scholarly journals Hearing Loss in Mucopolysaccharidosis

2020 ◽  
Author(s):  
Cibele Gomes Bicalho ◽  
Emília Katiane Embiruçu de Araújo Leão ◽  
Álvaro Muiños de Andrade ◽  
Angelina Xavier Acosta
Keyword(s):  
Hearing Loss ◽  
Physical Examination ◽  
Clinical Examination ◽  
Rare Diseases ◽  
Lysosomal Enzymes ◽  
Conductive Hearing Loss ◽  
Case Series ◽  
Hearing Rehabilitation ◽  
Conductive Hearing

Abstract Introduction Mucopolysaccharidosis (MPS) is a set of rare diseases caused by deficiency of lysosomal enzymes that lead to the accumulation of glycosaminoglycans (GAG) in tissues and organs, which, in turn, is responsible for the multisystemic clinical, chronic, and progressive symptoms. Objective To describe the profile of the otorhinolaryngological clinical examination and audiology tests of patients with MPS disease. Methods The present study is a case series. The evaluation was performed, initially, in 24 patients with MPS types I, II, IIIA, IV and VI. Results The most common hearing complaint was hearing loss, which was confirmed by audiology tests in almost 100% of the patients, most of whom presented conductive hearing loss. Conclusions It is important to evaluate the complaints, physical examination, and audiology tests in patients with MPS. The otorhinolaryngologistshould be part of the group of professionals that follows these patients to better monitor their hearing and provide early hearing rehabilitation.

Contribution of spectral pinna cues for sound localization in children with congenital unilateral conductive hearing loss after hearing rehabilitation

2020 ◽  
Vol 385 ◽  
pp. 107847 ◽  
Author(s):  
Katharina Vogt ◽  
Jan-Willem Wasmann ◽  
A. John Van Opstal ◽  
Ad F.M. Snik ◽  
Martijn J.H. Agterberg
Keyword(s):  
Hearing Loss ◽  
Sound Localization ◽  
Conductive Hearing Loss ◽  
Hearing Rehabilitation ◽  
Conductive Hearing

scholarly journals Audiological Performance of ADHEAR Systems in Simulated Conductive Hearing Loss: A Case Series with a Review of the Existing Literature

2021 ◽  
Vol 11 (4) ◽  
pp. 537-546
Author(s):  
Enrico Muzzi ◽  
Valeria Gambacorta ◽  
Ruggero Lapenna ◽  
Giulia Pizzamiglio ◽  
Sara Ghiselli ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Conductive Hearing Loss ◽  
Bone Conduction ◽  
Free Field ◽  
Hearing Threshold ◽  
Case Series ◽  
Binaural Hearing ◽  
Bone Conduction Implant ◽  
Conductive Hearing ◽  
Loudness Summation

A new non-invasive adhesive bone conduction hearing device (ABCD) has been proposed as an alternative solution for reversible bilateral conductive hearing loss in recurrent or long-lasting forms of otitis media with effusion (OME) in children that cannot undergo surgical treatment. Our aim was to assess the effectiveness of ABCD in children with OME. Twelve normal-hearing Italian-speaking volunteers, in whom a conductive hearing loss was simulated, participated in the study. The free-field average hearing threshold was determined and, to evaluate binaural hearing skills, loudness summation and the squelch effect were assessed. Five conditions were tested: (1) unaided without earplugs, (2) unaided with bilateral earplugs, (3) aided right ear with bilateral earplugs, (4) aided left ear with bilateral earplugs, and (5) bilateral aid with bilateral earplugs. Post-hoc analysis showed a significant statistical difference between plugged, unplugged, and each aided condition. The main results were a better loudness summation and a substantial improvement of the squelch effect in the bilaterally aided. Our results suggest that ABCD is a valid treatment for patients with conductive hearing loss that cannot undergo bone conduction implant surgery. It is also important to consider bilateral aids in order to deal with situations in which binaural hearing is fundamental.

scholarly journals Penetrating Middle Ear Trauma: A Report of 2 Cases

2005 ◽  
Vol 84 (1) ◽  
pp. 32-35 ◽  
Author(s):  
Michael C. Neuenschwander ◽  
Ellen S. Deutsch ◽  
Anthony Cornetta ◽  
Thomas O. Willcox
Keyword(s):  
Hearing Loss ◽  
Physical Examination ◽  
Middle Ear ◽  
Conductive Hearing Loss ◽  
Penetrating Trauma ◽  
Ossicular Chain ◽  
The Other ◽  
Ear Injury ◽  
The Right ◽  
Conductive Hearing

Penetrating middle ear injury can result in hearing loss, vertigo, and facial nerve injury. We describe the cases of 2 children with penetrating trauma to the right ear that resulted in ossicular chain disruption; one injury was caused by cotton-tipped swabs and the other by a wooden matchstick. Symptoms in both children included hearing loss and otalgia; in addition, one child experienced ataxia and the other vertigo. Physical examination in both cases revealed a perforation in the posterosuperior quadrant of the tympanic membrane and visible ossicles. Audiometry identified a moderate conductive hearing loss in one child and a mild sensorineural hearing loss in the other. Both children underwent middle ear exploration and reduction of a subluxed stapes. We discuss the diagnosis, causes, and management of penetrating middle ear trauma. To reduce the morbidity associated with these traumas, otologic surgeons should act promptly and be versatile in choosing methods of repairing ossicular chain injuries.

