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.

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.

Evaluating the Frequency of Ear Problems in Patients with Cleft Lip and Palate

2021 ◽  
Author(s):  
Fatemeh Mirashrafi ◽  
Babak Saedi ◽  
Mahtab Rabbani Anari ◽  
Gholamreza Garmaroudi ◽  
Roja Toosi ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Cleft Palate ◽  
Otitis Media ◽  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Conductive Hearing Loss ◽  
Otitis Media With Effusion ◽  
Pure Tone Audiometry ◽  
Hearing Disorders ◽  
Conductive Hearing

Background: Orofacial cleft is one of the most common congenital malformations of craniofacial region. Otitis media with effusion causing conductive hearing loss is a considerable challenge for many children with cleft lip and palate. The aim of this study was to evaluate the prevalence of hearing disorders and associated malformations in these patients. Methods: The research population consisted of patients with cleft palate, between years 2012 and 2014, who were referred to Children’s Medical Center and Vali-e-Asr hospital in Tehran, Iran. Otoscopic examination, tympanometry, pure tone audiometry and echocardiography were performed for each patient. Results: Among patients with cleft palate, 73% suffered from hearing disorders. There was no relationship between prevalence of hearing loss and sex, presence of other congenital anomalies and degree of cleft, but middle ear diseases were significantly higher in children younger than 2 years. Among patients with cleft lip or palate, 10% suffered from cleft lip, 63% suffered from cleft palate and 27% suffered from cleft lip and palate. There was at least one congenital anomaly in 53% of patients. Conclusion: This study demonstrates high prevalence of otitis media with effusion and conductive hearing loss in patients with cleft. However, audiologic problems are alleviated when patients become older.

Auditory Brainstem Responses in Children with Otitis Media with Effusion

1980 ◽  
Vol 89 (3_suppl) ◽  
pp. 200-206 ◽  
Author(s):  
Thomas J. Fria ◽  
Diane L. Sabo
Keyword(s):  
Hearing Loss ◽  
Otitis Media ◽  
Pure Tone ◽  
Conductive Hearing Loss ◽  
Otitis Media With Effusion ◽  
Auditory Brainstem ◽  
Auditory Brainstem Responses ◽  
School Age Children ◽  
School Age ◽  
Conductive Hearing

Auditory brainstem responses (ABR) were recorded in 14 infants and toddlers and 12 school-age children with a previous history of recurrent otitis media with effusion (OME), or otoscopic and tympanometric evidence of persistent OME, or both. ABR tests were performed immediately before and after myringotomy and tympanostomy tube insertion in the younger subjects. For the school-age children, ABR tests were performed following otoscopy, tympanometry, and pure tone audiometry. The results demonstrate that the latency of both wave I and wave V of the ABR was sensitive (82% and 100%, respectively) to the presence of OME. Wave I also identified the absence of OME (specificity = 100%) whereas wave V did not (specificity = 25%). ABR latency was significantly decreased postoperatively in ears found to have OME, but not in ears found to have no OME. In the school-age subjects the ABR was used to predict the conductive hearing loss at 4000 Hz with less than a 20 dB error in virtually all subjects. The ABR latency delay was also found to be related to conductive hearing impairment at lower pure tone frequencies and to the average conductive loss at a variety of pure tone frequencies. Predictions of the presence of a conductive hearing loss from these relationships promise to be impressively accurate. The results suggest that the ABR can be a valuable tool for detecting the presence of conductive hearing impairment in infants and young children suspected to have OME and perhaps as an estimate of the degree of impairment.

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.

scholarly journals Study on Hearing Impairment in Bangladesh

2021 ◽  
Vol 20 (1) ◽  
pp. 72-76
Author(s):  
Mohammad Mahabubul Alam Chowdhury ◽  
Kamrul Hassan Tarafder ◽  
Pran Gopal Datta ◽  
Mostafa Zaman ◽  
Nasima Akhtar ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Hearing Impairment ◽  
Otitis Media ◽  
Conductive Hearing Loss ◽  
Otitis Media With Effusion ◽  
Public Awareness ◽  
Chronic Suppurative Otitis Media ◽  
Suppurative Otitis Media ◽  
Ear Wax ◽  
Conductive Hearing

Background : Hearing impairment is one of the leading causes of disability in Bangladesh. Since half the cases can be prevented through public awareness, early detection and timely management, planning of public health interventions become necessary. To achieve this, a nationwide level of evidence is required. This survey was conducted with the aim of determining the prevalence of hearing impairment in Bangladesh. Materials and methods : The study was a cross-sectional one, conducted between January to May 2013, where a multistage, geographically clustered sampling approach was used. A total of 52 primary sampling units were selected, and from each unit households were selected at random followed by random selection of an individual from each household. In total, 5,220 people were targeted out of which data collection could be completed from 4260 individuals. (82%). Following clinical assessment of study subjects, hearing status was assessed by pure tone audiometry and otoacoustic emission test. Results : The mean age was 32 years among which 58% were females. Among men, there were 29% students, 28% agriculture workers and 14% business men. A major portion of females (63%) were home makers. On clinical examination, 11.5% respondents had impacted ear wax, 6.2% had chronic middle ear infections with eardrum perforation, and 5.3% had otitis media with effusion.On hearing assessment, 34.6% respondents had some form of hearing loss (>25dB in better ear). Conductive hearing loss was found in 12.0%, sensorineural in 4.5% and mixed in 3.8%, where the latter two increased in prevalence with age. Out of the total respondents, 9.6% had disabling hearing loss according to WHO criteria, with a higher prevalence in adults > 60 years(37%). Logistic regression analysis showed age, socioeconomic status, presence of ear wax, chronic suppurative otitis media, otitis media with effusion and otitis externa were significant predictors of disabling hearing loss. Conclusion : The major causes of conductive hearing loss are chronic suppurative otitis media and otitis media with effusion. Chatt Maa Shi Hosp Med Coll J; Vol.20 (1); January 2021; Page 72-76

