scholarly journals Ear Surgery in Treacher Collins Syndrome

1995 ◽  
Vol 104 (1) ◽  
pp. 31-41 ◽  
Author(s):  
Henri A. M. Marres ◽  
H. M. A. Marres ◽  
W. R. J. Cremers ◽  
Patrick L. M. Huygen
Keyword(s):  
Hearing Loss ◽  
Autosomal Dominant ◽  
Conductive Hearing Loss ◽  
Branchial Arch ◽  
Treacher Collins Syndrome ◽  
Ear Surgery ◽  
Arch System ◽  
Conductive Hearing ◽  
Outcome Of Surgery ◽  
Insight Into

The autosomal dominant hereditary Treacher Collins syndrome manifests itself phenotypically in dysmorphogenesis of particularly the first, but also the second branchial arch system. Consequently, 50% of patients with Treacher Collins syndrome have a congenital, generally pure conductive hearing loss resulting from a major or minor ear anomaly. The outcome of surgery to improve patients' hearing varies and is sometimes even disappointing. Thorough analysis of 33 cases (39 operated ears) and the strict application of a classification for the anomaly to each ear enabled us to gain insight into the most suitable surgical policy and to form a prognosis for reconstructive ear surgery.

Modification of auditory pathway functions in patients with hearing improvement after middle ear surgery

1998 ◽  
Vol 119 (1) ◽  
pp. 125-130 ◽  
Author(s):  
Juha-Pekka Vasama ◽  
Jyrki P. Mäkelä ◽  
Hans A. Ramsay
Keyword(s):  
Hearing Loss ◽  
Middle Ear ◽  
Pure Tone ◽  
Conductive Hearing Loss ◽  
Adult Patients ◽  
Middle Ear Surgery ◽  
Ear Surgery ◽  
Pure Tone Average ◽  
The Mean ◽  
Conductive Hearing

We recorded auditory-evoked magnetic responses with a whole-scalp 122-channel neuromagnetometer from seven adult patients with unilateral conductive hearing loss before and after middle ear surgery. The stimuli were 50-msec 1-kHz tone bursts, delivered to the healthy, nonoperated ear at interstimulus intervals of 1, 2, and 4 seconds. The mean preoperative pure-tone average in the affected ear was 57 dB hearing level; the mean postoperative pure-tone average was 17 dB. The 100-msec auditory-evoked response originating in the auditory cortex peaked, on average, 7 msecs earlier after than before surgery over the hemisphere contralateral to the stimulated ear and 2 msecs earlier over the ipsilateral hemisphere. The contralateral response strengths increased by 5% after surgery; ipsilateral strengths increased by 11%. The variation of the response latency and amplitude in the patients who underwent surgery was similar to that of seven control subjects. The postoperative source locations did not differ noticeably from preoperative ones. These findings suggest that temporary unilateral conductive hearing loss in adult patients modifies the function of the auditory neural pathway. (Otolaryngol Head Neck Surg 1998;119:125-30.)

scholarly journals Mild Form of Treacher Collins Syndrome Imitating Juvenile Otosclerosis

2012 ◽  
Vol 2012 ◽  
pp. 1-3
Author(s):  
Karol Zeleník ◽  
Pavel Komínek
Keyword(s):  
Early Childhood ◽  
Hearing Loss ◽  
Middle Ear ◽  
Developmental Disorder ◽  
Conductive Hearing Loss ◽  
Treacher Collins Syndrome ◽  
Mild Form ◽  
Ear Anomalies ◽  
Conductive Hearing ◽  
Surgical Corrections

Treacher Collins syndrome (TCS) is an inherited developmental disorder. More than 40% of individuals with TCS have conductive hearing loss attributed to external and middle ear anomalies. Mild cases of TCS often pass undiagnosed at birth or early childhood. The disease may be manifested as conductive hearing loss in teenagers and may resemble juvenile otosclerosis. Patients could suffer from slight facial variabilities including retrognathia (as in our case) and others, which point out to a possible middle ear anomaly. Surgical corrections of middle ear anomalies including TCS generally lead to poorer outcomes comparing with juvenile otosclerosis, which should be discussed with parents during preoperative counselling.

