Ossicular Anomalies Research Articles

Ossiculoplasty In Congenital Hearing Loss

We have presented an extensive review of congenital ossicular anomalies. These deformities occur in combination with one another or as isolated problems with equal frequency. They are so rare that even the busiest otologists have limited clinical experience with them. The astute otologist will keep these facts in mind when assessing congenital middle ear problems in the office and in the operating room. Careful, detailed history taking may reveal the presence of familial conductive hearing loss or other congenital syndromes. Complete head and neck examination as well as observation of the extremities may provide clues to the otologic diagnosis. Microscopic examination of the ear reveals abnormalities of the tympanic membrane, malleus, and incus. Audiometric and radiologic evaluation augments physical examination. At surgery, the otologist must be prepared to perform a variety of possible surgical procedures, from traditional ossiculoplasty and stapedectomy to fenestration. Hearing aids are an option for patients with congenital ossicular anomalies, and the possibility of inoperability must be kept in mind; the patient must be prepared for this eventuality. Congenital ossicular anomalies present a challenge to the otologic surgeon, and their successful treatment, the greatest reward.

鼓膜正常な耳小骨異常 診断と治療

In 116 ears of 106 patients with ossicular abnormalities with intact tympanic membranes, the preoperative diagnosis, differential diagnosis, operative findings and postoperative results were analyzed. The ossicular abnormalities in this study included 44 ears with otosclerosis, 48 with congenital anomalies and 22 with traumatic ossicular disruption.For the preoperative diagnosis, history taking and stapedial reflex tests were thought to be more reliable and useful diagnostic tools than tympanograms and radiological imaging.In congenital anomalies of the ossicular chain, surgeons should pay attention to auricular and facial anomalies even those of minimal degree, because there might be a close relationship between the ossicular anomaly and an auricular or facial anomaly.In traumatic ossicular disruption, history taking is indispensable and may disclose a history of minor head trauma such as falling down stairs in childhood.Reconstruction of the ossicular chain is most often successful for otosclerosis, and for traumatic disruption, but is not always satisfactory for congenital anomalies. Ossicular anomalies may be associated with other anatomical abnormalities such as facial nerve dehiscence, perilymph gusher etc. in exploratory tympanotomy. If so, the surgical results will not be satisfactory.In obtaining informed consent, all surgeons should be aware of these unexpected situations, and complete information regarding the surgical complications and the operative results should be given to the patient and his or her family.

耳介小奇形を伴った耳小骨奇形症例の検討

The relationship between auricular malformations and ossicular anomalies was investigated in 7 ears from 5 patients with congenital conductive hearing loss. A close relationship was found between the following ossicular anomalies and auricular malformations; 1) anomalies of the antihelical crus and a bony bridge from the pyramidal eminence and stapes; 2) absence of the inferior crus of the antihelix and malformation of the long process of the incus ; 3) anomalies of the tragus and a stenotic external auditory meatus. Careful examination of the auricle may provide a diagnostic clue in some types of ossicular anomaly.

Diagnosis of ossicular chain in the middle ear by high-resolution CT

This study was conducted to assess the usefulness and limitation of high-resolution CT for evaluating the condition of ossicular chain in the middle ear. Preoperative CT findings of the ossicular chain were compared with the operative findings of ossicles in 26 patients with chronic otitis media or congenital ossicular anomaly who underwent tympanoplasty. Total defect of head of the malleus, body of the incus and long process of the incus were completely detected by high-resolution CT. But the reliability in detecting the defect of handle of the malleus and superstructure of the stapes were 33.3% and 60%, respectively. Defect of the I-S joint (1 case) and partial defect of stapes crus (2 cases) could not be diagnosed correctly in the preoperative estimation. Although these findings demonstrate the limitations of high-resolution CT in the diagnosis of ossicular chain, it will be diminished by the advanced space resolution of CT in the future.

Congenital Ossicular Anomaly Accompanied with Congenital Cholesteatoma

Congenital Ossicular Anomaly Accompanied with Congenital Cholesteatoma

当科における耳小骨奇形の検討

当科における耳小骨奇形の検討

外耳奇形を伴わぬ耳小骨奇形の診断と治療

外耳奇形を伴わぬ耳小骨奇形の診断と治療

Townes-Brocks syndrome. Report of a case and review of the literature : Ferraz F.G., Nunes L., Ferraz M.E., Sousa J.P., Santos M., Carvalho C. and Maroteaux P.Ann. Genet. (1989) 32/2 (120–123)

The authors report a new case of Townes-Brocks syndrome with cardiac defect, ossicular anomalies and dominant transmission. The intrafamilial variability of the phenotype and the difficulty of diagnosis in isolated cases are underlined.

