp. 399-403 ◽  
Author(s):  
Steen Gimsing
Keyword(s):  
Hearing Loss ◽  
Soft Tissue ◽  
Conductive Hearing Loss ◽  
External Auditory Meatus ◽  
Ossicular Chain ◽  
Traumatic Origin ◽  
Stapedial Reflex ◽  
Conductive Hearing

AbstractIn ears with minor conductive hearing loss of traumatic origin, it is sometimes possible to invert the deflection of the stapedial reflex by application of a small underpressure in the external auditory meatus of the injured ear (probe ear). This inversion can be explained by an ossicular discontinuity bridge by soft tissue.

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