Management of hearing loss in Apert syndrome

2005 ◽  
Vol 119 (5) ◽  
pp. 385-390 ◽  
Author(s):  
D Rajenderkumar ◽  
D Bamiou ◽  
T Sirimanna
Keyword(s):  
Hearing Loss ◽  
Hearing Aids ◽  
Conductive Hearing Loss ◽  
Otitis Media With Effusion ◽  
Adult Life ◽  
Apert Syndrome ◽  
Glue Ear ◽  
Almost All ◽  
Congenital Hearing Impairment ◽  
Conductive Hearing

Background: Apert syndrome is one of the craniosynostosis syndromes, with abirth prevalence estimated to be between 9.9 and 15.5/million, and accounts for 4.5 per cent ofcraniosynostoses. Although conductive hearing loss is common in Apert syndrome there are contradicting reports regarding the cause of this hearing loss. There is also no detailed informationavailable on the management of hearing loss in Apert syndrome.Materials and methods: A retrospective analysis of case notes of Apert syndrome patients seen between 1970 and 2003 at Great Ormond Street Children’s Hospital, London, was undertaken.Results: Seventy case notes were obtained. The incidence of congenital hearing impairment was between 3 and 6 per cent. Almost all patients had otitis media with effusion (glue ear), which tended to persist into adult life. More than 56 per cent of cases developed permanent conductive hearing loss by 10–20 years. Repeated grommet insertion was common; even though 35 per cent had trouble with ear discharge and persistent conductive hearing loss. Statistically, grommets made no difference to the risk of developing permanent hearing loss.Conclusion:This study, of the largest number of Apert syndrome cases assembled to date, showed that early optimization of hearing with possible hearing aids needs to be considered. Repeated grommet insertion does not help in optimizing hearing, especially if ear discharge complicates the picture.

Congenital Conductive Hearing Loss in Apert Syndrome

1986 ◽  
Vol 95 (4) ◽  
pp. 429-433 ◽  
Author(s):  
Susan G. Phillips ◽  
Richard T. Miyamoto
Keyword(s):  
Hearing Loss ◽  
Otitis Media ◽  
Conductive Hearing Loss ◽  
Otitis Media With Effusion ◽  
Apert Syndrome ◽  
Review Of The Literature ◽  
Stapes Fixation ◽  
The Third ◽  
Hands And Feet ◽  
Conductive Hearing

Acrocephalosyndactyly (Apert syndrome) is a rare cranlosynostotic syndrome characterized by acrocephaly, syndactyly of the hands and feet, and—occasionally—-conductive hearing loss. We report three cases of conductive hearing loss in Apert syndrome. One patient was found to have bilateral stapes fixation. His daughter (the second case) had chronic bilateral otitis media with effusion. The third case involved a fixed Incus and hypomobile stapes. The management of these patients and a review of the literature are presented.

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

2008 ◽  
Vol 123 (5) ◽  
pp. 555-557 ◽  
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.

Use of hearing AIDS

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

scholarly journals Stapedotomy post-operative results and complications

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

<p class="abstract"><strong>Background:</strong> 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.</p><p class="abstract"><strong>Methods:</strong> This prospective observational study conducted on 30 patients of otosclerosis who fulfilled the inclusion and exclusion criteria.  </p><p class="abstract"><strong>Results:</strong> 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%.</p><p class="abstract"><strong>Conclusions:</strong> We conclude that stapedotomy is a relatively safe procedure with significant post-surgery hearing benefit.</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.

