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.

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 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.

scholarly journals Unusual case of unilateral conductive hearing loss: chronic lymphocytic leukaemia

2018 ◽  
pp. bcr-2017-223444
Author(s):  
Amelia Leigh Davis ◽  
Shane Gangatharan ◽  
Jafri Kuthubutheen
Keyword(s):  
Hearing Loss ◽  
Differential Diagnosis ◽  
Chronic Lymphocytic Leukaemia ◽  
Unusual Case ◽  
Lymphocytic Leukaemia ◽  
Conductive Hearing Loss ◽  
Cutaneous Lesion ◽  
External Ear ◽  
Ear Canal ◽  
Conductive Hearing

This presentation reports a novel case of chronic lymphocytic leukaemia (CLL), presenting with an early cutaneous lesion within the external auditory canal, in a patient being assessed for conductive hearing loss. It has previously been reported that infiltrative CLL can involve the head and neck; however, isolated external ear canal involvement is rare. Given that the incidence of CLL in Australia is rising, this case highlights the importance of considering CLL as a differential diagnosis for presentations of unilateral conductive hearing loss.

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.

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.)

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