Congenital stapedial suprastructure fixation with normal footplate mobility: case report

2009 ◽  
Vol 124 (6) ◽  
pp. 680-683 ◽  
Author(s):  
J H Lee ◽  
S H Jung ◽  
H C Kim ◽  
C H Park ◽  
S M Hong
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Rare Case ◽  
Conductive Hearing Loss ◽  
Branchial Arch ◽  
Fallopian Canal ◽  
Stapes Footplate ◽  
The Right ◽  
Conductive Hearing

AbstractObjective:We report a case of bilateral conductive hearing loss caused by stapedial suprastructure fixation with normal footplate mobility.Case report:A 50-year-old woman had suffered hearing loss in both ears since childhood. Exploratory tympanotomy revealed immobility of the stapes due to a bony bridge between the stapedial suprastructure and the fallopian canal. The incus was missing, while the malleus handle was minimally deformed. The mobility of the stapes footplate was normal. Post-operatively, the hearing in the right ear improved both subjectively and audiographically, while that in the left ear did not improve because of footplate subluxation during surgery.Conclusion:This is a rare case of congenital stapedial suprastructure fixation with normal footplate mobility. In this patient, development of the second branchial arch was arrested. When performing exploratory tympanotomy for stapedial fixation, one must keep in mind that normal footplate mobility is possible.

A bifid intra-tympanic facial nerve in association with a normal stapes

2006 ◽  
Vol 120 (5) ◽  
pp. 414-415 ◽  
Author(s):  
J Ahmed ◽  
P Chatrath ◽  
J Harcourt
Keyword(s):  
Hearing Loss ◽  
Facial Nerve ◽  
Middle Ear ◽  
Conductive Hearing Loss ◽  
Anatomical Variations ◽  
Middle Ear Surgery ◽  
Ear Surgery ◽  
Ossicular Reconstruction ◽  
Conductive Hearing

A rare facial nerve anomaly was incidentally discovered whilst performing a tympanoplasty and ossicular reconstruction on a patient with an acquired unilateral conductive hearing loss. The nerve was seen to bifurcate and straddle a normal stapes superstructure as it ran posteriorly through the middle ear, a unique and as yet unreported combination. This case highlights the importance of vigilance regarding facial nerve anatomical variations encountered during middle-ear surgery thus avoiding inadvertent damage. The purported embryological mechanism responsible for such anomalies of the intra-tympanic facial nerve is discussed.

Effect of Conductive Hearing Loss and Middle Ear Surgery on Binaural Hearing

1986 ◽  
Vol 95 (5) ◽  
pp. 525-530 ◽  
Author(s):  
Joseph W. Hall ◽  
Eugene L. Derlacki
Keyword(s):  
Hearing Loss ◽  
Middle Ear ◽  
Auditory Processing ◽  
Conductive Hearing Loss ◽  
Binaural Hearing ◽  
Middle Ear Surgery ◽  
Ear Surgery ◽  
Hearing Thresholds ◽  
Masking Level Difference ◽  
Conductive Hearing

This study investigated whether conductive hearing loss reduces normal binaural hearing advantages and whether binaural hearing advantages are normal in patients who have had hearing thresholds improved by middle ear surgery. Binaural hearing was assessed at a test frequency of 500 Hz using the masking level difference and interaural time discrimination thresholds. Results indicated that binaural hearing is often poor in conductive lesion patients and that the reduction in binaural hearing is not always consistent with a simple attenuation of the acoustic signal. Poor binaural hearing sometimes occurs even when middle ear surgery has resulted in bilaterally normal hearing thresholds. Our preliminary results are consistent with the interpretation that auditory deprivation due to conductive hearing loss may result in poor binaural auditory processing.

The deafness, pre-auricular sinus, external ear anomaly and commissural lip pits syndrome – otological, vestibular and radiological findings

1994 ◽  
Vol 108 (1) ◽  
pp. 13-18 ◽  
Author(s):  
H. A. M. Marres ◽  
C. W. R. J. Cremers ◽  
P. L. M. Huygen ◽  
F. B. M. Joosten
Keyword(s):  
Hearing Loss ◽  
Autosomal Dominant ◽  
Round Window ◽  
Large Family ◽  
Ossicular Chain ◽  
Branchial Arch ◽  
Hearing Improvement ◽  
Radiological Findings ◽  
Conductive Hearing

Commissural lip pits, pinna dysplasia, pre-auricular sinus and hearing loss constitute a recently described autosomal dominant branchial arch syndrome. In a large family, eight out of the 74 members were also affected by conductive hearing loss. No inner ear abnormalities could be demonstrated on the CT scans. In three patients (four ears) out of four patients (six ears), exploratory tympanotomy revealed serious ossicular chain anomalies. In one ear, round window aplasia was also present. Long-term hearing improvement could only be achieved in one ear.

