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.

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.

Congenital Anomalies Limited to the Middle Ear

1992 ◽  
Vol 106 (3) ◽  
pp. 285-287 ◽  
Author(s):  
Howard K. Herman ◽  
Charles P. Kimmelman
Keyword(s):  
New York ◽  
Hearing Loss ◽  
Middle Ear ◽  
Congenital Anomalies ◽  
Conductive Hearing Loss ◽  
Oval Window ◽  
Hearing Improvement ◽  
Surgical Indications ◽  
Ear Anomalies ◽  
Conductive Hearing

Congenital anomalies of the middle ear are occasionally encountered during surgery for conductive hearing loss and are unexpected in patients with no other deformities. We reviewed 12 such patients operated on at The New York Eye and Ear Infirmary from 1985 through 1989. Nine of the patients (75%) had unilateral conductive hearing loss whereas three (25%) had bilateral symptoms. One had bilateral congenital middle ear anomalies. Three patients (25%) had anomalies limited to the malleus and scutum. Five patients (47%) had agenesis of the oval window. After reconstructive surgery, 72% of patients had hearing improvement ranging from 13 to 38 dB. The etiology of these anomalies is discussed and their evaluation and surgical indications are presented.

Use of hearing AIDS

1976 ◽  
Vol 14 (12) ◽  
pp. 45-46
Keyword(s):  
Hearing Loss ◽  
Middle Ear ◽  
Hearing Aids ◽  
Conductive Hearing Loss ◽  
Auditory Training ◽  
Lip Reading ◽  
Conductive Hearing

Up to 3 million people in Britain might be helped by hearing aids.1 2 Most are over 65 years of age, but some are infants. All should be referred to specialist centres for assessment as soon as possible. Hearing aids generally help most in disorders of the middle ear (conductive hearing loss); they can also help those with sensorineural and other forms of hearing loss. The use of an aid often needs to be supplemented by lip reading and other means of auditory training.1 3

Conductive SISI Test

1974 ◽  
Vol 83 (1) ◽  
pp. 125-127 ◽  
Author(s):  
Vincent W. Byers
Keyword(s):  
Hearing Loss ◽  
Middle Ear ◽  
Statistical Difference ◽  
Conductive Hearing Loss ◽  
Bone Conduction ◽  
Sensitivity Index ◽  
Index Test ◽  
Bone Conduction Threshold ◽  
Conductive Hearing

The conductive SISI (short increment sensitivity index) test is an indirect procedure to estimate bone-conduction thresholds for middle ear pathology patients. A series of SISI tests are run, beginning at 20 dB S.L. and increasing in 10 dB S.L. steps, until a 100% SISI score is obtained. The following equation predicts the bone-conduction threshold: [Formula: see text] The results of 25 conductive SISI tests on a conductive hearing loss group indicate that the equation approximates the measured B.C. threshold. There was no statistical difference between the predicated B.C. thresholds (12.4 dB) and measured B.C. thresholds (10.4 dB) for the group.

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 Middle Ear Adenoma: Case Report and Discussion

2014 ◽  
Vol 2014 ◽  
pp. 1-4
Author(s):  
D. Isenring ◽  
T. F. Pezier ◽  
B. Vrugt ◽  
A. M. Huber
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Middle Ear ◽  
Conductive Hearing Loss ◽  
Surgical Excision ◽  
Histological Techniques ◽  
Preoperative Ct ◽  
Middle Ear Adenoma ◽  
Conductive Hearing ◽  
Ct And Mri

Introduction. Despite modern radiological workup, surgeons can still be surprised by intraoperative findings or by the pathologist’s report.Materials & Methods. We describe the case of a 52-year-old male who was referred to our clinic with a single sided conductive hearing loss. He ultimately underwent middle ear exploration and excision of a middle ear tumour followed by second look and ossiculoplasty a year later.Results. Though preoperative CT and MRI scanning were suggestive of a congenital cholesteatoma, the pathologist’s report diagnosed a middle ear adenoma.Discussion. Middle ear glandular tumors are extremely rare and, despite numerous histological techniques, continue to defy satisfactory classification. Most surgeons advocate surgical excision though evidence of the tumour’s natural course and risk of recurrence is lacking.

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 Neuroendocrine Adenoma of the Middle Ear (NAME)

2009 ◽  
Vol 88 (4) ◽  
pp. 874-879 ◽  
Author(s):  
Karen Leong ◽  
Marian M. Haber ◽  
Venu Divi ◽  
Robert T. Sataloff
Keyword(s):  
Hearing Loss ◽  
Otitis Media ◽  
Middle Ear ◽  
Conductive Hearing Loss ◽  
Carcinoid Tumors ◽  
Rare Tumor ◽  
Pathologic Findings ◽  
History Of ◽  
Conductive Hearing ◽  
Recurrent Otitis Media

Neuroendocrine adenoma of the middle ear (NAME) is a rare tumor. We report a case of NAME, the clinical and pathologic findings of which illustrate the biologic behavior of adenomatous tumors of the middle ear and their relationship with rare carcinoid tumors of the middle ear. A 29-year-old man presented with a history of recurrent otitis media, right conductive hearing loss, and aural fullness. The tumor was removed in its entirety. Otolaryngologists should be familiar with this unusual but important entity.

Modified sleeve tympanotomy approach for removal of congenital cholesteatoma

2008 ◽  
Vol 122 (12) ◽  
pp. 1365-1367 ◽  
Author(s):  
H J Park ◽  
G H Park ◽  
J E Shin ◽  
S O Chang
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Tympanic Membrane ◽  
Middle Ear ◽  
Conductive Hearing Loss ◽  
Tympanic Cavity ◽  
Operative Techniques ◽  
Congenital Cholesteatoma ◽  
The Right ◽  
Conductive Hearing

AbstractObjective:We present a technique which we have found useful for the management of congenital cholesteatoma extensively involving the middle ear.Case report:A five-year-old boy was presented to our department for management of a white mass on the right tympanic membrane. This congenital cholesteatoma extensively occupied the tympanic cavity. It was removed through an extended tympanotomy approach using our modified sleeve technique. The conventional tympanotomy approach was extended by gently separating the tympanic annulus from its sulcus in a circular manner. The firm attachment of the tympanic membrane at the umbo was not severed, in order to avoid lateralisation of the tympanic membrane.Conclusion:Although various operative techniques can be used, our modified sleeve tympanotomy approach provides a similarly sufficient and direct visualisation of the entire middle ear, with, theoretically, no possibility of lateralisation of the tympanic membrane and subsequent conductive hearing loss.

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