Hearing in Noise With Unilateral Versus Bilateral Bone Conduction Hearing Aids in Adults With Pseudo-conductive Hearing Loss

2020 ◽  
Vol 41 (3) ◽  
pp. 379-385
Author(s):  
Ohad Hilly ◽  
Meirav Sokolov ◽  
Reut Beck Finkel ◽  
Ofir Zavdy ◽  
Rafael Shemesh ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Hearing Aids ◽  
Conductive Hearing Loss ◽  
Bone Conduction ◽  
Hearing In Noise ◽  
Bone Conduction Hearing ◽  
Conductive Hearing

scholarly journals ADHEAR in Conductive Hearing Loss: a case study

2020 ◽  
Vol 30 (Supplement_2) ◽  
Author(s):  
T Marques ◽  
A Carvalho ◽  
A Miguéis
Keyword(s):  
Hearing Loss ◽  
Hearing Aids ◽  
Conductive Hearing Loss ◽  
Bone Conduction ◽  
Single Subject ◽  
Non Invasive ◽  
Middle Ear Cholesteatoma ◽  
Bone Conduction Hearing ◽  
Conductive Hearing ◽  
Hearing System

Abstract Introduction Bone conduction hearing systems can be applied through non-invasive devices, using soft bands that exert pressure on the skin, or they can be surgically implanted (Bone Anchored Hearing Aid - BAHA). However, these bone conduction devices are frequently not well accepted due to the pressure on the head. Therefore, a new non-surgical hearing system was developed not to exert pressure on the skin, the ADHEAR. The bone anchorage in ADHEAR is performed through an adhesive adapter and is indicated for patients with conductive hearing loss and normal inner ear function. Objectives Evaluate the audiological performance with the adhesive bone conduction hearing device (ADHEAR) in a patient with conductive hearing loss. Methodology The study was designed as a prospective single-subject repeated-measure study with the subject serving as his own control. A 29 year old female patient who had a primary surgery due to middle ear cholesteatoma, was adapted with unilateral non-invasive adhesive bone conduction system for the treatment of conductive hearing loss. Air and bone conduction thresholds, word recognition scores (WRS) and speech recognition thresholds (SRT) in quiet and noise were assessed to verify the inclusion criteria of the study. Aided and unaided pure tone audiometry at 0.5, 1, 2 and 4 kHz in free field and speech audiometry in quiet and noise were performed at baseline and after 4 weeks with the ADHEAR. Results The functional gain with the ADHEAR averaged over 0.5, 1, 2, and 4 kHz after 4 weeks of usage of the adhesive hearing system, improved from 55 dB HL to 31dB HL. Speech perception in quiet and noise improved significantly in the aided situation, with SRT in quiet improving from 60 to 35 dB HL, when compared to the unaided condition. Similar results were found in noise. The patient evaluated the ADHEAR system as being useful, and without complaints of skin pressure. There was no adverse skin reaction. Conclusion Hearing performance was significantly better with ADHEAR under all test conditions. Therefore, this transcutaneous hearing system seems to be an excellent alternative for patients who need a hearing solution for conductive hearing loss but for clinical reasons cannot undergo surgery or conventional hearing aids. Furthermore, it preserves skin over the mastoid and reduces the risk of infection. Otherwise it has benefits verified by absence of head pressure and improvement of patient’s quality of life.

scholarly journals Bilateral aberrant internal carotid arteries discovered during myringotomy: A case report and review of the literature

2018 ◽  
Vol 4 (1) ◽  
pp. 23
Author(s):  
Marc Antoine Hudon ◽  
Margaret Aron ◽  
Mireille Gervais
Keyword(s):  
Hearing Loss ◽  
Carotid Arteries ◽  
Internal Carotid ◽  
Conductive Hearing Loss ◽  
Otitis Media With Effusion ◽  
Vascular Anomaly ◽  
Review Of The Literature ◽  
The Right ◽  
Conductive Hearing ◽  
Internal Carotid Arteries

The aberrant course of the intratympanic carotid artery is a rare congenital vascular anomaly. We report the case of a 7-year-old patient who presented with bilateral conductive hearing loss, originally attributed to otitis media with effusion. After myringotomy, massive pulsatile bleeding occurred on the right side. The bleeding was successfully controlled with packing without any residual complication. Imaging confirmed aberrant internal carotid arteries abutting the tympanic membranes bilaterally.

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