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Major congenital ear malformations: surgical management and results.

Fifteen patients with major congenital aural atresia underwent operations using an anterior (trans-atretic bone) approach. Facial nerve monitoring was used in all cases and there were no instances of facial nerve injury. Postoperatively, two thirds of the patients had speech reception thresholds of 30 dB or better; the air conduction threshold (averaged for 500, 1,000, and 2,000 Hz) improved at least 25 dB in 80% of the patients and at least 30 dB in 60% of the patients (follow-up, 10 to 29 months). Minor revision surgery was necessary in three patients. On the basis of this series, several conclusions were made. First, one of the most important factors in proper patient selection is the degree of middle ear development on computed tomography, both in terms of size and ossicular formation. Second, every attempt should be made to keep the ossicular chain intact (versus use of a prosthesis), as this appears to optimize hearing results. Third, embryologic considerations and surgical experience predict a mobile stapes in the majority of major atresias. Lastly, facial nerve abnormalities are to be expected, especially in patients with significant microtia, but facial nerve monitoring will help minimize the risk of facial nerve injury.

Adolescent↗

Ossicular abnormalities in Down's syndrome.

Down's syndrome has been associated with hearing loss and otitis media. Because of difficulty in examination, however, there have been few detailed otologic studies on this population. An understanding of the nature and frequency of ear disease in Down's syndrome is important, since it is common and occurs in more than one in every 600 live births. To define the aural manifestation of Down's syndrome, complete otologic and audiometric examination was performed on 107 consecutive patients. This included micropneumatic otoscopy, pure-tone and speech audiometry, impedance tympanometry, and, in some cases, electronystagmography. Deficient hearing was found in 64% of these patients, and of these hearing losses, 83% were conductive. Surprisingly, middle ear effusion or tympanic membrane perforation accounted for only 60% of the conductive hearing losses. This finding prompted us to examine five temporal bones of children with Down's syndrome. These histologic sections revealed middle ear abnormalities including fixation and superstructure deformity of the stapes and dehiscence of the fallopian canal. Operative findings in 16 procedures on patients with Down's syndrome and conductive hearing loss suport those findings.

Adolescent↗

Surgical skills training in middle-ear surgery.

Until recently the practice of otological procedures on cadaver temporal bones was a common occurrence in otolaryngology departments. The difficulty in acquiring specimens has led to alternative techniques which involve artificial and computer-aided models. This article looks at the present situation in these rapidly developing areas and describes an artificial model developed by the senior author for training in middle-ear procedures.

Computer Simulation↗

Ossiculotympanic transplantation.

Continued investigations into current tympanoplasty problems have been carried out in the Eye and Ear Clinic, Royal Victoria Hospital, Belfast. Retrospective studies have been helpful in identifying some of the main problems responsible for many of our unsatisfactory results, both in the irradication of disease and the restoration of aural function. Prospective studies into the behavior of grafting materials for tympanic reconstruction in the experimental animal have led us to an overwhelming preference for natural materials as opposed to plastics or stainless steel. Both homologous and autologous bone and cartilage appear to offer good prospects in ossicular replacement techniques. The most satisfactory material we have used for repair of tympanic membrane defects continues to be either autologous or homologous temporalis fascia. The long-term success of these materials in functional restoration is utterly dependent on continued aeration of the tubotympanic cleft.

Animals↗