Pierre Robin syndrome: characteristics of hearing loss, effect of age on hearing level and possibilities in therapy planning

1996 ◽  
Vol 110 (9) ◽  
pp. 830-835 ◽  
Author(s):  
Jadranka Handžić-Ćuk ◽  
Višeslav Ćuk ◽  
Ranko Rišavi ◽  
Vladimir Katić ◽  
Damir Katušić ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Social Contact ◽  
Hearing Level ◽  
Pierre Robin Syndrome ◽  
Ventilation Tubes ◽  
Temporal Bones ◽  
Isolated Cleft Palate ◽  
Conductive Hearing ◽  
Pierre Robin ◽  
Effect Of Age

AbstractHearing loss was studied in 22 patients with Pierre Robin syndrome (PRS) aged three to 12 years (median 5.0 years). The results were compared to those obtained in 62 patients with isolated cleft palate (ICP) aged one to 27 years (median 5.5 years). Hearing loss was more frequently found in PRS (73.3 per cent) than in ICP (58.1 per cent) patients (p = 0.02). PRS patients had more ears with moderate (21–40 dB) and severe (>40 dB) hearing loss, disturbing their social contact, with no tendency to normalization with age (Spearman r = 0.065). In contrast to PRS, ICP patients showed a significant tendency to hearing level normalization with ageing (Spearman r = −0.453; p = 0.001). Planigraphs of temporal bones showed inadequately developed pneumatization of the mastoid bone in all PRS patients and in most ICP patients. No malformation of the inner or middle ear was found in either group. PRS patients have a significantly higher risk of conductive hearing loss than those with ICP. Use of tympanostomy (ventilation) tubes is therapy of choice in patients with Pierre Robin syndrome, and it should be introduced as early as possible, even at the same time as palatoplasty.

Hearing Levels in Pierre Robin Syndrome

1995 ◽  
Vol 32 (1) ◽  
pp. 30-36 ◽  
Author(s):  
Jadranka Handžić ◽  
Marijo Bagatin ◽  
Radovan Subotić ◽  
Višeslay Ćuk
Keyword(s):  
Hearing Loss ◽  
Middle Ear ◽  
Cleft Lip ◽  
Middle Ear Effusion ◽  
Pierre Robin Syndrome ◽  
Ear Malformations ◽  
Ventilation Tubes ◽  
Pierre Robin ◽  
Ear Effusion ◽  
Air Conduction

Hearing was tested in 18 patients with Pierre Robin syndrome (PRS). These results were compared with those obtained for 243 patients with either cleft lip or cleft palate, or both. None of the PRS group patients had middle ear or inner ear malformations, or sensorineural hearing loss in speech frequencies. Hearing loss in PRS is usually conductive, bilateral, and more frequent in PRS patients (30 ears or 83.33%) than in patients who do not have PRS (290 ears or 59.67%). A significantly higher (p < .01) mean of hearing loss for air conduction in speech frequencies (MHLSF = 24.5 dB) was found in PRS patients than in patients without PRS (MHLSF = 17.8 dB). The ears of the PRS patients with hearing loss were examined, revealing middle ear effusion. In all cases, hearing was restored to a normal level through suction and the use of ventilation tubes.

Dehiscence of Bone Overlying the Superior Semicircular Canal as a Cause of an Air-Bone Gap on Audiometry

2003 ◽  
Vol 12 (1) ◽  
pp. 11-16 ◽  
Author(s):  
Kenneth M. Cox ◽  
Daniel J. Lee ◽  
John P. Carey ◽  
Lloyd B. Minor
Keyword(s):  
Hearing Loss ◽  
High Resolution ◽  
Middle Ear ◽  
Semicircular Canal ◽  
Conductive Hearing Loss ◽  
Superior Canal Dehiscence ◽  
High Resolution Ct ◽  
Temporal Bones ◽  
Conductive Hearing ◽  
Canal Dehiscence

Dehiscence of bone overlying the superior semicircular canal can result in a syndrome of vertigo and oscillopsia induced by loud noises or by maneuvers that change middle ear or intracranial pressure. Patients with this disorder can also experience a heightened sensitivity to bone-conducted sounds in the presence of normal middle ear function. High-resolution CT scans of the temporal bones demonstrate the dehiscence. The authors describe a patient with bilateral superior canal dehiscence who had bilateral low-frequency conductive hearing loss, normal middle ear function, intact acoustic reflexes, and intact vestibular-evoked myogenic potentials. These findings would not be expected on the basis of a middle ear cause of the conductive hearing loss. A high-resolution CT scan of the temporal bones in this patient revealed bilateral superior canal dehiscence. Normal acoustic immittance findings in the presence of conductive hearing loss should alert clinicians to the possibility of inner ear cause of an air-bone gap due to superior canal dehiscence

Is the Conductive Hearing Loss in NOG-Related Symphalangism Spectrum Disorder Congenital?