scholarly journals Endoscopic Ear Surgery for the Management of Traumatic Ossicular Disruption with Intact Tympanic Membrane

2020 ◽  
Author(s):  
Chunlin Zhang ◽  
Dan Long ◽  
Yuan Deng ◽  
Mei Ynag ◽  
Dandan Guo ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Retrospective Study ◽  
Conductive Hearing Loss ◽  
Ossicular Chain ◽  
Endoscopic Ear Surgery ◽  
Ear Surgery ◽  
Temporal Bone Fracture ◽  
Hearing Outcomes ◽  
Conductive Hearing ◽  
Facial Function

Abstract Background Traumatic ossicular disruption (TOD) usually had a severe conductive hearing loss, the exploratory tympanotomy is critical for the diagnosis and improve hearing. Endoscopic ear surgery (EES) is becoming popular in the last decade, we conducted a retrospective study to explore the efficacy of EES for management of TOD and the accompanied injuries.Methods A retrospective study was performed on 18 ears (16 patients) of TOD with intact TM from May 2017 to Jun 2019 in our department. EES was conducted to check the ossicular chain anomalies, and to perform the ossiculoplasty and facial nerve (FN) decompression depending on the intraoperative findings. Hearing outcomes and surgical complications were assessed at 6 months postoperatively. Results The incus injury was the most common type of TOD, which was observed in 14 ears (77.8%), stapes suprastructure fracture was observed in 4 ears (22.2%). FN injury was found in 4 out of 13 ears with temporal bone fracture (TBF), the injury sites were mainly located in the perigeniculate area and the tympanic segment of FN. It showed the postoperative average pure-tone average (PTA) gain was 22.9 ± 9.5 dB, and the average ABG closure was 22.2 ± 8.3 dB, ABG closure to 20 dB or less and ABG closure to 10 dB or less were achieved in 18 ears (100%) and 14 ears (77.8%), respectively. The facial function achieved favorable recovery of House-Brackmann (H-B) grade Ⅰ (3 ears) and grade Ⅱ (1 ear) in all the 4 cases in 6 months after surgery. No iatrogenic FN paralysis and significant sensorineural hearing loss were observed. Conclusions ESS was effective in diagnosis and management of TOD and the accompanied otologic injuries, such as FN paralysis, it showed favorable surgical outcomes. ESS provides an alternative method to manage TOD with the advantage of excellent vision and less invasion.

scholarly journals Hearing Loss in Treacher-Collins Syndrome

2021 ◽  
Vol 51 (1) ◽  
Author(s):  
Fezzan Athama Fuadi ◽  
W Wijana
Keyword(s):  
Hearing Loss ◽  
Ossicular Chain ◽  
Treacher Collins Syndrome ◽  
Ear Surgery ◽  
Main Complaint ◽  
Sensory Neural ◽  
The Right ◽  
Facial Development ◽  
Conductive Hearing

Abstract Treacher Collins Syndrome is a craniofacial disorder that has dominant autosomal disorder in facial development, found 1 in every 50,000 births. The common manifestations can be mandibulofacial disorder, microtia, atresia of the ear canal, and hearing loss. This syndrome is also accompanied by malformations of the ossicular chain of bone in the middle ear, which can lead to conductive hearing loss up to 50% of cases and sensory neural hearing loss. The aim of this report was to present one case of Treacher-Collins Syndrome at the Audiology - Vestibular clinic Dr. Hasan Sadikin General Hospital Bandung. The main complaint of the patient is micrognathia and microtia with hearing loss in both ears. The right ear is smaller than the left ear, and supported to 2nd grade of Microtia. The patient had performed reconstruction ear surgery in the right ear, and had improvement from audiological examination after the 2nd stage of reconstruction. There was TCOF 1 gene mutation involved from this case. Conclusion: Treacher Collins syndrome is a rare inherited disorder, but the diagnose can be easily enforced. Early intervention with hearing rehabilitation, audio-verbal rehabilitation, and reconstruction ear surgery must be carried out for a better quality of life. Keyword : Treacher collins syndrome, micrognathia, microtia, hearing loss

Sign in / Sign up

Export Citation Format

Share Document