scholarly journals Hearing loss in the Saethre-Chotzen syndrome

1996 ◽  
Vol 110 (10) ◽  
pp. 952-957 ◽  
Author(s):  
Robbert J. H. Ensink ◽  
Henri A. M. Marres ◽  
Han G. Brunner ◽  
Cor W. R. J. Cremers
Keyword(s):  
Hearing Loss ◽  
Middle Ear ◽  
Conductive Hearing Loss ◽  
Ossicular Chain ◽  
Middle Ear Surgery ◽  
Ear Canal ◽  
Ear Surgery ◽  
Generation Family ◽  
Ear Infections ◽  
Conductive Hearing

AbstractA three-generation family with Saethre-Chotzen syndrome and an isolated case are presented. The proband presented with conductive hearing loss. His mother and grandmother showed minor features of the syndrome including conductive hearing loss.Symptoms of the craniosynostosis syndromes can include stapes ankylosis, a fixed ossicular chain in a too small epitympanum, and small or even absent mastoids. The proband was treated with a boneanchored hearing aid (BAHA) instead of reconstructive middle ear surgery. Current literature on the results of ear surgery is reviewed. In general, reconstructive middle ear surgery should only be considered if congenital anomalies of the middle ear are the only presenting symptom. In cases with additional anomalies such as atresia of the ear canal or damage due to chronic ear infections, the outcome of reconstructive surgery to correct the anomalous ossicular chain is unsatisfactory. In such cases the BAHA is probably the best solution.

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.)

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.

Hearing loss

2018 ◽  
Author(s):  
James Ramsden
Keyword(s):  
Hearing Loss ◽  
Facial Nerve ◽  
Middle Ear ◽  
Cranial Nerve ◽  
Nerve Palsy ◽  
Facial Nerve Palsy ◽  
Conductive Hearing Loss ◽  
Ear Canal ◽  
Eighth Cranial Nerve ◽  
Conductive Hearing

Hearing loss must be divided into conductive hearing loss (CHL) and sensorineural hearing loss (SNHL). CHL is caused by sound not reaching the cochlear (abnormality of the ear canal, tympanic membrane, middle ear, or ossicles), whereas SNHL is a condition affecting the cochlear or auditory (eighth cranial) nerve. Hearing loss may be accompanied by other cardinal signs of ear disease, such as pain or discharge from the ear, vertigo, facial nerve palsy, and tinnitus, which guide the diagnosis. This chapter describes the approach to the patient with hearing loss.

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.

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 Improvement in the Patients Operated on Otosclerosis by Right-Handed Surgeons

2020 ◽  
pp. 014556132096733
Author(s):  
Agnieszka Wiatr ◽  
Jacek Skladzien ◽  
Maciej Wiatr
Keyword(s):  
Hearing Loss ◽  
Human Factor ◽  
Conductive Hearing Loss ◽  
The Self ◽  
Middle Ear Surgery ◽  
Hearing Improvement ◽  
Ear Surgery ◽  
The Right ◽  
Similar Experience ◽  
Conductive Hearing

Background: Otosclerosis is a disease of the osseous labyrinth. The disease causes 5% to 9% of all cases of hearing loss and 18% to 22% of conductive hearing loss. The treatment of choice is a surgery. The hearing improvement after the operation is determined by various factors. Aims/Objectives: The aim of the analysis is to determinate changes in hearing after stapedoplasty in view of surgery side in the patients operated on otosclerosis by right-handed surgeons. Material and Methods: The analysis involved patients hospitalized and operated on otosclerosis between 2012 and 2018. Only patients with their first middle ear surgery due to otosclerosis were included in the study. The patients were operated by 2 right-handed surgeons who used the same surgical technique and had similar experience in otosclerosis surgery. The study included patients who were divided into 2 groups: with self-tightening prosthesis and with manually tightening prosthesis. Results: The procedure performed by right-handed operators on the left side using prostheses requiring manual fixation on the incus was associated with poorer audiometric results compared to the results of surgeries on the right side. In patients with the self-tightening prostheses, the audiometric improvement of hearing was bilaterally comparable independently from operation side. Conclusion: (1) The dependence of hearing improvement on the surgery side was demonstrated in cases of surgeries performed on the left ear by right-handed surgeons, particularly with manually tightening prosthesis. (2) Self-tightening prostheses in stapedotomy limit the human factor, reducing the risk of complications after otosclerosis surgery and provide repeatable hearing improvement.

