Isolated Congenital Round Window Atresia: Report of 2 Cases

Round window atresia (RWA) is an uncommon condition and can result in a conductive hearing loss. Two cases of nonsyndromal bilateral RWA in 2 members of the same family are reported. Both cases presented with a conductive hearing loss of 20 to 30 dB. High-resolution computed tomography scanning was used to diagnose the condition. The patients were rehabilitated with hearing aids. Review of the literature has shown disappointing results in hearing improvement with cochlear fenestration in an attempt to address this condition. Patients presenting with unexplained conductive hearing loss should be offered computed tomography scanning. The cases we report add to the literature to benefit future patients in preoperative counseling and better inform management.

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A Rare Stapes Abnormality

Case Reports in Otolaryngology ◽

10.1155/2015/387642 ◽

2015 ◽

Vol 2015 ◽

pp. 1-4 ◽

Cited By ~ 1

Author(s):

Hala Kanona ◽

Jagdeep Singh Virk ◽

Gaurav Kumar ◽

Sanjiv Chawda ◽

Sherif Khalil

Keyword(s):

Computed Tomography ◽

Hearing Loss ◽

High Resolution ◽

World Literature ◽

Surgical Intervention ◽

Conductive Hearing Loss ◽

High Resolution Computed Tomography ◽

Stapes Prosthesis ◽

Diagnostic Difficulties ◽

Conductive Hearing

The aim of this study is to increase awareness of rare presentations, diagnostic difficulties alongside management of conductive hearing loss and ossicular abnormalities. We report the case of a 13-year-old female reporting progressive left-sided hearing loss and high resolution computed tomography was initially reported as normal. Exploratory tympanotomy revealed an absent stapedius tendon and lack of connection between the stapes superstructure and footplate. The footplate was fixed. Stapedotomy and stapes prosthesis insertion resulted in closure of the air-bone gap by 50 dB. A review of world literature was performed using MedLine. Middle ear ossicular discontinuity can result in significant conductive hearing loss. This can be managed effectively with surgery to help restore hearing. However, some patients may not be suitable or decline surgical intervention and can be managed safely conservatively.

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Isolated Congenital round Window Absence

Annals of Otology Rhinology & Laryngology ◽

10.1177/000348940211100907 ◽

2002 ◽

Vol 111 (9) ◽

pp. 799-801 ◽

Cited By ~ 23

Author(s):

Christian Martin ◽

Stéphane Tringali ◽

Jean-François Pouget ◽

Pierre Bertholon ◽

Jean-Michel Prades

Keyword(s):

Computed Tomography ◽

Conductive Hearing Loss ◽

Round Window ◽

Virtual Endoscopy ◽

Rare Entity ◽

Computed Tomography Scan ◽

High Resolution Computed Tomography ◽

Conductive Hearing ◽

Rare Diagnosis ◽

Tomography Scan

Absence of the round window as a congenital defect is a rare entity that causes a conductive hearing loss. To the best of our knowledge, isolated and nonsyndromal round window absence has only been reported 3 times; the diagnoses were made during surgery. We are reporting on another case that was diagnosed before operation by virtual endoscopy of the middle ear during a high-resolution computed tomography scan. Although this new technique requires further validation, it may be an alternative to surgery as a means of confirming this rare diagnosis.

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A comparative study of hearing aids and round window application of the vibrant sound bridge (VSB) for patients with mixed or conductive hearing loss

International Journal of Audiology ◽

10.3109/14992027.2012.750431 ◽

2013 ◽

Vol 52 (4) ◽

pp. 209-218 ◽

Cited By ~ 22

Author(s):

Roberta Marino ◽

Nicola Linton ◽

Robert H. Eikelboom ◽

Elle Statham ◽

Gunesh P. Rajan

Keyword(s):

Hearing Loss ◽

Comparative Study ◽

Hearing Aids ◽

Conductive Hearing Loss ◽

Round Window ◽

Conductive Hearing

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High-Resolution Computed Tomography Imaging in Conductive Hearing Loss: What to Look for?

Current Problems in Diagnostic Radiology ◽

10.1067/j.cpradiol.2017.05.005 ◽

2018 ◽

Vol 47 (2) ◽

pp. 119-124 ◽

Cited By ~ 2

Author(s):

Manickam Subramanian ◽

Ashish Chawla ◽

Kabilan Chokkappan ◽

Tze Lim ◽

Jagadish Narayana Shenoy ◽

...

Keyword(s):

Computed Tomography ◽

Hearing Loss ◽

High Resolution ◽

Conductive Hearing Loss ◽

Computed Tomography Imaging ◽

High Resolution Computed Tomography ◽

Tomography Imaging ◽

Conductive Hearing

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The Value of High Resolution Computed Tomography in Detecting the Causes of Conductive Hearing Loss

Van Medical Journal ◽

10.5505/vtd.2018.79095 ◽

2018 ◽

Vol 25 (3) ◽

pp. 399-402

Author(s):

Fatma Beyazal Çeliker ◽

Suat Terzi ◽

Mehmet Beyazal ◽

Metin Çeliker ◽

Abdulkadir Özgür ◽

...