ORL ◽  
2021 ◽  
pp. 1-7
Author(s):  
Takahiro Nakashima ◽  
Akira Ganaha ◽  
Shougo Tsumagari ◽  
Takeshi Nakamura ◽  
Yuusuke Yamada ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Family History ◽  
Conductive Hearing Loss ◽  
Hearing Threshold ◽  
Auditory Brainstem ◽  
Spectrum Disorder ◽  
Hearing Level ◽  
Success Rates ◽  
Brainstem Response ◽  
Conductive Hearing

We describe a dominant Japanese patient with progressive conductive hearing loss who was diagnosed with <i>NOG</i>-related symphalangism spectrum disorder (<i>NOG</i>-SSD), a spectrum of congenital stapes fixation syndromes caused by <i>NOG</i> mutations. Based on the clinical features, including proximal symphalangism, conductive hearing loss, hyper­opia, and short, broad middle, and distal phalanges of the thumbs, his family was diagnosed with stapes ankylosis with broad thumbs and toes syndrome (SABTT). Genetic analysis revealed a heterozygous substitution in the <i>NOG</i> gene, c.645C&#x3e;A, p.C215* in affected family individuals. He had normal hearing on auditory brainstem response (ABR) testing at ages 9 months and 1 and 2 years. He was followed up to evaluate the hearing level because of his family history of hearing loss caused by SABTT. Follow-up pure tone average testing revealed the development of progressive conductive hearing loss. Stapes surgery was performed, and his post-operative hearing threshold improved to normal in both ears. According to hearing test results, the stapes ankylosis in our SABTT patient seemed to be incomplete at birth and progressive in early childhood. The ABR results in our patient indicated the possibility that newborn hearing screening may not detect conductive hearing loss in patients with <i>NOG</i>-SSD. Hence, children with a family history and/or known congenital joint abnormality should undergo periodic hearing tests due to possible progressive hearing loss. Because of high success rates of stapes surgeries in cases of SABTT, early surgical interventions would help minimise the negative effect of hearing loss during school age. Identification of the nature of conductive hearing loss due to progressive stapes ankylosis allows for better genetic counselling and proper intervention in <i>NOG</i>-SSD patients.

scholarly journals Canal cholesteatoma in canal stenosis: a case report

2019 ◽  
Author(s):  
Asma Abdullah ◽  
Wan Nabila Wan Mansor ◽  
Mark Paul ◽  
Noor Dina Hashim
Keyword(s):  
Hearing Loss ◽  
Conductive Hearing Loss ◽  
Advanced Stage ◽  
Radiological Evaluation ◽  
Corrective Surgery ◽  
Canal Stenosis ◽  
Canal Atresia ◽  
Temporal Bones ◽  
Conductive Hearing ◽  
Rule Out

Purpose : We present a-12-year old female with canal stenosis and canal cholesteatoma. We discuss the clinical, radiological and treatment option for canal cholesteatoma in canal stenosis Discussion : Congenital canal atresia is a failure of the development of the external auditory canal which comprises anomalies of variable severity involving pinna, external acoustic canal, middle ear structures and rarely inner ear leading to hearing impairment. Congenital canal stenosis is considered to be a subset of congenital canal atresia. This malformation results in mild to severe conductive hearing loss. Furthermore, risk of cholesteatoma increased when it is associated with stenotic ear. Radiological evaluation in the form of HRCT scan of temporal bones should always be done in these patients during their initial presentation and surgery should be planned accordingly. Their presence should be rule out prior any corrective surgery. Conclusion : Although the incidence of canal cholesteatoma is rare in congenital canal stenosis, all patients presenting with canal atresia or stenosis should be evaluated thoroughly due to their potential to develop cholesteatoma as it may be challenging to treat at an advanced stage in view of morbid complications. 