Ear Malformation and Hearing Loss in Patients with Treacher Collins Syndrome

1993 ◽  
Vol 30 (1) ◽  
pp. 97-103 ◽  
Author(s):  
Gaylene Pron ◽  
Cheryl Galloway ◽  
Derek Armstrong ◽  
Jeffrey Posnick
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Middle Ear ◽  
Conductive Hearing Loss ◽  
Treacher Collins Syndrome ◽  
External Ear ◽  
Ear Canal ◽  
Ear Malformations ◽  
Asymmetric Hearing Loss ◽  
Conductive Hearing

Although the hearing loss of patients with Treacher Collins syndrome is well documented, few studies have reported jointly on their hearing loss and ear pathology. This paper reports on the hearing loss and computerized tomography (CT) assessments of ear malformations in a large pediatric series of patients with Treacher Collins. Of the 29 subjects assessed by the Craniofacial Program between 1986 and 1990, paired audiologic and complete CT assessments were available for 23 subjects. The external ear canal abnormalities were largely symmetric, either bilaterally stenotic or atretic. In most cases, the middle ear cavity was bilaterally hypoplastic and dysmorphic, and ossicles were symmetrically dysmorphic or missing. Inner ear structures were normal in all patients. The majority of patients had a unilateral or bilateral moderate or greater degree of hearing loss and almost half had an asymmetric hearing loss. The hearing loss of all subjects was conductive, except for three whose loss was bilateral mixed. Two types of bilaterally symmetric hearing loss configurations, flat and reverse sloping, were noted. Conductive hearing loss in patients with Treacher Collins is mainly attributable to their middle ear malformations, which are similar for those of patients with malformed or missing ossicles.

scholarly journals Isolated Incudostapedial Cholesteatomas: Unique Radiologic and Surgical Features

2020 ◽  
pp. 014556132097378
Author(s):  
Bridget MacDonald ◽  
Krishna Bommakanti ◽  
Moises Mallo ◽  
Daniela Carvalho
Keyword(s):  
Hearing Loss ◽  
Soft Tissue ◽  
Ct Scan ◽  
Middle Ear ◽  
Conductive Hearing Loss ◽  
Soft Tissue Mass ◽  
Ossicular Chain ◽  
Ct Scans ◽  
Tissue Mass ◽  
Conductive Hearing

Objectives: Congenital cholesteatomas originate from epithelial tissue present within the middle ear in patients with an intact tympanic membrane, no history of otologic surgery, otorrhea, or tympanic membrane perforation. They are diagnosed by a pearl-like lesion on otoscopy and computed tomography (CT) scan showing an expansile soft-tissue mass. We describe a series of patients with no prior otologic history presenting with progressive unilateral conductive hearing loss and normal otoscopy. The CT scans showed ossicular erosion without obvious soft-tissue mass. Surgery confirmed incudostapedial erosion found to be cholesteatoma. In this study, we characterize the clinical course of patients diagnosed with isolated incudostapedial cholesteatoma (IIC) and review possible pathologic mechanisms. Methods: Retrospective review of IIC cases treated by the Department of Pediatric Otolaryngology, Rady Children’s Hospital, San Diego, 2014 to 2020. Data included patient demographics, clinical features, imaging, surgical findings, and audiologic data. Results: Five patients were diagnosed with IIC (3 [60%] female; mean age at presentation 10.7 years [range 5.5-16.0]). All patients presented with postlingual unilateral conductive hearing loss and normal otoscopy without any past otologic history; delay in diagnosis ranged from 4 months to several years. The CT scans showed ossicular chain erosion with an absent long process of the incus and/or stapes superstructure. All patients underwent middle ear exploration, revealing a thin layer of cholesteatoma in the incudostapedial region, confirmed by histopathology. Mean preoperative speech reception threshold was 55 dB and improved to a mean of 31 dB in the 4 patients who underwent ossicular chain reconstruction. Conclusion: Isolated incudostapedial cholesteatoma should be included as a possible etiology in pediatric patients with insidious onset of unilateral conductive hearing loss with normal otoscopy, unremarkable otologic history, and a CT scan showing ossicular abnormality/disruption without notable middle ear mass. These patients should be counseled preoperatively regarding the possibility of cholesteatoma and should undergo middle ear exploration with possible ossiculoplasty.

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