Keyword(s):

Computed Tomography ◽

Hearing Loss ◽

High Resolution ◽

Conductive Hearing Loss ◽

High Resolution Computed Tomography ◽

Conductive Hearing

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An approach to bilateral bone-anchored hearing aid surgery in children: contralateral placement of sleeper fixture

The Journal of Laryngology & Otology ◽

10.1017/s0022215108003952 ◽

2008 ◽

Vol 123 (5) ◽

pp. 555-557 ◽

Cited By ~ 3

Author(s):

J M Bernstein ◽

P Z Sheehan

Keyword(s):

Hearing Loss ◽

Hearing Aids ◽

Conductive Hearing Loss ◽

Hearing Aid ◽

Contralateral Side ◽

Two Stage ◽

Calvarial Bone ◽

Optimal Position ◽

Bone Anchored Hearing Aid ◽

Conductive Hearing

AbstractObjective:Bone-anchored hearing aid surgery in younger children is a two-stage procedure, with a titanium fixture being allowed to osseointegrate for several months before an abutment is fitted through a skin graft. In the first procedure, it has been usual to place a reserve or sleeper fixture approximately 5 mm from the primary fixture as a backup in case the primary fixture fails to osseointegrate. This ipsilateral sleeper fixture is expensive, is often not used, and is placed in thinner calvarial bone where it is less likely to osseointegrate successfully. The authors have implanted the sleeper fixture on the contralateral side, with the additional objective of reducing the number of procedures for bilateral bone-anchored hearing aid implantation, providing a cost-effective use for the sleeper.Methods:The authors implanted the bone-anchored hearing aid sleeper fixture in the contralateral temporal bone instead of on the ipsilateral side in seven successive paediatric cases with bilateral conductive hearing loss requiring two-stage bone-anchored hearing aids, treated at the Royal Manchester Children's Hospital, UK.Results:The seven patients ranged in age from five to 15 years, with a mean age of 10 years; in addition, a 20-year-old with learning disability was also treated. In each case, the contralateral sleeper fixture was not needed as a backup fixture, but was used in four patients (57 per cent) as the basis for a second-side bone-anchored hearing aid.Conclusions:In children with bilateral conductive hearing loss, in whom a bilateral bone-anchored hearing aid is being considered and the second side is to be operated upon at a later date, we recommend placing the sleeper fixture on the contralateral side at the time of primary first-side surgery. Our technique provides a sleeper fixture located in an optimal position, where it also offers the option of use for a second-side bone-anchored hearing aid and reduces the number of procedures needed.

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Use of hearing AIDS

Drug and Therapeutics Bulletin ◽

10.1136/dtb.14.12.45 ◽

1976 ◽

Vol 14 (12) ◽

pp. 45-46

Keyword(s):

Hearing Loss ◽

Middle Ear ◽

Hearing Aids ◽

Conductive Hearing Loss ◽

Auditory Training ◽

Lip Reading ◽

Conductive Hearing

Up to 3 million people in Britain might be helped by hearing aids.1 2 Most are over 65 years of age, but some are infants. All should be referred to specialist centres for assessment as soon as possible. Hearing aids generally help most in disorders of the middle ear (conductive hearing loss); they can also help those with sensorineural and other forms of hearing loss. The use of an aid often needs to be supplemented by lip reading and other means of auditory training.1 3

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Stapedotomy post-operative results and complications

International Journal of Otorhinolaryngology and Head and Neck Surgery ◽

10.18203/issn.2454-5929.ijohns20203085 ◽

2020 ◽

Vol 6 (8) ◽

pp. 1442

Author(s):

Hanumant S. Giri ◽

Ram C. Bishnoi ◽

Pooja D. Nayak ◽

Ninad S. Gaikwad

Keyword(s):

Hearing Loss ◽

Hearing Aids ◽

Conductive Hearing Loss ◽

Major Complication ◽

Safe Procedure ◽

Hearing Results ◽

Post Surgery ◽

Localized Disease ◽

Conductive Hearing

Background: Otosclerosis is a hereditary localized disease of the bone derived from the otic capsule. It is characterized by alternating phases of bone formation and resorption and patient presents with conductive hearing loss. Treatment of otosclerosis can be of two kinds: hearing aids and surgery. Stapedectomy and stapedotomy are the two surgical procedures done for treatment of otosclerosis. Present study was conducted on 30 patients with otosclerosis who underwent stapedotomy to assess the hearing results post-surgery by serial Audiometric studies and to study the complications of stapedotomy surgery.Methods: This prospective observational study conducted on 30 patients of otosclerosis who fulfilled the inclusion and exclusion criteria. Results: In this study of thirty cases of otosclerosis which were operated for small fenestra stapedotomy, we conclude that Hearing gain post-surgery was remarkable especially for patients with a pure conductive hearing loss. There was no deterioration in hearing after two years of follow-up. In our study on 30 patients we encountered minor complication in 4 patients (13.33%) and 1 major complication of profound sensorineural hearing loss 3.33%.Conclusions: We conclude that stapedotomy is a relatively safe procedure with significant post-surgery hearing benefit.

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

The Journal of Laryngology & Otology ◽

10.1017/s0022215111002738 ◽

2011 ◽

Vol 126 (1) ◽

pp. 76-78 ◽

Cited By ~ 4

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.

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