Otitis Media—Red Flags for Management: American Academy of Otolaryngology; Head and Neck Surgery 2013 Clinical Practice Guideline for Tympanostomy Tube Insertion

2014 ◽  
Vol 24 (1) ◽  
pp. 4-10
Author(s):  
Melody Harrison
Keyword(s):  
Hearing Loss ◽  
Otitis Media ◽  
Middle Ear ◽  
Conductive Hearing Loss ◽  
Otitis Media With Effusion ◽  
Neck Surgery ◽  
Tympanostomy Tube ◽  
Red Flags ◽  
Tube Insertion ◽  
Conductive Hearing

Otitis media with effusion (OME) is described as a collection of fluid in the middle ear, without signs or symptoms of acute ear infection ( Stool, Berg, Berman, & Carney, 1994). The accumulated fluid decreases the ability of both the tympanic membrane (eardrum) and the ossicles in the middle ear to vibrate, resulting in a mild conductive hearing loss. Although 28 decibels (dB) is the average hearing loss associated with OME, the range is quite wide. While some children experience no hearing loss, about 20% have hearing loss of 35 dB or greater ( Gravel, 2003).

scholarly journals Effect of enlarged adenoid in the development of otitis media with effusion under the Age of 12

2020 ◽  
Vol 22 (1) ◽  
pp. 48-52
Author(s):  
Raju Barua ◽  
Kamrul Hassan Tarafder ◽  
Mohammad Wakilur Rahman ◽  
Abu Naser Md Jamil ◽  
Farzana Haque ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Early Diagnosis ◽  
Otitis Media ◽  
Middle Ear ◽  
Conductive Hearing Loss ◽  
Otitis Media With Effusion ◽  
Middle Ear Pressure ◽  
Pressure Changes ◽  
Medical University ◽  
Conductive Hearing

Objective: To find out relation between enlarged adenoid and otitis media with effusion (OME), degree of hearing loss in OME cases with the size of the adenoids and degree of pressure changes in middle ear in OME cases with the size of adenoids. Method: This Prospective Study was carried out Bangabandhu Sheikh Mujib Medical University, Shahbag, Dhaka and Specialized ENT Hospital of SAHIC, Mohakhali, Dhaka from September 2010 to March 2011 (7 months). Randomly selected 50 children (Age below 12 years) with enlarged adenoid in outdoor & indoor. Patient with sensorineural hearing loss and conductive hearing loss with other than enlarged Adenoids are exclude in this study. Result: In this study, 58% had hearing loss in which 38% were mild and 20% were moderate loss. 54% of ears had negative pressure. 22 cases of severe adenoids of which 72.72% had OME.10 out of 16 OME with severe adenoids cases having hearing loss were in 26-40 db range and middle ear pressure is -201 to -400 dapa. Conclusion: Early diagnosis and treatment of enlarged adenoids should be aparciated to decrease the incidence, morbidity and complications of otitis media with effusion. Bangladesh J Otorhinolaryngol; April 2016; 22(1): 48-52

Acute otitis media and otitis media with effusion in children with bacterial meningitis

1997 ◽  
Vol 111 (10) ◽  
pp. 913-916 ◽  
Author(s):  
M. P. Richardson ◽  
A. Reid ◽  
T. J. Williamson ◽  
M. J. Tarlow ◽  
P. T. Rudd
Keyword(s):  
Hearing Loss ◽  
Bacterial Meningitis ◽  
Otitis Media ◽  
Conductive Hearing Loss ◽  
Otitis Media With Effusion ◽  
Acute Otitis Media ◽  
Auditory Brainstem Responses ◽  
Acute Bacterial Meningitis ◽  
Controlled Study ◽  
Conductive Hearing

AbstractAcute otitis media and otitis media with effusion (OME) have often been observed in children with bacterial meningitis. OME has also been proposed as the mechanism of reversible hearing loss after meningitis. In this controlled study, children with acute bacterial meningitis were studied using auditory brainstem responses (ABR), otoacousticemissions, tympanometry and otoscopy. An age- and sex- matched control was recruited for each patient and the incidence of acute otitis media and OME was compared between the twogroups. One hundred and twenty-four children with meningitis were studied. Ninety-two children (74 per cent) had meningococcal meningitis. Five patients (4 per cent) had conductive hearing loss (ABR threshold≥30 dB HL) at the time of discharge from hospital. None of the patients or controls had acute otitis media. Patients and controls were well matched for risk factors for OME and the prevalence of middle ear effusion in patients and controls was 7.2 per cent and 11.3 per cent respectively. The relative risk of OME in the children with meningitis was 0.64 (95 per cent confidence interval 0.29 to 1.42). After nine months, three of the five children with meningitis and conductive hearing loss had regained normal hearing.In contrast to previous reports, there was no relationship between bacterial meningitis and acute otitis media or OME in this study. Nevertheless, coincidental conductive hearing defects were identified as the cause of reversible hearing loss in three patients.

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