scholarly journals Bilateral Oval and Round Window Atresia on CT Temporal Bone: A Rare Anomaly Clinically Mimicking Otosclerosis in an Adult

2019 ◽  
Vol 2019 ◽  
pp. 1-5
Author(s):  
Manzoor Ahmed ◽  
Yogesh Indrasen More ◽  
Shaik Irfan Basha
Keyword(s):  
Hearing Loss ◽  
High Resolution ◽  
Temporal Bone ◽  
Conductive Hearing Loss ◽  
Round Window ◽  
Adult Case ◽  
High Resolution Ct ◽  
Rare Anomaly ◽  
Temporal Bones ◽  
Conductive Hearing

We present a rare adult case of bilateral oval and round window atresia. Clinical and audiologic findings were suggestive of otosclerosis. High resolution CT Temporal bones showed unequivocal findings of bilateral oval and round window atresia. Atresia of these windows is a rare temporal bone anomaly. Presentation as an adult can confound the clinicians and warranting a closer look on the CT for atretic windows and subtle signs of otosclerosis in patients with conductive hearing loss.

scholarly journals Bilateral glomus tympanicum tumors: Human temporalbone study

2018 ◽  
Author(s):  
Hisaki Fukushima ◽  
Hirotaka Hara ◽  
Michael M. Paparella ◽  
Mohamed F. Oktay ◽  
Patricia A. Schachern ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Conductive Hearing Loss ◽  
Black Female ◽  
Pulsatile Tinnitus ◽  
University Of Minnesota ◽  
Glomus Tympanicum ◽  
Temporal Bones ◽  
Conductive Hearing ◽  
Tympanic Membranes

To describe human temporal bones with bilateral glomus tympanicum tumors. Patient is 83-year-old black female who no pulsatile tinnitus. The histopathologic characteristics of human temporal bones after death were setting Department of Otolaryngology of University of Minnesota in USA. Histopathologic observation of temporal bones showed bilateral small glomus tympanicum tumors limited to the promontory. Although there was bilateral tinnitus, there was no pulsatile tinnitus, no conductive hearing loss and both of the tympanic membranes were intact. Histopathologic observation of temporal bones after death showed bilateral glomus tympanicum tumors. To our knowledge, this is the first reported case of bilateral glomus tympanicum tumors.

scholarly journals A giant cholesteatoma of the mastoid extending into the foramen magnum: A case report and review of literature

2018 ◽  
Vol 10 (1) ◽  
Author(s):  
Seidu A Richard ◽  
Li Qiang ◽  
Zhi Gang Lan ◽  
Yuekang Zhang ◽  
Chao You
Keyword(s):  
Hearing Loss ◽  
Tympanic Membrane ◽  
Squamous Epithelium ◽  
Conductive Hearing Loss ◽  
Foramen Magnum ◽  
Imaging Findings ◽  
Review Of Literature ◽  
Resonance Imaging ◽  
Temporal Bones ◽  
Conductive Hearing

Cholesteatomas are very rare benign, progressive lesions that have embryologic derivation and usually result in progressive exfoliation and confinement of squamous epithelium behind an intact or preciously infected tympanic membrane. To the best of our understanding no reports demonstrates the extension of cholesteatoma from the temporal bone into the foramen magnum. We therefore present a case of cholesteatoma extending down into the foramen magnum. We report a case of 67- year-old man with a giant cholesteatoma extending into the foramen magnum without substantial destruction of the mastoid and petrous temporal bones. The patient’s major symptoms were recurrent tinnitus in the left ear and dizziness with unilateral conductive hearing loss. A working diagnosis of cholesteatomas was made combining the symptoms and magnetic resonance imaging findings. He was then successfully operated on with very minimal postoperative complications. Cholesteatomas originating from the mastoid bone often linger with the patients for many years in a subclinical state and progress into a massive size before causing symptoms. Patients with unilateral conductive hearing loss who are otherwise asymptomatic and have a normal tympanic membrane should be suspected with a progressive cholesteatoma. Cholesteatoma should be one of the working diagnosis when an elderly patient present with unilateral conductive hearing loss that is associated with tinnitus and dizziness.

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