Anomalies of the middle ear

Malformations of the ossicles in 52 ears of 48 patients were analyzed on the basis of surgical findings. The external meatus of each of these ears was patent. The long process of the incus and the stapes are frequently involved in this series of observations. There are distinct groups of ossicular anomalies, in each of which patients show similar degrees of hearing loss and ossicular findings. These include spindle handle, malleus bar, and dislocated annulus. Spindle handle gets its name from the shape of the malleus handle. There is disconnection between the long process of the incus and the capitulum of the stapes. Malleus bar is a kind of malleus fixation, in which a small bony bar connects the tip of the malleus handle to the posterior wall of the tympanic cavity. In the dislocated annulus, the annulus is dislocated cranially in relation to the middle-ear structures. Anomalies of the ossicle are usually present. Surgical repairs for these conditions are discussed.

Correlation between high-resolution computed tomography and surgical findings in congenital aural atresia.

Congenital aural atresia poses a great challenge, even to a competent otologic surgeon, due to innumerable types of malformations affecting the conduction and perception of sound. Roentgenographic evaluation by plain roentgenography is inconclusive in most cases; polytomography is helping to some extent. Recent generations of high-resolution computed tomographic (CT) scanners are probably the best. Seven cases of unilateral and three cases of bilateral congenital aural atresia were evaluated with high-resolution CT. Subsequently, ten ears were operated on. Surgical findings were correlated with CT scan findings with respect to atresia plate, extent of pneumatization, ossicular anomalies, bony facial nerve canal, and inner ear. Use of CT scans bears considerable importance in the management of these types of cases. High-resolution CT scan, when targeted for maximal bony detail, is possibly the method of choice in congenital aural atresia when surgical correction is contemplated.

外耳道正常な耳小骨奇形の９症例

Five of the nine patients in this series had incudostapedial joint separation, two had stapes fixation, one had incudostapedial joint separation and incus fixation, and one had a dislocated stapes foot plate that had no contact with the oval window.Autografts were used to reconstruct the discontinuity in six cases, and good results were obtained. Stapes surgery was done in three cases, and was successful in two cases.The etiology of the anomalies observed in these patients is discussed from the standpoint of temporal bone embryology.The results of pre-operative auditory function tests were evaluated. However, it was difficult to predict pre-operatively by auditory examination which type of ossicular anomalies the patients had.

CHARGE Syndrome. Part II. Hearing loss

CHARGE is a mnemonic for a syndrome with multiple congenital anomalies that occurs with normal chromosomes. The unique external ear anomalies have been described in CHARGE Syndrome Part I in this journal. This report describes the distinctive middle ear and sensorineural losses that occur in the syndrome, both of which can be progressive and, in most cases, are moderate to severe. There is evidence to indicate that these losses are due to congenital ossicular anomalies, eustachian tube dysfunction from craniofacial malformation, and cochlear involvement that is greatest for high frequencies.

Congenital malformations of the external and middle ear: high-resolution CT findings of surgical import.

The external auditory canal, middle ear, and bulk of the ossicular chain develop from the first branchial groove, first and second branchial arches, and first pharyngeal pouch. Embryologic development of these structures is complex and only rarely are two anomalies identical. Development of the inner ear structures occurs independently of external ear structures, and concomitant involvement is unusual. This study includes 11 cases of unilateral external auditory canal atresia and two cases of bilateral atresia. Eight cases (four bilateral) of isolated congenital ossicular anomalies are also included. Emphasis is placed on findings of surgical import. All patients were studied with computed tomography only, because it was believed that the bony and soft-tissue detail achieved is superior to that with conventional multidirectional tomography.

外耳道正常な中耳奇形の発生学的検討 中耳奇形と耳介・あご・顔面形態との関連

外耳道正常な中耳奇形の発生学的検討 中耳奇形と耳介・あご・顔面形態との関連

Congenital heart disease, deafness, andskeletal malformations: A new syndrome?

The unusual occurrence of mitral insufficiency, conductive deafness, short stature,and skeletal anomalies in 3 members of one family is reported and appears to represent a new syndrome. The skeletal anomalies appear as bony fusion, involving the cervical vertebrae, ossicles, and carpal and tarsal bones. Except for deafness related to ossicular anomalies, no functional loss has resulted from the bone malformations. The basic defect involves only mesodermal structures and appears to be genetically transmitted as simple dominant.