Hearing characterization in oculoauriculovertebral spectrum: A prospective study with 10 patients

Goetze, Thayse Bienert; Sleifer, Pricila; Rosa, Rafael Fabiano Machado; Silva, Alessandra Pawelec da; Graziadio, Carla; Zen, Paulo Ricardo Gazzola

doi:10.1002/ajmg.a.38017

Cited by 10 publications

(16 citation statements)

References 15 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Sometimes a sensorineural hearing loss also coexists due to damage to the inner ear, but this type of hypoacusis is less common [21,21,22]. The degree of hearing loss can range from mild to moderate disorders of the conductive type (such as with cases of chronic otitis media with effusion) to severe hearing impairment with profound sensorineural hearing loss [23,23]. It is common knowledge that hearing is important for speech development, and in patients with OAV this is one of the leading problems that can significantly affect quality of life.…”

Section: Hypoacusis In Oavdmentioning

confidence: 99%

Hearing Impairment In Oculoauriculovertebral Dysplasia (Goldenhar Syndrome): Literature Review

Porowski¹,

Skarżyńśki²,

Skarżyński³

et al. 2019

J Hear Sci

View full text Add to dashboard Cite

This paper reviews the literature on patients with oculoauriculovertebral dysplasia (OAVD, Goldenhar syndrome) in the context of methods to improve their hearing, an area of interest to otorhinolaryngologists, audiologists, and phoniatrists. The papers reviewed describe individual cases and groups of patients diagnosed with the syndrome. They are of interest due to the range of therapeutic approaches used and the percentage of favourable results. The available literature is sparse, particularly in terms of improving hearing in OAVD. In these patients, degrees of hearing loss or deafness are major problems resulting from complex external, middle, and inner ear defects, which can also lead to speech development disorders. There is therefore a need for a closer look at the syndrome, including how to identify patients, indications for surgical treatment, use of other methods to improve hearing, and methods of rehabilitation.

show abstract

Section: Hypoacusis In Oavdmentioning

confidence: 99%

Hearing Impairment In Oculoauriculovertebral Dysplasia (Goldenhar Syndrome): Literature Review

Porowski¹,

Skarżyńśki²,

Skarżyński³

et al. 2019

J Hear Sci

View full text Add to dashboard Cite

show abstract

“…It widely thought to due to abnormalities of the first and second branchial arches [1]. It has been associated with a variety of possible causes including environmental, teratogenic, vascular insufficiencies, and twin pregnancies [3]. Though known to have an embryologic etiology, there have also been theories surrounding teratogenic exposures including thalidomide, primidone, anticonvulsants, retinoic acid, and smoking [4,5].…”

Section: Introductionmentioning

confidence: 99%

Clinical Presentation of Congenital Cholesteatoma of the External Auditory Canal in Goldenhar Syndrome: A Case Series

Hodge¹,

Peterson²,

O’Connell³

et al. 2019

IJO

View full text Add to dashboard Cite

Background: Goldenhar Syndrome, also called Oculoauricular Vertebral Dysplasia, includes abnormalities of the first and second branchial arches. Ear abnormalities are present in approximately 92% of those with Goldenhar Syndrome [1]. There is a wide spectrum for ear anomalies; they can range from preauricular tags and pits, microtia, external auditory canal (EAC) atresia, ossicular malformations, and facial nerve hypoplasia, which can lead to an array of clinical findings [5]. Hearing loss is an unfortunate consequence of these clinical findings, and often results in subsequent speech and language delays [4]. Despite the widely reported auricular and audiologic manifestations of this disease, a review of the literature revealed no detailed description or analysis of congenital canal cholesteatoma specifically in this patient population. Objective: This study aims to characterize congenital external auditory canal cholesteatoma as a feature of Goldenhar Syndrome. Method: A retrospective case series was conducted with patients seen at the UNC Craniofacial Center in Chapel Hill, NC. Inclusion criteria included patients with a diagnosis of Goldenhar Syndrome also found to have congenital cholesteatoma necessitating surgery. A total of three patients were identified who met inclusion criteria. Results: Three patients with Goldenhar Syndrome were identified who also had congenital external auditory canal cholesteatoma. All three patients underwent surgical management of their disease. Conclusion: Congenital external auditory canal cholesteatoma appears to be a feature of Goldenhar Syndrome not widely described in current literature. Though rare, it is a relevant disease process with significant clinical implications for both hearing as well as speech and language development. Awareness of this condition can thus help guide practitioners in the care of these patients.

show abstract

“…In addition, malformations of the middle and inner ear have been described, including the absence of oval and round windows, malformed auditory ossicles, hypoplasia of the cochlea, and a short or wide internal auditory canal 13,14 . Consequently, these ear anomalies may cause varying types and degrees of hearing loss 12,15 .…”

mentioning

confidence: 99%

Hearing impairment and ear anomalies in craniofacial microsomia: a systematic review

Rooijers

Tio

Schroeff

et al. 2022

International Journal of Oral and Maxillofacial Surgery

View full text Add to dashboard Cite

The aim of this systematic review was to review the literature on hearing impairment and ear anomalies in patients with craniofacial microsomia and to determine their prevalence. Sixty-two records including 5122 patients were included. Ear anomalies were present in 52-100% of patients. The most reported external ear malformations were microtia, pre-auricular tags, and atresia of the external auditory canal. Ossicular anomalies were the most reported middle ear malformations, whereas the most reported inner ear malformations included oval window anomalies, cochlear anomalies, and anomalies of the semicircular canals. Hearing loss in general was reported in 29-100% of patients, which comprised conductive hearing loss, mixed hearing loss, and sensorineural hearing loss. Between 21% and 51% of patients used hearing aids, and 58% underwent a surgical intervention to improve hearing. The relationship between different phenotypes of craniofacial microsomia and the type and severity of hearing loss is mostly unclear. In conclusion, the high prevalence of ear and hearing anomalies in patients with craniofacial microsomia underlines the importance of audiological screening in order to facilitate individual treatment.

show abstract

Hearing characterization in oculoauriculovertebral spectrum: A prospective study with 10 patients

Cited by 10 publications

References 15 publications

Hearing Impairment In Oculoauriculovertebral Dysplasia (Goldenhar Syndrome): Literature Review

Hearing Impairment In Oculoauriculovertebral Dysplasia (Goldenhar Syndrome): Literature Review

Clinical Presentation of Congenital Cholesteatoma of the External Auditory Canal in Goldenhar Syndrome: A Case Series

Hearing impairment and ear anomalies in craniofacial microsomia: a systematic review

Contact Info

Product

Resources

About