Children with Cleft Palate: Predictors of Otologic Issues in the First 10 Years

2019 ◽  
Vol 160 (5) ◽  
pp. 902-910 ◽  
Author(s):  
Jamie L. Funamura ◽  
Janet W. Lee ◽  
Samantha McKinney ◽  
Ahmed G. Bayoumi ◽  
Craig W. Senders ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Cleft Palate ◽  
Conductive Hearing Loss ◽  
Univariate Analysis ◽  
Case Series ◽  
Study Data ◽  
Close Monitoring ◽  
Academic Center ◽  
Tube Function ◽  
Conductive Hearing

Objective To evaluate the characteristics of children with cleft palate associated with persistent otologic issues in the first 10 years of life. Study Design Case series with chart review. Setting Single academic center. Subjects and Methods Children born with cleft palate from 2003 to 2007 and treated by the UC Davis Cleft and Craniofacial Team between January 2003 and December 2017 were included in the study. Data from 143 patients were analyzed via Wilcoxon rank sum and Fisher exact tests for univariate analysis and logistic regression to determine adjusted odds ratios. Results The median length of follow-up was 9.9 years, and the age at last ear examination was 10.7 years. At the last evaluation, unresolved otologic issues were common, with at least 1 ear having a tympanic membrane (TM) perforation (16.1%), a tympanostomy tube (36.2%), or conductive hearing loss (23.1%). After adjusting for demographic and clinical characteristics, history of palate revision or speech surgery was associated with having a TM perforation ( P = .02). The only clinical variables associated with conductive hearing loss was the presence of a TM perforation ( P < .01) or a genetic abnormality ( P = .02). Severity of palatal clefting was not associated with specific otologic or audiologic outcomes after adjusting for other characteristics. Conclusion A large proportion of children with cleft palate have persistent otologic issues at age 10 years and would benefit from continued close monitoring well after the age when most children have normalized eustachian tube function. Prolonged otologic issues were not found to be associated with cleft type.

scholarly journals The Bonebridge in Adults with Mixed and Conductive Hearing Loss: Audiological and Quality of Life Outcomes

2019 ◽  
Vol 24 (2) ◽  
pp. 90-99 ◽  
Author(s):  
Piotr H. Skarżyński ◽  
Anna Ratuszniak ◽  
Bartłomiej Król ◽  
Magdalena Kozieł ◽  
Kamila Osińska ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Hearing Loss ◽  
Word Recognition ◽  
Conductive Hearing Loss ◽  
Bone Conduction ◽  
Speech Understanding ◽  
Hearing Rehabilitation ◽  
Patient Reported ◽  
Conductive Hearing

Background: Considering that hearing loss has a significant impact on social functioning, everyday activity and a person’s emotional state, one of the most important goals of hearing rehabilitation with bone conduction devices is improvement in a patient’s quality of life. Objectives: To measure self-assessed quality of life in patients implanted with the Bonebridge, a bone conduction device. Method: Prospective, observational, longitudinal study with one treatment group. Twenty-one patients with mixed or conductive hearing loss were included, and each individual served as its own control. The Abbreviated Profile of Hearing Aid Benefit (APHAB) was used to measure patient-reported quality of life before intervention and at 3 and 6 months after activation of the device. At the same time frames, pure-tone audiometry and speech understanding in quiet and in noise were tested. Results: Hearing-specific quality of life increased significantly after intervention and remained stable up to 6 months. Both word recognition in quiet and speech reception threshold in noise were significantly better after 6 months compared to before surgery. Outcomes of aided speech understanding were independent of initial bone conduction thresholds and equally high (word recognition score >75%) across the device’s indication range. Conclusions: The Bonebridge provides not only significant audiological benefit in both speech understanding in quiet and in noise, but also increases self-perceived quality of life in patients suffering from mixed and conductive hearing loss. Together with a very low rate and minor nature of adverse events, it is the state-of-the-art solution for hearing rehabilitation in patients with mixed or conductive hearing loss up to a bone conduction threshold of 45 dB HL.

scholarly journals A CASE SERIES OF PHYLLOID HYPOMELANOSIS

2021 ◽  
pp. 8-9
Author(s):  
S.N. Agrawal ◽  
Nisha M Varma ◽  
Manali Patil ◽  
Mohini Bhagwat
Keyword(s):  
Hearing Loss ◽  
Mental Retardation ◽  
Corpus Callosum ◽  
Conductive Hearing Loss ◽  
Case Series ◽  
Skin Lesions ◽  
Agenesis Of Corpus Callosum ◽  
Skeletal Defects ◽  
Conductive Hearing

THE PHYLLOID HYPOMELANOSIS is a defined as a distinct syndrome consisting of achromic phylloid skin lesions in combination with extracutaneous anomalies such as mental retardation , agenesis of corpus callosum , conductive hearing loss ,coloboma and various skeletal defects[1].

scholarly journals Cone-Beam CT compared to Multi-slice CT for the diagnostic analysis of conductive hearing loss, a pilot study

2016 ◽  
Vol 130 (S3) ◽  
pp. S188-S188
Author(s):  
Pieter Kemp ◽  
Jiska van Stralen ◽  
Pim de Graaf ◽  
Erwin Berkhout ◽  
Jan Wolff ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Pilot Study ◽  
Conductive Hearing Loss ◽  
Cone Beam Ct ◽  
Cone Beam ◽  
Diagnostic Analysis ◽  
Conductive Hearing

Long-term Compliance and Satisfaction With Percutaneous Bone Conduction Devices in Patients With Congenital Unilateral Conductive Hearing Loss

2015 ◽  
Vol 36 (5) ◽  
pp. 826-833 ◽  
Author(s):  
Rik C. Nelissen ◽  
Emmanuel A. M. Mylanus ◽  
Cor W. R. J. Cremers ◽  
Myrthe K. S. Hol ◽  
Ad F. M. Snik
Keyword(s):  
Hearing Loss ◽  
Conductive Hearing Loss ◽  
Bone Conduction ◽  
Conductive Hearing

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.

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